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Familial frontotemporal dementia with ubiquitin inclusion bodies and without motor neuron disease

Leuba, G
Saini, Krishan
Anastasiu, R
Miklossy, J
Published in Acta Neuropathologica. 2000, vol. 100, no. 4, p. 421-426
Abstract Frontotemporal dementia (FTD) is the second most common degenerative dementia after Alzheimer's disease and its Lewy body variant. Clinical pathology can be subdivided in three main neuropathological subtypes: frontal lobe dementia, Pick's disease and FTD with motor neuron disease (MND), all characterised by distinct histological features. Until recently the presence of ubiquitin-positive intraneuronal inclusions in the dentate gyrus, and the temporal and frontal cortex was usually associated with the MND type. Such inclusions were also observed in a few sporadic cases of FTD without or with parkinsonism (FTDP) in the absence of MND. We present here clinical, neuropathological and immunohistochemical data about a Swiss FTD family with FTDP-like features but without MND. Spongiosis and mild gliosis were observed in the grey matter. No neurofibrillary tangles, Pick bodies, Lewy bodies, senile plaques or prion-positive signals were present. However, ubiquitin-positive intracytoplasmic inclusions were detected in various structures but predominantly in the dentate gyrus. These observations support the existence of a familial form of FTDP with ubiquitin-positive intracytoplasmic inclusions (Swiss FTDP family).
Keywords AgedDementia/genetics/pathologyDentate Gyrus/pathologyFemaleGenes, DominantGliosis/pathologyHumansInclusion Bodies/chemistryMaleMotor Neurons/pathologyNerve Tissue Proteins/analysisPedigreeSwitzerlandSyndromeUbiquitins/analysis
PMID: 10985702
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Research group Groupe Bouras Constantin (neuropsychiatrie) (1)
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KOVARI, Eniko Veronika et al. Familial frontotemporal dementia with ubiquitin inclusion bodies and without motor neuron disease. In: Acta Neuropathologica, 2000, vol. 100, n° 4, p. 421-426. https://archive-ouverte.unige.ch/unige:26606

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Deposited on : 2013-03-06

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