[Amyotrophic lateral sclerosis with dementia (ALSD)].
Patients with ALSD show characteristic mental and behavioral changes, represented by lack of insight into their tragic condition. Psychiatric symptoms usually precede life-threatening motor neuron symptoms. Cerebral SPECT, especially 3D-SSP, exercises its power in the diagnosis of ALSD by demonstrating constant and sharp blood flow reduction in the prefrontal region. The neuropathology of the cerebral cortex is characterized by most prominent and probably earliest degeneration in the medial side cortex of the temporal pole, border zone between the CA1 and subiculum, ambient gyrus, and amygdala as well as 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 more prominent Bunina bodies and less affected pyramidal tract. The substantia nigra is usually 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 exhibited upper motor neuron-dominant involvement, showing the possibility that the category of ALSD may be widened than considered so far.
Nakano
Imaharu
I
Division of Neurology, Department of Medicine, Jichi Medical University School of Medicine.
D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D001921:Brain / Q000473:pathology; D003704:Dementia / Q000209:etiology* / Q000473:pathology; D006801:Humans; D009046:Motor Neurons / Q000473:pathology