Background. CNS lupus is a serious but potentially treatable illness which, though long recognised, 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 south-west of England and South Wales, covering the period 1990-2002. Results We found that primary neurological 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 ESR or plasma viscosity were elevated during neurological episodes while CRP 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 neurological deficits, and normal or show wholly non-specific 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-SLE, perhaps particularly in the elderly, and its management.
|Translated title of the contribution||CNS lupus - a study of 41 patients|
|Pages (from-to)||644 - 654|
|Number of pages||13|
|Publication status||Published - Aug 2007|