TABLE 7-1.

Diagnostic Breakdown of Non–Jakob-Creutzfeldt Disease Rapidly Progressive Dementia Referrals to Three Jakob-Creutzfeldt Disease Referral Centers^a

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

^aIf only a single case of any given condition presented to a cohort, it was placed under “other.”

^bNumbers in parentheses indicate the number of pathology-proven cases.

^cIncludes 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.

^dIncludes two cases of mixed Alzheimer disease with Lewy bodies.

^eOther 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.