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This Article Figures Only Full Text Full Text (PDF) CME: Take the course for this article: Volume 69, Number 07, August 14, 2007 Correspondence: Submit a response Correspondence: View responses 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 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 Joseph, F. G. Articles by Scolding, N. J. Search for Related Content PubMed PubMed Citation Articles by Joseph, F. G. Articles by Scolding, N. J. Related Collections Autoimmune diseases Lupus

CNS lupus

A study of 41 patients

From the Institute of Clinical Neurosciences (F.G.J., N.J.S.), Frenchay Hospital, Bristol, and Department of Pathology (G.A.L.), University of Wales College of Medicine, Cardiff, UK.

Address correspondence and reprint requests to Dr Scolding, Institute of Clinical Neurosciences, Frenchay Hospital, Bristol, UK BS16 1LE n.j.scolding{at}bristol.ac.uk

Background: CNS lupus is a serious but potentially treatable illness, which, though long recognized, may still present very difficult diagnostic challenges. We believed that further detailed study of patients with neuropsychiatric lupus would yield clinical information of practical value in improving both recognition and management of this difficult illness.

Methods: A retrospective case analysis of 41 patients with CNS systemic lupus erythematosus (CNS-SLE) was performed largely in the southwest of England and South Wales, covering the period 1990 to 2002.

Results: We found that primary neurologic presentation of SLE was not rare (10/41 patients), and there was an unexpected emergence of movement disorders (particularly parkinsonism and myoclonus) early in the disease course (4/10 patients). These showed a good response to immunosuppressants, but not to standard dopaminergic therapy. Typically, the erythrocyte sedimentation rate (ESR) or plasma viscosity was elevated during neurologic episodes while C-reactive protein levels were normal, and lupus-related serum antibody tests usually supportive. But, significantly, neither a normal ESR nor negative serology excluded CNS lupus. MR brain imaging is more commonly abnormal in patients with focal neurologic deficits and normal or shows wholly nonspecific change with more diffuse manifestations (cognitive decline, epilepsy). Abnormal CSF correlated significantly with poorer outcome. At the end of the period of study, 54% had no more than minor functional disability, the remainder having a severe or fatal outcome.

Conclusions: Our observations, particularly the emergence of non-choreic movement disorders, the blood, serum, and imaging findings, and the prognostic importance of CSF abnormalities, should help improve both the recognition of CNS systemic lupus erythematosus, perhaps particularly in elderly individuals, and its management.

Fady Joseph was supported by the Neurological Research Fund established by Dr. M.J. Campbell.

Disclosure: The authors report no conflicts of interest.

Received January 1, 2007. Accepted in final form March 12, 2007.

This article has been cited by other articles:

				E. Avalos Herrera, S. Y. Alvarez, C. E. Pacay, N. Scolding, and F. Joseph CNS LUPUS: A STUDY OF 41 PATIENTS Neurology, April 29, 2008; 70(18): 1648 - 1649. [Full Text] [PDF]

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