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Most cases of dementia with hippocampal sclerosis may represent frontotemporal dementia

From the Department of Pathology (Drs. Hatanpaa, Bigio, and White), University of Texas Southwestern Medical School, Dallas, and Departments of Psychiatry and Behavioral Sciences (Dr. Blass), Pathology (Drs. Pletnikova, Crain, Hedreen, and Troncoso), and Neurology (Drs. Crain and Troncoso), Johns Hopkins University School of Medicine, Baltimore, MD.

Address correspondence and reprint requests to Dr. K.J. Hatanpaa, Department of Pathology, UT Southwestern Medical School, 5323 Harry Hines Blvd., Rm. H2.130, Dallas, TX 75390-9073; e-mail: Kimmo.Hatanpaa{at}UTSouthwestern.edu

Hippocampal sclerosis dementia (HSD) is a disease of unknown etiology and pathogenesis. To determine whether HSD cases could be reclassified as variants of frontotemporal dementia (FTD), a heterogeneous group of disorders, 18 brain autopsy cases previously diagnosed as HSD were re-evaluated. In 11 cases, ubiquitinated neuronal inclusions, similar to those of motor neuron disease inclusion dementia (MNDID), were found. Brain levels of soluble and insoluble tau were normal. Most patients with pathologic findings of HSD may actually have the MNDID variant of FTD.

Received August 22, 2003. Accepted in final form February 11, 2004.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the August 10 issue to find the title link for this article.

See also pages 414 and 492

The current address of Dr. Bigio is Northwestern University Feinberg School of Medicine, Chicago, IL. The current address of Dr. Hedreen is Harvard University School of Medicine, Belmont, MA.

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