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Dementia with Leukoencephalopathy in Systemic Lupus Erythematosus

Published online by Cambridge University Press:  18 September 2015

Andrew Kirk ,
Andrew Kertesz  and
Marsha J. Polk
Andrew Kirk*
Affiliation:
Department of Clinical Neurological Sciences, Lawson Research Institute, St. Joseph's Health Centre, University of Western Ontario, London
Andrew Kertesz
Affiliation:
Department of Clinical Neurological Sciences, Lawson Research Institute, St. Joseph's Health Centre, University of Western Ontario, London
Marsha J. Polk
Affiliation:
Department of Clinical Neurological Sciences, Lawson Research Institute, St. Joseph's Health Centre, University of Western Ontario, London
*
Department of Medicine (Neurology), Royal University Hospital, Saskatoon, Saskatchewan, Canada S7N 0X0
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Abstract:

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Neurologic manifestations, afflicting up to 70% of SLE patients, include psychosis, seizures, chorea, neuropathies, and stroke. MRI is useful in evaluation of lupus patients and several reports have documented cerebral atrophy or focal hyperintensities. We report an unusual MRI appearance in a 56-year-old woman with SLE, diagnosed on the basis of pleuritis, lymphopenia, anti-DNA antibodies, and neurologic involvement. She reported recent onset of Raynaud's phenomenon and generalized macular rash. She presented after two months of gradual deterioration with memory loss, flattened affect, dysphagia, dysarthria, anomia, and somnolence, without focal neurologic signs. Investigations included elevated ESR, reduced complement, normal CSF without oligoclonal bands, negative viral serology, normal hormone and vitamin levels, normal renal and hepatic function. Neuropsychologic testing showed widespread impairment (WAIS-R: FSIQ-63; WMS-69; DRS-98; RCPM-14; WAB AQ-78.8). CT was normal but MRI showed strikingly symmetric, confluent hyperintensities extensively involving cerebral and cerebellar white matter on Tl and T2 weighted scans. Basal ganglia and subependymal and subcortical white matter were spared. Treated with prednisone, the patient made a gradual, but incomplete, recovery. These MRI findings may reflect widespread vasculopathy or direct immunologic brain insult with or without imunologic blood-brain barrier disruption.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 18 , Issue 3 , August 1991 , pp. 344 - 348
Copyright
Copyright © Canadian Neurological Sciences Federation 1991

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