Authors: Yuichiro Ii Akihiro Shindo Ryogen Sasaki Yutaka Naito Keiko Tanaka Shigeki Kuzuhara
Publish Date: 2008/05/21
Volume: 28, Issue: 12, Pages: 1277-1280
Abstract
We report a 49yearold woman with neuromyelitis optica NMO spectrum disorder coexisting with Sjögren’s syndrome SS She presented with acute brainstem symptoms and transverse myelitis Brain MRI showed focal high signal intensity lesions in the hypothalamus and the pontine tegmentum on T2weighted and FLAIR images MRA revealed stenotic changes of the bilateral middle cerebral artery MCA posterior cerebral arteries PCA and basilar artery BA Spinal MRI revealed hyperintense lesions within the cord extending from the T4 to the T6 level on the T2weighted image The patient fulfilled the clinical criteria of primary SS In addition antiAQP4 antibody which is highly specific for NMO was detected in the serum at the acute phase The patient excellently responded to IVIg while methylprednisolon pulse therapy was not effective Followup MRA displayed complete resolution of the stenosis of the MCA PCA and BA
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