The Japanese journal of neuropsychology

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ArticleTitle	Neuropathology of amyotrophic lateral sclerosis with dementia
Language	J
AuthorList	Imaharu Nakano
Affiliation	Division of Neurology, Department of Medicine, Jichi Medical University
Publication	Japanese Journal of Neuropsychology: 22 (3), 171-177, 2006
Received
Accepted
Abstract	Patients with amyotrophic lateral sclerosis with dementia (ALSD) show characteristic mental and behavioral changes, represented by lack of insight into their tragic condition. Psychiatric symptoms usually precede motor neuron symptoms, which determine the life prognosis of the patients. In this condition, the neuropathology is classified as cerebral pathology and motoneuron one. The former is usually emphasized and probably earliest observed in the medial side cortex of the temporal pole, border zone between the CA1 and subiculum, ambient gyrus and entorhinal area, and amygdala. Another finding unique to the cerebral pathology of ALSD is cytoplasmic ubiquitinated inclusion bodies in the dentate gyrus granular neurons and other cortical small neurons. Motoneuron pathology is almost the same as that in classic ALS except for a tendency for Bunina bodies to be more prominent and for the pyramidal tract to be less affected in this condition. The substantia nigra is more or less degenerated without Lewy bodies. A condition recently proposed as motor neuron disease-inclusion dementia seems to be a forme froste of ALSD. Several cases of ALSD with upper motor neuron-dominant involvement have been also reported, showing the possibility that extension of ALSD will be widened than considered so far.
Keywords	amyotrophic lateral sclerosis, neuropathology, temporal lobe change, CA1-subiculum transitional zone lesion, ubiquitinated inclusion