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NEUROLOGY 1984;34:1096
© 1984 American Academy of Neurology

Familial amyloid polyneuropathy

A new kinship of German ancestry Carolyn Riester O'Connor, MD, Alan Rubinow, MD, Sydney Brandwein, MD and Alan S. Cohen, MD

From the Arthritis and Connective Tissue Disease Section, the Thorndike Memorial Laboratory and Division of Medicine, Boston City Hospital, Boston University School of Medicine, Boston, MA.

Familial amyloid polyneuropathy (FAP) is an autosoma1 dominant inherited disorder that primarily affects the peripheral and autonomic nervous systems and usually becomes symptomatic in the third or fourth decade. Because of the confusion with other genetically transmitted neurologic conditions, the diagnosis is often delayed until advanced stages. We describe a new German kinship with FAP in which the disorder was detected in two asymptomatic family members who were seeking genetic counseling.

Address correspondence and reprint requests to Dr. Cohen, Arthritis Center, Boston University School of Medicine, 71 East Concord Street, Boston, MA 02118.

Supported by grants from the United States Public Health Service; National Institute of Arthritis, Metabolism and Digestive Diseases (AM 04599 and AM 07014); National Institute of Health Multipurpose Arthritis Center (AM 20613): the General Clinical Research Centers Branch of the Divisions of Research Resources, National Institutes of Health (RR 533): and the Arthritis Foundation.

This material was presented in part at the regional meeting of the American Rheumatism Association, Boston. October 24, 1982.

Accepted for publication December 6, 1983.