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Arch Neurol. 2008 Jul;65(7):971-3. doi: 10.1001/archneur.65.7.971.

Sporadic fatal insomnia masquerading as a paraneoplastic cerebellar syndrome.

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  • 1Neuroimmunology Unit, Department of Neurology, University of Rochester Medical Center, 601 Elmwood Ave, Box 605, Rochester, NY 14642, USA. lahar_mehta@urmc.rochester.edu

Abstract

BACKGROUND:

Sporadic fatal insomnia is a rare prion disease that has recently been recognized.

OBJECTIVE:

To report a unique case of sporadic fatal insomnia in a woman with progressive cerebellar deterioration who was originally thought to have a paraneoplastic cerebellar syndrome.

DESIGN:

Case report describing a patient with autopsy-proven sporadic fatal insomnia.

PATIENT:

A 56-year-old woman with progressive cerebellar ataxia who was found to have a retroperitoneal non-Hodgkin lymphoma.

RESULTS:

Autopsy demonstrated marked degenerative changes in the thalamus, cerebellum, and inferior olivary nucleus. A mild spongiform change was present in the thalamus and cortical gray matter. Western blot analysis confirmed the presence of abnormal, protease-resistant prion protein (PrP(Sc)), characteristic of sporadic fatal insomnia.

CONCLUSIONS:

Clinicians should be aware of this rare prion disease and should strongly consider the importance of autopsy toward the investigation of unusual neurological diseases.

PMID:
18625868
[PubMed - indexed for MEDLINE]
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