Practice Interactive Case Report

A woman with acute myelopathy in pregnancy: case progression

BMJ 2009; 339 doi: (Published 19 November 2009) Cite this as: BMJ 2009;339:b4025

Re: A woman with acute myelopathy in pregnancy: case progression

Aquaporin is amongst a family regulatory transport of water in central nervous system [1]. A new autoantibody found AQP4-Ab, NMO-IgG has been described in neuromyelitis optic (NMO), transverse myelitis and recurrent optic neuritis. During the acute phase the spinal cord develops diffuse swelling over multiple spinal segments. Also associated with this are perivascular inflammatory infiltration. The pattern of tissue inflammation in early demyelination with a unique perivascular pattern suggested a role of humoral autoimmunity in the pathogenesis of disease. The autoantibody is associated with many autoimmune disease such as systemic lupus erythematosus, sjogren syndrome, autoimmune thyroiditis, type I diabetes mellitus, myasthenia gravis where B cell activation is suggested [2][3]. However, one of the studies done in South America nullifies the association of aquaporin-4 antibodies with neurological manifestation in sjogren syndrome [4]. A disease like lupus may also cause central nervous system involvement, the coexistence may reflect a predisposition of patients with NMO to multiple autoimmune disease[5]. Seropositivity of aquaporin-4 antibody can help differentiate between systemic lupus erythematosus nervous system involvement and NMO (neuromyelitis optica) [6][7]. Thus, in conclusion, myelitis and optic neuritis in a patient with lupus should alert the co-existence of NMO for testing the presence of aquaporin-4 antibody.

1. Amiry - Moghaddam M, Ottersan OP.The molecular basis of water transport in brain. nat.Rev Neurosci 2003 ; 4:991 – 1001.
2.Jarius S,Jacobi C , De Seze J et al.Frequency and syndrome specificities of antobodies to aquaporin -4 in neurological patienst with rheumatic disorders.Multi SCler 2011 May 4.
3.Sergio P,Mariana B,ALbertoo et al.Association of neuromyelitis optica (NMO) with autoimmune disorder: A report of 2 cases and literature review . Clin .Rheumatol . 20120 ;29:1335 -8.
4.Dellavance ,Allesandra , ALverang et al.Anti-aquaporin -4 antibodies are highly specific for neuromylitis optica and show no association with sjogren syndrome and other autoimmune disease.Arthritis & Rheumatism ,Volume 60 ,Oct 2009 . Abstract Suppl.
5.Pittick SJ,Lennon VA,SezeJ et al.Neuromyelitis optica and non-organic specific antibody.Arch Neurol 2008 , 65:78 -83.
6.Bimbaum J , Kern D. Devic’s syndrome in a women with SLE diagnostic and therapeutic implication of testing for the neuromyelitis optica IgG antibody . Arthritis Rheumatism 2007;57 :347-51.
7. Lennon VA,Wingerchunk DM,Kryner TJ, et al.A serum antibody marker of neuromyelitis optica : Distnction from multiple sclerosis .lancet 2004 ;314:2106 -12.

Competing interests: No competing interests

03 January 2013
Kaushik Sanyal
Department of Rheumatology
East Surrey Hospital
Redhill , Surrey