Neuropsychiatric Systemic lupus Erythematosus Presenting as Bickerstaff’s Brainstem Encephalitis: a Case Report
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Neuropsychiatric events are commonly reported in patients with systemic lupus erythematosus and they are an important contributor to both morbidity and mortality. Neuropsychiatric systemic lupus erythematosus (NPSLE) mainly involves the central nervous system, and rarely invades the peripheral nervous system. A 12-year-old girl presented with sudden-onset motor weakness in her lower extremities and face, as well as drowsiness. Brain magnetic resonance imaging and angiography produced no abnormal findings, and a cerebrospinal fluid examination revealed only pleocytosis. Reticular erythema in the extremities, oral ulcers, and a dry mouth were noted at her first visit, but a malar rash appeared on the third day with positivity for serum antinuclear and anti-dsDNA antibodies. Therefore a high- dose steroid was administered based on a diagnosis of NPSLE. An ophthalmologic examination, salivary gland scan, and biopsy of minor salivary gland resulted in the additional diagnosis of Sjogren’s syndrome. The reported patient represents a rare pediatric case of NPSLE accompanied with Sjogren’s syndrome, presenting as Bickerstaff’s brainstem encephalitis and being successfully treated with early steroid pulse therapy.
Abstract
Neuropsychiatric events are commonly reported in patients with systemic lupus erythematosus and they are an important contributor to both morbidity and mortality. Neuropsychiatric systemic lupus erythematosus (NPSLE) mainly involves the central nervous system, and rarely invades the peripheral nervous system. A 12-year-old girl presented with sudden-onset motor weakness in her lower extremities and face, as well as drowsiness. Brain magnetic resonance imaging and angiography produced no abnormal findings, and a cerebrospinal fluid examination revealed only pleocytosis. Reticular erythema in the extremities, oral ulcers, and a dry mouth were noted at her first visit, but a malar rash appeared on the third day with positivity for serum antinuclear and anti-dsDNA antibodies. Therefore a high- dose steroid was administered based on a diagnosis of NPSLE. An ophthalmologic examination, salivary gland scan, and biopsy of minor salivary gland resulted in the additional diagnosis of Sjogren’s syndrome. The reported patient represents a rare pediatric case of NPSLE accompanied with Sjogren’s syndrome, presenting as Bickerstaff’s brainstem encephalitis and being successfully treated with early steroid pulse therapy.
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