Dementia with Motor Neuron Disease: A Review

Abstract

Dementia associated with motor neuron disease has been described as a new clinico-pathological entity, and more than 100 cases have been reported in Japan since 1964. The clinico-pathological criteria in the diagnosis of dementia with motor neuron disease are (1) frontotemporal lobe-type dementia with insidious onset, mostly in the presenile period; (2) neurogenic muscular wasting during the course of the illness (ALS- or SPMA-like symptoms); (3) duration from the onset of illness to death, 2–5 years (average, 30.6 months); (4) both extrapyramidal symptoms and definite sensory deficits are less commonly present; (5) no characteristic abnormalities in the CSF or EEG in screening; (6) no known parental consanguinity or familial occurrence; and (7) nonspecific, mild to slight degenerative changes in the frontotemporal cortex, hypoglossal nuclei, 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 the hippocampal dentate granule cells.