Term
| Central Pontine myelinolysis |
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Definition
loss of myelin, symmetetric pattern; basis pontis and tegmentum, sparing periventricular and subpial
related to rapid correction of hyponatremia; rapid quadriplegia, myelin loss without inflam, neurons and axons preserved |
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Term
| hydrocephalus ex vacuo, neuritic plaques |
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Definition
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Term
diffuse AD plaques
vs
neuritic AD plaques |
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Definition
| AB42 for diffuse; AB40 and 42 for neuritic |
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Term
| components of neurofibrillary tangles of AD |
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Definition
| tau, paired helical filament, MAP2, ubiquitin,neuropil threads |
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Term
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Definition
| elongated, glassy, eosinophilic bodies of paracrystalline arrays of beaded filaments with actin as there major component; found in AD wihin hippocampal pyramidal cells |
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Term
| asymmetrical lobar atrophy |
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Definition
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Term
| cytoplasmic round to oval filamentous inclusions that are weakly basophilic and do not survive the death of host neurons |
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Definition
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Term
| truncal rigidity with dysequilibrium and nuchal dystonia; pseudobulbar palsy, abnormal speech, ocular disturbances, mild dementia |
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Definition
| progressive supranuclear palsy |
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Term
| ballooned neurons, tufted astrocytes, coiled bodies; tau positive threads in gray and white matter |
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Definition
| corticobasal degeneration |
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Term
| extrapyramidal rigidity, asymmetric motor disturbances (jerky limbs), sensory cortical dysfunctions (apraxia, language), cognative decline |
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Definition
| corticobasal degeneration |
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Term
| Lewy bodies starting in the medulla, progressing to midbrain; dementia, depigmentation of substantia nigra, preservation of cortex, hippocampus, amygdala |
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Definition
| dementia with lewy bodies |
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Term
| glial cytoplasmic inclusions, typically a-synuclein within the cytoplasm of oligodendrocytes; dominant symptoms either parkinsonism, cerebellar dysfunction or autonomic dysfunction |
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Definition
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Term
| autosomal recessive, first decade of life- gait ataxia, clumbsiness (hands), dysarthria, decreased DTR and +babinski |
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Definition
| fredreich ataxia- GAA trinucleotide repeat frataxin protein (mitochondrial encephalopathy) |
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Term
| decrease in DRG neurons, enlarged heart, degeneration of clarke column, brainstem (8, 10, 12 nuceli), CB and Betz |
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Definition
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Term
| ataxic dyskinetic syndrome, with subsequent telangiectasia in conjunctiva and skin |
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Definition
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Term
| loss of purkinje and granule cells, degernation of dorsal colmuns, spinocerebellar tract, anterior horn cells and peripheral neuropathy, in addition to amphicytes, hyoplastic gondads, thymus and LN |
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Definition
| ataxia-telangiectasia; mutation in ATM (ds dna breaks) |
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Term
| spinocerebellar disease which usually causes death within second decade and many affected individuals develop lymphoid neoplasms, leukemias, gliomas and carcinomas |
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Definition
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Term
| mutation in copper zinc superoxide dismutase gene on chromosome 21 (SOD1) |
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Definition
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Term
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Definition
| neurons containing PAS positve cytoplasmic inclusions (remnants of autophagic vacuoles) found in ALS |
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Term
| ALS in only lower motor neuron |
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Definition
| progressive muscular atrophy |
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Term
| bulbar or progressive bulbar ALS |
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Definition
| degeneration of lower brainstem cranial motor nuclei occurs early and pregresses rapidly; deglutition, phonation, |
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Term
| X-linked adult onset; limb amyotrophy, bulbar signs, androgen insensitivity, gynecomastia, testicular atrophy, oligospermia |
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Definition
| kennedy syndrome (bublospinal atrophy) |
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Term
| nuclear inclusions containing aggregated androgen receptor |
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Definition
| bulbospinal atrophy, kennedy syndrome |
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Term
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Definition
| ALS in children, selective loss of anterior horn cells an atrophy of anterior spinal roots |
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Term
| mutation in the palmitoyl protein enzyme leads to neuronal dysfunction of blindness, mental and motor deterioration and seizures |
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Definition
| neuronal ceroid lipofuscinoses |
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Term
| globoid cells in the parenchyma and around blood vessels |
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Definition
| krabbe disease (leukodystrophy, galactocerebroside beta-galactosidases) |
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Term
| mutation leads to alternate catabolic pathway leads to generation of galactosylphingosine which causes oligodendrocyte injury |
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Definition
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Term
| deficiency of arylsulfatase A; leads to an accumulation of sulfatides, especially cerebroside sulfate leading to demylination with gliosis. macrophages have vaculoated cytoplasms that are metachromatic |
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Definition
| metachromatic leukodystrophy |
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Term
psychiatric symptoms leading to motor issues in adults
motor symptoms progressing to death in children |
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Definition
| metachromatic leukodystrophy |
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Term
| X linked disease; present in early school years with neuro symptoms and adrenal insufficiency |
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Definition
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Term
| inability to catabolize very long chain fatty acids |
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Definition
| adrenoleukodystrophy, atrophy of adrenal cortex, VLCFA accumulation seen in remaining cells |
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Term
| the two X-linked leukodystrophies |
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Definition
| Pelizaeus-Merzbacher, adrenoleukodystrophy, |
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Term
| pendular eye movements, hypotonia, choreoathetosis and pyramidal signs, follled by spasticity, dementia, and ataxia |
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Definition
| pelizaeus-merzbacher disease |
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Term
| tigroid apperance of myelin |
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Definition
| pelizaeus-Merzbacher disease |
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Term
| megalocephaly, severe mental deficits, blindness, white matter injury starting in infancy |
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Definition
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Term
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Definition
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Term
| accumulation of n-acetylaspartic acid as a consequence of mutation in gene for aspartoacylase enzyme |
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Definition
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Term
| megalencephaly, seizures and progressive psychomotor retardation |
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Definition
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Term
| accumulation of rosenthal fibers around blood vessels, in subpial and subependymal zones in brain parenchyma |
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Definition
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Term
| ataxia, seizures during first few years of life; eIF2B reduced throughout body |
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Definition
| vanishing white matter leukodystrophy |
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Term
| most common neurological syndrome caused my mitrochondria |
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Definition
| MELAS: encephalomyopathy, lactic acidosis and strokelike episodes; tRNA mutaiton |
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Term
| myoclonus, seizure disorder, myopathy, ataxia, mutations in tRNA, mitochondrial disorder |
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Definition
| MERRF (myoclonic epilepsy and ragged red fibers) |
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Term
| lactic acidemia, arrest of psychomotor development, feeding problems seizures, extra-ocular palsies, weakness with hypotonia; death within 1-2 years |
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Definition
| Leigh syndrome (subacute necrotizing encephalopathy) |
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Term
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Definition
| neuropathy, ataxia, retinitis pigmentosa; higher fraction of normal myochondria in Leigh Syndrome (heteroplasmy) |
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Term
| spongiform change in gray and white matter, neuronal loss most evident in CB; ataxia, external opthalmoplegia, pigmentary retinopathy, cardiac conduction defects |
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Definition
| Kearn-Sayre Syndrome (ophthalmoplegia plus) due to large deletion/rearrangement in mito dna |
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Term
| neurosymptoms along with hepatic dysfunction including hepatitis an dbile duct proliferation; developmental delay, hypotonia, ataxia and cortical blindness; spongiform degenration of gray matter |
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Definition
| alpers; mutation in gene for DNA poly gamma- mitochondrial genome |
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Term
| tracts affected by vitamin B12 deficiency |
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Definition
| dorsal columns and descending pyramidal tracts; aka subacute combined degeneration of the spinal cord |
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Term
| Main neuronal cell layers affected by CO (and most other toxins, deprivations, etc) |
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Definition
| Neurons from layer 3/5, also sommer (CA1) sector of hippocampus, purkinje cells of CB |
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Term
| what does methanol toxicity preferentially affect? |
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Definition
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Term
| bergmann gliosis and loss of purkinje cells |
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Definition
| ethanol affects on CB (chronic) |
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Term
| coagulative necrosis and edema; white matter lesions |
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Definition
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Term
| drowsiness, ataxia, confussion, leading to recover OR to death; dystrophic mineralization of axons and cell bodies, gliosis, |
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Definition
| methotrexate and radiation induced CNS injury |
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