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A rapidly progressive dementia with genetic linkage to chromosome 17q21 was observed.The mean age of onset was 51 years and the average duration to death was 3 years.Two patients started with speech disturbances leading to a progressive,nonfluent aphasia,one patient had onset symptoms of leg apraxia and akinesia and muscular rigidity and in one patient reckless driving was the first symptom.Loss of spontaneous speech later developed in all patients and emotional bluntness in three of the patients.Cerebral perfusion was decreased in the frontal areas in all patients.In the person with apraxia as the onset symptom,the cerebral blood flow was also diminished in the left hemisphere,where a slight atrophy was detected on magnetic resonance imaging scans.At the postmortem examination,slight gliosis of the parietal lobes was observed in this patient.In all patients there was a frontocentral degeneration of the cortex with discrete microvacuolation and gliosis.Clinical features of frontotemporal dementia, parkinsonism,an early age of onset,a rapid disease progression and variable onset symptoms were seen in these patients.Two other clinically distinct diseases,dementia with pallido-ponto-nigral degeneration and a disinhibition-dementia,parkinsonism,amyotrophy complex,have recently been mapped to chromosome 17q21.In the family described in this article,genetic linkage was detected to the same region,suggesting the possibility that these diseases may originate from pathogenic mutations in the same gene. |
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aphasia
chromosome 17
dementia
dementia,familial
dementia,frontal lobe type
dementia,frontotemporal
dementia,presenile
dementia,rapidly progressive
mortality
neuropathology
Parkinson disease
review article
speech disorder
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