for medical students

1. CNS 1
BASHAIR AL-MDAWI
OMCF-12-091

7. Upper and
lower neurons
lesions:
1) Upper motor neurons: pyramidal
tract, Convey the messages from brain
to Spinal cord(corticospinal) and
brainstem (corticobulbar) , Pronator
sign is an early sign of UMN lesion
2) Lower motor neurons: from anterior
horn cells and motor nuclei of cranial
nerves in brain stem, Convey voluntary
and autonomic impulses and signals to
muscles .

8. Headache:
Classified to
• *Primary : due a primary malfunction of neurons.
Eg: Tension-type headache, Migraine, Cluster Headache, Primary stabbing Headache.
•Secondary: Symptomatic of some underlying pathology.
Eg: From raised intracranial pressure and space occupying lesions.

10. Tension-type Headache:
SYMMETRICAL HEADACHE. ONSET : GRADUAL. PAIN CHARACTER: TIGHTNESS,
A BAND PRESSURE.

11. Migraine:
•Without Aura: accounts for 90% of migraine.
•Duration: episodes last 1 -72 hours.
•Location: mainly bilateral but can be unilateral.
•Character of pain: pulsatile, over frontal or temporal area.
•Associated symptoms: unpleasant gastrointestinal disturbances
like vomiting, nausea or abdominal pain. Also, photophobia,
and phonophobia.
•Aggravating factors: physical activity .
•Relieving factors: sleep.

12. • With aura: 10% of migraine.
• The headache is preceded by an aura (visual, sensory, or motor).
• Features: absence of problems between episodes and the
frequent presence of premonitory symptoms (tiredness, difficulty
concentrating, autonomic features, etc.).
•Most common : Visual disturbance.
•Including :
- negative phenomena, such as hemianopia (loss of half the
visual field) or scotoma (small areas of
visual loss).
- positive phenomena such as fortification spectra (seeing zigzag
lines).

13. • Symptoms of tension-type headache or a migraine often overlap.
• part of the same pathophysiological continuum.
• There is a genetic predisposition, with first-degree and second-degree
relatives often also
Affected.
Triggered by:
1. disturbance of inherent biorhythms, such as late nights or early rises.
2. stress, or winding down after stress at home or school.
3. Certain foods, e.g. cheese, chocolate, and caffeine.
4. In girls, headaches can be related to menstruation.

14. Raised intracranial pressure and
space-occupying lesions
* Worse when lying down and morning vomiting is characteristic.
* cause night-time waking.
* change in mood, personality, or educational performance.
* visual field defects – from lesions pressing on the optic pathways, e.g. craniopharyngioma (a
pituitary tumor).
* cranial nerve abnormalities causing diplopia, new-onset squint or facial nerve palsy.

16. Management

17. * Rescue Treatments.
* Prophylactic Treatments.
* Psychosocial Support.

18. Rescue :
• Analgesia – paracetamol and nonsteroidal
anti-inflammatory drugs (NSAIDs).
• Antiemetics – prochlorperazine or cyclizine, for
nausea.
• Triptans (serotonin (5-HT1) agonists), e.g. sumatriptan. A nasal
preparation of this is
particularly useful in children, early in a migraine attack, together with
a NSAID or paracetamol.
•Physical treatments such as cold compresses, warm pads, topical
forehead balms.

19. Prophylactic :
• sodium channel
blockers –
topiramate or
valproate.
• beta-blockers –
propranolol;
contraindicated in
asthma.
• tricyclics:
pizotifen (5-HT2
antagonist) –
cause weight gain
and sleepiness, or
amitriptyline – can
cause dangerous
arrhythmias in
overdose.
• acupuncture.

20. Psychosocial support
* Psychological support – is it required to ameliorate a
particular stressor, e.g. bullying, anxiety over
exams, or illness in friends or family.
* Relaxation and other self-regulating techniques, addressing
life-style issues: ensuring adequate and regular rest, play, sleep,
water, and food.

21. Seizures

22. Epileptic seizures:
. Abnormal electrical activity in the brain.
(cerebral cortex).
. due to excessive and hypersynchronous
electrical activity.
. difficult to tell from a non-epileptic (especially
a syncopal seizure) clinically.

25. Diagnosis:
Primarily based on a detailed history from the child and eyewitnesses, substantiated by a video if
available.
* INVESTIGATIONS
1. ECG
2. EEG
3. Brain Imaging
4. OTHERS

26. ECG
. Especially convulsive seizures
. Done to avoid convulsive syncope due to an arrhythmia.
Eg: long QT syndrome.

27. EEG
. categorize the epilepsy type and severity.
. Eg : suspected childhood absence epilepsy and suspected infantile
spasms.
. If normal, a sleep or sleep-deprived record can be helpful.
. 24 hour ambulatory EEG or a 5-day video-telemetry.
. For assessment prior to surgery, more invasive techniques such as
subdural electrodes.

28. Brain Imaging
Structural:
MRI and CT brain scans are generally required routinely for
childhood epilepsies unless there is a characteristic history of
childhood absence epilepsy, juvenile absence epilepsy,
juvenile myoclonic epilepsy, and childhood Rolandic epilepsy.
Functional:
allow functional imaging to detect areas of abnormal
metabolism suggestive of epileptogenic
zones. These include PET (positron emission tomography)
and SPECT (single photon emission
computed tomography).

29. Others
. Metabolic investigations :
if there is developmental arrest or regression, or seizures are related to feeds or fasting.
.in epilepsies (i.e. not including febrile seizures) starting in the first 2 years of life.
. Genetic tests: in intractable epilepsies with developmental arrest or delay (“epileptic
encephalopathies”).

30. Guidance to the
Management:
. Not all children with epileptic seizures require antiepileptic
drug (AED) therapy.
. The decision should be based on the seizure type, epilepsy
type, and frequency.
. choose an appropriate AED for the seizure and epilepsy.
. Monotherapy at the minimum dosage to prevent the
seizures without adverse effects.
. All AEDs have potential unwanted effects and these should
be discussed with the child and Parent.
. AED levels are not measured routinely, but may be useful to
check for concordance (adherence) or to see if a dose
increase could be considered if a high dose is not working.
. children with prolonged epileptic seizures are given rescue
therapy to keep with them. This is usually buccal midazolam.
. may be discontinued after 2 years free of seizures. Needs
tapering for 3 months . DON’T STOP COMPLETELY!!!!!!!!!

33. Febrile
seizures:
. An epileptic seizure accompanied by a fever in the
absence of intracranial infection.
. Occur in children, between the ages of 6 months and 6
years.
. Strong genetic predisposition .
. Usually occurs early in a viral infection when the
temperature is rising rapidly.
. brief generalized tonic-clonic seizures.

34. Types
a. Generalized tonic-clonic attack + fever
b. Last for≤15 minutes
c. No reoccurrence during 24 hours
d. Does not affect intellectual
performance
e. No risk of developing epilepsy
a. Focal seizure attack + fever
b. Last more than 15 minutes
c. May reoccur during 24 hours
d. Has risk of subsequent epilepsy

35. If the child is unconscious or has
cardiovascular instability, lumbar
puncture is contraindicated and
antibiotics should be started
immediately.
Investigation Treatment Counseling of parent
• Focused history in
the causes of fever
• Infections screen (
blood culture – urine
culture – LP)
• Airway : high flow O2 +
glucose
• Rectal diazepam is given
at time of reoccurrence
of febrile seizures
• Buccal midazolam is
given if there is history if
seizure > 5 minutes*
• Need reassurance
and information.
• Learn first aid
management of
seizure .

36. Neural Tube
Defects:
. Result from failure of normal fusion of the neural plate to
form the neural tube during the first 28 days following
conception.
. Incidence decreased due to folic acid supplementation .
* TYPES:
. Anencephaly .
. Encephalocele .
. Spina Bifida Occulta.
. Meningocele and Myelomeningocele.

37. * Anencephaly :
• Failure in the development of most of the cranium and
brain .
• Affected infants are stillborn/ die shortly after birth.
• detected by ultrasound during pregnancy .
* Encephalocele
• Extrusion of brain and meninges through midline skull
defect.
• Can be corrected surgically .
• Can be associated with cerebral malformations .

38. * Spina Bifida Occulta:
• Failure of fusion of the vertabral arches.
• Incidental finding on X-Ray.
• Hidden by skin .
• May be associated with skin lesions : birth marks,
dermal sinus, lipoma ,and hairy patches .
• may be underlying tethering of the cord
(diastematomyelia), which, with growth may cause
neurological deficits of bladder function and lower limbs.
• Neurosurgical relief of tethering is usually indicated.

39. * Meningocele :
• a sac protruding from the spin
• sac includes spinal fluid, but does not contain neural tissue
• covered with skin or with meninges
• Good prognosis after surgery.
* Myelomeningoceles
may be associated with:
• variable paresis of the lower limbs with hypotonia
• muscle imbalance, which may cause dislocation of
the hip and talipes
• sensory loss
• bladder denervation (neuropathic bladder)
• bowel denervation (neuropathic bowel)
• scoliosis
• hydrocephalus

41. Management
• Back lesion usually closed soon after birth
• Physiotherapy for paralysis and muscle imbalance.
• Walking aids / wheelchairs
• Skin care to prevent damage and ulcer ( due to sensory loss chances)
• Indwelling catheter or intermittent urinary catheterization( to manage the
neuropathic bladder)
• regular check for hypertension , renal function and urinary infection.
• prophylactic antibiotic.
• Bowel denervation require regular toileting , laxatives and suppositories ,
and low roughage diet .
• Scoliosis require surgical treatment

42. Duchenne
muscular
dystrophy
. Inherited as an X-linked
recessive disorder.
. Results from a deletion
of the gene for
dystrophin, which
connects the
cytoskeleton of a muscle
fibre to the surrounding
extracellular matrix
through the cell
membrane.
. There is an influx of
calcium ions, a
breakdown of the calcium
calmodulin complex and
an excess of free radicals,
ultimately leading to
myofibre necrosis.
. creatine kinase (CK) is
markedly elevated.

43. Clinical features
a)Waddling gait
b) Early school age : children with DMD tend to be slower and clumsier than peers.
c) Language delay + learning difficulty
d) 10-14 years : not ambulant
e) Gowers sign ( the need to turn prone to rise).
f) Late twenties : death due to respiratory failure .
g) Pseudo hypertrophy of calves .
h) complication: scoliosis
i) Nocturnal hypoxia due to weakness of intercostal muscles
j) irritability
k) daytime headache
l) Loss of appetite

44. Management:
1) Physiotherapy
2) Overnight CPAP* / non invasive positive pressure
ventilation
3) Tendoachillis lengthening + scoliosis surgery
4) Ambulant children treated with corticosteroid to preserve
mobility and prevent scoliosis.
5) Ataluren *( for nonsense mutation ).
Investigations
1) CK level
2) DNA analysis

45. Spinal muscular atrophy
. autosomal recessive degeneration of the anterior horn cells.
. survival motor neurone (SMN1) gene.
. leading to progressive weakness and wasting of skeletal muscles.

46. TYPES
Type 0
• Most severe type
• Diagnosed in
newborn infants
• weak children ,
survive to few
weeks.
Type 3Type 1
• Werdnig Hoffman disease
• Present from birth to 3 months
• At pregnancy : diminished fetal
movement .
• At birth : Arthrogryposis
• About 12 months : death due to
respiratory failure .
• Never sit unaided.
Typical signs :
• Alert expression
• Fasciculation of tongue
• Symmetrical flaccid paralysis
• Absent deep tendon reflexes
• Intercostal recession
• Weak cry and poor suck with
pooling of secretions
Type 2
•Most common
•Present at 3-15
months
•Can sit but never
walk
independently.
•Kugelberg – Welander
syndrome.
•Present after 1 year of
life
•Learn to walk .

47. Match the following defects to the corresponding picture
Meningocele
Myelomeningocele
Spina Bifida occulta