Neuropsychiatric manifestations in juvenile systemic lupus erythematodes

Introduction: Nervous system manifestations in systemic lupus erythematodes (SLE) can occur at any time of the disease and are described in up to 91% of adult cases. The prevalence in juvenile patients is not known. Cognitive dysfunction, mood disorders and headache are the most common problems followed by cerebovascular disease and seizures. Myelopathy and polyneuropathy are infrequent. We report on the spectrum of neuropsychiatric symptoms in 6 juvenile SLE patients.

Results: Over the past 6 years, 7 female patients with SLE have been treated in our institution. Neuropsychiatric symptoms, according to the nomenclature of the American College of Rheumatology 1999, were the presenting symptom in 2 patients at time of diagnosis. One had mononeuritis multiplex with paresis of ocular muscles, the other severe mood disorder. In another 2 patients neuropsychiatric symptoms occured during the first manifestation of the disease. The first had recurrent epileptic seizures, the other asymptomatic signs of vasculitis in the MRI. One patient developed choreatic movements during activ disease and later diffuse alterationes of the white matter in the cerebral MRI, an other girl had an episode with tinnitus and ischaemic retinopathy during remission which resolved after termination of cyclosporin A therapy. Only 1 patient with juvenile SLE was free of neuropsychiatric symptoms over a mean follow up of 4 years.

Conclusion: Patients with isolated neuropsychiatric symptoms may at first escape thorough clinical workup for systemic autoimmun disease.

Juvenile SLE is rare and multicenter studies are necessary to reveal the frequency of neuropsychiatric symptoms. The differential diagnosis to infections, side effects of the therapy, hypertonic or metabolic encephalopathy can be difficult.

Current therapies are empirical and include symptomatic, immunosuppressive and anticoagulant medications.