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

This Article 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 Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Rinne, J. O. Articles by Marsden, C. D. Search for Related Content PubMed PubMed Citation Articles by Rinne, J. O. Articles by Marsden, C. D.

Nigral degeneration in neuroacanthocytosis

University Department of Clinical Neurology (Drs. Rinne, Harding, and Marsden); the Parkinson's Disease Society Brain Bank (Dr. Daniel); and the Department of Neuropathology (Drs. Daniel and Scaravilli), Institute of Neurology, London, England; and the Department of Neurology (Dr. Rinne), University of Turku, Turku, Finland.

We counted nerve cells in different subregions of the substantia nigra in three patients with neuroacanthocytosis and compared the results with those of age-matched Parkinson's disease and control patients. Two patients with neuroacanthocytosis and clinical parkinsonism in life had a reduced neuronal density in the substantia nigra, while in a third patient without parkinsonism, this number was at the lower limit of the control range. In neuroacanthocytosis with parkinsonism and in Parkinson's disease, the ventrolateral region of the substantia nigra was most severely affected, although in neuroacanthocytosis cases, nigral neuronal loss was more widespread.

Address correspondence and reprint requests to Dr. C. David Marsden, University Department of Clinical Neurology, The National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, England.

J.O.R. is financially supported by grants from the Medical Research Council of the Academy of Finland and the Finnish Cultural Foundation. S.E.D. is supported by the Parkinson's Disease Society of UK.

Received November 10, 1993. Accepted in final form February 23, 1994.

This article has been cited by other articles:

				R. H. Walker, H. H. Jung, C. Dobson-Stone, L. Rampoldi, A. Sano, F. Tison, and A. Danek Neurologic phenotypes associated with acanthocytosis Neurology, January 9, 2007; 68(2): 92 - 98. [Abstract] [Full Text] [PDF]

				R. H. Walker, S. Morgello, B. Davidoff-Feldman, A. Melnick, M. J. Walsh, P. Shashidharan, and M. F. Brin Autosomal dominant chorea-acanthocytosis with polyglutamine-containing neuronal inclusions Neurology, April 9, 2002; 58(7): 1031 - 1037. [Abstract] [Full Text] [PDF]