Document Detail

Motor neurone disease-inclusion dementia.
MedLine Citation:
PMID:  9117546     Owner:  NLM     Status:  MEDLINE    
We describe nine patients, five women and four men (age at death 58-83 years), who developed isolated progressive frontotemporal dementia over 4 to 12 years. These cases represent nine of the 385 (2.3%) cases from a series of autopsy cases of dementia in a large teaching hospital. One had a mother with a history of frontotemporal dementia and marked frontal lobe atrophy. Another had multiple affected family members with frontotemporal dementia, motor neurone disease or both. None of the nine had clinical evidence of either an upper or lower motor neurone disorder. In each case neuropathological examination revealed cortical pathology identical to that described previously as typical of dementia associated with motor neurone disease. There was variable macroscopic atrophy and neuronal loss in the frontal and temporal lobes. All cases had cortical microvacuolation, in seven limited to cortical layer II, and transcortical in two. There was variable cortical and subcortical gliosis. Intraneuronal ubiquitin-immunoreactive inclusions, characteristic of the extra-motor involvement of motor neurone disease, were found in the hippocampal dentate granule cells and residual neurones in layer II of the frontotemporal cortex of all cases. Similar inclusions were also seen in the nucleus ambiguus of three cases. The hypoglossal nuclei showed no neuronal loss, gliosis or ubiquitin-immunoreactive inclusions. Ubiquitin-immunoreactive dystrophic neurites were detected within affected cortex, being most conspicuous in layer II in areas containing microvacuolation. Dystrophic neurites were not detected in subcortical structures. Spinal cords were unavailable for examination because of limited autopsy consent. The finding of intraneuronal ubiquitin-immunoreactive inclusions characteristic of motor neurone disease in patients with frontotemporal dementia, without clinical or pathological evidence of motor system degeneration, extends the clinical spectrum of diseases associated with such inclusions. We propose the term motor neurone disease-inclusion dementia (MNDID) for these cases.
M Jackson; G Lennox; J Lowe
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Neurodegeneration : a journal for neurodegenerative disorders, neuroprotection, and neuroregeneration     Volume:  5     ISSN:  1055-8330     ISO Abbreviation:  Neurodegeneration     Publication Date:  1996 Dec 
Date Detail:
Created Date:  1997-04-24     Completed Date:  1997-04-24     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9209022     Medline TA:  Neurodegeneration     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  339-50     Citation Subset:  IM    
Department of Neurology, Queen Elizabeth Hospital, Birmingham.
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MeSH Terms
Aged, 80 and over
Dementia / genetics,  metabolism,  pathology*
Dentate Gyrus / metabolism,  pathology
Frontal Lobe / metabolism,  pathology
Inclusion Bodies / metabolism,  ultrastructure*
Middle Aged
Motor Neuron Disease / genetics,  metabolism,  pathology*
Neurons / metabolism
Temporal Lobe / metabolism,  pathology
Ubiquitins / metabolism
Reg. No./Substance:

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