Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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September 3, 2015
Brain Calcification in Systemic Lupus Erythematosus (SLE)
- Background:
- Brain calcifications have been reported in SLE with neuropsychiatric manifestations. The most frequent location involved is the basal ganglia, with less frequent involvement of the cerebral white matter and cerebellum.
- The underlying mechanism of calcification is unknown, but may be dystrophic following microinfarctions due to primary vascular damage and ongoing venous inflammation.
- Clinical Presentation: CNS manifestations of SLE including seizures or focal neurological deficits, occurring in ~20% of patients.
- Key Diagnostic Features:
- Cerebral atrophy is the most common finding on cranial CT. Basal ganglia calcification is present in up to 25% of cases.
- MRI shows cerebral involvement in approximately 70% of patients with SLE. The most frequent imaging finding in SLE is multifocal high-signal intensities on FLAIR and T2WI in the subcortical white matter. SLE also can present with diffuse white matter abnormality, infarcts, cortical atrophy, cerebral edema, intracranial hemorrhage, and calcifications.
- DDx:
- Bilateral striopallidodentate calcinosis (Fahr disease)
- Hyperparathyroidism
- (Pseudo)hypoparathyroidism
- Lead intoxication
- AIDS
- Radiation therapy
- Rx: Mainstay of treatment for SLE is immunosuppressive agents, including steroids.
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