TY - JOUR
T1 - Central nervous system Sjögren's syndrome in a child
T2 - Case report and review of the literature
AU - Gottfried, Jay A.
AU - Finkel, Terri H.
AU - Hunter, Jill V.
AU - Carpentieri, David F.
AU - Finkel, Richard S.
PY - 2001
Y1 - 2001
N2 - We describe a case of pediatric Sjögren's syndrome with progressive neurologic involvement. At age 4 years, she had been diagnosed with Melkersson-Rosenthal syndrome. After being stable with facial diplegia and swelling for 5 years, she acutely presented with diplopia, vertigo, and ataxia. Cranial magnetic resonance imaging (MRI) showed a left dorsal midbrain lesion. Serologic and histopathologic findings confirmed primary Sjögren's syndrome. She responded well to intravenous methylprednisolone, with subsequent clinical improvement and MRI resolution. This report reviews the pediatric literature and underscores the importance of considering Sjögren's syndrome in a child with unexplained facial weakness and in the differential diagnosis of pediatric stroke.
AB - We describe a case of pediatric Sjögren's syndrome with progressive neurologic involvement. At age 4 years, she had been diagnosed with Melkersson-Rosenthal syndrome. After being stable with facial diplegia and swelling for 5 years, she acutely presented with diplopia, vertigo, and ataxia. Cranial magnetic resonance imaging (MRI) showed a left dorsal midbrain lesion. Serologic and histopathologic findings confirmed primary Sjögren's syndrome. She responded well to intravenous methylprednisolone, with subsequent clinical improvement and MRI resolution. This report reviews the pediatric literature and underscores the importance of considering Sjögren's syndrome in a child with unexplained facial weakness and in the differential diagnosis of pediatric stroke.
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U2 - 10.1177/088307380101600911
DO - 10.1177/088307380101600911
M3 - Article
C2 - 11575610
AN - SCOPUS:0034775273
SN - 0883-0738
VL - 16
SP - 683
EP - 685
JO - Journal of child neurology
JF - Journal of child neurology
IS - 9
ER -