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

This Article Figures Only Full Text 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 Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Shafiq, M. Articles by Lippa, C.F. Search for Related Content PubMed Articles by Shafiq, M. Articles by Lippa, C.F.

Frontotemporal dementia: Report of a familial case

From the Department of Neurology (Drs. Shafiq and Lippa), MCP–Hahnemann University, Philadelphia, PA; Center for Neurodegenerative Disease Research (Drs. Lee and Trojanowski), Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA; and the National Institute of Neurological Disorders and Stroke (Drs. Grafman and Tresser and L. Nee), Bethesda, MD.

Address correspondence and reprint requests to Dr. Carol F. Lippa, Department of Neurology, MCP–Hahnemann University, Philadelphia, PA 19129; e-mail: carol.lippa{at}drexel.edu

The authors describe a 49-year-old woman (R.K.) who presented with one year of progressive frontal lobe dysfunction, including signs of expressive aphasia. Signs of parkinsonism were absent until late in the clinical course. Neuropsychologic testing and neuroimaging studies are described. The patient died at age 55, after 7 years of symptoms. Family history was remarkable for a mother who died at the age of 45, after experiencing 7 years of progressive aphasia. R.K.’s brain showed asymmetric frontotemporal atrophy, which was more severe on the left side. Histopathologic analysis was remarkable for numerous tau-positive neurons with some classic-appearing Pick bodies and many ballooned neurons. Tau-positive glial cells were also present. The authors suggest that the abnormal tau aggregates are related to the symptoms experienced by affected members of this family.