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From the Archives
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A form of familial degeneration of the cerebellum. By Gordon Holmes, M.D. (From the Neurological Research Department, National Hospital, Queen Square, London.) Brain 1907: 30; 466–489: A clinical study of an heredo-familial disease resembling disseminated sclerosis. By Fergus R. Ferguson and Macdonald Critchley. (From the National Hospital, Queen Square, W.C.) Brain 1929: 52; 203–225; and The clinical features and classification of the late onset autosomal dominant cerebellar ataxias: a study of 11 families, including descendants of ‘the Drew family of Walworth’ by A.E. Harding. (From the MRC Clinical Genetics Unit, Institute of Child Health, London). Brain 1982: 105; 1–28.
Although accounts of familial ataxia begin with Pierre Marie, the cases he described were a mixed bag, and MD Sanger Brown had already described a four generation pedigree, showing dominant inheritance, characterised by ataxia, optic atrophy, ophthalmoplegia and cognitive impairment (MD Sanger Brown: On hereditary ataxy, with a series of twenty-one
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