K. Tsuchiya et al., Atypical amyotrophic lateral sclerosis with dementia mimicking frontal Pick's disease: a report of an autopsy case with a clinical course of 15 years, ACT NEUROP, 101(6), 2001, pp. 625-630
This report concerns an autopsy case of atypical amyotrophic lateral sclero
sis (ALS) with dementia mimicking frontal Pick's disease. The patient was a
Japanese woman without hereditary burden who was 45 years old at the time
of death. She developed abnormal behavior and amnesia at age 30, followed b
y disinhibition, aspontaneity, urinary incontinence, abulia, and rectal inc
ontinence. Neurological signs compatible with ALS developed about 14 years
after the disease onset. No respirator was used throughout the clinical cou
rse. Macroscopically, neuropathological examination showed atrophy of the f
rontotemporal lobes with accentuation in the convexities of the frontal lob
es. Histologically, there was neuronal loss in the cerebral cortex, parahip
pocampal gyrus, amygdala, caudate nucleus, substantia nigra, brain stem mot
or nuclei, and anterior horns of the spinal cord, in addition to marked deg
eneration of the pyramidal tracts. Ubiquitin-immunoreactive neuronal inclus
ions were present in the frontotemporal cortical layer II neurons and motor
neurons in the brain stem and spinal cord. In the hippocampal dentate gran
ular cells, many ubiquitin-immunoreactive neurites were present without ubi
quitin-immunoreactive intraneuronal inclusions. Based on these clinicopatho
logical findings and a review of the literature, we concluded that our case
was atypical ALS with dementia of long disease duration. We also note the
possibility that motor neuron disease-inclusion dementia with a long clinic
al course may develop into ALS in the final stage of the illness.