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Dementia with motor neuron disease
Author(s) -
Mitsuyama Yoshio
Publication year - 2000
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1046/j.1440-1789.2000.00302.x
Subject(s) - amyotrophic lateral sclerosis , frontotemporal dementia , motor neuron , dementia , medicine , hippocampal sclerosis , pathology , neuroscience , disease , spinal cord , temporal lobe , psychology , epilepsy , psychiatry
Dementia with motor neuron disease has been described as a new clinicopathologic entity and more than 100 cases have been reported in Japan since 1964. The clinicopathologic criteria in the diagnosis of dementia with motor neuron disease are: (i) frontotemporal lobe‐type dementia with insidious onset, mostly in the presenile period; (ii) neurogenic muscular wasting during the course of the illness (amyotrophic lateral sclerosis‐ or SPMA‐like symptoms); (iii) duration from the onset of illness to death of 2–5 years (average, 30.6 months); (iv) both extrapyramidal symptoms and definite sensory deficits are present less commonly; (v) no characteristic abnormalities in the cerebrospinal fluid or electroencephalogram on screening; (vi) no known parental consanguinity or familial occurrence; and (vii) non‐specific, mild to slight degenerative changes in the frontotemporal cortex, hypoglossal nuclei and spinal cord, and frequently in the substantia nigra. Dementia with motor neuron disease is characterized by ubiquitin‐immunoreactive intraneuronal inclusions in cortical layer II and hypocampal dentate granule cells.