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Extensive cerebral calcification in a patient with systemic lupus erythematosus R H Andres, G Schroth and L Remonda J. Neurol. Neurosurg. Psychiatry 2008;79;365doi:10.1136/jnnp.2007.125393
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Editorial commentaries and, on simple Mendelian principles, nonrecessive deleterious genes are apt to be strongly selected out of the population gene pool. This would be still be true for genes such as APOE-e4, the expression of which, in relation to Alzheimer’s disease, manifests itself of course later than usual reproductive age. Taken together, the Willemse-van Son et al and Hans et al. studies suggest that there may be conditions in which the APOE-e4 gene actually confers a compensatory beneficial effect thus accounting for its continuing presence in the human gene pool.
At all events the ‘‘received wisdom’’ of purely negative effects of the APOE-e4 gene would seem to be in need of revision and the action of the gene to be in need of further research.
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Competing interests: None. J Neurol Neurosurg Psychiatry 2008;79:364–365. doi:10.1136/jnnp.2007.138081
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Corder EH, Saunders AM, Strittmatter WJ, et al. Gene dose of apolipoprotein-e type-4 allele and the risk of alzheimers-disease in late-onset families. Science 1993;261:921–3.
Teasdale GM, Murray GD, Nicoll JAR. The association between APOE epsilon 4, age and outcome after head injury: a prospective cohort study. Brain 2005;128:2556–61. Willemse-van Son AHP, Ribbers GM, Hop WCJ, et al. Association between apolipoprotein-e4 and long-term outcome after traumatic brain injury. J Neurol Neurosurg Psychiatry 2008;79:426–30. Jennett B, Bond M. Assessment of outcome after severe brain-damage–practical scale. Lancet 1975;7905:480–4. Han SD, Drake AI, Cessante LM, et al. Apolipoprotein E and traumatic brain injury in a military population: evidence of a neuropsychological compensatory mechanism? J Neurol Neurosurg Psychiatry 2007;78:1103–8.
Neurological picture Extensive cerebral calcification in a patient with systemic lupus erythematosus 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. 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.2 3
Figure 1 Native axial CT scan showing extensive calcification, from left, of the brainstem, cerebellar hemispheres, basal ganglia and centrum semiovale.
R H Andres,1 G Schroth,2 L Remonda2 1 2
Department of Neurosurgery, University of Berne, Inselspital, Berne, Switzerland; Department of Neuroradiology, University of Berne, Inselspital, Berne, Switzerland
REFERENCES
Correspondence to: Robert H Andres, Department of Neurosurgery, University of Berne, Inselspital, CH-3010 Berne, Switzerland;
[email protected]
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Competing interests: None declared.
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J Neurol Neurosurg Psychiatry 2008;79:365. doi:10.1136/jnnp.2007.125393
J Neurol Neurosurg Psychiatry April 2008 Vol 79 No 4
West SG. Neuropsychiatric lupus. Rheum Dis Clin North Am 1994;20:129–58. Raymond AA, Zariah AA, Samad SA, et al. Brain calcification in patients with cerebral lupus. Lupus 1996;5:123–8. 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.
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