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 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.
Dementia Research Group, National Hospital for Neurology and Neurosurgery, London, UK.
J
J
Brown
P L
PL
Lantos
M N
MN
Rossor
D001284:Atrophy / Q000473:pathology; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology; D003704:Dementia / Q000150:complications / Q000235:genetics* / Q000473:pathology*; D005260:Female; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000150:complications / Q000175:diagnosis; D016874:Neurofibrillary Tangles / Q000473:pathology; D009483:Neuropsychological Tests; D010375:Pedigree; D013702:Temporal Lobe / Q000473:pathology*