Neurologic disorders

Cerebral palsy
RF
·       Prematurity
·       Low birth weight
Clx
·       Delayed motor milestones
·       Abnormal tone, hyperreflexia
·       Comorbid seizures, intellectual disability
Dx
·       MRI of the brain
·       ± Electroencephalography
·       ± Genetic/metabolic testing
Tx
·       Physical, occupational, speech therapies
·       Nutritional support
·       Antispastic medications
 Febrile SZ 👶🏼
S/S
·       Bilateral Tonic-Clonic generalized
·       : <15 m + Return to baseline quickly
·       Do not Rx within 24 h
Dx
Age: 3m – 6ys
No previous AFEBRlLE seizure
No meningitis or encephalitis
No acute metabolic cause
Tx
·       Abortive therapy (≥5 minutes) → Rectal Diazepam
·       Reassurance + Discharge pt (do not admit) + No need for F/U
·       Hospitalize only for 24h if did not return to baseline
Prog
development/intelligence
-30% risk of recurrence
<5% risk of epilepsy
Acute causes of hemiplegia in 👶🏼
Cause  
?
SZ
·       Hx of generalized limb jerking or LOC
·       Postictal confusion or Todd temporarily paralysis
Intracranial
hemorrhage
·       Hx of trauma &/or bleeding disorder
·       Signs of ⤴️ICP (vomiting, bradycardia)
·       + imaging 
Ischemic stroke
·       Hx of prothrombotic disorder (eg, antithrombin Ill deficiency) or cardiac disease (eg, patent
·       foramen ovale)
·       FND (eg, hemiplegia, aphasia, ataxia)
Hemiplegic migraine
·       Onset in adolescence & often positive FHx
·       Hx of headache & visual aura
·       Sx self-resolve
Bacterial Meningitis in 👶🏼 (age >1 month)
Clx
·       Fever
·       ⤴️ ICP (Headache, vomiting, AMS)
·       Meningeal irritation (nuchal rigidity)
Dx
·       CBC + Blood cultures
·       LP for CSF analysis
Tx
·       IV vancomycin & ceftriaxone OR cefotaxime
·       Dexamethasone for Haemophi/us influenzae type b meningitis
Comp
·       Intellectual/behavioral disabilities
·       Hearing loss
·       Cerebral palsy
·       Epilepsy
 Sturge-Weber Syndrome
Path
Mutation in GNAQ gene
S/S
·       Port wine stain (trigeminal N /CN VI/V2)
·       Leptomeningeal capillary-venous malformation
·       SZ ± hemiparesis
·       Intellectual disability
·       Visual field defects
·       Glaucoma
Dx
 MRI with contrast
Tx
·       Laser therapy
·       Antiepileptic drugs
·       Intraocular pressure reduction
 Tuberous Sclerosis
?
·       Neurocutaneous autosomal dominant 
·       hamartin and tuberin form a complex that down regulates mTOR signaling
·       when this pathway is 🆎 → hamartomas everywhere
S/S
·        infantile spasms in infants and have hypsarrhythmias on EEG
·       SZ
·       Arrhythmia 2ndary to cardiac rhabdomyomas
Dx
Genetic testing
Tx
Corticotropin for infantile spasms

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