Rapidly progressive aphasic dementia and motor neuron disease

Richard J. Caselli, Anthony J. Windebank, Ronald C. Petersen, Takashi Komori, Joseph E. Parisi, Haruo Okazaki, Emre Kokmen, Rita Iverson, Robert P. Dinapoli, Neill R. Graff‐Radford, Steven D. Stein

Research output: Contribution to journalArticlepeer-review

203 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)200-207
Number of pages8
JournalAnnals of neurology
Issue number2
StatePublished - Feb 1993

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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