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Neurology 2000;55:1584-1586
© 2000 American Academy of Neurology


Brief Communications

Ataxia with vitamin E deficiency: Biochemical effects of malcompliance with vitamin E therapy

M. Schuelke, MD, B. Finckh, PhD, E. A. Sistermans, PhD, M. G. E. M. Ausems, MD, C. Hübner, MD and A. von Moers, MD

From the Department of Neuropediatrics (Drs. Schuelke, Hübner, and von Moers), Charité, Humboldt University, Berlin and the Children’s Hospital (Dr. Finckh), University of Hamburg, Germany; and the Department of Human Genetics (Dr. Sistermans), University Medical Center Nijmegen and the Department of Medical Genetics (Dr. Ausems), University Medical Center Utrecht, the Netherlands.
From the Neurology Unit (Drs. G. Modi, Martinus, and Saffer), Department of Medicine, and the Department of Radiology (Dr. M. Modi), Chris Hani Baragwanath Hospital, and the Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
From the Department of Neurology, Klinikum Grosshadern, Ludwig-Maximillians-University, Munich, Germany.
From the Departments of Neurology (Drs. E.M. Frohman, Zhang, Dewey, Hawker, Racke, and T.C. Frohman) and Ophthalmology (Dr. E.M. Frohman) and the Center for Immunology (Dr. Racke), University of Texas Southwestern Medical Center, Dallas.
From the Department of Neurology, Haukeland University Hospital (Drs. Myhr, Nyland, and Vedeler), University of Bergen, Norway; and the Institute for Inflammation Research (Drs. Ross and Bendtzen), Rigshospitalet University Hospital, Copenhagen, Denmark.
From the Departments of Neurological Sciences (Drs. Kompoliti, Comella, Goetz, and J.A. Jaglin) and Preventive Medicine (Dr. Leurgans and R. Raman), Rush–Presbyterian–St Luke’s Medical Center, Rush University, Chicago, IL.
From the Department of Geriatric Medicine (Drs. Hu, Okamura, Arai, Higuchi, Matsui, Tashiro, Shinkawa, and Sasaki), Tohoku University School of Medicine, and the Cyclotron and Radioisotope Center (Drs. Itoh and Ido), Tohoku University, Sendai, Miyagi, Japan.
From INSERM EPI 9906 (Drs. Raux, Gantier, Hannequin, Frebourg, and Campion), Rouen; CHU Bellevue (Dr. Thomas–Anterion), St. Etienne; CHR Bourg en Bresse (Dr. Boulliat); and INSERM U 289 (Drs. Verpillat and Brice), Paris, France.
From the Departments of Neuropathology (Dr. Ince) and Neurology (Drs. Duffey and Shaw), University of Newcastle upon Tyne; Department of Pathology (Dr. Cochrane), Sunderland General Hospital; and the Department of Neuropathology (Dr. Lowe), Queens Medical Centre, Nottingham, United Kingdom.
From the Biopsychology Subprogram (Rev. Murphy and Dr. Weber), Hunter College, the Graduate School and University Center of the City of New York; and the Department of Neurology (Drs. Rowland, Albert, and Weber, and Rev. Murphy and M.L. Del Bene), the Eleanor and Lou Gehrig MDA/ALS Center (Drs. Albert and Rowland, Rev. Murphy, and M.L. Del Bene), and the Gertrude H. Sergievsky Center (Drs. Albert and Weber), Columbia University, New York, NY.

Address correspondence and reprint requests to Rev. Peregrine L. Murphy, Hunter College, Department of Psychology, 695 Park Avenue HN 611, New York, NY 10021.

To prevent neuronal damage, patients with ataxia with isolated vitamin E deficiency need lifelong supplementation with high doses of vitamin E. Short interruptions of therapy, such as occur in malcompliance, do not lead to clinical symptoms. However, the authors show that even short withdrawals may cause a prolonged decrease of the total radical trapping capacity of plasma; its major contributors, such as urate and sulfhydryl groups, fail to compensate for the missing vitamin E.–1586




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B. N Ames, I. Elson-Schwab, and E. A Silver
High-dose vitamin therapy stimulates variant enzymes with decreased coenzyme binding affinity (increased Km): relevance to genetic disease and polymorphisms
Am. J. Clinical Nutrition, April 1, 2002; 75(4): 616 - 658.
[Abstract] [Full Text] [PDF]




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