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Convulsive Disorders
 

Convulsive Disorders

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    Convulsive Disorders Convulsive Disorders Presentation Transcript

    • Convulsive Disorders Alina Valdes, M.D.
    • Epilepsy
      • Chronic condition
        • Seizures characterized by sudden and unusually unprovoked attacks
          • subjective experiential phenomena
          • altered consciousness
          • involuntary movements
      • Seizures
        • Result from abnormal brain electrical activity
        • Common sign of brain dysfunction
      • Not all seizures imply epilepsy
    • Causes
      • Reversible medical or neurologic illness – symptomatic seizures
        • Usually self-limited
        • Seizures resolve if underlying disorder corrected
      • Physiologic stress (e.g., sleep deprivation) , fever, and alcohol or sedative drug withdrawal
        • Lowered seizure threshold
      • Genetic factors
      • Unrecognized previous CNS injury
      • Unknown
    • Causes of Symptomatic Seizures
      • Acute electrolyte disorders
        • Na <120 or >155 mEq/L
        • Osmolality > 310 mOsm/L
        • Ca < 7 mg/dl
      • Drugs
        • INH, PCN’s, theophylline, ephedrine, lidocaine, TCA’s, cocaine, amphetamines, EtOH withdrawal
      • CNS disease
        • Hypertensive/hepatic encephalopathy, eclampsia, SLE, meningitis, stroke, sickle cell disease, brain tumor
    • Etiology
      • Can begin at any time of life
      • Incidence higher among young children and elderly
      • Incidence higher in developing countries and inner-city areas
      • About 70% of adults and 40% of children with new-onset epilepsy have partial (focal) seizures
        • Imply cerebral injury or lesion
      • In children
        • Idiopathic/cryptogenic: 67%
        • Congenital: 20%
        • Trauma, infection, vascular, neoplastic, degenerative
      • In adults
        • Idiopathic/cryptogenic: 55%
        • Vascular: 15%
        • Neoplastic, trauma, degenerative, congenital, infection
    • Classification
      • International League Against Epilepsy
      • Classified by clinical symptoms and signs
      • Manifestations depend on
        • Whether most or only part of cerebral cortex involved at beginning
        • Functions of cortical areas where originates
        • Subsequent pattern of spread
      • Seizures are dynamic and evolve depending on extent and manner of spread of electrical discharge
    • International League Against Epilepsy Classification
      • Classification of Seizures
        • Partial (Focal) Seizures
          • Simple partial seizures (consciousness not impaired)
            • with motor signs
            • with sensory signs
            • with psychiatric symptoms
            • with autonomic symptoms
          • Complex partial seizures (consciousness is impaired)
            • simple partial onset followed by impaired consciousness
            • with impairment of consciousness at onset
            • with automatisms
          • Partial seizures evolving to secondarily generalized seizures
        • Generalized Seizures of Nonfocal Origin
          • Absence seizures
          • Myoclonic seizures; myoclonic jerks (single or multiple)
          • Tonic-clonic seizures
          • Tonic seizures
          • Atonic seizures
      • Classification of Epileptic Syndromes
        • Idiopathic Epilepsy Syndromes (Focal or Generalized)
          • Benign neonatal convulsions
          • Benign partial epilepsy of childhood
          • Childhood absence epilepsy
          • Juvenile myoclonic epilepsy
          • Idiopathic epilepsy, otherwise unspecified
        • Symptomatic Epilepsy Syndromes (Focal or Generalized)
          • West’s syndrome (infantile spasms)
          • Lennox-Gastaut syndrome
          • Partial continuous epilepsy
          • Temporal lobe epilepsy
          • Frontal lobe epilepsy
          • Post-traumatic epilepsy
        • Other Epilepsy Syndromes of Uncertain or Mixed Classification
          • Neonatal seizures
          • Febrile seizures
          • Reflex epilepsy
          • Adult nonconvulsive status epilepticus
    • Partial Seizures
      • Simple partial
        • Epileptic electrical discharge limited to focal area of cortex
        • Patients can interact normally with their environment except for limitations on localized brain function
        • Include subjective sensory and psychological phenomena
        • Auras affect about 60% of patients with focal epilepsy
        • Location of focus often predicts nature of pathology and directs testing
      • With motor signs
        • Begin with clonic (rhythmic jerking) or tonic (stiffening) movements in body part
        • Large cortical representation
          • involves muscles of face and hand
        • “ Jacksonian march ” – uncommon
          • homunculus representation: begins in primary motor cortex and spreads to involve rest of precentral gyrus
        • “ Fencer’s posture ” – common
          • ictal discharges involve secondary motor areas of frontal lobe
          • contralateral flexion and elevation of arm, contralateral turning of head and eyes, and tonic expression of ipsilateral arm
        • Speech arrest, vocalizations, and eye blinking
        • Todd’s paralysis
          • Transient neurologic abnormality – last <48 hours
          • Postictal depression of epileptogenic cortical area
            • motor seizure – focal weakness
            • sensory seizure – numbness
            • occipital lobe seizure – blindness
          • Focal abnormalities useful clues to site of origin
      • Complex Partial
        • Impair consciousness and produce unresponsiveness
        • 70% to 80% arise from temporal lobe
        • Remaining cases arise mainly from frontal lobe
        • Many evolve from simple partial as progresses
        • Uncinate fits
          • preceded by olfactory aura
          • arise in or near uncus of temporal lobe
    • Generalized Seizures
      • Begin diffusely and involve both cerebral hemispheres simultaneously from outset
      • Subdivided on basis of motor manifestations
      • Must be distinguished from focal seizures that become secondary generalized
      • Generalized tonic-clonic
        • Grand mal convulsions
          • abrupt LOC
          • bilateral tonic extension of trunk and limbs (tonic phase)
          • loud vocalization (“epileptic cry”)
          • bilaterally synchronous muscle jerking (clonic phase)
        • Urinary incontinence common
        • Fecal incontinence rare
        • Seizure itself not usually last >90 seconds
        • Postictal phase
          • transient deep stupor
          • 15-30 minutes lethargic, confused state with automatic behavior
          • with recovery, patients complain of headache, muscle soreness, mental dulling, lack of energy, or mood changes for as long as 24 hours
        • Physiologic changes – transient
          • Blood hypoxia, lactic acidosis
          • Elevated plasma catecholamines
          • Increased serum CPK, prolactin, corticotropin, cortisol, Beta-endorphin, and GH
        • Complications
          • Oral trauma
          • Vertebral compression fractures
          • Shoulder dislocation
          • Aspiration pneumonia
          • Sudden death – acute pulmonary edema, cardiac arrhythmia, suffocation
      • Absence
        • Petit mal seizures
        • Mainly in children
        • Sudden, momentary lapses in awareness, staring, rhythmic blinking, and few small clonic jerks of arms or hands
        • Immediate return to normal
        • No postictal period
        • Usually no recollection of seizure
        • Most last <10 seconds
      • Atypical absence
        • More gradual lapses of awareness
        • Not resolve as abruptly
        • Autonomic features or loss of muscle tone
        • Most often in mentally retarded children
        • Not respond as well to drugs
      • Myoclonic
        • Rapid, recurrent, brief muscle jerks
        • Can occur bilaterally, synchronously or asynchronously, or unilaterally without LOC
        • Often cluster shortly after waking or while falling asleep
      • Atonic
        • “ Drop attacks ”
        • Most often in children with diffuse encephalopathies
        • Sudden loss of muscle tone
          • falls with self-injury
      • Reflex
        • Precipitated by specific stimulus
          • touch, musical tune, reading, etc .
    • Febrile Seizures
      • Most common cause of convulsions in children
      • Most occur between ages of 6 months and 4 years
      • About 30% of the children have more than one attack
      • Greatest chance of recurrence if seizure <1 y.o. or family history
      • Most times no long-term consequences but increase risk of developing epilepsy later
    • Benign Partial Epilepsy of Childhood With Central-Midtemporal Spikes
      • Rolandic epilepsy
      • One of most common in children
        • 15% of all pediatric epilepsies
      • Usually begin between 4 and 13 years old and disappear by mid to late adolescence
      • Seizures principally at night
      • EEG shows stereotyped epileptiform discharges over central and midtemporal regions
      • Outcome not affected by treatment
      • Carbamazepine prevents recurrent attacks
    • Juvenile Myoclonic Epilepsy
      • Idiopathic generalized epilepsy
      • Most often begins between 8 and 20 years old
      • Morning myoclonic jerks, generalized tonic-clonic seizures occurring just after awakening
      • Family history of similar seizures
    • Lennox-Gastaut Syndrome
      • Heterogeneous group of early childhood epileptic encephalopathies
      • Physical brain abnormalities
      • Mental retardation
      • Uncontrolled seizures
    • Temporal Lobe Epilepsy
      • Most common cause in adults
        • At least 40% of epilepsy cases
      • Begin in late childhood/adolescence
      • Often history of febrile seizures
      • Almost all patients have complex partial seizures, some secondarily generalize
      • Most often arises from mesial temporal lesion – hippocampal sclerosis
    • Post-Traumatic Epilepsy
      • Directly related to severity of head injury
        • Cerebral contusion
        • Intracerebral or intracranial hematoma
        • Unconsciousness or amnesia >24 hours
        • Persistent abnormalities on neuro exam
        • 2/3 of patients have partial or secondary generalized
      • Mild head injuries not increase risk
    • Diagnosis
      • Evaluation
        • Determine if patient has epilepsy
        • Classify the seizures – if clinical data fit syndrome
        • Identify underlying cause
      • History – patient or witness description
        • Setting, previous history, pattern
      • Physical exam normal in most cases
        • Skin, retina, focal neurologic signs
        • Absence seizures can be triggered by hyperventilation
    • Lab Tests – EEG
      • Most important for epilepsy
        • Establishing diagnosis
        • Classifying seizures
        • Identifying syndromes
        • Making treatment decisions
      • Epileptiform patterns – spikes or sharp waves
        • Focal vs. generalized activity
      • Establish epilepsy only if record characteristic discharge during representative clinical attack
      • 40% - 50% of epileptics have abnormalities on initial EEG
      • Increase chance of capturing by sleep deprivation 24 hours before test and sleep during a portion of recording
      • Serial EEG’s increase yield
    • electroencephalography Brainwave Patterns The electrical activity of the brain was first demonstrated in 1929 by the German psychiatrist Hans Berger. The scientific professions were slow in giving proper attention to Berger's discovery of the brain rhythms he named alpha waves, but since then at least three other standard brainwave patterns have been isolated and identified. Alpha waves are fast, medium-amplitude oscillations, now known to represent the background activity of the brain in the physically and psychologically healthy adult. They are most characteristically visible during dream-sleep or when a subject is relaxing with eyes closed. Delta waves are large, slow-moving, regular waves, typically associated with the deepest levels of sleep. In children up to the age of puberty the appearance of high-amplitude theta waves, having a velocity between those of alpha and delta rhythms, usually signals the onset of emotional stimulation. The presence of theta waves in adults may be a sign of brain damage or of an immature personality. Beta rhythms are small, very fast wave patterns that indicate intense physiological stress, such as that resulting from barbiturate intoxification.
    • Neuroimaging Studies
      • Brain MRI
        • Identifies structural lesions that may be related
        • Obtain complete study : T1- and T2-weighted images in coronal and axial planes
        • Should be obtained in all adults with epilepsy and children with partial seizures , abnormal neuro findings, or focal slow-wave abnormalities on EEG
      • PET and SPECT
        • Functional views of brain
        • Physiologically active, radiolabeled tracers
          • Metabolic activity – PET
          • Blood flow – SPECT
        • Abnormalities often seen when MRI normal
    • Other Tests
      • Routine bloods not helpful
      • Baseline electrolytes,LFT’s, cbc prior to treatment
      • Bloods in older patients with acute or chronic disease
      • Drug screen for substance abuse if unexplained generalized seizures
      • Lumbar puncture only if suspect meningitis or encephalitis – repeated seizures may increase CSF protein and wbc’s
      • EKG if history – arrhythmia, valvular disease
    • Differential Diagnosis
      • Misdiagnosis – not respond to treatment
      • Nonepileptic paroxysmal disorders
        • Sudden, discrete events with abnormal behavior, variable responsiveness, changes in muscle tone, and various postures or movements
        • More common and variable in presentation in children
    • Disorders That May Resemble Seizures
      • Movement disorders: myoclonus, episodic ataxias
      • Migraine: confusional, vertebrobasilar
      • Syncope
      • Behavioral and psychiatric disorders: panic, hyperventilation, dissociation
      • Cataplexy
      • TIA
      • Alcoholic blackouts
      • Hypoglycemia
      • Syncope
        • Transient global decrease in cerebral perfusion
        • LOC lasts seconds with rapid recovery
        • Convulsive syncope : if ischemia severe, may get brief tonic posturing or clonic jerks of arms and legs
      • Migraine
        • Especially basilar artery migraine in adolescents and young adults: lethargy, mood changes, confusion, disorientation, vertigo, bilateral visual disturbances, and LOC
      • Panic attacks
        • Muscle twitching or spasms with hyperventilation
        • Patients may faint
    • Treatment
      • Correct underlying cause if possible
      • If seizures recurrent, try to stop attacks with antiepileptic drugs:
        • Define type of seizure and give indicated medication in increasing doses until either control seizures or side effects occur
        • Infrequent seizures treated with slow dose changes
        • Pick another agent if persistent seizures or major side effect
        • Add another drug before stop first to prevent status epilepticus
        • If seizures persist after tried two agents to toxicity, refer to specialist
        • Seizures can be caused by toxic levels of antiepileptics (especially phenytoin and carbamazepine
      • Surgery:
        • For medically intractable epilepsy: about 20% of patients
        • Can eliminate seizures and restore normal neuro function in selected patients
        • Must accurately locate seizure focus
    • Drugs in Treatment
      • Simple and complex partial and secondary generalized
        • Carbamazepine, phenytoin, valproate, gabapentin, lamotrigine, topiramate
      • Primary generalized
        • Tonic-clonic: valproate, carbamazepine, phenytoin, lamotrigine
        • Absence: ethosuximide, valproate, lamotrigine
        • Myoclonic and tonic: valproate, clonazepam
    • Status Epilepticus
      • Major generalized motor status
        • Seizures follow one another quickly that patient not have chance to recover
        • Can cause brain damage
        • Most common cause is abrupt anticonvulsant medication withdrawal
        • Other causes:
          • alcohol or drug withdrawal from abuser
          • cerebral infection
          • trauma
          • hemorrhage
          • neoplasm
        • Treatment is urgent
        • Cause must be sought
      • Partial motor status
        • Partial continuous epilepsy
        • Uncommon
        • Can last for hours, days, or longer
        • Seizure frequency range from one every three seconds to several per second
        • Motor attacks range from highly focal, myoclonic twitches to jerks that involve half of body
        • Cerebral lesions cause in face or distal upper extremity
        • Brain stem or spinal cord lesions cause proximal myoclonic activity
        • Causes:
          • severe hyperglycemia – can also produce partial complex
          • stroke
          • trauma
          • neoplasms
          • encephalitis
          • unknown
        • Often resistant to treatment
      • Partial complex status
        • Sustained state of confusion with stereotyped motor and autonomic automatisms: hours to days
        • May have abrupt-onset schizophreniform or other bizarre activity
        • Patients may resist help in abnormal state
        • EEG: continuous, usually asymmetric slow and spike activity over temporal lobe(s)
        • Treat rapidly due to possible memory and intellect impairment
      • Absence status
        • Petit mal status
        • Two forms
        • More common form similar to partial complex status
          • confused automatic behavior
          • closely spaced or continuous runs of 3- to 4-Hz spike and wave activity on EEG
          • occurs mostly in adolescents with known petit mal
          • most episodes last <30 minutes
          • most stopped with IV diazepam
    • Pregnancy
      • >90% of women on antiseizure meds have healthy infants
      • Hereditary risks to fetus
      • Women have higher risks of complications of pregnancy
      • Anticonvulsant meds need readjustment due to changes in blood volume and drug interactions
      • Breast feeding not contraindicated if taking meds
      • Not discontinue meds during pregnancy but can consider prior to conception
    • Prognosis
      • 60% to 70% of people with epilepsy have 5-year remission of seizures within 10 years of diagnosis
      • About half of these seizure-free without meds
        • Idiopathic epilepsy
        • Normal neuro exam
        • Onset in early to middle childhood
    • Discontinuing Drugs
      • Many patients seizure-free on meds
      • Some can stop meds and remain seizure-free
        • Controlled with monotherapy
        • Few seizures
        • Normal EEG and neuro exam prior to stopping meds
        • Seizure-free interval of at least 4 years