Juvenile systemic lupus erythematosus with primary neuropsychiatric presentation

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiple manifestations in several organs and systems. Neuropsychiatric manifestations can occur in 22-95% of paediatric cases, being much less frequent as an initial clinical event. We report a case of SLE, presenting primarily with neuropsychiatric symptoms. An African-descendant 7-year-old girl was admitted with a 4-day history of ataxia, diplopia and morning vomiting, as well as severe headache, psychiatric symptoms and cognitive dysfunction beginning 1 year prior to admission. Brain MRI was suggestive of encephalitis. Investigation excluded infectious aetiology. Immunological markers revealed high titre of antinuclear and anti-double-stranded DNA antibodies. Neuropsychiatric lupus (NPL) was considered, and cyclophosphamide and methylprednisolone pulses were started, with good initial response. Clinical deterioration motivated therapy with azathioprine with subsequent clinical stabilisation and a latent cognitive dysfunction. In unusual encephalitis presentation, a wide range of differential diagnosis has to be considered. Primary NPL presents difficult diagnostic and therapeutic challenges.

Figure 1

First brain MRI: multiple lesions of both cerebral hemispheres, with irregular borders and intermediate intensity on T2-weighted images, located at the grey matter–white matter junction, in both temporal lobes (with hippocampal involvement), right insula, right anterior superior frontal lobe and left frontal lobe; marked cerebellar atrophy. These aspects were described as ‘highly suggestive of encephalitis’.

Figure 2

Brain MRI after two pulses of methylprednisolone and cyclophosphamide: nearly resolution of all lesions; there is only an image of hyperintensity in the inner portion of both temporal lobes, involving the hippocampus, with complete resolution of all the other lesions.