Familial dementia lacking specific pathological features presenting with clinical features of corticobasal degeneration

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Familial dementia lacking specific pathological features presenting with clinical features of corticobasal degeneration.

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 feature...

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OBJECTIVE To characterise the neuropsychiatric symptoms of patients with corticobasal degeneration (CBD). METHODS The neuropsychiatric inventory (NPI), a tool with established validity and reliability, was administered to 15 patients with CBD (mean (SEM), age 67.9 (2) years); 34 patients with progressive supranuclear palsy (PSP) (66.6 (1.2) years); and 25 controls (70 (0.8) years), matched fo...

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Neuropathological features of corticobasal degeneration presenting as corticobasal syndrome or Richardson syndrome.

Patients with corticobasal degeneration can present with several different clinical syndromes, making ante-mortem diagnosis a challenge. Corticobasal syndrome is the clinical phenotype originally described for corticobasal degeneration, characterized by asymmetric rigidity and apraxia, cortical sensory deficits, dystonia and myoclonus. Some patients do not develop these features, but instead ha...

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ژورنال

عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry

سال: 1998

ISSN: 0022-3050

DOI: 10.1136/jnnp.65.4.600