Abstract | BACKGROUND: OBJECTIVES: To evaluate the prevalence of even mild CNS involvement in patients with MG and to identify features indicative of neuromyelitis optica-like disease. DESIGN: Cohort study. SETTING: Outpatient clinic. PATIENTS: A cohort of 164 patients with MG. METHODS: In 24 patients with MG, signs of CNS involvement were detected; 15 of these patients had at least 1 additional paraclinical indication of neuromyelitis optica-like disease (presence of antibodies against AQP4, pathological visual evoked potentials, or white matter lesions detected on brain and/or spinal magnetic resonance imaging scans) and fulfilled the inclusion and exclusion criteria for our study. RESULTS: Of the 15 patients who had at least 1 additional paraclinical indication of neuromyelitis optica-like disease, 14 had abnormal visual evoked potentials, and in 6 of 9 patients in whom magnetic resonance imaging was performed, there was evidence of lesions in the white matter of the brain and/or spinal cord. Anti-AQP4 antibodies were detected in 7 patients (out of the 14 tested). Thymic enlargement ( hyperplasia or thymoma) was more frequent in patients with MG who had signs of CNS involvement than in patients with MG who did not. CONCLUSIONS: The incidence of CNS involvement in MG is higher than previously reported and is expressed predominantly as a pyramidal syndrome accompanied by optical tract involvement (frequently subclinical). These features bear some resemblance to neuromyelitis optica spectrum disease, supported also by the presence of anti-AQP4 antibodies in 7 of the 14 patients tested. This association may represent a new nosological entity or may indicate that an autoimmune process targeting AQP4 is an integral part of the immunopathogenetic mechanisms in MG.
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Authors | Adi Vaknin-Dembinsky, Oded Abramsky, Panayiota Petrou, Tamir Ben-Hur, Marc Gotkine, Livnat Brill, Talma Brenner, Zohar Argov, Dimitrios Karussis |
Journal | Archives of neurology
(Arch Neurol)
Vol. 68
Issue 12
Pg. 1557-61
(Dec 2011)
ISSN: 1538-3687 [Electronic] United States |
PMID | 21825214
(Publication Type: Journal Article)
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Chemical References |
- AQP4 protein, human
- Aquaporin 4
- Autoantibodies
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Topics |
- Adult
- Aquaporin 4
(immunology)
- Autoantibodies
- Cohort Studies
- Evoked Potentials, Visual
(physiology)
- Female
- Humans
- Male
- Middle Aged
- Myasthenia Gravis
(diagnosis, immunology)
- Neuromyelitis Optica
(diagnosis, immunology)
- Thymoma
(diagnosis, immunology)
- Thymus Neoplasms
(diagnosis, immunology)
- Young Adult
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