Reversible stenosis of large cerebral arteries in a patient with combined Sjögren’s syndrome and neuromyelitis optica spectrum disorder
We report a 49-year-old 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 T2-weigh...
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| Veröffentlicht in: | Rheumatology international 2008-10, Vol.28 (12), p.1277-1280 |
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| creator | Ii, Yuichiro Shindo, Akihiro Sasaki, Ryogen Naito, Yutaka Tanaka, Keiko Kuzuhara, Shigeki |
| description | We report a 49-year-old 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 T2-weighted 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 T2-weighted image. The patient fulfilled the clinical criteria of primary SS. In addition, anti-AQP4 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. Follow-up MRA displayed complete resolution of the stenosis of the MCA, PCA and BA. |
| doi_str_mv | 10.1007/s00296-008-0611-y |
| format | Article |
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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 T2-weighted 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 T2-weighted image. The patient fulfilled the clinical criteria of primary SS. In addition, anti-AQP4 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. 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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 T2-weighted 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 T2-weighted image. The patient fulfilled the clinical criteria of primary SS. In addition, anti-AQP4 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. Follow-up MRA displayed complete resolution of the stenosis of the MCA, PCA and BA.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer-Verlag</pub><pmid>18493764</pmid><doi>10.1007/s00296-008-0611-y</doi><tpages>4</tpages></addata></record> |
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| ispartof | Rheumatology international, 2008-10, Vol.28 (12), p.1277-1280 |
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| subjects | Anti-Inflammatory Agents - administration & dosage Arterial Occlusive Diseases - complications Arterial Occlusive Diseases - drug therapy Arterial Occlusive Diseases - pathology Basilar Artery - pathology Case Report Cerebral Arterial Diseases - drug therapy Cerebral Arteries - pathology Drug therapy Female Humans Immunoglobulins, Intravenous - therapeutic use Immunologic Factors - therapeutic use Magnetic Resonance Angiography Medicine Medicine & Public Health Methylprednisolone - administration & dosage Middle Aged Neuromyelitis Optica - complications Neuromyelitis Optica - drug therapy Pulse Therapy, Drug Rheumatology Sjogren's Syndrome - complications Sjogren's Syndrome - drug therapy |
| title | Reversible stenosis of large cerebral arteries in a patient with combined Sjögren’s syndrome and neuromyelitis optica spectrum disorder |
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