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Rapidly progressive aphasic dementia and motor neuron disease
Author(s) -
Caselli Richard J.,
Windebank Anthony J.,
Petersen Ronald C.,
Komori Takashi,
Parisi Joseph E.,
Okazaki Haruo,
Kokmen Emre,
Iverson Rita,
Dinapoli Robert P.,
GraffRadford Neill R.,
Stein Steven D.
Publication year - 1993
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410330210
Subject(s) - gliosis , dementia , atrophy , aphasia , medicine , neuroscience , magnetic resonance imaging , anterior horn cell , motor neuron , upper motor neuron , amyotrophic lateral sclerosis , disease , psychology , pathology , radiology
Articulatory and language impairment heralded rapidly progressive motor neuron disease in 7 patients aged 54 to 77 years. One patient had a family history of a similar disorder. Severe nonfluent aphasia developed in all 7 patients and 4 were anarthric within a year. Other cognitive domains were impaired, yet 2 patients lived alone until 1 month before their deaths. Four died within 2 years. Abnormalities were found on electromyography, computed tomography, magnetic resonance imaging, single‐photon emission computed tomography, and electroencephalography. Neuropathological examination in 3 patients showed bilateral hemispheric atrophy with neuronal loss and gliosis predominantly of superficial cortical layers. Pigmented and hypoglossal nuclei were relatively preserved. At all spinal levels there was degeneration of corticospinal tracts and loss of anterior horn cells with gliosis. Rapidly progressive aphasic dementia and motor neuron disease are a distinctive clinical entity whose nosology is poorly understood.