Familial British dementia

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Familial British dementia is a form of dementia. It was first reported by Cecil Charles Worster-Drought in 1933 [1] and is therefore also known as Worster-Drought syndrome. It is caused by a mutation in the ITM2B gene (also known as BRI2); a different mutation of the same gene causes the similar syndrome of familial Danish dementia. The combination of amyloid pathology and neurofibrillary tangles has led to comparison with the pathology of Alzheimer's disease.[2]


  1. ^ C Worster-Drought; Hill, TR; McMenemey, WH; et al. (1933). "Familial Presenile Dementia with Spastic Paralysis". J Neurol Psychopathol. s1-14 (53): 27–34. doi:10.1136/jnnp.s1-14.53.27. PMC 1038860Freely accessible. PMID 21610757. 
  2. ^ J Ghiso; Révész, T; Holton, J; Rostagno, A; Lashley, T; Houlden, H; Gibb, G; Anderton, B; et al. (2001). "Chromosome 13 dementia syndromes as models of neurodegeneration". Amyloid. 8 (4): 277–84. doi:10.3109/13506120108993826. PMID 11791622.