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KMID : 0391520150230030130
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Journal of the Korean Child Neurology Society
2015 Volume.23 No. 3 p.130 ~ p.134
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Neuropsychiatric Systemic lupus Erythematosus Presenting as Bickerstaff’s Brainstem Encephalitis: a Case Report
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Choe Ha-Yeong
Cheon Kyeong-Ryeol
Song Tae-Yang
Jeong Ku-Mi
Park Soo-Min
Woo Young-Jong
Kim Young-Ok
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Abstract
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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 highdose
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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KEYWORD
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Lupus erythematosus, Systemic, Sjogren’s syndrome, Brainstem, encephalitis, Child
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