Term
Congenital failure of the cerebellar vermis to develop
Presents with dilated 4th ventricle with absent cerebellum |
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Definition
| Dandy-walker malformation |
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Term
| Congenital extention of cerebellar tonsils through the foramen magnum |
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Definition
| Arnold-Chiari malformation |
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Term
Cystic degeneration of the spinal cord at C8-T1
Usually occurs with bilateral loss of pain and temperature on the upper extremity |
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Definition
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Term
| Viral infection that degenerates the anterior motor horn |
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Definition
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Term
Inherited degeneration of the anterior motor horn
Presents as a "floppy baby". Death in the first few years of life |
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Definition
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Term
| Degenerative disorder of both upper and lower motor neurons of the corticle spinal tract |
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Definition
Amyotrophic Lateral Sclerosis
Distinguish ALS between syringomelia due to the fact that ALS has:
Impaired sensory (touch, vibration etc) |
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Term
Degenerative disorder of the cerebellum and spinal cord:
Ataxia and weakness in lower extremities
GAA trinucleotide repeat |
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Definition
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Term
Causes of:
Thrombotic stroke
Hemorrhagic stroke
Lacunar stroke |
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Definition
Thrombotic stroke - rupture of a plaque (arthlerosclerosis) causes activation of coagulation cascade to create a thrombus to block blood (pale infarct)
Hemorrhagic - due to an emboli that can easily become lysed. Blood re-enters the infarcted area
Lacunar - due to arteriolar sclerosis which causes dilation of small arteries to create cystic deteriations |
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Term
| Bleeding on the underside of the brain due to ruptured Berry aneurysm, or marfan's disease, or PKD |
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Definition
Subarachnoid hemorrhage
Berry aneurysm lacks an media layer during the branch points.
"worst headache of my life" |
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Term
Damage to a high pressure vasculature system causing lens shaped lesion on CT.
Presents with a lucid interval with rapid death afterwards |
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Definition
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Term
Damage due to tearing of the bridging veins
Presents with a crescent shaped lesion on CT |
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Definition
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Term
| Type of herniation that causes death due to cardiopulmonary arrest |
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Definition
| Tonsillar herniation - cerebellar tonsils into the foramen magnum |
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Term
| Type of herniation that causes infarction of the anterior cerebral artery |
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Definition
| Subfalcine herniation - cingulate gyrus under the falx cerebri |
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Term
| Type of herniation that causes a "blown out" pupil and "down and out eye" because it involves the CNIII |
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Definition
Uncal herniation - Displacement of temporal lobe under the tentorium cerebelli
Compression of PCA can lead to infarction of the occipital lobe |
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Term
Autoimmune destruction of the CNS myelin and oligodendrocytes
Associated with HLA-DR2
Relapsing neurological deficits with periods of remission |
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Definition
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Term
| Debilitating encephalitis due to persistant infection of brain by measels virus |
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Definition
| Subacute sclerosing panencephalitis |
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Term
| Rapidly progressive neurological signs in individuals who are immunosuppressed (HIV/Leukemia) who are infected by this polyoma virus... |
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Definition
Progressive multifocal leukoencephalopathy
via JC virus! |
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Term
Presents with "locked in syndrome"
due to rapidly correcting hyponatremia too quickly |
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Definition
| Central Pontine myelinolysis |
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Term
Slow onset memory loss
Loss of learned motor skills
Changes in behavior/personality |
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Definition
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Term
Degenerative disease of the frontal and temporal cortex
Presents with behavioral and language symptoms as a result |
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Definition
Pick Disease
Pick bodies (round aggregates of tau protein) |
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Term
| Degenerative loss of dopaminergic neurons in the substantia nigra of the basal ganglia |
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Definition
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Term
Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia
CAG trinucleotide repeats during spermatogenesis
Chorea |
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Definition
Huntington Disease
Suicide is a common cause of death
Autosomal dominant in Chr. 4 |
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Term
Disease caused by normal prion (PrPc - alpha helix) to the pathologic prion (PrPsc - Beta pleated)
Damage to neurons and glial cells is characterized by intracellular vacuoles |
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Definition
| Spongiform encephalopathy |
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Term
| GAA trinucleotide repeat of the frataxin gene responsible for mitochondrial iron regulation |
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Definition
Friedreich's ataxia
Excess iron, free radial damage due to the fenton reaction |
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Term
Blurred vision
Vertigo
Scanning speech
Internuclear ophthalmoplegia
Hemiparesis
Lower extremity loss of sensation
Bowel, bladder, and sexual dysfunction |
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Definition
Multiple sclerosis
HLA-DR2 |
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Term
MRI reveals plaques (areas of white matter demyelination)
Lumbar puncture reveals increased lymphocytes, immunoglobulins with oligoclonal IgG bands, myelin basic protein |
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Definition
Multiple Sclerosis
CNS destruction of myelin and oligodendrocytes
Tx: High dose steroids |
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Term
Morphologic features:
Neuritic plaques (AB amyloid with neuritic processes)
Neurofibrillary tangles (hyperphosphorylated tau proteins)
Cerebral atrophy (widening sulci, thinning gyri) |
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Definition
Alzheimer disease
AB amyloid - amyloid precursor protein that was cleaved into B fragments that cannot break down
Tau proteins - microtubule associated protein. |
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Term
Dementia that is associated with increased risk with:
Epsilon 4 allele of apolipoprotein E
Down Syndrome
Presenilin 1 and Presenilin 2 |
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Definition
Alzheimer Disease
Presenilin 1/2 occurs in early familial cases (younger adults) |
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Term
Tremor (pill rolling at rest)
Rigidity (cogwheel)
Akinesia/Bradykinesia
Postural instability (shuffling gait) |
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Definition
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Term
Loss of pigmented neurons in substantia nigra
Round eosinophilic inclusions of Alpha synuclein |
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Definition
Parkinson Disease
Lewy Bodies - alpha synuclein |
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Term
Most common form of spongiform encephalopathy
Rapid dementia with ataxia
Startle myoclonus
Spike and wave complexes on EEG |
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Definition
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