This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Report of a Case

Karl R. Meyers, MD; Donald G. Dorencamp, MD; Kinuko Suzuki, MD

Arch Neurol. 1974;30(1):84-89.

Abstract
A 41-year-old male former boxer had amyotrophic lateral sclerosis (ALS) for about eight years prior to his death from bronchopneumonia. Neuropathologic examination revealed, in addition to the classical changes of ALS, numerous neurofibrillary changes (tangles) of the neurons in the cerebrum and brain stem. The tangles were most prominent in Ammon horn, amygdaloid nuclei, and locus caeruleus. Other findings included a moderate degree of neuronal loss and gliosis of the substantia nigra. Granulovacuolar degeneration and Hirano bodies were seen in the neurons of Ammon horn. Senile plaques were not found. The distribution of the tangles resembled that reported in Guamanian ALS. Ultrastructurally, the intracytoplasmic fibrils had a twisted configuration and were similar to those described in Alzheimer disease and Guamanian ALS.

The presence and distribution of the neurofibrillary changes in a patient with ALS could well be coincidental; however, the occurrence of the Guamanian type of ALS in the northeastern population of the United States is worth reporting in view of the etiologic and epidemiologic considerations of this form of ALS.

Author Affiliations
Philadelphia

From the Electron Microscopy Laboratory, Division of Anatomic Pathology, and Neurology Section, Veterans Administration Hospital, and the Department of Neurology, Medical College of Pennsylvania (Dr. Dorencamp); and the Division of Neuropathology, departments of pathology and neurology, University of Pennsylvania School of Medicine (Drs. Meyer and Suzuki), Philadelphia. Dr. Meyers is now with the National Board of Medical Examiners, Philadelphia. Dr. Suzuki is now with the Department of Pathology, Albert Einstein College of Medicine, Bronx, NY.

Footnotes
Accepted for publication Aug 1, 1973.

Reprint requests to Department of Pathology, Albert Einstein College of Medicine, 1300 Morris Park Ave, Bronx, NY 10461.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

The ALS/PDC syndrome of Guam and the cycad hypothesis
Steele and McGeer
Neurology 2008;70:1984-1990.
ABSTRACT | FULL TEXT