NMO-IgG positive neuromyelitis optica in a patient with myasthenia gravis but no thymectomy

Kay, Cláudia Suemi Kamoi; Scola, Rosana Hermínia; Lorenzoni, Paulo José; Jarius, Sven; Arruda, Walter Oleschko; Werneck, Lineü Cesar

doi:10.1016/j.jns.2008.06.038

Cited by 35 publications

(20 citation statements)

References 25 publications

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“…Most of the cases of NMO developing in patients with MG had had a thymectomy done7 20; our patient did not have a thymectomy. Kay et al 13 reported a case similar to ours, in which a patient with MG developed NMO without a thymectomy having been done. The report by Kay et al 13 proposed that a thymectomy is not necessarily a prerequisite of NMO occurrence in patients with MG.…”

Section: Discussionsupporting

confidence: 76%

Neuromyelitis optica and myasthenia gravis in a young Nigerian girl

Balarabe¹,

Adamu

Watila

et al. 2015

BMJ Case Reports

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Neuromyelitis optica (NMO) and myasthenia gravis (MG) are rare autoimmune disorders. The coexistence of the two disorders, although rare, has been documented. This is a case report of a 16-year-old student who presented with recurrent episodes of transverse myelitis and optic neuritis, 8 years after diagnosis of MG. She presented with visual impairment, relapsing and remitting weakness, numbness and paraesthesia of her lower limbs, with bladder and bowel incontinence. Her examination revealed bilateral optic atrophy, spastic paraparesis of the lower limbs and patchy sensory loss up to thoracic level (T4-5). She had a positive acetylcholine receptor antibody, a positive aquaporin-4 antibody and chest CT finding of thymic enlargement. We therefore confirmed the previous diagnosis of MG and performed a recent diagnosis of background NMO. A high index of suspicion is needed to make a diagnosis of this rare coexistence of NMO and MG in resource-limited settings such as Nigeria.

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Section: Discussionsupporting

confidence: 76%

Neuromyelitis optica and myasthenia gravis in a young Nigerian girl

Balarabe¹,

Adamu

Watila

et al. 2015

BMJ Case Reports

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“…Pathological examination of the thymus revealed hyperplasia in 7 of 9 documented cases (78%) [5][6][7][8]11]. NMO-IgG was positive in 5 of 8 cases (63%) [7][8][9][10]13]. Anti-AQP4 antibody was positive in our 2 cases only.…”

Section: Literature Reviewmentioning

confidence: 69%

“…We have summarized 13 previously reported cases showing association of NMO with MG [5][6][7][8][9][10][11][12][13], in addition to our 2 cases, in Table 1. Of the 15 patients, 14 were females, and this predominance fits the clinical characteristics of MG [4] and NMO.…”

Section: Literature Reviewmentioning

confidence: 99%

“…Anti-AQP4 antibody was positive in our 2 cases only. MG had remitted in 5 of 9 cases (56%) [7,9,11,13], and only 1 of the 9 cases (11%) [6,7,[10][11][12][13] showed negative serum anti-AChR antibody at the onset of NMO. Serum anti-nuclear antibodies were detected in 9 of 13 cases (69%) [6-9,11-13].…”

Section: Literature Reviewmentioning

confidence: 99%

“…Coexistence of NMO and MG is rare, but at least 13 such cases have been reported to date [5][6][7][8][9][10][11][12][13]. All 13 patients developed MG before the onset of NMO.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Association of anti-aquaporin-4 antibody-positive neuromyelitis optica with myasthenia gravis

Uzawa

Mori

Iwai

et al. 2009

Journal of the Neurological Sciences

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Myasthenia Gravis–Associated Neuromyelitis Optica–Like Disease

Vaknin‐Dembinsky¹

2011

Arch Neurol

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Background: Although overt involvement of the central nervous system (CNS) in myasthenia gravis (MG) is considered rare, hyperreflexia is a common and yet unexplained finding. Aquaporin 4 (AQP4), the target autoantigen in neuromyelitis optica, is expressed both in the CNS and in the neuromuscular junction.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 opticalike 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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NMO-IgG positive neuromyelitis optica in a patient with myasthenia gravis but no thymectomy

Cited by 35 publications

References 25 publications

Neuromyelitis optica and myasthenia gravis in a young Nigerian girl

Neuromyelitis optica and myasthenia gravis in a young Nigerian girl

Association of anti-aquaporin-4 antibody-positive neuromyelitis optica with myasthenia gravis

Myasthenia Gravis–Associated Neuromyelitis Optica–Like Disease

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