| Term | Definition |
| Alzheimer's disease | most common cause of dementia in the elderly. widespread cortical atrophy with decreased Ach. beta-amyloid plaques and tau protein neurofibrillary tangles |
| Pick's disease | dementia, aphasia, parkinsonian aspects; intracellular aggregated tau protein bodies; frontotemporal lobe atrophy |
| Lewy body dementia | parkinsonism with dementia and hallucinations. alpha-synuclein defect |
| Creutzfeldt-Jakob disease | rapidly progressive dementia with myoclonus & spongiform cortex |
| vitamin B12 deficiency | dementia; mamillary body degeneration |
| Wilson's disease | dementia, clumsiness, behavior changes, parkinsonism; changes in basal ganglia, putamen, and globus pallidus |
| neurosyphilis | 'general paresis of the insane' - dementia and general paresis |
| HIV dementia | metabolic encephalitis with activation of brain macrophages and microglia |
| neurosyphilis | tabes dorsalis (demyelination of posterior columns) |
| multiple sclerosis | autoimmune inflammation and demyelination of CNS. optic neuritis, MLF syndrome, hemiparesis, hemisensory sx, bladder/bowel incontinence. increased IgG in CSF, periventricular plaques |
| progressive multifocal leukoencephalopathy | demyelination of CNS due to destruction of oligodendrocytes. associated with JC virus, often in AIDS patients |
| acute disseminated encephalomyelitis | multifocal perivenular inflammation and demyelination after viral infection or vaccinations |
| metachromatic leukodystrophy | autosomal-recessive lysosomal storage disease, usually due to arylsulfatase A deficiency. impaired myelin sheath production |
| Charcot-Marie-Tooth disease | group of progressive hereditary nerve disorders related to defective production of proteins involved in PNS function |
| Sturge-Weber syndrome | congenital disorder with port-wine stains and ipsilateral leptomeningeal angioma. glaucoma, seizures, hemiparesis, MR |
| tuberous sclerosis | hamartomas in CNS, skin, organs; several associated tumors; MR, seizures, hypopigmented 'ash leaf spots' |
| Neurofibromatosis type I | cafe-au-lait spots, LIsch nodules, neurofibromas in skin |
| von Hippel-Lindau disease | cavernous hemangiomas in skin, mucosa, organs; renal cell carcinoma, hemangioblastoma in retina, brain stem, cerebellum. autosomal dominant |