Dementia with motor neuron disease.
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 hippocampal dentate granule cells.
Mitsuyama
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Department of Psychiatry, Miyazaki Medical College, Kihara, Japan. miyama@post.miyazaki-med.ac.jp
D003704:Dementia / Q000150:complications* / Q000473:pathology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D006801:Humans; D016472:Motor Neuron Disease / Q000150:complications* / Q000473:pathology* / Q000503:physiopathology; D009410:Nerve Degeneration / Q000473:pathology / Q000503:physiopathology; D013116:Spinal Cord / Q000473:pathology / Q000503:physiopathology