TABLE 7-1.
University of California, San Francisco (UCSF), Cohort N = 104 (21)b |
% | German Cohort7 N = 124 (37)b |
% | National Prion Disease Pathology Surveillance Center (NPDPSC) Cohort8 N = 304 (304)b |
% |
---|---|---|---|---|---|
Autoimmune/antibody-mediatedc | 13 | Alzheimer disease | 27 | Alzheimer disease | 51 |
Unclassified dementia | 13 | Unclassified dementia | 16 | Vascular disease | 12 |
Psychiatric | 12 | Cerebrovascular (vascular dementia, cerebrovascular accident) |
9 | Immune mediated | 9 |
Dementia with Lewy bodies | 8 | Encephalitis, unknown | 8 | Neoplasia | 8 |
Encephalitis, not otherwise specified | 8 | Parkinson disease | 5 | Infections | 5 |
Neoplasm | 8 | Psychiatric | 5 | Unspecified degenerative disease | 3 |
Frontotemporal dementia with or without motor neuron disease |
7 | Motor neuron disease | 2 | Frontal lobe degeneration | 3 |
Corticobasal syndrome or corticobasal degeneration |
6 | Multiple sclerosis | 2 | Metabolic | 2 |
Alzheimer diseased | 5 | Paraneoplastic | 2 | Hippocampal sclerosis | 2 |
Central nervous system vasculitis | 3 | Toxicity | 2 | Dementia with Lewy bodies | 1 |
Encephalopathy, not otherwise specified |
3 | Alcohol induced | 2 | Tauopathy, not otherwise specified | 1 |
Leukoencephalopathy | 3 | Brain tumor | 2 | Hereditary diffuse leukoencephalopathy with spheroids |
1 |
Progressive supranuclear palsy | 3 | Chronic epilepsy | 2 | Progressive supranuclear palsy | 1 |
Vascular dementia | 2 | Corticostriatonigral degeneration |
2 | Othere | 2 |
Othere | 8 | Familial spastic paraplegia | 2 | Total | 100 |
Total | 100 | Hashimoto encephalopathy | 2 | ||
Hereditary ataxia | 2 | ||||
Huntington disease | 2 | ||||
Metabolic disorder | 2 | ||||
Primary central nervous system lymphoma |
2 | ||||
Othere | 4 | ||||
Total | 100 |
If only a single case of any given condition presented to a cohort, it was placed under “other.”
Numbers in parentheses indicate the number of pathology-proven cases.
Includes anti–voltage-gated potassium channel complex, glutamic acid decarboxylase (GAD65), Yu, Hu, CV2, adenylate kinase5, α-amino-3-hydroxy-5-methylisoxazole-4-proprionic acid (AMPA) receptor, glial, neuropil, and other antibodies as well as Hashimoto encephalopathy.
Includes two cases of mixed Alzheimer disease with Lewy bodies.
Other UCSF cohort cases include hydrocephalus, mesial temporal sclerosis, vertigo, germinoma, methylmalonic acidemia, multiple sclerosis, methotrexate toxicity, and pathology-proven sarcoid. Other German cohort cases include leukoencephalopathy, amyloid angiopathy, fatal familial insomnia, hypoxia, and Niemann-Pick lipoid histiocytosis. Other NPDPSC cohort cases include corticobasal degeneration, adult polyglucosan body disease, Huntington disease, Marchiafava-Bignami disease, and superficial siderosis.