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NEUROLOGY 1989;39:58
© 1989 American Academy of Neurology

Thalamic dementia and motor neuron disease

F. Deymeer, MD, T. W. Smith, MD, U. DeGirolami, MD and D. A. Drachman, MD

Department of Pathology (Neuropathology), University of Massachusetts Medical Center, Worcester, MA. (Drs. Deymeer, Smith, and DeGirolami)
Department of and Neurology, University of Massachusetts Medical Center, Worcester, MA. (Drs. Deymeer, Smith, DeGirolami, and Drachman)

A 46-year-old woman developed a progressive neurologic disorder over the course of 30 months which was characterized by profound dementia complicated by a motor neuron disorder that became evident 10 months prior to death. Postmortem examination of the nervous system disclosed extensive neuronal loss and gliosis of the thalamus, predominantly involving the dorsomedial nuclei, as well as severe degeneration of the corticospinal tracts, spinal anterior horns, and hypoglossal nuclei. The disease could not be transmitted to experimental animals by intracerebral inoculation of the patient's brain tissue. This case represents a unique dementing disorder, possibly familial, with associated motor neuron disease.

Address correspondence and reprint requests to Dr. Smith, Department of Pathology (Neuropathology), University of Massachusetts Medical Center, 55 Lake Avenue North, Worcester, MA 01655.

Presented in part at the thirty-fourth annual meeting of the American Academy of Neurology, Washington, DC, April 1982.

Received March 18, 1988. Accepted for publication in final form July 5, 1988.




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