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102 Cards in this Set
- Front
- Back
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Sporadic congenital disorder with port-wine stains in V1 ophthalmic distribution
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Sturge-Weber
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Ilpsilateral leptomeningeal angiomas, phochromocytoma, can cause glaucoma, seizures, hemiparesis, mental retardation
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Sturge-Weber
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Hamartomas in CNS, skin, organs; cardiac rhabdomyoma, renal angiomyolipoma, subependymal giant cell astrocytoma, mitra regurgitation, seizures, hypopigmented ash leaf spots, sebaceous adenoma, shagreen patch
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Tuberous sclerosis
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How is Tuberous sclerosis inherited?
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AD
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Cardiac manifestations of tuberous sclerosis?
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Mitral regurg
Cardiac rhabdomyoma |
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Skin manifestations of tuberous sclerosis
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Hamartomas in CNS
Subependymal giant cell astrocytoma Seizures |
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NF1 on chromosome?
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17
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NF2 on chromosome?
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22
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Cafe-au-lait spots, Lisch nodules (pigmented iris hamartomas), neurofibromas in skin, optic gliomas, pheochromocytomas
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NF1
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Optic gliomas and pigmented iris hamartomas affecting every generation
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NF1 - autosomal dominant
Cafe-au-lait spots, Lisch nodules (pigmented iris hamartomas), neurofibromas in skin, optic gliomas, pheochromocytomas |
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Cavernous hemangiomas of skin, mucosa, organs, bilateral renal cell carcinoma, hemangioblastoma in retina, brain stem, cerebellum, pheochromocytoma
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VHL
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Mutation in VHL?
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Tumor suppressor VHL on chromosome 3 (autosomal dominant)
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Associated with chronic hypertension; affects small vessels in basal ganglia, thalamus
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Charcot-Bouchard microaneurysms
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Rupture of berry aneurysms leads to?
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Hemorrhagic stroke/subarachnoid hemorrhage
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Berry aneurysms associated with?
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Adult polycystic kidney disease
Ehlers-Danlos Marfan's Age, HTN, smoking, race (blacks) |
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On CT, biconvex disk not crossing suture lines
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Epidural hemorrhage
Can cross falx, tentorium |
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Epidural hemorrhage usually due to?
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Rupture of middle meningeal artery (branch of maxillary artery)
Secondary to fracture of temporal bone |
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Complications of epidural hemorrhage
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Rapid expansion under systemic arterial pressure --> transtentorial herniation (compression of corticospinal tracts), CN II Palsy
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Rupture of bridging veins
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Subdural hematoma
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Subdural hematoma on CT?
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Crescent-shaped hemorrhage that crosses suture lines
Gyri preserved (pressure distributed equally) |
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Subdural hematoma due to?
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Rupture of bridging veins
Elderly, alcoholics, blunt trauma, shaken baby |
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Rupture of an aneurysm
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Subarachnoid hematoma
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Rupture of AVM
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Subarachnoid hematoma
(or aneurysm) "Worst headache of life" |
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Bloody or yellow (xanthrochromic spinal tap) and nuchal rigidity is caused by what intracranial hemorrhage?
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Subarachnoid
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Patient with subarachnoid hemorrhage is at risk for?
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2-3 days after: vasospasm due to blood breakdown products that irritate vessels
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Treatment for vasospasm due to subarachnoid hemorrhage breakdown products
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Beta blockers
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Parenchymal hematoma caused by?
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HTN, amyloid angiopathy, diabetes, tumor
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Parenchymal hematoma typically occurs where?
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Basal ganglia, internal capsule
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Ischemic brain disease causes irreversible damage after how long?
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5 minutes
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Brain areas most vulnerable to ischemia
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Hippocampus
Neocortex Cerebellum Watershed areas |
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Irreversible neuronal injury in ischemia - timeline?
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Red neurons (12-48 hours)
Necrosis + neutrophils (1-3 days) Macrophages (3-5 days) Reactive gliosis + vasc proliferation (1-2 weeks) Glial scar (>2 weeks) |
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Veins from face drain into?
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Superior ophthalmic vein --> meet sphenoparietal sinus at cavernous sinus
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Main location of CSF return via arachnoid granulations
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Superior sagittal sinus
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Wet, wobby, wacky
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Normal pressure hydrocephalus
Normal CSF pressure; does NOT result in increased subarachnoid space volume |
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Normal pressure hydrocephalus leads to?
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Expanded ventricles distorts fibers of corona radiata --> dementia, ataxia, urinary incontinence
(Wet Wild Wacky) |
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What causes normal pressure hydrocephalus?
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Gradual blockage of CSF drainage (normal CSF pressure)
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Decreased CSF absorption by arachnoid villi leading to increased ICP, papilledema, herniation
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Communicating hydrocephalus
(e.g. arachnoid scarring post-meningitis) |
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Obstructive (noncommunicating) hydrocephalus due to?
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Structural blockage of CSF circulation (e.g. stenosis of aqueduct of Sylvius)
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Stenosis of Sylvius aqueduct causes?
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Obstructive hydrocephalus
Paralysis of upward gaze (Parinaud's) (most common in newborns) |
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Hydrocephalus ex vacuo seen in?
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Alzheimer's, advanced HIV, Pick's
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Subarachnoid space extends to?
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S2
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Spinal cord extends to?
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L1-L2
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Seen in UMN lesion
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Weakness, increased reflexes, tone, Babinski, spastic paralysis, clasp knife spasticity (increased resistance to passive stretch abruptly stops)
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Seen in LMN lesion
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Atrophy, fasciculation
Lowered reflexes, tone |
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Bilateral loss of pain and temperature sensation (C8-T1) with preservation of touch sensation
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Syringomyelia (damages anterior white commissure of spinothalamic tract)
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Syringomyelia associated with what congenital condition?
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Arnold-Chiari II
(cerebellar tonsillar herniation through foramen magnum) |
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Cause of Arnold-Chiari type II?
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Cerebellar tonsillar herniation through foramen magnum with aqueductal stenosis and hydrocephaly
Presents with syringomyelia and thoracolumbar myelomeningocele |
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Large posterior fossa; absent cerebellar vermis with cystic enlargement of 4th ventricle
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Dandy Walker
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What is missing in Dandy Walker malformation
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Cerebellar vermis --> cystic enlargement of 4th ventricle
Can lead to hydrocephalus and spina bifida |
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LMN lesions only; due to destruction of anterior horns --> flaccid paralysis
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Wernig-Hoffmann
Poliomyelitis |
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Random and asymmetric lesions due to demyelination; presents with scanning speech, intention tremor, nystagmus
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Multiple sclerosis
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Combined UMN and LMN deficits with NO SENSORY DEFICITS
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ALS
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Degeneration of dorsal roots and dorsal columns; presents with impaired proprioception and locomotor ataxia
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Tabes dorsalis (3 syphilis)
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Demyelination of dorsal columns, lateral corticospinal tracts, spinocerebellar tracts
Present with ataxic gait, hyperreflexia, impaired position and vibration sense |
Vit B12 neuropathy
Vit E deficiency Friedriech's ataxia |
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Vit B12 neuropathy
Vit E deficiency Friedriech's ataxia Share what lesions? |
Dorsal columns
Lateral corticospinal tracts Spinocerebellar tracts |
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Poliovirus characteristics?
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ss+ RNA, nonenveloped, icosahedral
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Wernig-Hoffman inheritance?
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AR
Median age of death 7 months |
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ALS caused by?
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Defect in SOD1
Betel nut ingestion |
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Friedreich's ataxia inheritance?
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AR (GAA repeat; frataxin gene)
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Friedreich's ataxia due to?
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GAA repeat causes impaired mitochondrial function --> iron homeostasis disrupted, cells prone to apoptosis
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Cause of death in Friedreich's ataxia
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Hypertrophic cardiomyopathy
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Staggering gait, frequent falling, nystagmus, dysarthria, pes cavus, hammer toes in a child with scoliosis
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Friedreich's ataxia
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Parinaud's syndrome due to?
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Lesion in superior colliculi (e.g. pinealoma) --> paralysis of conjugate vertical gaze
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Opthalmoplegia, ophthalmic and maxillary sensory loss due to mass effect
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Cavernous sinus syndrome
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CNs in cavernous sinus
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III, IV, V1, V2, VI, postganglionic sympathetic fibers
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Hyperacusis from paralysis of stapedius muscle, inability to ipsilateral eye
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Bell's palsy (destruction of VII nucleus or branchial efferent fibers)
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Acute, painless monocular loss of vision; pale retina and cherry red macula
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Retinal artery occlusion
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Macula blood supply
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Choroid artery
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Retinal necrosis and edema leading to atrophic scar
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Retinitis
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Changes in lens due to aging
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Sclerosis, decreased elasticity
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Dilator/radial muscle function
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alpha1 --> mydriasis
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Sphincter/circular/constrictor muscle function
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M3 --> miosis
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Ciliary muscle function
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M3 --> accomodation
Near vision (muscle contraction --> zonular fibers relax --> lens relaxes --> more convex) Far vision (relaxation --> lens flattens) |
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Dry/atrophic ARMD due to?
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Fat deposits --> gradual loss of central vision (scotoma)
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Wet ARMD due to?
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Neovascularization --> hemorrhage and cell death (rapid)
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Visual pathway that loops around inferior horn of lateral ventricle
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Meyer's loop (temporal lobe)
L/R upper quadrantic anopia |
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Visual pathway that goes through internal capsule
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Dorsal optic radiation (parietal lobe)
L/R lower quandratic anopia |
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Early onset Alzheimer's genes
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APP (21)
Presenilin-1 (14) Presenilin-2 (1) |
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Late onset Alzheimer's genes
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ApoE4 (19)
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Protective gene in Alzheimer's
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ApoE2 (19)
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Dementia, aphasia, parkinsonian aspects, changes in personality that spares parietal lobe and posterior 2/3 of superior temporal gyrus
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Pick's
(frontotemporal dementia) |
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What are Pick bodies?
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Intracellular, aggregated tau protein
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Lewy body dementia features?
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Parkinsonism with dementia and hallucinations
alpha-synuclein defect with Lewy bodies |
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Lewy bodies are?
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eosinophilic, intracellular inculsions
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Dementia with myoclonus; bubbles and holes seen on imaging
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CJD
(prions --> alpha helix to beta sheets resistant to proteases) |
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Second most common cause of dementia in elderly
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Multi-infarct
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Causes of dementia
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Alzheimer's
Pick's Lew body CJD Multi-infarct syphilis HIV Vit B12 Wilson's |
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Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with preservation of axons
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MS
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Demyelination of CNS due to destruction of oligodendrocytes; rapidly progressive, fatal
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Progressive multifocal leukoencephalopathy (PML)
Associated with JC virus (polyomavirus - DS DNA, circular) |
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JC virus is a?
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Polyomavirus - dsDNA, circular
Associated with PML |
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Multifocal perivenular inflammation and demyelination after infection (chickenpox, measles) or vaccinations (rabies, smallpox)
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Acute disseminated (postinfectious) encephalomyelitis
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Acute disseminated encephalomyelitis occurs when?
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After infection (chickenpox, measles) or vaccinations (rabies, smallpox)
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Metabolic disease featuring central and peripheral demyelination with ataxia, dementia
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Metachromatic leukodystrophy (arysulfatase A deficiency --> buildup of sulfatides)
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Buildup of sulfatides in metachromatic leukodystrophy causes?
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Impaired production of myelin sheath
Direct toxicity to oligodendrocytes and Schwann cells |
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Hereditary nerve disorder --> defective production of proteins involved in structure and function of peripheral nerves or myelin sheath
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Charcot-Marie-Tooth
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Peroneal nerve neuropathy, atrophy of leg muscles; "inverted bottle" appearance in AD inheritance
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Charcot-Marie-Tooth
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Partial seizures original from?
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Mesial temporal lobe
Simple (consciousness intact) Complex |
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Unilateral; 4-72 hours of pulsating pain with nausea, photophobia, phonophobia, +/- aura
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Migraine
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Migraine due to?
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Irritation of CN V and release of substance P, calcitonin gene-related peptide, vasoactive peptides
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Treatment of migraines?
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Propanolol, NSAIDS, sumatriptan (acute)
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Bilateral; > 30 minutes of steady pain; no aura, not aggravated by light or noise
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Tension headache
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Unilateral; repetitive brief headaches
Periorbital pain, ipsilateral lacrimation, rhinnorhea, Horner's |
Cluster headache
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