Presenile Dementia With Motor Neuron Disease –An Additional Case Report–

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Abstract
A 51 yr old male patient has suffered the gradual onset of difficulty with memory, concentration and cognition at age 50. He has progressively developed severe dementia accompanied by muscle wasting and fasciculation prominent in the upper arms and shoulders and the bulbar muscles. Electromyogram and muscle biopsy revealed denenervation patterns and a computed tomographic scan showed moderate cerebral atrophy. Cases of presenile dementia with motor neuron disease reported in Japan have been reviewed and the possibility of a new clinico-pathologic entity is discussed.