Atypical amyotrophic lateral sclerosis with dementia mimicking frontal Pick's disease: a report of an autopsy case with a clinical course of 15 years

Citation
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
Citations number
24
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Neurosciences & Behavoir
Journal title
ACTA NEUROPATHOLOGICA
ISSN journal
0001-6322 → ACNP
Volume
101
Issue
6
Year of publication
2001
Pages
625 - 630
Database
ISI
SICI code
0001-6322(200106)101:6<625:AALSWD>2.0.ZU;2-B
Abstract
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.