HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Pearlman, R. L. Articles by Turel, A. P. Search for Related Content PubMed PubMed Citation Articles by Pearlman, R. L. Articles by Turel, A. P.

Clinical significance of types of cerebellar amyloid plaques in human spongiform encephalopathies

Division of Neurology, The Pennsylvania State University College of Medicine, Hershey, PA (Drs. Pearlman and Tenser)
Department of Pathology, The Pennsylvania State University College of Medicine, Hershey, PA (Dr. Towfighi)
Armed Forces Institute of Pathology, Washington, DC (Dr. Pezeshkpour)
Department of Neurology, Geisinger Medical Center, Danville, PA (Dr. Turel).

We report three patients with both spongiform encephalopathy and cerebellar amyloid plaques; one showed kuru-like plaques and was diagnosed as having Creutzfeldt-Jakob disease (CJD), and two had multicentric plaques and were diagnosed as having Gerstmann-Sträussler-Scheinker disease (GSSD). Evaluation of these cases and review of others previously reported suggests a clinicopathologic correlation between type of cerebellar plaque and neurologic clinical course. CJD patients who showed kuru-like plaques generally had disease with early onset (average age, 49.1 years) and long duration (average, 34 months), as compared with CJD patients without kuru-like plaques. GSSD patients usually had multicentric cerebellar plaques, and cases were usually familial, had early age of onset (average, 42.7 years), and were of long duration (average, 73 months). Myoclonus was infrequent in GSSD patients and pathologically spongiform change was minimal; spinal tract degeneration was common.

Address correspondence and reprint requests to Dr. Tenser, Division of Neurology, The Pennsylvania State University College of Medicine, Hershey, PA 17033.

Received August 13, 1987. Accepted for publication in final form December 14, 1987.

This article has been cited by other articles:

				Institute of Food Science & Technology (UK) BSE - Bovine Spongiform Encephalopathy ('Mad Cow Disease') Perspectives in Public Health, October 1, 1996; 116(5): 322 - 333. [Abstract]