Vascular
Deep branch of ACA- supplies anterior limb of internal capsule, inferior head caudate nucleus, anterior globus pallidus
Recurrent artery of Heubner
Definition of vasogenic edema
Definition of cytotoxic edema
V.E.- extracellular edema. BBB damage (e.g. tumor)
C.E.- intracellular edema. Cellular membrane damage (e.g. ischemia)
Juvenile Myoclonic Epilepsy: EEG frequency/appearance
4 to 6 Hz polyspikes
Direct pathway: inhibitory or excitatory to cortex?
Excitatory (increases thalamic excitation to cortex)
MC type of CMT
Mode of inheritance
Axonal or demyelinating
CMT1
Autosomal Dominant
Demyelinating
Normal variant with vascular supply to both medial thalami
Artery of Percheron
Petechial hemorrhage in the brain after trauma with bone fracture
Side effects related to which AEDs?
Nephrolithiasis (2 meds)
Auto-induction of metabolism
Reduces the effectiveness of combined estrogen-progestin contraceptives
Topiramate and Zonisamide
Carbamazepine
Lamotrigine
MC gene mutated in hereditary Parkinson's disease
Leucine- rich repeat kinase 2 (LRRK2)
Deletion in peripheral myelin protein 22 (PMP22) gene
Hereditary neuropathy with liability to pressure palsies
CADASIL gene mutation
NOTCH3
This process is suspected w/ neurologic worsening in SAH with secured aneurysm, and b/w 3-15 days
Vasospasm
Syndrome. Severe myoclonic epilepsy of infancy
Another syndrome. Myoclonic- astatic epilepsy (now myoclonic atonic epilepsy)
Dravet's syndrome- SCN1A gene mutation
Doose's syndrome- "drop attack"
*keto diet can be effective for both
Filipino with dystonia and parkinsonism
Lubag: X- linked dystonia- parkinsonism
DYT3 mutation
Demyelinating neuropathy with monoclonal gammopathy. Which anti-body is associated?
Anti- MAG
Pons, midbrain, or internal capsule- weakness with ataxia out of proportion to weakness
Ataxic hemiparesis lacunar syndrome
This marker if elevated, after cardiac arrest, predicts poor neurologic outcome
Neuron- specific enolase (NSE)
Triad of:
Infantile spasms, hypsarrhythmia, and psychomotor delay or regression
West' syndrome
Episode of ataxia with nystagmus and dysarthria: diagnosis, gene, triggers, treatment
Episodic ataxia type II. Gene: CACN1A4. Trigger: alcohol, fatigue, stress. Tx: Acetazolamide
Episodic ataxia type I. Gene: KCN1A. Trigger: exercise, startle. Tx: AEDs like Tegretol. See ataxia w/ facial twitching
Antibodies associated in acute motor axonal neuropathy (AMAN)? (4)
GM1, GM1b, GD1a, GalNac-GD1a
Ipsilateral seventh nerve palsy with contralateral hemiplegia
Millard- Gubler syndrome: lesion in the pons
Pathologic waves on ICP monitor- asso. w/ intracranial HTN and decreased intracranial compliance
Lundberg A waves or "plateau waves"
Sustained duration b/w 5 and 20 minutes
Amplitude high: range of 50-100mmHg
Gene/ protein affected in Unverricht- Lundborg syndrome (a type of Progressive Myoclonic Epilepsy)
CSTB gene- makes Cystatin B protein
Stimulus sensitive myoclonus (eg photic), worse with action
Tegterol and Phenytoin make it worse
EPM1
"Eye of the tiger"
Dx associated?
Location/intensity of MRI findings?
Seen in PKAN (pantothenate-kinase-associated-neurodegeneration)
Hyperintensity surrounded by hypointensity in basal ganglia
Painful sensory polyneuropathy
Autonomic dysfunction
Cardiac/ renal involvement
Family hx of the same
Familial Amyloid Polyneuropathy type 1, transthyretin deposition
*If X linked inheritance: consider Fabry's disease*