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

Uzawa, Akiyuki; Mori, Masahiro; Iwai, Yuhta; Kobayashi, Makoto; Hayakawa, Sohachiro; Kawaguchi, Naoki; Kuwabara, Satoshi

doi:10.1016/j.jns.2009.08.040

Cited by 46 publications

(20 citation statements)

References 21 publications

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“…This is the first reported case in our region; however, other cases may not have been reported due to failure of diagnostic acumen and unavailability of diagnostic facilities. The findings in this patient are in keeping with other previous reports of most patients being women and with almost all cases having features of MG predating the development of NMO 8 9 11–14 20. The age of our patient was younger than those mostly reported.…”

Section: Discussionsupporting

confidence: 91%

“…The exact mechanism for the coexistence of the two condition is not fully known, but may be due to possible shared autoimmune predisposition 9 22. This theory is supported by the fact that IgG1 antibodies activate complementally to mediate both diseases, indicating that MG and NMO are both T-helper (Th2)-dominant disorders 23.…”

Section: Discussionmentioning

confidence: 99%

“…Antibodies in NMO might possibly be produced as a paraneoplastic response in patients with MG with thymoma. An alteration in the immune system functioning caused by thymectomy and immunosuppressive treatment for MG may lead to the development of NMO 8 9 14…”

Section: Introductionmentioning

confidence: 99%

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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: 91%

Section: Discussionmentioning

confidence: 99%

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Neuromyelitis optica and myasthenia gravis in a young Nigerian girl

Balarabe¹,

Adamu

Watila

et al. 2015

BMJ Case Reports

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“…We previously reported two patients with MG who presented with NMO after thymectomy [8]. In that report, we showed longitudinal change in anti-AQP4 antibody titre for more than 16 years, but the titres were measured only after NMO onset.…”

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confidence: 81%

Seroconversion of anti-aquaporin-4 antibody in NMO spectrum disorder: a case report

et al. 2011

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“…Sixteen patients had both disorders, and four out of five patients whom stored sera where available had AQP4-IgG present 4 to 16 years before NMOSD onset [2]. Another case series suggested that those who underwent thymectomy maybe at higher risk for developing NMO [3,4]. Because of the association of two rare autoimmune diseases, authors suggested that patients diagnosed with AChR-MG might be at risk of developing NMO, and vice versa [2].…”

Section: Discussionmentioning

confidence: 99%

A Patient with NMO and Ulcerative Colitis: Is it only Autoimmunity?

Al-Eissa¹

2013

J Trauma Treat

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Keywords: NMO; Ulcerative colitis; Aquaporin Case ReportA 35-year-old lady referred for recurrent bilateral severe optic neuritis. Her past medical history is significant for ulcerative colitis (UC) diagnosed in 2006 and treated successfully with azathioprine (AZA).In August 2010, one year after AZA discontinuation, she had her first attack of severe right optic neuritis that caused blindness for which she received 5-day-course of intravenous methylprednisolone (IVMP) with complete recovery. Three months later, she had similar attack that was treated with another course of IVMP but with partial improvement. In May 2012 she had another episode of simultaneous bilateral severe optic neuritis not responding to 2 courses of IVMP. The MRI showed the enhancement of both optic nerves, chiasm, optic tracts, and lateral geniculate bodies (Figure 1a and 1b).Upon presentation to our hospital 5 months after the last attack, she was blind and had no light perception. Her pupils were not reactive to light; she had pale optic discs, and otherwise normal neurological exam. A repeated MRI showed residual optic nerve atrophy with no brain or spinal cord lesions.Patient's serum sent for neuromyelitisoptica (NMO) antibodies in Mayo clinic laboratories were positive with a titer of >1:160, and the diagnosis of NMO spectrum disorder (NMOSD) was confirmed. Other serological investigations showed negative rheumatoid factor, ANA, anti-DNA, anti-Ra, anti-La antibodies. A spinal tap showed normal levels of protein, glucose, and cells; oligoclonal bands were absent.While the patient was scheduled to receive rituximab infusion, she had another relapse in December 2012 causing left sided weakness and hemianasthesia. The repeated MRI showed a "longitudinally extensive" lesion involving the right internal capsule and right cerebral peduncle (Figures 1b-1h). Her spine MRI was normal. She received 5-day-course IVMP with good recovery, followed by rituximab infusion of 1000 mg biweekly (Table 1).Six months after treatment, her neurological exam came back to baseline and she continued to receive rituximab 1000 mg biweekly every 6 months. She also remains in remission from her ulcerative colitis since 2006. DiscussionCoexistence between NMO and both organ-specific and nonorgan-specific systemic autoimmune diseases is not uncommon. In a cohort study of 153 patients with NMOSD, 4 (2.6 %)patients diagnosed with ulcerative colitis, 26 (17.0%) with autoimmune thyroid disease, 5 (3.2%) with systemic lupus erythematosus or sjogren's syndrome, 2 with idiopathic thrombocytopenic pupura (1.3%), 2 with rheumatoid arthritis (1.3%), 2 with myasthenia gravis (1.3%), one with Raynaud phenomenon (0.7%), one with polymyositis (0.7%), and one with celiac disease (0.7%) [1]. A recent multinational study highlighted the association with acetylcholine receptor antibody positive myasthenia gravis (AChR-MG) [2]. Sixteen patients had both disorders, and four out of five patients whom stored sera where available had AQP4-IgG present 4 to 16 years before NMOSD onset [2]. An...

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

Cited by 46 publications

References 21 publications

Neuromyelitis optica and myasthenia gravis in a young Nigerian girl

Neuromyelitis optica and myasthenia gravis in a young Nigerian girl

Seroconversion of anti-aquaporin-4 antibody in NMO spectrum disorder: a case report

A Patient with NMO and Ulcerative Colitis: Is it only Autoimmunity?

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