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.
Brown
J
J
Dementia Research Group, National Hospital for Neurology and Neurosurgery, London, UK.
Lantos
P L
PL
Rossor
M N
MN
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*