Neurology Flashcards

1
Q

Dandy Walker malformation

A

A genesis or hypoplasia of cerebella vermis, cystic dilation of fourth ventricle, enlargement of posterior fossa

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2
Q

Anterior cerebral artery supply

A

Medial, frontal, parietal lobes, caudate

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3
Q

Middle cerebral artery supplies

A

Lateral hemispheres

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4
Q

Posterior cerebral artery supplies

A

Midbrain, occipital lobes, inferior temporal lobes

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5
Q

Cerebral perfusion pressure (CPP)

A

CPP = MAP - ICP

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6
Q

Differential of neonatal encephalopathy

A
Metabolic
Infectious
Thromboembolic
Intracranial hemorrhage
Genetic syndromes
Hypoxic-ischemic
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7
Q

Neonatal encephalopathy not HIE. Work up

A

Ammonia, urine ketones

Consider: imaging, echo, PAA, urine AA, UOA, acylcarnitine profile

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8
Q

Additional work up for refractory seizures not HIE

A

EEG
MRI
plasma and csf studies including AA and pipecolic acid and lactate, glucose
Biotinidase level
Plasma uric acid, urine sulphites, urine purine, FAs, copper and ceruplasmin
Consider gene panel

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9
Q

Diffuse selective neuronal injury d/t

A

Severe prolonged insults

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10
Q

Cerebral cortex deep nuclear structure injury d/t

A

Moderate to severe relatively prolonged insults

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11
Q

Deep nuclear structures (basal ganglia, thalamus, globus pallidus) injury d/t

A

Severe abrupt insult

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12
Q

Proposed mechanism of periventricular hemorrhagic infarction

A

IVH obstructs BF in terminal vein leading to venous infarction in distribution of medullary veins (drain the cerebral white matter into the terminal vein)

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13
Q

Hypoglycemia neuropathology

A

Most common pattern of injury is bilateral posterior occipital cortex but can be diverse and include MCA infarction and basal ganglia/thalamic abn

Risk of injury related to duration of hypoglycemia rather than degree of hypoglycemia

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14
Q

Disorders of LMN

A

Anterior horn (hypoxic-ischemic myelopathy, SMA, neurogenic arthrogryposis)
NMJ (transient or congenital myasthenia gravis, drugs impacting acetylcholine release - aminoglycoside toxicity, c botulinum, c tetani, hypermag)
Muscle (congenital myopathy - myotubular myopathy; muscular dystrophy - congenital myotonic dystrophy, congenital muscular dystrophy, duchenne dystrophy)
Metabolic and multi system (phoshpofructokinase deficiency, peroxisomal disorders, mitochondrial disorders, pompe disease, debrancher deficiency, carnitine deficiency, phosphorylase deficiency)

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15
Q

Phenobarbital MOA

A

Increase time Cl channels are open by acting on GABA-A receptor which depresses the CNS

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16
Q

Phenytoin MOA

A

Blocks voltage gated Na channels, thus blocking repetitive firing of action potentials

17
Q

Levetiracetam MOA

A

Binding to the synaptic vesicles protein SV2A reducing the rate of vesicle release

18
Q

Midazolam MOA

A

Increases GABA activity

19
Q

Lidocaine MOA

A

Blocks Na channels but true MOA of AED unknown

20
Q

Topiramate MOA

A

Blocks Na channels and enhances GABA activated Cl channels and inhibits excitatory NTs

21
Q

Bumetanide MOA

A

Inhibits Na-K-Cl co transporter and is effective for temporal lobes epilepsy with altered chloride homeostasis

22
Q

Neurocutaneous syndromes

A
Sturge-Weber 
Tuberous sclerosis
Neurfibromatosis
McCune Albright
Von Hippel Lindau
23
Q

Tuberous Sclerosis

A

AD
Ch 9, 16
Ash leaf macules
CNS tumors, eye involvement, seizures, rhabdomyomas

24
Q

Neurofibromatosis

A
AD
Ch 17
Cafe au lait spots
Freckled axillae, inguinal, perineum
Macrocephaly, aqueductal stenosis
Associated tumors 
Seizures
25
Q

McCune Albright

A
Sporadic
Fibrous dysplasia of bones
Precocious puberty
Hyperthyroidism
Hyperparathyroidism
Pituitary adenomas
26
Q

Von Hippel Lindau

A
AD
Ch 3p
Over expression of transcription factor hypoxia-inducible factor which leads to increased tumor growth
CNS tumors
Systemic hemangiomata
Retinal angiomas
Pheochromocytomas