PRESENILE-DEMENTIA WITH MOTOR-NEURON DISEASE

Seventy-one Japanese cases of presenile dementia with motor neuron dis ease were reviewed. The clinico-pathological features were: (1) progre ssive dementia with insidious onset, mostly in the presenile period: ( 2) neurogenic muscular wasting in the course of illness (ALS- or SPMA- like symptoms); (3) duration from the onset of the illness to death: 2 -5 years (average 30.6 months) (4) extrapyramidal symptoms and definit e sensory deficits are less commonly present; (5) no characteristic ab normalities in the CSF or EEG; (6) no known consanguinity or familial occurrence: (7) non-specific mild to moderate degenerative changes in the fronto-temporal cerebral cortex, hypoglossal nuclei and spinal cor d, and frequently in the substantia nigra. The author was interested i n discovering whether the frequency and topology of lesions in the bra in of patients with presenile dementia and motor neeuron disease diffe red characteristically from the distribution found in cases of Alzheim er's disease, Pick's disease, Creutzfeldt-Jakob disease or progressive subcortical gliosis. Presenile dementia with motor neuron disease mig ht be a new disease entity.