CCC3: Radiology of Head
Reasons to order a CT/MRI of brain/spine:
S/S for stroke
Very severe headaches/back pain
Sensory examination
– The sensory exam should includepinprick,vibration,toeposition,and Romberg’s as a minimum; might
Presents as rapidly progressiveascending polyneuropathy:wkness of feets or hands and quickly moves up the
extremities, dea...
Attention – orientation to and action upon sensory/cognitiveoperations
Language – evaluate comprehension, repetition, flue...
Ha  confusion/memory loss/hemiparesis,DX on MRI/CT
Sx drainagecould be curative
HIV dementia
Some degree of cortical atro...
 Typical MRI findings
◦ Multifocal lesionsin the periventricular regions of the brain
◦ Spinal lesionsincreasethelikeliho...
Chronic Inflammatory demyelinatingpolyneuropathy – chronic formof GB
CCC11: Movement Disorders
Essential Tremor
Tremor wit...
Orofacial movements prominent, progressive,rarely resolves,usually elderly
Lipsmacking,tongue movements
Tx: withdraw offen...
i. Antibodies againstthe acetylcholinereceptors at the neuromuscular junction prevent
acetylcholinefromcompleting the neur...
a. mitochondrial (e.g.,Kearns-Sayre,MELAS, MERRF)
b. glycogen storage diseases (e.g., Pompe’s, Andersen’s, and Cori’s)
c. ...
f. Prognosis
i. Depends on the myopathy
1. Most are progressiveand without cure
2. Disability dueto worseningweakness, gai...
e. Associated features: Congenitally absentpectoralis,biceps,or
brachioradialis;CK and aldolaseelevated in approximately 5...
Ocular movement spared
May occur with collagen-vascular disorders
Onset after 35 y/o, progression over weeks  reaches pla...
Tx: stretching, massage,conditioning,trigger pointinjections,analgesics,relaxers,sleep aids,IV
lidocaine,topical ketamine
H = hypotonia
A = asynergy (lack of coordination)
N = nystagmus: abnormalities of target-directed eye movements
Metastatic tumor
Freidreich’s Ataxia
MC early onset ataxia
Spinal cord may be thinn...
Common pediatric epileptic syndromes
West syndrome
Triad begins in 1st year of life:infantilespasms,hypsarrhythmic EEG, ps...
CCC16: Pediatric MuscleWeakness and Cerebral Palsy (Hillary’s notes)
See lecture for MuscleWeakness Lecture – itwas mainly...
- Low tone of trunk and extremities, usually with normal DTR’s
- Support their weight with their knees extended ...
- Daily physical therapy for 6 to I 2 months after surgery
- Post-op complicationsarerareand includetransientparesthesiasa...
- CNS infection (encephalitis,meningitis)
- Meds/Toxins (Shiga toxin, salicylates,anticholinergics,cocaine,tricyclics,...
- Epilepsy syndromes: Seizure syndromes incl.infantilespasms,benign epilepsy of childhood,Lennox-Gastaut,
and juvenilemyoc...
Lumbar puncture only if meningitis or encephalitis suspected and potential for brain herniation isruled out
Blood or urine...
epilepticus.The outcome of convulsivestatus epilepticuslargely depends on etiology, but prompt treatment can
improve outco...
*cough + pass out = tonsillarherniation
Emergency management
Increased intracranial pressurecan kill!
Deep Venous...
Pituitary adenoma
Tumors of endocrine system, are they even cancer?
Do not have high growth rates or met
Big question: are...
iii. Enters anterior perforated substanceand runs to the olfactory cortex; NOTE: there is no
relay in the thalamus – this ...
i. Loss of vision in oneor both eyes
ii. Visual field cuts
iii. Afferent pupillary defect(APD, or Marcus-Gunn pupil)
d. Di...
III. Cranial Nerve III (oculomotor nerve) (Moore & Dalley,pp. 968-973;Blumenfeld, pp. 530-532)
a. Function(s)
i. General S...
i. Horner’s syndrome – ptosis,miosis,anhydrosis –all ipsilateral to the lesion
d. Differential diagnosis of dysfunction
i. General Somatic Afferent (GSA) – Sensory fibers to the face, mouth, anterior two-thirds
of the tongue, meninges, and na...
i. General Somatic Efferent (GSE) – Motor fibers to the lateral rectus muscle,which
abducts the eye
b. Anatomy
i. Leaves t...
i. Both cerebral cortices supply fibers for the forehead to both facial nervenuclei in the
pons. The cerebral cortex suppl...
i. Vestibular schwannoma (a.k.a. acoustic neuroma) or cerebellopontineangle
ii. Ischemic damage to the nerve
ii. General Visceral Efferent (GVE) – Parasympathetics to heart, lungs,and the upper GI
tract (to the splenic flexure)
i. Have the patient turn the chin into your hand (note: away from the sideof the
functioningsternocleidomastoid muscleand ...
Antidepressants (Wellbutrin,any SSRIs,TCAs)
USE medicines AS SOON As HA starts!
Acute Migraine Attacks
Treatment: IV, anti...
Sinus Headaches – NOT a diagnosisanywherebut in the US
Usually a migraine that causes nasal symptoms
Only acute, purulent ...
Lucid interval,cerebral edema often develops
Stroke Diagnosis Mimics:
Sudden onset of focal numbness/wkness Hypoglycemia
Pediatric Stroke
Ischemic strokes aredeeper than cortical
Vascular occlusivelesions areintracranial vs.extracranial
Workup may include:
Cardiac enzymes glucose drug screen alcohol level
Pupillary Responses
Normal – not a structural lesion of the CNS
Unilateral dilation – structural injury
B/L dilation unres...
I – infectious &
T – trauma and toxins E – endocrine/epilepsy/emotional
A – Anoxic
M – metabolic
I – iatrogenic
M - neopla...
Acute – encephalopathy,tremor, myoclonus,seizures,eventual posturing
Chronic – mild confusion slowingprogressingto dementi...
LMN syndrome: UMN Syndrome (everything up!):
Flaccid weakness spastic weakness
Decreased tone increased tone
Decreased DTR...
L/o proprioception,vibration sensein legs with sensory ataxia and + Romberg’s sign
B/L corticospinal tractdysfunction =spa...
CCC Neuro Exam Notes.docx - VCOM Student Portal - VCOMDO.COM
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  1. 1. CCC3: Radiology of Head Reasons to order a CT/MRI of brain/spine: Trauma S/S for stroke Very severe headaches/back pain New onset seizure in adults Trauma Epidural hematoma (extradural acutebleed with fracture), lenticular shape Subdural hematoma (acute, subacute, chronic) CTA – screen for potential vascular diseasewith contrast,done if suspected stroke MRA – images of vessels without contrast Carotid Ultrasound Imaging – depicts plaquethat narrows arteries,detects narrowing,stenosis or carotid artery which increases the risk for stroke Lacunar infarcts –small strokes within/around thevassal ganglia,could bean incidental finding Hemorrhagic stroke: Intracerebral hemorrhage (HTN) or subarachnoid hemorrhage(aneurysm rupture) Meningitis – not diagnosed by imaging,a clinical diagnosis! Functional MRI – useblood-oxygen-level-dependant effects to localizeregional cerebral blood flowchanges during neuronal activity *Map brain functions in relationship to intracranial tmors,seizurefoci,or vascularmalformations PET scan – dye goes to metabolically activetissues CCC6: Peripheral Nerve Disorders Axonal neuropathy – axon is primary siteof injury with myelin sheath intact Demyelinating neuropathy – myelin sheet is primary siteof injury with axon intact(segmental or diffuse) History  Nature of chief complaint:weakness, sensory disturbance,pain,autonomic dysfunction,atrophy.  Time courseof symptoms: acute, subacute,chronic. Relapsing,recurrent,progressive  Anatomic pattern of symptoms: symmetric, asymmetric, focal or diffuse – Polyneuropathy, mononeuropathy, etc.  Specific questions to help narrow etiology: Trauma, Toxin exposures, infections,dietary deficiencies,medication used,presence of other medical conditions, constitutional symptoms.Family history *Weakness = motor *Burning/numbness = sensory Exam Cranial nerves involvement: Think Guillain-Barresyndrome(GBS), Sarcoid,carsinomatosis,diphtheria
  2. 2. Sensory examination – The sensory exam should includepinprick,vibration,toeposition,and Romberg’s as a minimum; might also do temperature sense and lighttouch. Most neuropathy have distal,symmetric,sensory loss in lower extremities first. – Typical pattern is called ‘stockinghypesthesia’in the lower limbs,and ‘glove hypesthesia’in the upper limbs. Motor examination 1). Look for atrophy and distribution 2). Distribution of weakness: most polyneuropathy affect distal muscles of lower extremities first. Most demyelinatingand certain acute motor and toxic neuropathy affects all muscles of limbs,trunk,neck and some facial muscles 3) The extensor digitorum brevis muscleis likely to be the firstmuscleto demonstrate atrophy. 4) Fasciculations may be seen in the weakened muscles.(Fasciculations area lower motor neuron sign.) Reflex examination DTR’s arelikely to be reduced or absent= LMN sign Coordination and gait – Sensory ataxia if lostsenseof proprioception Autonomic involvement: edema, hair loss,skin reddening, impotence, incontinence,or gastroparesis. Labs Serum general screen 24-hour urine Muscle Studies Nerve conduction velocity study - measures speed of conduction Electromyogram study - depends on health of nerve, measures inherent electrical activity of muscles Etiologies: Diabetic neuropathy - Demyelination – MC pathological process Prone to compressive/entrapment and cranial neuropathies Carpal tunnel syndrome at wrist(median nerve) Cubital tunnel syndrome atelbow (ulnar nerve) Peroneal nerve entrapment at fibular head Radial nerveinjury athumeral groove Bell’s palsy (VII weakness) Trigeminal/glossopharyngeal neuralgia Proximal diabetic neuropathy (amyotrophy – atrophy due to neuropathy) Sudden onset of severe thigh pain  weakness of proximal legmuscles  atrophy EMG is diagnostic,oppositelegmay be affected later than the first(milder too) CIDP Chronic,progressivedemyelination where axons eventually degenerate Progressivesensory loss  wkness  atrophy  autonomic changes Tx: immunomodulators,plasmapheresis,chemo, IV Ig Other causes of Peripheral Nerve Disorders: Guillian-Barre Autoimmune, demyelination of peripheral nerve at junction of dorsal/ventral roots
  3. 3. Presents as rapidly progressiveascending polyneuropathy:wkness of feets or hands and quickly moves up the extremities, death usually resultsfromrespiratory compromisedue to paralysisof respiratory muscles Areflexic with minimal sensory symptoms May followa viral or C.jejuni infection CSF = very elevated protein Dx: LP, NVC, EMG, serology studies TM: plasmapheresis or IVIg Toxic and Metabolic neuropathies: Alcoholic neuropathy – MC Common, but mild in wkness – marked sensory changes and pain Associated with poor general nutrition/multifactoral Due to axonal degeneration Endocrinopathies – hypothyroidism,acromegaly Uremia – symmetric, distal sensorimotor polyneuropathy RLS, cramps,severe burningof the feet Amyloidosis Liver disease/general nutrition Sensory or a sensorimotor neuropathy Drugs, especially statins, chemo, cardiac drugs, colchicines, paclitaxel AIDS Sensory symptoms predominant in axonal,distal symmetric sensorimotor neuropathy Often inflammatory demyelination linked to autoimmune or infection Tm: immunomodulators Vasculitis, Connective tissue diseases Polyarteritis nodosa,allergicangiitis,Wegener’s, Giantcell arteritis,RA, SLE, Sjogren’s syndrome, Scleroderma, CMTD Neoplasia Common nerve compression Invasion of epineuriem Paraneoplastic syndromes (immune-mediated) Anti-Hu antibodies  sensory neuropathy of DRG cells Plasma cell dyscrasias –monoclonal serumantibodies Monoclonal Gammopathy of Undetermined Significance Hereditary motor/sensory neuropathies Charcot-Marie-Tooth – MC hereditary Nerves areenlarged, slowly progressive,sensorimotor forms or severe pain CCC7: Dementias Dementia – acquired loss of cognitivefunction due to an abnormal brain conditions. Requires functional declinethat interferes with work or social activities pluscognitivedecline. Spheres of cognition: Memory – encoding, storage, and retrieval of info
  4. 4. Attention – orientation to and action upon sensory/cognitiveoperations Language – evaluate comprehension, repetition, fluency, and naming Aphasia –acquired disturbancein comprehension and formulation of verbal messages Executive function – coordination basic cognitivecapacities and emotion, regulatingbehavioral responses to environmental demands High sensory perception Praxis –skilled movements Apraxia – failureof integration of motor function with visual perception,somatic sensation and/or motor intension = improperly executed motor function Calculation - Emotion Evaluation of Disordered Cognition MMSE – to assess cognitivemental status,detect impairment, followcourseo fillness,monitor responseto tx Labs: B12, thyroid, syphilis,ANA, HIV, ESR, Protein electrophoresis,Genetic testing Neurophysical EEG – for seizures or encephalopathy, CJD Neuroimaging – CT, MRI, PET/SPECT Common cognitive Disorders: Alzheimer’s disease – MC degenerative dementia ~Widespread cerebral cortical atrophy with neuritic(senile) plaques and neurofibrillary tangles ~Loss of synapses ~Deposition of amyloid in neuropil and cerebral vessels =NFT formation ~ApoE status is 2nd MC risk factor,agingis 1st(ApoE4 alleleincreases risk) Clinical features:dementia, apraxia,aphasia,agnosia,psychiatric symptoms,disturbed sleep/wake cycles, incontinence Treatment: Acetylcholinesteraseinhibitors, atypical antipsychotics,antidepressants Vascular Dementia or Multi-infarct Dementia (MID) ~Large cortical infarctor numerous small lacunar infarcts ~Hx of HTN, sudden onset of dementia, step-wise progression of deficits ~UMN signs (pseudobulbar affectw/ emotional lability) TM: control HTN, rule out stroke, antiplatelettherapy, AChE inhibitors often used Pseudodementia Major depression impairscognition enough to be confused with primary demtnai Attempt tx with appropriateantidepressant Other common cognitive disorders: Pick’s disease (frontotemporal dementia) Generalized cortical atrophy with significantatrophy or one lobe (usually frontal) Swollen Pick’s bodies,earlier onset than AD Behavioral changes aremore signifantthan in Alzheimer’s Chronic subdural hematoma Elderly,symptoms develop weeks/months after minimal head trauma
  5. 5. Ha  confusion/memory loss/hemiparesis,DX on MRI/CT Sx drainagecould be curative HIV dementia Some degree of cortical atrophy and enlarged ventricles Longer has disease,more likely to develop dementia Apathy, inertia,depression,behavioral changes,cognitivesymptoms,motor abnormalities Transmissible Spongiform Encephalopathy Sporadic form90% Mental or physical deterioration with death w/I 6 months after onset No way to specifically diagnoseor Treat. Normal Pressure Hydrocephalus Result of an imbalancebetween production/resorption of CSF Triad:progressivedementia + gaitdisturbance+ urinary incontinence Tx: reduce CSF volume Causes of DEMENTIAS: D – degenerative, depression, rage E – endocrine M – metabolic, myelin E – epilepsy N – nutrition, neoplasia T – toxic, trauma I – infection, inflammation, infarction A – atherosclerosis/vascular S – Structural, system CCC8: DemyelinatingDisorders Multiple Sclerosis – CNS demyelination 20-40 y/o with female predominance Young women w/ neurological symptoms = THINK MS! Diagnosis LP multiplesclerosispanel:Oligoclonal bands,IgGindex,cell count, protein, glucose, culture Serum MS panel:Oligoclonal bands,IgGindex *CSF should havehigher IgG than serum = focal immune response *CSF should havehigher oligoclonal bandsthan serum= immune inflammation goingon in the CNS that is absent from the serum  FIRST look atthe clinical presentation –for diagnosis,thepatient must have 2 neurological symptoms separated in space(2 locations in thenervous system) and time (more than a month apart) Sensory disturbances arethe MC presenting symptom!! Typical Hx + typical MRI OR Typical Hx + positiveLP (immune components on MS panel)
  6. 6.  Typical MRI findings ◦ Multifocal lesionsin the periventricular regions of the brain ◦ Spinal lesionsincreasethelikelihood of MS in the correct clinical setting ◦ Cervical spinelesions ◦ Enhancinglesions arehighly suspiciousfor MS in the correct clinical setting  Acutely activeinflammatory lesions –leakageof the gadlimium  LP results ◦ IgG index elevated ◦ 2 or more oligoclonal bands in theCSF  Paraclinical tests ◦ Visual Evoked Potentials slowin optic neuritis ◦ Somatosensory Evoked Potentials slowin spinal cord lesions  Tibial or median nerve, give impulsemeasure in brain – good but do not know where the demyelination is Tx: Steroids, IVmethylpredisolone daily for 5 days Immunomodulators for MS to SLOW progression Copaxone, Avonex, Rebif, Betaseron Symptomatic therapy: urinary incontinence,spasticity,pain,fatigue Forms of MS  Relapsing-Remitting ◦ 85% of patients start with RRMS – distinctrelapses thatimprove to some degree ◦ Diseaseitself never remits, constantinflammation in all partsof the nervous system and sometimes you get an acute flare-up  Relapsing-Progressive ◦ Relapses may improve, but between relapses there is functional decline  Primary Progressive ◦ 10% of patients have a progressivefunctional declinefromthe onset – usually a moresevere form of the disease ◦ Very difficultto manage (males + older age at onset more likely)  Secondary Progressive 50% of patients with initially RRMS will go on to a steadily progressivecoursein about10-15 years Multiple Sclerosis Exacerbation = abruptonset of new symptoms or worsening of old symptoms in response to some stressful situation Tx: Corticosteroids to suppress immune system Guillian-Barre – PNS demyelination ~Tingling,rapid progression of weakness ~decreased/loss of reflexes = LMN/peripheral lesion ~ascendingparalysis VERY increased protein in CSF Self-limitingdiseasebuthas a high risk of respiratory depression Tx: IV Ig(cleans out the overabundance of patient Abs within 2-3 days)
  7. 7. Chronic Inflammatory demyelinatingpolyneuropathy – chronic formof GB CCC11: Movement Disorders Essential Tremor Tremor with action or sustension,notpresent at rest Worsewith: Stress, sleep deprivation,meds, caffeine DDx: drug-induced, thyrotoxicosis,sedative/alcohol withdrawal,cerebellartremor, parkinson’s Tx: anythingto dampen the CNS response, but if tremor doesn’t bother pt – don’t use drugs, drugs can do more harm than good Beta blockers Primidone Benzodiazepines Antiepileptics Tourette’s Syndrome: Motor and vocal tics +young age + symptoms of longer than 1 year Tics do not hurt, more likean itch you have to scratch –sometimes worse if they suppress them Common to also haveOCD DDx: simpletics,syndenham,dystonia Tx: Antipsychotics (haloperidol,pimozide,clonidine,fluphenazine) Huntington’s Disease Loss of Ach neurons in the indirectpathway leads to decreased inhibition of the thalamus =increased excitation of the cortex = need to block the dopamine! Autosomal dominantdiseasedue to a trinucleotiderepeat (CAG) with anticipation and 100%penetrance (if you have the gene, you will get Huntingdon’s) Parkinson’s disease Loss of dopaminergic neurons in the substantia nigra =lack of disinhi bition of thalamus =inhibited thalamus = inhibited cortex Clinical features:pill-rolling,restingtremor, absentfacies,depression,bradykinesia,gaitimbalance,dysphagia,drooling, dry eyes, cognitiveslowingand dysfunction Tx: Dopamine agonists,Anticholinergics,ergotamines *Too much dopaminecan produce dyskinesias Syndenham’s chorea Autoimmune disorder caused by Group B Streptococcus (1 of PANDAS), affects basal gangliaand cerebellum Self-limited,under age of 15 Associated with personality change Beware of streptococcal rheumatic disease Tardive dyskinesia (orofacial dyskinesia) Previous exposure to dopamine blockers
  8. 8. Orofacial movements prominent, progressive,rarely resolves,usually elderly Lipsmacking,tongue movements Tx: withdraw offending agent, sedatives,antiepileptics CCC12: Muscular Disorders I. Myasthenia Gravis a. Definition i. “Myasthenia gravis”means “grave muscleweakness” ii. Myasthenia gravis is an autoimmunedisease,in which the immune system is activated against the neuromuscular junction iii. Acquired disorder iv. Fatigablemuscleweakness, worse with exerciseand better after rest b. Clinical features i. Epidemiology 1. Affects 2/100,000 people 2. Any age, either gender can be affected 3. Most common in women under the age of 40 and men over the age of 60 4. Family members of people who have other autoimmune disorders such as rheumatoid arthritis,lupus,or scleroderma may be at higher risk for developingmyasthenia gravis ii. Types of myasthenia 1. Approximately 85-90%of patients with myasthenia gravis have generalized muscle weakness, while10-15% of patients have ocular myasthenia,which affects only extraocular muscles and eyelids 2. Lambert-Eaton myasthenic syndrome (LEMS) results from autoantibodies againstthe voltage-gated calciumchannelsin thepresynaptic terminal of the neuromuscular junction;weakness actually improves with exercise in this form of the disease 3. The autoimmune myasthenia shows no direct inheritancepattern; however, there are raregenetic mutations that resultin a myasthenic syndrome based on defects in the acetylcholinereceptor or acetylcholinesteraseproteins 4. Neonatal myasthenia affects 10-20% of children born to mothers with myasthenia. This form of myasthenia results from maternal antibodies affectingthe newborn’s neuromuscular junction. The condition is usually temporary,with symptoms disappearingwithin 2-3 months after delivery iii. Symptoms are variable,dependingon which muscles are involved,and may be intermittent 1. Ptosis 2. Double vision/weakness of extraocular muscles 3. Dysphagia 4. Dysarthria 5. Unsteady gait 6. Limb weakness 7. Shortness of breath 8. Neck muscleweakness iv. Myasthenic crisis 1. Respiratory muscles weaken to the pointthat breathingbecomes difficult 2. Requires ventilation 3. Triggers include a. Infection or fever b. Pregnancy c. Medication reaction d. Physical overexertion or emotional stress 4. Treatment a. Plasmapheresis b. IVIg c. Etiology
  9. 9. i. Antibodies againstthe acetylcholinereceptors at the neuromuscular junction prevent acetylcholinefromcompleting the neurochemical transmission fromnerve to muscle ii. The thymus gland may contain lymphoid hyperplasia,clusters of immune cells thatnormally develop in the spleen and lymph nodes duringan activeimmune response; 15% of myasthenic patients have a thymus tumor, and 60-80%of myasthenic patients have thymic hyperplasia d. Diagnosis i. Diagnosisis often delayed, as weakness is a common complaintof many disorders,notjust myasthenia ii. Exam often shows impaired extraocular movements, ptosis,and neck and limb muscle weakness; loss of sensation is nota feature iii. Anti-acetylcholinereceptor antibodies arefrequently present in the blood iv. Edrophonium test 1. Edrophonium is an acetylcholinesteraseinhibitor and therefore increases acetylcholineatthe neuromuscular junction. 2. A dose of edrophonium is given to the patient, and neurological examination is repeated to assess for improvement in muscleweakness v. Nerve conduction studies/electromyography 1. Repetitive nerve stimulation –an electrical stimulusisapplied repeatedly to a nerve; myasthenia is suggested if the response decreases with this repeated stimulation 2. Singlefiber EMG – singlemusclefibers aregiven an electrical stimulus;repeated stimuli resultin reduced response vi. Additional studies assistwith management, not necessarily diagnosis 1. CT chest with and without contrastto evaluate for thymic hyperplasia or tumors 2. Pulmonary function tests to assess respiratory function e. Treatment i. Neostigmine and pyridostigmine ii. Immunosuppression 1. Azathioprine 2. Prednisone 3. Cyclosporine iii. Thymic resection reduces symptoms in young women even in the absence of thymoma and may be curativein some patients with thymic hyperplasia iv. IVIg – especially useful in myasthenic crisis v. Plasmapheresis –also useful in crisis f. Prognosis i. Myasthenia does not usually shorten lifeexpectancy ii. Myasthenia can spontaneously remit, with return of normal musclestrength and no need for medications;remissions areoften temporary iii. Thymectomy may be curativein some patients II. Myopathies – Look for proximal weakness a. Definition i. Myopathy is a defect in the musclefiber itself that produces weakness b. Clinical features i. Epidemiology 1. Myopathies can occur in any age, either gender, any race 2. Certain myopathies are more likely in differentpopulations 3. Symptoms from mitochondrial myopathies startin childhood or early adulthood 4. Congenital myopathies produce symptoms in infancy 5. Polymyositis,dermatomyositis,and inclusion body myositisaremore common in older patients ii. Types of myopathies (diversegroup of disorders) 1. Congenital - skeletal and facial abnormalitiesusually presentfrom birth 2. Metabolic
  10. 10. a. mitochondrial (e.g.,Kearns-Sayre,MELAS, MERRF) b. glycogen storage diseases (e.g., Pompe’s, Andersen’s, and Cori’s) c. myoglobinurias (e.g., McArdle, Tanuia,DiMauro diseases) 3. Inflammatory a. dermatomyositis - inflammation of skin and muscle b. polymyositis 4. Myositis ossificans - bone grows in the muscles 5. Familial periodic paralysis 6. Inclusion body myopathy 7. Neuromyotonia, stiff-man syndrome 8. Common musclecramps 9. Tetany - spasms in the limbs 10. Infectious a. HIV iii. Symptoms 1. Muscleweakness (usually proximal; causes problems risingfroma chair and walking down stairs) 2. Musclecramps 3. Muscletenderness (with myositis) 4. Musclestiffness 5. Musclespasms c. Etiology i. Due to genetic mutations or ii. Inflammatory/auto-immune or iii. Congenital metabolic dysfunction iv. Medication toxicity (-statin myopathy, steroid myopathy) v. Infections (HIV) d. Diagnosis i. Electromyography often shows abnormalities of muscleaction potentials - small amplitude potentials atrest are characteristic ii. Musclebiopsy with histochemical stainingcan identify certain myopathies iii. Serum CK and aldolaseareoften elevated (CK is usually many hundred times normal in some of the myopathies, especially myositis) iv. Metabolic panels to characterizemetabolic myopathies v. CSF analysishelps to identify some of the congenital and metabolic myopathies vi. Labs to ruleout muscleweakness from other reasons 1. Calcium,phosphorous,potassium,magnesium 2. Consider evaluation for myasthenia,if symptoms are suggestive 3. TSH to evaluate for hypothyroidism 4. Basic metabolic panel,cortisol levels to evaluatefor hypoadrenalism e. Treatment i. Depends on the cause 1. Inflammatory myopathies a. Anti-inflammatory medications (prednisone,etc.) 2. Congenital myopathies a. Often no specific or curativetreatment b. Supportive care(braces to improve function,respiratory supportin some cases) 3. Metabolic myopathies a. No curativetreatment b. Creatine replacement in creatinedeficiency myopathy has shown variable efficacy in clinical trials c. Lactic acidosisfrommitochondrial myopathies mustbe addressed when it occurs;vitamin C, menadione, niacin,riboflavin,and ubiquinonetogether have been used
  11. 11. f. Prognosis i. Depends on the myopathy 1. Most are progressiveand without cure 2. Disability dueto worseningweakness, gaitinstability,falls,and (in some cases) respiratory depression or cardiac arrhythmias III. Muscular Dystrophies a. Definition i. Muscular dystrophies areinherited forms of musclediseasecharacterized by initially normal muscledevelopment, followed by weakness, then finally muscleatrophy ii. Although these areprimarily considered muscular disorders,there may be a neuropathological component, as well b. Clinical features i. Family history of musclediseaseis common ii. Weakness often develops before muscleatrophy iii. Distribution of the weakness and musclewastingdepends on the form of dystrophy iv. Reflexes are often depressed even before weakness develops v. In late stages, differentiation of which dystrophy is present is difficultdueto diffusemuscle weakness and wasting vi. Types of muscular dystrophy 1. Duchenne muscular dystrophy a. Mode of inheritance: X-linked recessive; almostexclusively presentin males (rarefemale cases havebeen reported) b. Age of onset: firstdecade c. Distribution: proximal legmuscles,then shoulders,then spreads distally d. Exam findings: Reflexes lost,except for anklejerks; macroglossia; pseudohypertrophy of the calves,deltoids,infraspinatusmuscles e. Associated features: EKG abnormalities,osteopenia,scoliosis,fatty heart infiltrates,pathological fractures,respiratory infections,lowIQ in 30% of patients, contractures f. Treatment: Steroids initially help strength but do not affect overall prognosis g. Prognosis: Death from cardiac failureor respiratory infections within 6 years of onset, frequently by age 20 2. Becker’s muscular dystrophy a. Similar to Duchenne, but age of onset in teens to thirties and slower progression 3. Limb girdledystrophy a. Mode of inheritance: Autosomal recessiveusually,butcan also be dominant or sporadic b. Age of onset: Second or third decade c. Distribution: Weakness in proximal muscles,either the pelvic or shoulder girdle,with slowprogression distally d. Exam findings: Depressed proximal reflexes with spared anklejerks, pseudohypertrophy in calves,deltoids,and lateral quadriceps e. Associated features: CK and aldolasemay be slightly elevated or normal f. Treatment: Supportive; no specific treatment g. Prognosis: No affect on expected lifespan 4. Facioscapulohumeral dystrophy a. Mode of inheritance: Autosomal dominant, occasionally recessive b. Age of onset: Second through fifth decades c. Distribution: Faceinvolved first,then shoulder girdle d. Exam findings: Reduced reflexes atthe biceps and triceps; pseudohypertrophy of forearm muscles;true hypertrophy of deltoids
  12. 12. e. Associated features: Congenitally absentpectoralis,biceps,or brachioradialis;CK and aldolaseelevated in approximately 50% of cases f. Treatment: No specific treatment g. Prognosis: No affect on lifespan 5. Myotonic dystrophy a. Mode of inheritance: Autosomal dominant b. Age of onset: Any age, usually third decade c. Distribution: Generalized weakness in hands,ptosis,facial weakness, weakness and atrophy of quadriceps and tibialisanterior d. Exam findings: Atrophy in arms,facial muscles,“swan neck” appearance, “hatchet” face, depressed reflexes diffusely,EMG shows characteristic myotonic discharges e. Associated features: Cataracts,frontal baldness,gynecomastia, bronchiectasis,testicular atrophy,slowed bowel motility,cardiac abnormalities f. Treatment: No specific treatment g. Prognosis: Premature death due to cardiac failureor respiratory infections 6. Myotonia congenital a. Mode of inheritance: Autosomal dominant; the autosomal recessiveform results in progressiveweakness b. Age of onset: At birth c. Distribution: Myotonia (inability to relax a muscleafter contracting) present diffusely d. Exam findings: Diffusemusclehypertrophy startingin the second decade, slowed and stiff movements are worse in cold e. Associated features: None f. Treatment: Procaineamide,phenytoin, or quinidinework well to reduce myotonia g. Prognosis: Normal lifeexpectancy 7. Ocular dystrophies a. Mode of inheritance: Autosomal dominant or sporadic forms b. Distribution: Extraocularmovement weakness, eyelid weakness, and facial weakness; 25% of patients have limb girdleweakness,as well c. Exam findings: Ptosis,eye movement abnormalities,dysphagia in half d. Associated features: (in some families) retinitis pigmentosa,cerebellar dysfunction,cardiac abnormalities,endocrinedysfunction,visual changes; EMG/nerve conduction studies indicatea neurogenic component in some e. Treatment: No specific treatment CCC13: MusclePain/Myopathies Inflammatory Myopathies (rare) Inclusion Body myositis Distal limb weakness often confined to legs over 50 y/o, dyspagia Wkness of long finger flexors Normal CK Beta-amyloid precur protein!! Inclusion body = lymphocytes that invade and destroy the muscle, inflammation is rarely seen Does not respond to steroids Polymyositis Neck and proximal limb weakness  eventual total body weakness
  13. 13. Ocular movement spared May occur with collagen-vascular disorders Onset after 35 y/o, progression over weeks  reaches plateau and permanent disability CK elevated 10x or more above normal CD8+ infiltration Anti-Jo antibodies Tx: Steroid responsive Dermatomyositis Peaks in puberty and around 40 y/o Females > Males 1st manifestations simultaneous in SKINand muscle RASH: butterfly distrubtion over nose, edema/erythema affect eyelids,periungal skin,extensor surfaces of joints,upper chest MUSCLE: proximal weakness,ache, tender to palpation Proximal wkness with decreased DTRs, dysphagia CK elevated (not nearly as high as polymyositis) Abnormal EMG Biopsy:degeneration, regeneration, edema, lymphocytic infiltration (Bcells) Tx: steroid responsive Sarcoid myopathy Clinically similar to polymyositis Granulomas seen on muscle biopsies ACE levels are elevated Eosinophilic myositis Patient has generalized pain w/ proximal > distal wkness Biopsy = eosinophilic infiltrateinstead of lymphocytes Seen as responseto drug, chemical or parasitic exposure= allergicresponseto toxin Non-inflammatory disorders Myofascial pain syndrome – very common Radiatingpain in defined,localizingpattern (constant,aggravated with motion, worse upon awakening, ROM is restricted), taut bands and trigger points with radiation on palpation Onset is typically after minor,repeated trauma or abruptly after severe trauma Trigger points are activeor latent EMG is abnormal within trigger points Biopsy – normal Neuro exam – normal
  14. 14. Tx: stretching, massage,conditioning,trigger pointinjections,analgesics,relaxers,sleep aids,IV lidocaine,topical ketamine Fibromyalgia syndrome 7x MC in Females, numerous comorbidities Clinical Symptoms – Chronic,diffusemusculoskeletal pain, with focal areas of tenderness in earlier stages,but more widespread tenderness as diseaseprogresses. • The pain often begins after trauma, whether physical or psychological. • Pain is described as deep achingand throbbing. • Pain is continuous with intermittent exacerbations often triggered by weather changes, increased physical activity,trauma,or intercurrent illness. – Additionally,patients haveaffective and cognitivedysfunction,dizziness,chronic insomnia or non- restorativesleep, nocturnal myoclonus, morningstiffness,abdominal or pelvic pain,dysuria,and diarrhea or constipation. – FMS patients have abnormal reactivity to pain (similar to allodynia). Objectivepain measurements indicatethat FMS patients feel minimally painful stimuli as strongly painful. – Comorbidities includeTMJ syndrome, migraines,irritablebowel syndrome (IBS), interstitial cystitis(IC), dysmenorrhea, rheumatoid arthritis,depression,anxiety,and chronic fatiguesyndrome. • These comorbidities occur within FMS at a significantly higher ratethan in the non-FMS population Pathophys: Central sensitization Neuroendocrine, neurosensory,and NT disturbances Diagnostic Critiera Widespread pain in ¾ body quadrants Tenderness to palpation in 11/18 tender points on body Tx: exercise,antidepressants,neuromodulators,analgesics,psychological support Prognosis:non-fatal,butseverely life-altering CCC14: Cerebellar Lesions Cerebellar anatomy – 3 lobes,2 fissures,and 3 peduncles Superior peduncle – connects cerebellum  midbrain Middle– connects cerebellum  pons Inferior – connects cerebellum  medulla (largestpeduncle) Cerebellar function: Coordination center Discrete, skilled movements Regulates movements and posture indirectly Comparator – compares intension with performance, adjusts appropriately Cerebellar lesions: COMMON findings = HANDS Tremor
  15. 15. H = hypotonia A = asynergy (lack of coordination) N = nystagmus: abnormalities of target-directed eye movements Slow-conjugate movement way from lesion D = dysarthria:slurred,garbled speech slowor staccato in nature S = station and gait(imbalance/ataxia) Tremor = courseintension tremor Cerebellar ataxia Ataxia of gait= incoordination of walking,steps arewide-based, unequal, zig-zagged Truncal ataxia =incoordination of all truncal movements (extremities flail) Dysmetria – inaccuracy in range/direction Dysprosody – disturbanceof stress,pitch, rhythm of speech Localization Midlinecerebellum truncal ataxia/musculatureand body equilibrium Rostral – stance/gait Caudal – truncal ataxia Hemispheres IPsilateral extremity dysmetria Pancerebellar  entire cerebellum Evaluation: Test motor skills Romberg’s Test – tests cerebellar and posterior column function Disorders: Cerebellar infarction Thrombotic/embolic occlusion of vessel 6 D’s: Decreased consciousness,Dz,Dysequilibrium,Diplopia,Dysarthria,Dysphagia MC = PICA infarct Acute postinfectious cerebellitis Children between 2-7 y/o M = F Explosiveonset w/ ataxia atmaximum at onset Sersoriumis clear despitesevere ataxia Symptoms remit after a few days,normal gaitrequires 3 wks/5 months to regain Dx of exclusion,Tx – self-limiting Cerebellar hemorrhage Abrupt onset of vertigo, Ha, Vomiting, inability to stand/walk with absenceof hemiparesis/hemiplegia Tendency for abrupt deterioration to coma/death Cerebellar neoplasm 50% of neoplasms in kids:Posterior fossa neoplasms: Cerebellar astrocytoma Medulloblastoma Ependymoma of 4th ventricle Brainstemglioma Rare in adults:
  16. 16. Hemangioblastoma Metastatic tumor Schwannoma Meningioma Freidreich’s Ataxia MC early onset ataxia Spinal cord may be thinner than normal = degeneration/sclerosis in posterior columns,SC tracts,and CS tracts GAA tripletrepeat – deficiency of frataxin Clinical features:gaitataxia,armataxia,scolio,dysarthria,l/o DTRs,l/o vibration/position sense,musclewkness, atrophy, Hcardiomopathy,pes cavus,abnormal ocular motility, diabetes,deafness Course: progression,cannotwalk after 15 years of onset, death from infection or cardiac disease Tx: nothing influences the course *UMN + LMN symptoms!* Cerebellar Herniation Upward (transtentorial) –obliterates aqueduct and cisterns =hydrocephalus/obtundation/coma Downward (tonsillar) –compression of medulla = respiratory arrest/death CCC15: New Onset Seizures in Children 75% of epilepsy presents before 20 years of age. If begin before 2 y/o – grave prognosis Neonatal seizures: May be only symptom of CNS disorder or symptom of another treatable, serious condition Majority arepartial seizures Risk factors:premie, lowbirth weight, HIE Diagnosis:observation w/ and w/o EEG Classification –based on clinical manifestation Subtle Tonic Clonic Myoclonic Autonomic Special types:  Benign neonatal familial convulsion 1. Dominantly inherited, chromosome 20. 2. Seizures occurringin the second or third day of life 3. Characterized by clonic or apneic seizures 4. without specific EEG marker  Pyridoxinedependency and biotinidasedeficiency 1. Variableageof onset from neonatal period to later infancy 2. Various seizuretypes  Benign neonatal sleep myoclonus 1. EEG and physical examis normal and prognosis isexcellent. Treatment: determine cause,provideadequate ventilation/glucose,Phenobarbital/phenytoin
  17. 17. Common pediatric epileptic syndromes West syndrome Triad begins in 1st year of life:infantilespasms,hypsarrhythmic EEG, psychomotor delay Flexor spasms,extensor postures/focal motor features are common Etiology: prenatal/perinatal brain injury,metabolic,degen disorders,neurocutaneous disorders,cerebral malformations Neurodevelopment is only normal in 10% of patients Tx: benzos, valproate, steroids,corticotrophin,vigabatrin,focal cortical dysplasia Poor prognosis –onset before 3 months, symptomatic etiology, multipleseizure types Lennox-Gastaut syndrome Multipleseizures types, MR, slowspike-wave EEG discharges beginning1-7 y/o Tonic, atonic,atypical absence,tonic-clonicseizures common Developmental regression + gradual/abruptonsetof symptoms Tx: Corticosteroids,ketogenic diet, valproate,lamotrigine,topiramate, felbamate Prognosis:POORespecially if onsetis before2 years/age Febrile convulsions MC seizures in early life Simple - <15 mins,lack focality Complez - >15 mins,focal manifestations,recur within 24 hours Tx: rectal diazepam,avoid prophylactic AEDs Prognosis:30%will haverecurringseizure,only 2-5% develop afebrileseizure Idiopathic partial epilepsy syndrome BECTs – most common epilepsy syndromein childhood. Partial seizures occur w/I hours of fallingasleep = sensorimotor symptoms 25% of pts develop secondarily generalized seizures Tx: 1st lineAEDs Benign childhood epilepsy with occipital paroxysms Visual phenomena includingsimple/complex hallucinations,visual distortions,hemianopsia,amaurosis Misdiagnosed as migraineoften Prognosis:Good Tx: 1st lineAEDs Childhood absence epilepsy 4-8 years with frequenc absence seizures 40% develop tonic-clonicseizures when approachingpuberty Normal development, usually remitby adolence Tx: 1st lineAEDs Juvenile myoclonic epilepsy Generalized tonic-clonic seizures after awakeningbeginningin adolescence Myoclonus in droppingthings,or unnoticed for years Tx: valproate= DOC, indefinitely b/c less than 20% grow out of this.
  18. 18. CCC16: Pediatric MuscleWeakness and Cerebral Palsy (Hillary’s notes) See lecture for MuscleWeakness Lecture – itwas mainly justa differential diagnosislist Cerebral Palsy - Difficulty in neuromotor control,anonprogressivebrain lesion,injury to the brain that occurred before it was fully mature (<3 yrs), and a static encephalopathy must exist. Prevalence - 2-5 / 1000 livebirths (ratedecreases with age) - There is a definite correlation between birth weight/ gestational ageand the incidenceof CP. Etiology - Hypoxic/ischemic incidents occurringperinatally havebeen blamed for the majority of CP - In > 50% of CP pts, etiology may not be evident - Although prematurity is the MC antecedent of CP, the majority of infants were full term Classification - Neuroimaging techniques may help define the anatomic deficit,but will not reveal any pathology in the majority of children - 20% to 30% of infants who appear to fit the criteria for the diagnosis of CP at 1 year of age will notshow any manifestations of a motor deficitby 7 years of age. - In the pastdecade, the number of patients who have spastic diplegiahas increased,proportional to increased survival rates of preterm infants (33%). - Kernicterus and CP: Bilirubin encephalopathy with resultingkernicteruscausesathetosis-likeCP Diagnosis - Any infantwhose has motor delay must be suspectfor havingCP. - Diagnosisis very difficult<6 mo. - Delay in achievingmotor milestones at an appropriateageis common for children who are retarded but who do not have CP. (present w/ hypotonia) - Highly suspicious for CP if there is motor delay with normal cognitivedevelopment. - >50% of children with CP are retarded. - It is necessary to decide where the pathology exists anatomically: - centrally (eg brain) – indicates CP - peripherally (egspinal cord,anterior horn cell,peripheral nerve,myoneuraljxn,or muscle) - Make sureit’s not progressive:clues are: - Regression (could be a latesign) - Consanguinity - Congenital skeletal anomalies such as pescavusor scoliosis - Neurocutaneous stigmata TYPES: Athetoid CP (type of Dyskinetic) - Damage to Basal Gangliaor Cerebellum - Kernicterus causes this type - Obligatory asymmetric tonic neck reflex. - Delay in achievingmotor milestones - 12 -18 months of age: infantwill showathetoid-dystonic posturingon voluntary movement. - Hypotonia gradually changes to increased tone of the rigid or cogwheel type. - Hypertonus and the involuntary movements may increasein severity over the next year. SPASTIC DIPLEGIA - Many preterm infants who have CP will develop spastic diplegia(33%) - Delayed sitting,crawling,standing,and ambulation - Low tone of the LE may be present. - 1 year of age hypertonia (spasticity) - Increased DTRs and occasionally unsustained bilateral clonus will bepresent. - Abnormal postural reactions,e.g., scissoringof the legs due to spasticity of the hip adductors.
  19. 19. ATAXIC CP - Low tone of trunk and extremities, usually with normal DTR’s - Support their weight with their knees extended so as to lock them in position,and use a very wide basefor support. (Rare to see any overt signs of ataxia) - May be a spastic componentto the condition. - Infantusually will behypotonic and not spastic,buthyperreflexia will indicatethe presence of pyramidal tract involvement. SPASTIC QUADRIPARESIS - Infants who have this type of CP also pass through a stage of hypotonia prior to the appearanceof spasticity and increased muscletone. - Early manifestations of spasticity aresimilar to those described in other spastic clinical types and affect both lower and upper extremities. Management of CP - The long-range management of children who have CP addresses both motor dysfunction and associated nonmotor deficits. Motor Development Goals - Improve function and prevent secondary complications of the musculoskeletal system. - Establish realisticexpectations for the child’s motor function - Ensure that progressivecontractures and deformities do not interfere with optimal outcome. - Promote gross motor skills. - Occupational therapy concentrates on eye-hand coordination and UE motor control to attain the highest level of independence in self-careand other activities of daily lifethatinvolvehand fxn. - Pseudobulbar palsy in bilateral impairmentof the motor system leads to oral motor dysfunction manifested as early sucking,swallowing,and chewing difficulties, can lead to impaired speech. - Parental involvement and trainingin special feedingtechniques AssistiveDevices Biofeedback Bracing Antispasticity Medications - Affect muscletone through different mechanisms of action. - Diazepam - a centrally actingmusclerelaxant - Dantrolene -Na reduces spastic hypertonicity by the contractileproperties of skeletal mm fibers - Baclofen - a gamma-aminobutyric acid derivativethatis thought to inhibitneurotransmission. - More effective for spasticity of spinal origin,butit may be used for adjunctivetreatment in CP that is accompanied by severe spasticity. - Topical injection of phenol,alcohol solution,or botulismtoxin into the motor points or motor nerves of a spastic musclecreates a temporary neurolysis and consequenttone reduction lasting4 to 6 months. Most often is applied to alleviatespasticity of the ankleplantar flexors and hip adductors. Surgical Therapy Orthopedic - lengthening of a tight or contracted heel cord corrects toe walkingand enhances the efficiency of gait. - releaseof spastic hip and knee flexors and tenotomy of the hip adductors - In severely affected children,the primary reason for surgery is to prevent progressiveskeletal deformities that inevitably followspastic muscleimbalance. - Acquired hip dislocation,scoliosisareseverecomplications of spasms Neurosurgical - Selective posterior rhizotomy is the current neurosurgical approach to alleviatespasticity. - L2 to L5 laminectomy to localizethe posterior roots in the caudaequina. - Electrical stimulation applied to the individual rootlets is monitored by electromyography. - Rootlets that produce clonic or sustain stimulusresponse,especially with spread to other musclegroups are severed becausethey are thought to be responsiblefor spastic disinhibition - Recommended mostly for those children who have spastic diplegiabutwho can walk and have adequate strength, motor control,balance,and reasonableintelligence.
  20. 20. - Daily physical therapy for 6 to I 2 months after surgery - Post-op complicationsarerareand includetransientparesthesiasand lossof bladder control. - Although the procedure reduces spastic hypertonicity,whether it significantly influences the natural courseof motor disability remains a matter of debate. ASSOCIATED DYSFUNCTION Hearing - High-f hearingloss:70%of athetoid CP - Other types have 30% frequency of sensori-neural hearinglossis approximately 30% Speech and languagedevelopment Uncontrolled drooling:sx of pseudobulbar palsy,improves spontaneously by 5-10 yo in some Vision - Refraction errors and retinopathy of prematurity - High incidenceof strabismus(75%) esp in spastic type–tx to prevent amblyopia Seizures - ~50% of the children who have CP develop some form of seizure disorder. - highest incidencein spastic hemiparesisand quadriparesis. Mental Retardation: 50% of those affected, others may have LD Walking:Energy expenditure for walkingand other physical activities is several times abovenormal Outcome - Walkingis notexpected of children who cannot sitby 4 years of age. - Motor control of the upper extremities is adequate to perform ADLs in diplegia and usually in cases of mild ambulatory quadriparesis. - Some children who have quadriparesis&rely on wheelchair for mobility may achievepartial independence in self- care. ~25% of this group requires help for all activities. CCC17: Febrile vs.Non-febrile seizures (hillary’s notes) Febrilevs. Non-Febrile Seizures in Children FEBRILE SEIZURES EPIDEMIOLOGY: about 5% in N. America INCIDENCE - MC between ages of 6 months and 5 years (peak between 18-24 mo) RISK FACTORS GeneralPopulation - Family hx of febrilesz in 1st or 2nd degree relatives - Developmental delay - Nursery stay > 30 days - Daycareattendance ChildrenwithFebrileIllness - Family hx of febrilesz in 1st or 2nd degree relatives - Peak T duringthe illness(notT at time of sz) - Nature of underlyingillness(eg.AGE, OM, HHV-6 or 7) PATHOPHYSIOLOGY - Pathophysiology is unclear,maybeIL-1, genetic factors,multifactorial - The PEAK temp is significant notthe rate of riseor fall SUBTYPES AND CLINICAL FINDINGS COMPLEX - Focal - Duration >15 minutes - Multipleszwithin the samefebrile illness (within 24 hr) - 15-20% of febrilesz SIMPLE - Generalized - Duration < 15 minutes - About 75% of all febrilesz
  21. 21. DDx - CNS infection (encephalitis,meningitis) - Meds/Toxins (Shiga toxin, salicylates,anticholinergics,cocaine,tricyclics,amphetamines,etc) - Metabolic disorder (hypoglycemia,hypocalcemia) - Shaken baby syndrome - Head trauma - Underlyingbrain disease(epilepsy,tuberous sclerosis,etc.) Diagnostic Workup:Hx, PE, neuro exam, look sick?,LP, Lytes, imaging,EEG AAP Guidelines LumbarPuncture - Strongly consider in infant< 1 y/o - Probably “mandatory” in infant< 6 m/o - Remember meningeal signs subtleto non-existent in 12 to 18 month age group - Not necessary in child >18 m/o if H & P not suspiciousfor meningitis - Recommended in all children with complex febrilesz, especially if focal or prolonged BloodStudies - Children 6 m/o and over of limited valueunless suspicioushx (eg. vomiting, diarrhea) - Infants < 6 m/o detailed lab studies may be helpful in select cases ImagingStudies - Limited valueand not necessary prior to LP - EXCEPTION: Neuroimaging indicated in febrileszof 30 min or more (febrilestatus epilepticus).MRI is the method of choice. EEG - Little role except for febrilestatus epilepticus TREATMENT - No Tx for simplefebrileseizures - For those at high risk for recurrence, rectal diazepamcan be given as ABORTIVE THERAPY ParentalEducationandCounseling - Reassurance:szwon’t causebrain damageand chanceof developing epilepsy small (2-10%) - Advisethem itcould recur especially in first24 hours (30%recurrence in 1st year) - Teach mgmt of sz: liechild on side,maintain airway,call EMS if sz> 3 min - Morbidity and mortality essentially zero NON-FEBRILE SEIZURES EPIDEMIOLOGY AND INCIDENCE - Overall incidenceof childhood epilepsy frombirth to 16 years is about1-2% - 1% of children will haveatleastone afebrileszby age 14 years - Epilepsy is the MC chronic neurologic disorder in childhood CLASSIFICATION - Partial o Simple partial:brief tonic-clonicmovement of face, neck, extremities; head turning; eye deviation o Complex partial:consciousnessimpaired;children may pick at things,blink,and stare o Onset at any age - Generalized o Generalized tonic-clonic:alternatingstiffening/shaking,tongue biting, LOC, incontinence,and postictal recovery phase o Generalized absence: brief LOC (rarely >30 sec) but w/o loss of postural tone o Others: Myoclonic,tonic,clonic,atonic
  22. 22. - Epilepsy syndromes: Seizure syndromes incl.infantilespasms,benign epilepsy of childhood,Lennox-Gastaut, and juvenilemyoclonic epilepsy EVALUATION AND DIAGNOSIS DDx - Generalized tonic-clonic:Syncope,breath-holding,cardiac arrythmia - Generalized absence: Behavioral staring,complex partial seizures,tic disorder - Complex partial:Sleep walking,night terrors, benign paroxysmal vertigo,migrainedisorders,self- stimulatory behavior - Epileptic myoclonus:Physiologic in sleep,startlemyoclonus Diagnostic Workup - Hx, PE, EEG, MRI, LP,labs Treatment - AED selection based on seizuretype - ~80% of pts w/1° generalized seizure epilepsy & 65% of pts w/ partial seizureepilepsy achievesatisfactory control with AED tx CCC18: Seizures in Adults  Seizure: the clinical manifestation of an abnormal and excessiveexcitation and synchronization of a population of cortical neurons.  Seizure is a symptom, not a disease!!  Occur b/c of too much excitation,too littleinhibition =hyperexcitablenetwork  Epilepsy: two or more recurrent seizures unprovoked by systemic or acute neurological insults.  Epilepsy is a disease,a syndrome, or a neurological disorder. Classification of Epileptic Seizures (refer to word document if further description is warranted)  Partial Seizures( Seizures beginninglocally) 1. simplepartial seizures ( consciousness notimpaired) 2. Complex Partial seizures ( With impairmentof consciousness) 3. Partial seizures( simpleor complex),secondarily generalized  Generalized Seizures - bilaterally symmetric, without localized onset, l/o consciousness 1. Absence seizures 2. Myoclonic seizures 3. Clonic seizures 4. Tonic seizure 5. Tonic-clonic seizures (“grand mal”) 6. Atonic seizures Seizure risk factors – Family history – History of febrileseizures – History of head trauma – History of meningitis – History of stroke or other structural lesion Evaluation of a 1st seizure: History,physical Blood tests: CBC, electrolytes, glucose,Calcium,Magnesium,phosphate, hepatic and renal function
  23. 23. Lumbar puncture only if meningitis or encephalitis suspected and potential for brain herniation isruled out Blood or urinescreen for drugs Electroencephalogram CT or MR brain scan Seizure Precipitants  Metabolic and Electrolyte Imbalance  Stimulant/other proconvulsantintoxication  Sedative or ethanol withdrawal  Sleep deprivation  Antiepileptic medication reduction or inadequate AED treatment  Hormonal variations  Stress  Fever or systemic infection  Concussion and/or closed head injury Factors to consider for a woman with epilepsy: Drug if on OCP? Extra folateb/c seizures can lead to fetal malformation Life style modifications:  Adequate sleep  Avoidance of alcohol,stimulants,etc.  Avoidance of known precipitants  Stress reduction — specific techniques Non-medical treatment options for epilepsy  Ketogenic diet ( Anti-seizure effect of ketosis)  Vagal nerve stimulator (Intermittent programmed electrical stimulation of left vagus nerve)  Epilepsy surgery Psychogenic Seizures  A common symptom of conversion or somatization disorder.  Maintain a high degree of suspicion when seizure arerefractory to therapy or when atypical features are present.  Diagnosisis madeby recordingtypical events with video-EEG. Status Epilepticus Status epilepticus is defined as:1) an episodeof more than 30 minutes of continuous seizureactivity,or 2) two or more sequential seizures spanningthis period withoutfull recovery between seizures.Clinically,however, most seizures lastless than 5 minutes, and those persistinglonger areunlikely to stop spontaneously. Therefore, one should initiatetreatment for the seizures lastinglonger than 5 minutes. The incidenceof status epilepticus is atleast60,000 cases/year in theU.S., with higher rates among the very young and very old. Status epilepticus is an emergency becauseof its morbidity and mortality,and any seizuretype may manifest as status
  24. 24. epilepticus.The outcome of convulsivestatus epilepticuslargely depends on etiology, but prompt treatment can improve outcome. From a practical standpoint,status epilepticus may be divided into convulsiveand nonconvulsiveforms.The convulsiveforms may be generalized or partial.Thenonconvulsiveforms aredifficultto classify on clinical grounds, but areoften divided electroencephalographically into absencestatus (in which the EEG demonstrates generalized spike-wave activity) and complex partial status (in which the EEG may show a variety of localized rhythmic discharges. CCC19: Brain tumors 35% of Pediatric solid tumors occur in the CNS Most come from supportivecells (astrocytes,oligodendrocytes,ependymal,pinealocytes) Only a few come from neurons Cell of origin: Neural stem cells  lead to gliomas Prevalence Adults: meningiomas > glioblastomas >other astrocytomas Kids:Pilocytic astrocytomas >Medulloblastomas >other astrocytomas Risk factors for childhood tumors: Prenatal x-rays Cranial radiation in childhood Viruses,industrial chemicals,cell phones HIV infection  CNS lymphoma Genetic predisposition for NF1 (loss of NF1 tumor suppressor gene) and Li -Fraumeni (loss of p53 tumor suppressor gene) S/S: *Head ache, behavioral change,GI upset, balanceor gait change, “Alzheimer’s”, school work suffers,falling, shortened attention span,visual or hearingimpairment,Mom is in your officemore than once! *Pupillary changes,gaitdifficulties,papilledema,seizures,speech defect, neglect, memory changes, visual field cut, galactorrhea,menigismus. Diagnosis: MRI is the best test b/c it shows blood flow and water movement Ring enhancement often means GBM CT’s miss low-grades Herniation Syndromes Uncal – temporal lobe impinges the PCA, contralateral peduncle, midbrain Unilateral dilatd pupil (CNIII compression),oculomotor palsy,contra hemiparesis Central – midlinetumors compress basal ganglia(midbrain symptoms) Tonsillar –posterior fossa tumors compress downward into the foramen magnum
  25. 25. *cough + pass out = tonsillarherniation Emergency management Increased intracranial pressurecan kill! Seizures Deep Venous Thrombosis – brain tissueis clotagenic Treatments: Resection surgery Decision to operate includes:age, performance status,location of tumor, feasibility of debulking/complete resection, if recurrence – time since Radiation Therapy Goal:damage DNA of tumor cells,so try to localize tx to sparenormal brain and fractionate to exploit differences in repair Chemo principles –works best when tumor burden is small,combineactivedrugs Radiosurgery = GammaKnife/Cyberknife/Synergy S Glial Brain Tumors Astrocytes (provideblood vessel structure support) Low-Grade Seizure as 1st sign Not cured with resection aloneunless a rarepilocytic tumor High-grade (Anaplastic Astrocytoma- 3 year survival & Glioblastoma Multiforme(GBM 80% of high grade, survive1 year)) Ring-like,enhancing,edematous – risk of herniation Don’t let the “capsule” fool you  spreads,necrotic Oligodendrocytes (provideaxonal support) Low-grade Present with seizureor subtleneurological impairment MRIs show calcifications Chemosensitive High-grade Meningioma B9, off the dura “dural tail”,20%of all intracranial neoplasms Sx for symptomatic, sometimes discovered incidentally Primary CNS Lymphoma B-cell,non-Hodgkin’s Lymphoma Prevalent in immunocompromised Tx: high dose methotrexate
  26. 26. Pituitary adenoma Tumors of endocrine system, are they even cancer? Do not have high growth rates or met Big question: arethey secretingor not? Are they causingmass effects or not? Tx: may leave aloneif asymptomatic,medical for secretingtumors, transsphenoidal resection if mass lesion,usually handled by endocrinologist CCC20: Brain tumors in Kids 2/3 of pediatric brain tumors can be cured usingsome combination of rx, chemo, and/or radiation,butthe younger brains aremore susceptibleto Side effects Medulloblastomas 25% of pediatric tumors, less than 3 years/age usually Cerebellum = medulloblastoma,clipsoff the 4th ventricle S/S: obstructivehydrocephalus,Ha,N/V, ataxia,and drowsiness Tx: managingthe ICP + tumor resection  then adjuvanttherapies (sx will notcurealone) Low Grade Astrocytomas Pilocytic predominates Cerebellar – rare Enpendymoma Majority in posterior fossa Brain stem gliomas Diagnosisby MRI and suspicion (is notremoved) Intracranial Germ cell tumors Pineal region is the majority S/S – panhypopituitarism,diabetes insipidus,visual disturbances Biopsy – need to determine b/w germinoma vs. non-germa vs. teratoma Pure Germinoma - Radiation aloneis curative Non-germanomatous – worse prognosis Craniopharyngioma Epithelial cellsin Rathke’s pouch Calcification and cystic appearanceareclassicfinding CCC21: Cranial Nerve Disorders I. Cranial Nerve I (olfactory nerve) a. Function(s) i. Special Somatic Afferent (SSA) – Smell b. Anatomy (Moore & Dalley, p.1133; Blumenfeld, p. 465) i. Intranasal fibers run through the cribriformplateto the olfactory bulb ii. Olfactory bulb to the olfactory tractjustinferior to the frontal lobes and superior to the optic nerves and chiasm
  27. 27. iii. Enters anterior perforated substanceand runs to the olfactory cortex; NOTE: there is no relay in the thalamus – this is the only sensory pathway that does not run through the thalamus c. Symptoms of dysfunction i. Inability to smell (anosmia) ii. Disordered taste – often lack of taste or bad taste – becausemuch of the taste of food is derived from smell rather than taste d. Differential diagnosis of dysfunction i. Trauma, either local or generalized head injury thattears the fragilefibers crossingthe cribriformplate ii. Dry or clogged airways iii. Allergic rhinitis iv. Nasal polyps v. Foreign bodies vi. Autoimmune/inflammatory disease(e.g., Wegener’s granulomatosis) vii. Medications – antihistamines,antibiotics,anti-inflammatories,anti-thyroid,anti- metabolites viii. Tumors of the olfactory groove – meningiomas,craniopharyngiomas ix. Bad smells (burningrubber,skunks) may occur in the setting of seizure, are intermittent, and are often followed by alteration in consciousnesswith or without generalized seizureactivity x. Sense of smell normally declines with age xi. Psychogenic e. Evaluation i. Test sense of smell by occludingone nostril,ask patientto identify a smell through the unoccluded nostril (peppermint, vanilla,lemon) ii. Brain imagingis very important to ruleout structural lesion II. Cranial Nerve II (optic nerve) (Moore & Dalley,pp. 1132-1136;Blumenfeld, pp. 431-453) a. Function(s) i. Special Somatic Afferent (SSA) – Vision b. Anatomy i. Special fibers in the retina collectdata regardinglight,color,movement, and shapes ii. Retinal fibers join to form the optic nerve iii. The optic nerve continues straightback from the back of the eye, below the frontal lobes,and then join one another at the optic chiasm iv. The optic chiasmconsistsof crossingnasal fibersfromeach eye; the lateral edges of the optic chiasmcontain non-crossingtemporal fibers fromeach eye 1. NOTE 1: The nasal fibers arethosefibers that arisefromthe nasal aspectof the retina; however, due to the lenses in the eyes, the nasal fibers actually pick up information from the temporal visual field in each eye. The temporal fibers in the optic nerve and chiasmarisefromthe temporal aspect of the retina but carry information aboutthe nasal visual fields. 2. NOTE 2: Know and understand the diagrams on Moore & Dalley,p. 1134-1135 or Blumenfeld, p. 442) v. The temporal fibers from the ipsilateral eye and the nasal fibers fromthe contralateral eye join to form the optic tract, which starts justposterior to the optic chiasm vi. The optic tract synapses in the lateral geniculatenucleus (or body), LGN, justlateral to the midbrain. vii. Optic radiationsspread outfrom the LGN and travel through the temporal and parietal lobes to reach their destination in the visual cortex of the occipital lobes. c. Symptoms of dysfunction
  28. 28. i. Loss of vision in oneor both eyes ii. Visual field cuts iii. Afferent pupillary defect(APD, or Marcus-Gunn pupil) d. Differential diagnosis of dysfunction i. Loss of vision in oneeye (or both) 1. Optic neuritis (think multiplesclerosis) 2. Microvasculardamageto the nerve 3. Trauma 4. Tumor of the optic nerve or optic nerve sheath 5. Intra-ocular pathology (does not usually causeAPD) ii. Visual field cut 1. Bitemporal field cut – chiasmal lesion such as pituitary tumor or aneurysm 2. Homonymous hemianopsia –contralateral hemispheric lesion such asstrokeor tumor 3. Superior quadrantinopsia –contralateral temporal lobe lesion such as strokeor tumor 4. Inferior quadrantinopsia –contralateral parietal lobe lesion such as strokeor tumor 5. Homonymous hemianopsia or quadrantinopsia with macular sparing – contralateral occipital lobe lesion iii. Afferent pupillary defect (No discussion in Moore& Dalley;use Blumenfeld, p. 541, 544- 545) 1. A flashlightaimed into the normal pupil causes consensual pupil constriction; when the flashlightis moved to the abnormal eye (“swingingflashlighttest”), both pupils dilatebecausethe abnormal optic nerve “sees” less lightand triggers CN III to dilateboth pupils 2. Also known as “Marcus-Gunn”pupil; a. caused by ischemia or demyelination/inflammation (optic neuritis) in the optic nerve ipsilateral to the dilatingpupil;think multiplesclerosis in a young person and ischemia in an older person b. Other causes: tumor, trauma e. Evaluation i. Pupillary reflex (Afferent = CN II,Efferent = CN III) ii. Fundoscopic evaluation iii. Loss of vision in oneeye (or both) 1. Opthalmology evaluation – look at the fundus with a fundoscope 2. Think multiplesclerosis –brain MRI to look for lesions and spinal tap to evaluate cerebrospinal fluid (CSF) 3. Visual Evoked Response (VER) – the patient is shown a series of patterns on a screen, and the electrical impulses fromthe eyes are measured in the brain;a slowed responseindicates demyelinatinglesion in theoptic nerve iv. Visual field cut 1. Brain MRI to look for the structural lesion –know your anatomy; where areyou lookingfor the abnormality? 2. Formal visual field tests –the patient’s head is positioned facinginto a large bowl; lights areflashed into the bowl, and the patient indicates to the technician when a lightis seen. A map of the patient’s visual field isderived from the pattern of lights seen and not seen. v. Afferent pupillary defect 1. Brain MRI to look for lesions and spinal tap to evaluateCSF for multiple sclerosis
  29. 29. III. Cranial Nerve III (oculomotor nerve) (Moore & Dalley,pp. 968-973;Blumenfeld, pp. 530-532) a. Function(s) i. General Somatic Efferent (GSE) – Motor to levator palpebraeand all extraocular muscles other than superior oblique(CN IV) and lateral rectus (CN VI) ii. General Visceral Efferent (GVE) – Parasympathetics to papillary constrictor and ciliary muscles for lens accommodation b. Anatomy i. Leaves the posterior midbrain and tracks alongthesuperior cavernous sinus and enters the orbitthrough the superior orbital fissure ii. The nerve fibers divideinto superior and inferior divisions;the superior division carries motor fibers to the superior rectus and levator palpebraesuperioris;the inferior division carries motor fibers to the other oculomotor-innervated extraocular muscles and the parasympathetic fibers iii. Parasympathetic fibers synapsein the ciliary ganglion (located justposterior to the eyeball in the orbital socket) and connect to the papillary sphincters c. Symptoms of dysfunction i. Double vision (diplopia) ii. Inability to raiseeyelid (ptosis) iii. Loss of pupil lightresponseand dilated pupil on examination d. Differential diagnosis of dysfunction i. Horizontal doublevision results fromlack of conjugateeye movement in the horizontal directions of gaze; oculomotor palsy causes failureof eye adduction on the side ipsilateral to the nerve lesion,e.g., intracranial massor oculomotor nerve ischemia ii. Ptosis results fromoculomotor lesion ipsilateral to the lesion,e.g., intracranial mass or oculomotor nerve ischemia iii. Dilated pupil results fromcompression of the oculomotor nerve. Autonomic fibers run on the surfaceof the nerve and, when compressed, resultin pupillary dilation. Causes may includeaneurysm,increased intracranial pressure(causes dilated,fixed pupils eventually), tumor, intracranial bleeding,fractureof the cavernous sinus. iv. A complete CN III palsy includes ptosis,dilated pupil with loss of pupillary lightresponse, and turning of the eyeball down and out (temporally) e. Evaluation i. Careful examination to isolatewhich extraocular functionsareaffected, pupil evaluation,and look for ptosis (ptosis in a CNIII lesion is usually quiteobvious) ii. Brain imaging – head CT or MRI to look for mass lesion (aneurysm,tumor, bleeding) IIIb. Cervical Sympathetic Chain – OK, this isn’ta cranial nerve, but its anatomy is worth reviewing with CN III becausedamage to the chain causes symptoms that can be confused with CN III pathol ogy if the examiner is not careful… (No discussion in Moore& Dalley;use Blumenfeld pp. 542-544) a. Function(s) i. Sympathetic innervation of the pupillary sphincter – causes pupil dilatation b. Anatomy i. Starts in the spinal cord atT1, runs to the superior cervical ganglion atthe carotid bifurcation,where the nerves synapse ii. Runs superiorly alongthe internal carotid into the superior orbital fissureto terminate on the pupillary dilators c. Symptoms of dysfunction
  30. 30. i. Horner’s syndrome – ptosis,miosis,anhydrosis –all ipsilateral to the lesion d. Differential diagnosis of dysfunction i. Pancoasttumor in the upper lung lobe can compress the cervical sympathetic chain ii. Carotid dissection or aneurysminterrupts the cervical sympathetic fibers iii. Trauma to the neck from surgery, whiplash,etc. e. Evaluation i. Consider the history – chest X-ray,carotid ultrasound or angiogrammay be indicated IV. Cranial Nerve IV (trochlear nerve) (Moore & Dalley,p. 1142; Blumenfeld, pp. 538-539) a. Function (s) i. General Somatic Efferent (GSE) – Motor to the superior obliquemuscle,which ANATOMICALLY pulls theeye down and out and intorts the eye medially;CLINICALLY, the musclepulls the eye down when it is already adducted (the “cheating” muscle) and intorts the eye when itis already abducted b. Anatomy i. Decussates completely around the periaqueductal gray matter – the only cranial nerve to completely cross ii. Exits from the posterior surfaceof the midbrain – the only cranial nerveto exit from the posterior midbrain iii. Wraps forward around the brainstem,enters the cavernous sinus iv. Crosses over CN III to enter the posterior orbitthrough the superior orbital fissureand terminates on the superior obliquemuscle c. Symptoms of dysfunction i. Double vision when lookingdownward ii. The eye deviates upward and outward when CN IV is lesioned iii. The person can rotate the head to compensate for the double vision –head tiltis to the sideopposite the injured trochlear nerve iv. Rare to have an isolated CN IV lesion d. Differential diagnosis of dysfunction i. Head injury tears the fragilenerve, often bilaterally ii. Mass lesion to the dorsal midbrain –tumor, hemorrhage, aneurysm iii. Congenital (children) iv. Ischemia (especially older adults) e. Evaluation i. Careful extraocular movement evaluation,typically with a red lens (Right eye is covered with a red lens,then a white lightis shined into both eyes. The relationship of the red image to the white lightis compared as the patient looks straightahead,to the sides, up, and down – people go through fellowships to learn how to do this so don’t strain too hard right now to try to understand this test; justtrust me that it can be done) ii. Brain imagingwith CT or MRI if mass is suspected;a mass typically causes a litany of other findings,notjustan isolated trochlear palsy V. Cranial Nerve V (trigeminal nerve) (Moore & Dalley pp.1139-1142;Blumenfeld pp. 474-478) a. Function (s)
  31. 31. i. General Somatic Afferent (GSA) – Sensory fibers to the face, mouth, anterior two-thirds of the tongue, meninges, and nasal sinuses ii. Special Visceral Efferent (SVE) – Motor fibers to muscles of mastication and tensor tympani in the ear b. Anatomy i. Arises from the mid-pons, passes anterolaterally acrossthesubarachnoid space,where it forms a largeganglion justover the tip of the petrous bone; note that the trigeminal nuclei extend all the way into the upper cervical cord ii. After the ganglion,the nerve divides into three branches 1. V1 – Opthalmic branch (sensory only) a. Passes through the cavernous sinus with CN IV, then enters the orbit through the superior orbital fissure b. Branches run to the lacrimal gland,skin over the forehead and scalp, and sensory fibers to the cornea,medial eyelid, inner nose 2. V2 – Maxillary branch (sensory only) a. Passes through the inferior cavernous sinus,leaves theskull through foramen rotundum, then enters the orbitthrough the inferior orbital fissure b. Branches run to the skin of the maxillary region,teeth and bums of the upper jaw, upper lip,lower eyelid,and meninges. Two branches run to the sphenopalatineganglion,which controlsthe secretion of the lacrimal gland 3. V3 – Mandibular branch (sensory and motor) a. One branch runs with CN VII to providesensation to the auditory meatus and tympanic membrane b. Other branches register sensation fromthe lower jaw, lower lip,lower teeth, chin;and provide fibers to trigger salivation in theparotid, sublingual,and anterior lingual glands c. Motor to the tensor tympani, mylohyoid, anterior digastric,and mastication muscles c. Symptoms of dysfunction i. Pain in one or more distributionsof the nerve (trigeminal neuralgia,or tic doloreaux) ii. Difficulty chewing,with mandibledeviatingipsilateral to (toward) the lesion iii. Facial numbness,includinglossof corneal reflex d. Differential diagnosis of dysfunction i. Trigeminal neuralgia may resultfromdemyelinating lesion to the nerve as it exits the mid-pons (as in multiplesclerosis),fromherpes zoster, or from small vessels wrapping around and compressingthe nerve ii. Facial numbness and weakness of muscles of mastication may resultfrom pontine stroke, trauma, tumors, herpes zoster, aneurysms,or meningeal infections iii. Watch the associated symptoms; lesions of the spinal nucleus of the trigeminal nerve may present in conjunction with other spinal symptoms,includingcervical spinal level, arm symptoms, etc. e. Evaluation i. Corneal reflex (Afferent = CN V, Efferent = CN VII) ii. Brainstemimaging(must be MRI; cannotuse CT, which does not image the brainstem well because of artifactfrom nearby bone) iii. Consider multiplesclerosisin cases of trigeminal neuralgia in younger people, especially women – to evaluate, perform MRI brain and cervical spineand spinal tap VI. Cranial Nerve VI (abducens nerve) (Moore & Dalley,pp. 1142-1143;Blumenfeld, pp. 539-540) a. Function (s)
  32. 32. i. General Somatic Efferent (GSE) – Motor fibers to the lateral rectus muscle,which abducts the eye b. Anatomy i. Leaves the brainstemat the pontomedullar junction,crosses thebasilar artery in the subarachnoid space ii. Enters the dura over the clivus,bends over the petrous portion of the temporal bone, then enters the cavernous sinus iii. Runs parallel to the internal carotid through the cavernous sinus,then enters the posterior orbitand innervates the lateral rectus muscle c. Symptoms of dysfunction i. Double vision frominability to abductthe eye ipsilateral to the injured nerve ii. At rest, the affected eye may deviate medially becauseof unopposed action of the medial rectus d. Differential diagnosis of dysfunction i. Isolated lesion of the abducens nerve from tumor or ischemic lesion ii. Increased intracranial pressurecauses thebrain to compress the nerve as itpasses over the temporal bone; this compression is often bilateral,butnot always iii. Aneurysm of the Circleof Willisor the internal carotid in thecavernous sinus iv. Cavernous sinus thrombosis or tumor e. Evaluation i. Vestibulo-ocular reflex or calorics(Afferent = CN VIII,Efferent = CN VI) ii. Brain imaging(CT or MRI) VIb. A special situation involving CN VI: Intranuclear opthalmoplegia (No discussion in Moore& Dalley; use Blumenfeld, p. 549) A shorttract connects the nucleus of CN VIII to the ipsilateral nucleus of CN VI. The medial longitudinal fasciculus(MLF) connects the nucleus of CN VI to the contralateral nucleus of CN III. Normally,when the head turns, CN VIII communicates with CN VI on the sideaway from the head turn so that CN VI tells the lateral rectus to hold the gaze by turningthe eye laterally. At the same time, a signal travelsfromthat CN VI (1) to suppress the movement of the contralateral CNVI and (2) through the MLF in the central midbrain to the contralateral CNIII nucleus to tell the other eye to deviate medially. In a lesion of the MLF, CN VI cannot communicate with CN III. As a result,the intactCN VI laterally deviates its eye, but the oppositeeye does move pastmidline. The intacteye then develops nystagmus in lateral gaze, as if the eye is tryingto recruitthe other eye to move with it. This finding is called intranuclear opthalmoplegia. The lesion is in theMLF on the sideof the eye that failsto medially deviate. VII. Cranial Nerve VII (facial nerve) (Moore & Dalley,pp.1143-1146;Blumenfeld, pp.479-484) a. Function (s) i. General Visceral Efferent (GVE) – Motor fibers to the muscles of facial expression, includingplatysma,frontalis,stapedius(dampens loud sounds),orbicularis ii. General Somatic Afferent (GSA) – Sensation from the external auditory canal iii. Special Visceral Afferent (SVA) – Taste from the anterior 2/3 of the tongue b. Anatomy
  33. 33. i. Both cerebral cortices supply fibers for the forehead to both facial nervenuclei in the pons. The cerebral cortex supplies fibers for the cheek and jawto only the contralateral facial nervenucleus in the pons. (Text in Moore & Dalley,pp. 1143-1146;Illustration in Blumenfeld, p. 483) ii. The nerve exits at the pontomedullary junction as a largemotor nerve and a smaller intermediate nerve that carries sensory and parasympathetic fibers. The nerve passes across a longintraosseus coursethrough the skull,sendingoff fibers to the chorda tympani (taste), stapedius muscle,and the greater petrosal nerve. iii. The nerve exits the skull through the stylomastoid foramen and passes through the parotid gland,where it breaks into five major branches to the muscles of facial expression 1. Temporal TWO 2. Zygomatic ZEBRAS 3. Buccal BIT 4. Marginal Mandibular MY 5. Cervical CAT c. Symptoms of dysfunction i. This is the cranial nervemost likely to suffer injury ii. Facial droop 1. Upper motor neuron – droop of the nasolabial fold and lipscontralateral to the lesion,forehead spared 2. Lower motor neuron – droop of the entire face ipsilateral to the lesion (Bell’s palsy) iii. Loss of taste to the anterior 2/3 of the tongue (not usually evidentclinically,as theother sideis typically functioning) iv. Hyperacusis ipsilateral to the lesion – hearingmay be louder because the stapedius is not dampening loud sounds in the inner ear d. Differential diagnosis of dysfunction i. Bell’s palsy 1. Viral is mostcommon cause 2. Trauma 3. Tumor, especially of the parotid gland ii. Central lesion 1. Stroke 2. Tumor e. Evaluation i. Corneal reflex (Afferent = CN V, Efferent = CN VII) ii. Brain imaging(MRI is best to visualizethe brainstem) VIII. Cranial Nerve VIII (vestibulocochlear nerve) (Moore & Dalley,pp. 1146-1147;Blumenfeld, pp 484- 494) a. Function (s) i. Special Somatic Afferent (SSA) – Hearing through the cochlear portions of the nerve ii. Special Sensory Afferent (SSA) – Fibers to the vestibular apparatus in theinner ear b. Anatomy i. The nerve exits the upper medulla near the pontomedullary junction and extends straightinto the internal acoustic meatus,where it splits into the vestibular nerveto the vestibular apparatus and the cochlear nerve to the cochlea c. Symptoms of dysfunction i. Dysequilibrium,usually vertigo ii. Loss of hearing – sensorineural deafness d. Differential diagnosis of dysfunction
  34. 34. i. Vestibular schwannoma (a.k.a. acoustic neuroma) or cerebellopontineangle meningioma ii. Ischemic damage to the nerve iii. Meniere’s disease iv. Brainstemstroke v. Multiplesclerosiswith lesion in the medulla vi. Vascular anomaly or tumor in the medulla e. Evaluation i. Vestibulo-ocular reflex (Afferent = CN VIII,Efferent = CN IX) ii. Dix-Hallpikemaneuver – have the patient lay backward quickly with the head turned 45 degrees toward the examiner; vestibular dysfunction will causenystagmus within 1 minute after layingdown with the head turned toward the malfunctioningnerve/ vestibular apparatus iii. Electro- or video-nystagmogram – usually doneby ENTs, a video or electronic measurement of nystagmus direction and intensity iv. Brain imaging(CT or MRI) for vestibular schwannoma v. Brain imaging(MRI is better) for brainstemstroke or MS IX. Cranial Nerve IX (glossopharyngeal nerve) (Moore & Dalley,pp. 1147-1150;Blumenfeld, pp. 495- 496) a. Function (s) i. General Somatic Afferent (GSA) – Sensation from the posterior oropharynx,posterior 1/3 of the tongue, middleear, and external auditory meatus ii. Special Visceral Afferent (SVA) – Taste from the posterior 1/3 of the tongue and chemo- and baroreceptors of the carotd body iii. Special Visceral Efferent (SVE) – Parasympathetic fibers to the parotid gland to mediate salivation iv. General Visceral Efferent (GVE) – Motor fibers to stylopharyngeus muscle b. Anatomy i. Nucleus in the upper medulla;fibers leave justinferior to the pontomedullary junction and leave the skull through the jugular foramen ii. Fibers pass through the lesser petrosal nerve, synapsein the otic ganglion,and provide parasympathetics to the parotid gland iii. Other fibers travel to their ultimate destinations in thetongue, carotid body, and ear iv. Only muscleinnervated by the glossopharyngeal isstylopharyngeus,which helps to elevate the palate c. Symptoms of dysfunction i. Lost sensation to the posterior oropharynx ii. Glossopharyngeal neuralgia(intense,paroxysmal pain in theposterior oropharynx) d. Differential diagnosis of dysfunction i. Tumors near the jugular foramen,often in association with other cranial nervepalsies ii. Deep neck injury e. Evaluation i. Gag reflex (Afferent = CN IX,Efferent = CN X) ii. Brainstemimaging(MRI is best) X. Cranial Nerve X (vagus nerve) (Moore & Dalley,pp. 1150-1151;Blumenfeld, pp. 496-498) a. Function (s) i. Special Visceral Efferent (SVE) – Motor fibers to pharyngeal muscles (swallowing,gag reflex) and laryngeal muscles (voicebox)
  35. 35. ii. General Visceral Efferent (GVE) – Parasympathetics to heart, lungs,and the upper GI tract (to the splenic flexure) iii. General Somatic Afferent (GSA) – Sensation from pharynx,meninges, and a small area near the external auditory meatus iv. Special Visceral Afferent (SVA) – Taste from epiglottis and pharynx v. General Visceral Afferent (GVA) – Chemo- and baroreceptors from the aortic arch b. Anatomy i. Exits the brainstemfrom the upper medulla then enters the skull through the jugular foramen ii. After leavingthe skull,this nerve spreads into fibers thatgo all over the place,including oropharynx,aortic arch,GI tract, etc. c. Symptoms of dysfunction i. Unusual to have a lesion justof the vagus nerve ii. Palatedroops on the sideof the lesion,causingtheuvula to deviate AWAY FROM the lesion (uvula is drawn toward the normal sideof the palate,which rises and pulls the uvula upward) iii. Dysphagia (troubleswallowing) iv. Voice hoarseness due to laryngeal muscleweakness or recurrent laryngeal nerveinjury v. Aphonia (loss of voice) and inspiratory stridor vi. Unusually,denervation of the heart results in restingtachycardia d. Differential diagnosis of dysfunction i. Head and neck tumors ii. Neck surgery may traumatize the recurrent laryngeal nerve iii. Thoracic surgery may traumatize the fibers of the vagus that travel to the heart and GI tract e. Evaluation i. Clinical evaluation is best;very difficultto visualizethis nerve in imagingor by other methods ii. Valsalvamaneuver and carotid body massagewill producebradycardia in a normally functioningvagus nerve XI. Cranial Nerve XI (spinal accessory nerve) (Moore & Dalley,pp.1151-1153;Blumenfeld, p. 499) a. Function (s) i. Special Visceral Efferent (SVE) – Motor fibers to sternocleidomastoid and upper trapezius muscles to laterally rotatethe head and elevate the shoulders (as in shoulder shrug) b. Anatomy i. Arises from the upper cervical spinal cord,where the spinal accessory nucleussits between the horns of the spinal cord gray matter ii. Fibers leavethe nucleus and exit the lateral spinal cord justdorsal to the dentate ligament iii. After leavingthe spinal cord,fibers ascend to leavethe skull through the jugular foramen and terminate in the sternocleidomastoid and upper trapezius muscles c. Symptoms of dysfunction i. Inability to turn the head away from the lesion ii. Inability to raisethe shoulder on the sideof the lesion d. Differential diagnosis of dysfunction i. Neck injury ii. Jugular foramen tumor e. Evaluation
  36. 36. i. Have the patient turn the chin into your hand (note: away from the sideof the functioningsternocleidomastoid muscleand nerve); have the patient shrug the shoulders up againstyour hands ii. Brainstemimaging(MRI is best) XII. Cranial Nerve XII (hypoglossal nerve) (Moore & Dalley, pp. 1153-1154;Blumenfeld, pp. 499-500) a. Function (s) i. General Somatic Efferent (GSE) – Motor fibers to the tongue intrinsic muscles b. Anatomy i. Rootlets exit the ventral medulla and pass through the skull via thehypoglossal foramen c. Symptoms of dysfunction i. Tongue deviation TOWARD the lesion d. Differential diagnosis of dysfunction i. Neck injury ii. Skull basefracture e. Evaluation i. Brainstemimaging(MRI is best) ii. Skull baseX-rays if trauma in the history CCC22: Cephalgia without Fever Focus on DDX of Ha and medicines (not dosage) Most Has have a vascular origin. Migraines (with and without aura) Designed to get your attention. Electrochemical/vascularproblem Diagnosis: *5 attacks lasting4-72 hours with 2 of the 4 followingcharacteristics: Unilateral location Pulsatingquality Moderate/severe intensity (inhibits daily activities) Aggravation by routine physical activities *During HA, 1 of 2 must occur: Phonophobia/photophobia Nausea and/or vomiting Migrainephysiology: Some factor reaches a critical threshold  neuronal depolarization fromoccipital lobeto frontal lobes (aura *Signals sentto Trigeminal nucleus =release of inflammatory factors producing vasodilation *trigeminal nucleus also stimulates thalamus = pain sensation signals to the cortex Do not requirefurther workup if: Neuro exam is normal,no change in frequency/severity/character, and within typical agerange MigrainePrevention: Antiepileptics (Valproic acid,topiramate,zonisamide,gabapentin,lamotrigine,levtiracetam) Calmabnormal electric activity,uselower doses than you would for an epileptic Blood pressuremeds (Propranolol,Verapamil) Vitamins/herbals (Mg,Riboflavin,Coenzyme Q10)
  37. 37. Antidepressants (Wellbutrin,any SSRIs,TCAs) USE medicines AS SOON As HA starts! Acute Migraine Attacks Treatment: IV, antiemetics, triptans,NSAIDs, isometheptene, musclerelaxants,other painkillers,narcotics Tension Headaches Diagnosis: *Lasting 30 minutes to 7 days *At least2 of the following: Pressing/tighteningquality Mild/mod intensity but does not prohibitactivity B/L location No aggravation by routine physical activity *Both of the following: No N/V Photo/phonophobia are absent Evaluation:PE, no imagingis useful Pathophys: Tense muscles exert traction on lower meninges  referred pain to frontal region B/L Tension builds throughout the day Mechanical problem– meds do not work, requiremechanical solutions Cluster Headaches Diagnosis: *At least5 attacks of severe unilateral orbital,supraorbital and/or temporal pain lasting15-180 minutes untreated with one or more of the followingsigns occurringon the same sideas the pain: Conjunctival injection Miosis Lacrimation Ptosis Nasal congestion eyelid edema Rhinorrhoea Forehead/facial sweating *Frequency from one every other day to 8 per day Eval: H & P Treatment: Prevention (Li, valproate,verapamil) Acute: Oxygen, indomethacin Rebound Headache *Chronic, daily,can beworse than original headache,usually wakeup wi th them in the morning *Due to analgesic overuse(triptans,*narcotics,*acetaminophen, NSAIDs, salicylates,caffeine Treatment: startwith a preventer, gently find other meds that they can take alternatively
  38. 38. Sinus Headaches – NOT a diagnosisanywherebut in the US Usually a migraine that causes nasal symptoms Only acute, purulent sinusitis causes HA Treatment: as for migraines + decongestant Status Migrainosus - Debilitatingmigraineattack lastingfor more than 72 hours, stuck in a migrainecycle Diagnosis:Typical of previous attacks exceptfor duration + Both of these (>72 hours,severe intensity) + no other disorders thatcould causethis Someone you want to image, make sure they don’t have a fever. Treatment: in or outpatient with “migraine breakers,” requires specific treatment Valproate, Robaxin,Solumedrol,Ergotamine, Topiramate, Combo of Aspirin,lorazepam, diphenhydramine, and prochlorperazine CCC23: Cerebrovascular Diseaseand Stroke Focus on localization,work-up and treatment, do not focus on stroke syndromes. Stroke – vascular event leadingto a focal brain injury lastinglonger than 24 hours 80% ischemic (cardioembolic >lacunar >other) – part of brain lacksoxygen! Stenosis/occlusion of vessels due to atherosclerosis =largevessel disease Atherothrombosis – thrombus alonga vessel wall previously damaged by atherosclerosis,occur during/justafter sleep when bp is lowand flow is reduced Embolism – from artery elsewhere (heart mc) and lodges at a bifurcation in the head, Abrupt onset  fluctuatingsymptoms over 48 hours Occur duringactivity,larger/moresevere than thrombotic strokes Hypovolemia/hypotension – reduced CO or hypovolemia = decreased perfusion Even without stenosis can causea “watershed” infarct Risk factors:orthostasis,perioperative,hrt ischemia,arrythmias ,stenosis Vasospasm – resultingin focal hypoperfusion/focal tissuedamage Caused by trauma, aneurysm rupture, certain drugs Arterial dissection Intracranial venous thrombosis – clotin drainingveins of brain result in pressurebuildup and compression of surroundingtissues,could resultin venous rupture/hemorrhagic stroke Small vessel occlusive disease – occludesmall penetratingarteries = lipohyalinosis related to HTN = lacunar infarcts 20% Hemorrhagic Intraparenchymal/intracerebral Subarachnoid hemorrhage – due to aneurysmrupture, AVM or trauma ExplosiveHa with alteration in consciousness,vomiting,stiff neck, fever Complications:hydrocephalus,arterial spasm,ischemia distal to rupture Subdural hematoma – post blunthead trauma, due to tearing of bridgingveins,slowly progressing symptoms Epidural hematoma – postsharp head trauma, tear in meningeal artery
  39. 39. Lucid interval,cerebral edema often develops Stroke Diagnosis Mimics: Sudden onset of focal numbness/wkness Hypoglycemia Double vision/visual loss Tumor Spinning/vertigo Seizure Slurred speech, trouble speaking MS, encephalitis Limb/gait incoordination Trauma Unexplained, severe HA Hemorrhage Evaluation: ABCs, IV fluids Neuro consult Treat hypoglycemia,hypoxia,hyperthermia Trauma eval PT/PTT/INR/CBC/BMP CT for edema, hemorrhage r-tPA on standby (must be given within 1st 3 hrs) Positiveprognostic factors for acuteepisode: Younger age less severe deficits Normal CT (CT without bleed/lg stroke) no diabetes or cardiac disease Low bp on admission Positiveprognostic factors for longterm recovery: Young age small/rapidly improvingdeficit Modifiablerisk factors Early treatment Treatment: GOAL = avoid stroke extension, protect from ischemic “penumbra - ” neural tissuethat is rapidly killed in the setting of vascular compromise Normal saline,O2,regulate blood sugars/temperature Aspirin,Plavixor Aggrenox Do not lower bp unless >210/110,if you have to – lower it slowly Early therapies:physical,occupational,speech/swallow=activeor passiverehabilitation Long term treatment: Aspirin Clopidogrel Aspirin/extended releasedipyridamole Anticoag with warfarin Treatment (hemorrhage) Monitor closely for further bleeding/swelling may need neurosurgery Keep bp <160/90 NO antiplatelets Prevention: Treat risk factors (modifiable!) Meds: aspirin,plavix,aggrenox,warfarin (afib,valvedisease,CHF, cardiac thrombus),statins
  40. 40. Pediatric Stroke Ischemic strokes aredeeper than cortical Vascular occlusivelesions areintracranial vs.extracranial Intracerebral/subarachnoid hemorrhageare more common in kids than adults TIA – vascularevent leadingto focal brain symptoms,not necessarily injury Last less than 24 hours, resolvein about 1 hr CCC24: Aphasias and Facial NerveDisorderS Disorders of speech Dysphonia – inability to vocalizedue to laryngeal disorder/innervationsproblem Dysarthria –lack motor control of speech organs = garbled speech Alexia – loseability to read Agraphia – loseability to write Broca’s Aphasia/expressive/motor aphasia Brodmann’s area 44 affected due to infarction,trauma,tumor, or infection Nonfluent, reduced verbal output that is poorly articulated and takes a lotof effort May or may not contain appropriatemeaning, worse aresparse Comprehension > verbal output Reading comprehension is ok, readingoutloud is NOT, compromise in writingskills Neuro Exam: R sided wkness/hyperreflexia with above speech deficits Wernicke’s Aphasia/receptive/sensory aphasia Auditory association cortex in the L temporal lobedue to infarction,trauma,tumor, infxn Fluent verbal output but impaired comprehension Rapid empty speech devoid of meaning/fluent, well-articulated but meaningless Repetition/naming impaired,writingis abnormal 2 varieties:comprehension of spoken worse than written vs. written worse than spoken Neuro exam: Sensory loss,butmainly an unrevealingexam Conduction Aphasia Difficulty in repetition and confrontational naming Disturbed writing Global Aphasia All Language functions are seriously impaired Verbal output, comprehension of spoken/written language, repetition, naming, readingand writing Usually associated with hemiplegia,sensory deficits,visual field loss
  41. 41. Workup may include: CT LP CXR UA ABG EKG D-dimer CBC ESR BMP Cardiac enzymes glucose drug screen alcohol level Coagulation panel Bell’s Palsy – LMN lesions = affects one entire side of face Idiopathic paralysisor paresisof half of the face involvingCNVII distribution 70-80% patients recover without treatment Viral prodromein 60% MC causeHSV infection Tx: Acyclovir and corticosteroids,prevent eyes from dryingout Neuro Exam: Asymmetric facial wkness,impaired hearingipsilateral Numbness or pain of face/ear/tongue Reduction in tearing/saliva Eyelid may not closeall theway Supranuclear Facial Paresis – UMN lesions so wkness spares forehead on affected side Causes:tumors, infarction,seizure,abscess CCC25: Coma and Encephalopathies Consciousness – a state of awareness of self and surroundings Dependent on: intactReticular activatingsystemand both cerebral hemispheres 2 types of alterations in consciousness Affect arousal –coma and encephalopathy Affect cognitiveand affective mental function – dementia, delusion,psychiatricdisorders States of altered consciousness Alert/awake – normal state of arousal Lethargy – fatigued with minimal difficulty maintainingalertness Stupor – mod reduction in alertness with decreased interest in environment, response to stimuli Obtunded – unresponsivewith arousal only to painful stimuli Comatose – unresponsiveto noxious stimuli Glasgow Coma Scale Eye opening (4) 4 eyes Verbal response (5) Jackson 5 Motor response(6) V6 motor Examination of Comatose Patient Verbal response Eye opening Spontaneous eye moving Oculocephalic responses Oculovestibular responses Respiratory pattern Motor responses DTRs Tone
  42. 42. Pupillary Responses Normal – not a structural lesion of the CNS Unilateral dilation – structural injury B/L dilation unresponsiveto light“fixed” – severe brain injury B/l midposition unresponsiveto light – midbrain injury Small pinpointpupils –medication effect or pons injury Respiratory Patterns Cheyne-Stokes – b/l hemisphere dysfxn Central neurogenic hyperventilation – b/w midbrain/pons Apneustic breathing – pons dysfxn Ataxic breathing – medullary dysfxn,closeto death! Coma with hyperventilation - metabolic Coma with hypoventilation – drug OD, COPD Response to pain: Purposeful Semi-purposeful Random, non-purposeful Decorticate Decerebrate Emergency Assessment ABCs Stat labs (glucose,electrolytes,CVC, BUN, Cr, osmolality,ABG, LP, tox screen, LFTs, ammonia,coags,TSH, cortisol,AED levels,Bcx, UCx, EEG/SSEP) Signs of Increased ICP/Herniation Unilateral dilated pupil or b/l small poorly reactivepupils 3rd/6th nerve palsies Papilledema Deterioration in metal status,pupils or motor exam Withdrawal to pain  withdrawal to flexor (decorticate)  extensor posturing(decerebrate) Treatment of ICP Hyperventilation reduces immediately Mannitol Mannitol + Furosemide Casehyperosmolality with 3% NaCl DDX of Coma Metabolic Symmetric motor findings +small reactivepupils Asterixis,myoclonus,tremor, and seizures arecommon Infectious Check hx, fever, nuchal rigidity,kernigs,brudzinski,rash Bacterial meningitis vs.Subarachnoid hemorrhage – check CT, but startempiric therapy and do an LP Etiologys: VITAMIN D & E V – vascular D - drugs
  43. 43. I – infectious & T – trauma and toxins E – endocrine/epilepsy/emotional A – Anoxic M – metabolic I – iatrogenic M - neoplasm Conditions that mimic coma Locked-in syndrome: quadriplegic with lower cranial nervepalsy,but completely alert/aware Persistentvegetative state: absentcognitive function but retained vegetative components Catatonia:mute, with marked decreasein motor activity Prognosis of Coma Cannot be predicted 100% unless patientis brain dead Only about 15% in a nontraumatic coma make a satisfactory recovery Functional recovery is related to causeof coma Worst: structural damage Intermediate: hypoxia-ischemia Best: metabolic causes Longer coma lasts,less likepatientwill regain independent functioning Traumatic coma – do better than nontraumatic patients Encephalopathy Diffusecerebral dysfunction leadingto alteration in cortical function and disturbanceof consciousness rangingfrom mild confusion to coma Dysfunction in Reticular activatingsystemor both hemispheres Creutzfeldt-Jakob Disease – Seizures + massiveincoordination +dementia Sporadic – MC form, normal prion protein in brain is changed to abnormal Genetic- inherited abnormal prion protein Iatrogenic – accidentally transmitted through medical procedures (Growth hormome in kids) Toxins: Arsenic – risein temp + Ha + vertigo + V + anxiety + seizures Hyperexcitability  lethargy  coma  death Lead Kids:listlessness+confusion +ataxia  coma, seizures,respiratory arrest Adults: sleep disturbance,loss of libidos,irritability,memory loss,delirium,coma CO – DZ, Ha, N, impaired vision Manganese – asthenia,anorexia,apathy,Has,personality changes Diagnosisof exclusion Anoxic/hypoxic/ischemic Typically occurswith cardiac arrest,is reversibleif cardiac event is brief Renal failure
  44. 44. Acute – encephalopathy,tremor, myoclonus,seizures,eventual posturing Chronic – mild confusion slowingprogressingto dementia Hepatic failure – liver’s inability to clear the blood leads to build up of toxins in the CNS Pulmonary failure – Ha, somnolence, confusion,papilledema,asterixis,myoclonus,confusion,coma Hypernatremia HTN Hypocalcemia Hyponatremia Wernicke’s – ataxia +confusion + opthalmoparesis Nystagmus, 6th nerve palsy,horizontal gazepalsy,gaitataxia Vitamin B12 – PNS complaints normally B6 deficiency – seen in general malnutrition Drug-induced Hypothyroidism/myxedema madness – coma, hypothermia, respiratory depression,areflexia Hyperglycemia – diabetic ketoacidosisand hyperosmolar non-ketotic hyperglycemia lead to impaired Hypoglycemia – due to insulin overdose Epilepsy CCC26: Spinal Cord Disorders Anatomy – from the atlas to L1 in adult,L3 at birth 31 pairs of spinal nerves Gray matter in cord has dorsal/ventral horns (dorsa=sensory,ventral = motor) Anterior horn – efferent motor neurons, LMN lesion Posterior horn – afferent sensory neurons, sensory deficits Lateral horn – T1-L2 preganglionic sympathetic & S2-S4 of parasympathetic = auto dysfxn White matter in cord has ascending/descendingtracts Spinothalamic Tracts (coursetouch, pain/temperature) Posterior funiculus:fasiculisgracilis (upper limb) and faciculuscuneatus (upper limb) Spinocerebellar tracts (proprioception) Major ascendingtracts –sensory goes UP Lateral spinothalamic=pain/temperature Spinoreticulothalamic=poorly localized pain Ventral spinothalamic =lighttouch Dorsal colum= discriminativetouch/proprioception Descending tracts – motor goes DOWN Corticospinal (pyramidal) –voluntary motor function, UMN lesion Arises from contralathemisphere, crosses in pyramids and descends
  45. 45. LMN syndrome: UMN Syndrome (everything up!): Flaccid weakness spastic weakness Decreased tone increased tone Decreased DTR increased DTRS, + babinki’s Profound muscleatrophy minimal atrophy Fasciculations no fasciculations Syndromes: Complete spinal cord transaction All motor, sensory,autonomic function is gone from below the site of lesion. DTRs increased and + babinski’s belowlesion Disease:Transverse myelopathy Acute, inflammatory process localized over several segments in the cord, functionally,transects primarily demyelinates,could beasymmetric Symptoms progress over Occurs in trauma,tumor, MS, vasculardisorders,herniated disks,parainfectious *Sudden onset of wkness/sensory disturbancein legs/trunk is presentingfeature. Paresthesia occurs first Eventually sphincter dysfunction occurs. Hemisection Loss of pain/temperature contralateral to hemisection Ipsilateral lossof priorprioception Ipsilateral spasticweakness Segmental LMN and sensory signs atlevel of lesion Disease:Brown-Sequard Syndrome Occurs with trauma or extrinsic compressiom(tumor, infectious mass) Central Cord Syndrome (syringomyelia) Spinothalamic tractdecussatingfibers arecompromised initially Posterolateral column syndromes
  46. 46. L/o proprioception,vibration sensein legs with sensory ataxia and + Romberg’s sign B/L corticospinal tractdysfunction =spasticity,hyperreflexia,b/l Babinski’s Disease:Vitamin B12 deficiency, AIDS, HTLV, Cervical spondylosis, Tabes dorsalis Sensory changes:paresthesias in feet Loss of propriocetion/vibration Pain/temp remain intact Anterior Horn Cell syndromes Diffuseweakness, atrophy, fasciculation in muscles,tonereduced Reflexes depressed or absent Sensory tracts arespared Diseases:variations of Spinal muscular atrophy (Werdnig-hoffman, intermediate, Kugelberg-welander, progressive SMA) Diffuseweakness, atrophy, fasciculations. Reduced muscletone, depressed DTRs Combined anterior horn cell and pyramidal tract syndromes LMN + UMN signs Diseases:ALS Degenerative changes in the anterior horn cell and the cortico-spinal tract DiffuseLMNs and UMN dysfunctions Vascular Syndrome Most involvethe anterior spinal artery or watershed areas ASA supplies anterior 2/3 of the spinal cord ASA – preservation of posterior column, l/o pain/temp, LMN signs early,UMN late, bladder/bowel incontinence Disease:ASA infarct Abrupt onset, radicularor “girdlepain” Loss of motor function within minutes/hours below lesion Impaired bowel/bladder control Preservation of the dorsal column (Posterior spinal arteries) Other disorders: Conus medullaris lesions Saddleanesthesia,urinary incontinence Caused by trauma and infection HIV/AIDS Self Assessment Quizzes: • HIV causes human diseaseprimarily by disruptingtheimmune system and this is measured by what? – Measured by CD4 cell depletion • How is the Diagnosis of AIDs made?