[Skip to Navigation]
January 1974

Amyotrophic Lateral Sclerosis With Diffuse Neurofibrillary Changes: Report of a Case

Author Affiliations

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.

Arch Neurol. 1974;30(1):84-89. doi:10.1001/archneur.1974.00490310086013

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.

Add or change institution