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NEUROLOGY 2005;65:1636-1638
© 2005 American Academy of Neurology
Brief Communications

Similar brain tau pathology in DM2/PROMM and DM1/Steinert disease

C. A. Maurage, MD, PhD, B. Udd, MD, PhD, M. M. Ruchoux, MD, PhD, P. Vermersch, MD, PhD, H. Kalimo, MD, R. Krahe, PhD, A. Delacourte, PhD and N. Sergeant, PhD

From INSERM U422 (Drs. Maurage, Delacourte, and Sergeant), Faculté de Médecine, Lille; Département d’Anatomie Pathologique (Drs. Maurage and Ruchoux), CHRU, and EA2691, Faculté de Médecine, Lille; Department of Neurology (Dr. Udd), Vaasa Central Hospital, and the Folkhalsan Institute of Genetics, University of Helsinki, Finland; Service de Neurologie D (Dr. Vermersch), CHRU and Faculté de Médecine, Lille, France; Karolinska Institute (Dr. Kalimo), Stockholm, Sweden; and Section of Cancer Genetics (Dr. Krahe), Department of Molecular Genetics, University of Texas M.D. Anderson Cancer Center, Houston, TX.

Address correspondence and reprint requests to Dr Maurage, INSERM U422, Faculté de Médecine, place Verdun, F-59045 Lille cedex, France; e-mail: ca-maurage{at}chru-lille.fr

Neurofibrillary degeneration (NFD) occurs in the brains of patients with myotonic dystrophy (DM) type 1. The authors report a similar tau pathology in the CNS of a patient with DM2 and compare it to that of patients with DM1. A reduced expression of tau exon 2 and exon 3 epitopes is observed in both DM1 and DM2. This suggests a similar physiopathologic process that may contribute to common neurologic features in patients with DM.

Supported by INSERM and AFM, by grants from EA 2691 MENRT, the Liv and Halsa Foundation in Finland, and the Research Funds of the Vasa Central Hospital.

Disclosure: The authors report no conflicts of interest.

Received July 27, 2004. Accepted in final form August 4, 2005.






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