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Extensive cerebral calcification in a patient with systemic lupus erythematosus

R H Andres¹, G Schroth², L Remonda²

¹ Department of Neurosurgery, University of Berne, Inselspital, Berne, Switzerland
² Department of Neuroradiology, University of Berne, Inselspital, Berne, Switzerland

Correspondence to:
robert.andres{at}neurochirurgie-bern.ch

A 53-year-old woman with a 20-year history of systemic lupus erythematosus (SLE) with antiphospholipid antibodies presented with psychotic episodes, progressive depression, insomnia, psychomotor slowing, gait disturbance and tremor of both hands. Neurological examination revealed rigidity of the upper extremities, ataxia, tetrahyperreflexia and bilateral pyramidal tract signs. A cranial CT scan showed extensive calcification of the basal ganglia, centrum semiovale, cerebellar hemispheres and brainstem without contrast enhancement (fig 1). Bilateral F-18 fluorodeoxyglucose positron emission tomography hypometabolism, mainly of the parieto-occipital and temporal regions, confirmed the diagnosis of central nervous system lupus. Despite high-dose immunosuppressive therapy, the patient’s condition was progressively deteriorating and she required long-term psychiatric care.

View this figure (90K): in this window | in a new window | PowerPoint for Teaching   Figure 1 Native axial CT scan showing extensive calcification, from left, of the brainstem, cerebellar hemispheres, basal ganglia and centrum semiovale.

 

Cerebral manifestations are found in about 20% of patients with SLE.¹ Cerebral calcification is present in about 30% of these cases and does not necessarily correlate with the severity of neuropsychiatric symptoms.² The pathogenetic mechanism of cerebral calcification in SLE is unknown. Although the globus pallidus seems to be affected in all patients with SLE with cerebral calcification, involvement of other brain regions such as the putamen, the head of the caudate nucleus, the thalamus, the centrum semiovale and the cerebellum has been found to be less common.² Extensive calcifications of multiple brain areas, as reported in this case, are rare.²³

This article has been adapted from Andres R H, Schroth G, Remonda L. Extensive cerebral calcification in a patient with systemic lupus erythematosus Journal of Neurology, Neurosurgery and Psychiatry 2008;79:365

Competing interests: None declared.

1. West, SG. Neuropsychiatric lupus. Rheum Dis Clin North Am 1994;20:129–58.[Medline]
2. Raymond, AA, Zariah, AA, Samad, SA, et al. Brain calcification in patients with cerebral lupus. Lupus 1996;5:123–8.[Abstract/Free Full Text]
3. Garcia Raya, P, Gil Aguado, A, Simon Merlo, MJ, et al. Massive cerebral calcification in systemic lupus erythematosus: report of an unusual case. Lupus 1994;3:133–5.[Abstract/Free Full Text]

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