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- CLINICA NEUROL, 43: 775－778, 2003
- Vol.43 No.11 contents
The 44th Annual Meeting of the Japanese Society of Neurology
Educational Lecture 9:
Familial non-Alzheimer dementia
Takeshi Tabira
National Institute for Longevity Sciences
This is an abstract of my lecture on familial non-Alzheimer dementia.
1. Vascular dementia
1) Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by autosomal dominant inheritance, smooth muscle cell degeneration and granular osmiophilic material (GOM) in arterioles, and Notch 3 mutations.
2) CARASIL
This is an autosomal recessive vascular dementia with unknown etiology.
3) Familial amyloid angiopathy
Familial cerebral hemorrhage and dementia is caused by mutations in amyloid precursor protein, cystatin c, and Bri genes.
2. Familial non-Alzheimer degenerative dementia
1) Dementia with Lewy bodies
This is characterized by Alzheimer like dementia, visual hallucination and diffuse Lewy bodies which are formed by ubiquitinated α -synuclein. Occasionally, familial forms are reported, but gene mutations are unknown.
2) Frontotemporal dementia (FTD)
FTDP-17 is characterized by tau mutations, character and personal changes, and disinhibition. The gene mutations were also found in familial forms of Pick's disease, corticobasal degeneration, and other tauopathies.
3) Familial British dementia (FBD), familial Danish dementia (FDD)
FBD and FDD are characterized by Abri amyloid deposits, amyloid angiopathy and dementia. Mutations in Bri gene are reported.
4) Familial encephalopathy with neuroserpin inclusion bodies (FENIB)
FENIB is characterized by dementia, Collins body and neuroserpin gene mutation.
(CLINICA NEUROL, 43: 775－778, 2003)
key words: CADASIL, amyloid angiopathy, FTDP-17, FBD, FENIB
(Received: 17-May-03)
