Familial dementia lacking specific pathological features presenting with clinical features of corticobasal degeneration
- aDementia Research Group, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK, bDepartment of Neuropathology, Institute of Psychiatry, De Crespigny Park, Denmark Hill, London, UK, cDepartment of Neurology, Addenbrooke’s Hospital, Hills Road, Cambridge, UK
- Dr M N Rossor, Dementia Research Group, National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK.
- Received 7 March 1997
- Revised 22 April 1998
- Accepted 23 April 1998
Abstract
A family is described in which one member presented with symptoms and signs suggestive of corticobasal degeneration and a sibling presented with features of a frontal lobe dementia. Their mother developed a presenile dementia and movement disorder. At postmortem examination the member with clinical corticobasal degeneration had non-specific pathological features. Therefore, the clinical features of corticobasal degeneration can occur with non-specific pathological changes. Within a pedigree, different members can present with different clinical syndromes, which may reflect variation in the distribution and severity of the pathological process.
