Abstract
BackgroundThe association of the anti-aquaporin-4 (AQP-4) water channel antibody with neuromyelitis optica (NMO) syndrome has been described from various parts of the world. There has been no large study describing this association from southern Africa, an HIV endemic area. HIV patients often present with visual disturbance or features of a myelopathy but seldom both either simultaneously or consecutively. We report our experience of NMO in the era of AQP-4 testing in HIV-positive and HIV-negative patients seen in KwaZulu-Natal, South Africa.MethodsA retrospective chart review was undertaken of NMO cases seen from January 2005 to April 2016 in two neurology units serving a population of 7.1 million adults. The clinical, radiological and relevant laboratory data were extracted from the files and analysed.ResultsThere were 12 HIV-positive patients (mean age 33 years), 9 (75%) were women and all 12 were black patients. Of the 17 HIV-negative patients (mean age 32 years), 15 (88%) were women and 10 (59%) were black people. The clinical features in the two groups ranged from isolated optic neuritis, isolated longitudinally extensive myelitis or combinations. Recurrent attacks were noted in six HIV-positive patients and six HIV-negative patients. The AQP-4 antibody was positive in 4/10 (40%) HIV-positive patients and 11/13 (85%) HIV-negative patients. The radiological changes ranged from longitudinal hyperintense spinal cord lesions and long segment enhancing lesions of the optic nerves. Three patients, all HIV-positive, had tumefactive lesions with incomplete ring enhancement.ConclusionThis study confirms the presence of AQP-4-positive NMO in southern Africa in both HIV-positive and HIV-negative patients. The simultaneous or consecutive occurrence of optic neuritis and myelitis in an HIV-positive patient should alert the clinician to test for the AQP-4 antibody. It is important to recognise this clinical syndrome as specific therapy is available. We further postulate that HIV itself may act as a trigger for an autoimmune process.
Highlights
Neuromyelitis optica (NMO) or Devic’s disease is a central nervous system inflammatory disorder originally described as a monophasic illness but recurrent cases have been noted.[1]
A number of laboratory tests were performed on each patient, the focus was on the following tests: HIV and human T-lymphotropic virus 1 (HTLV-1) antibodies, rapid plasma reagin (RPR), antinuclear factor (ANF), serum AQP-4 antibody, serum angiotensin converting enzyme (SACE), routine CSF chemistry and cell count, CSF microscopy, culture and PCR for tuberculosis, CSF fluorescent treponemal assay (FTA) and oligoclonal bands in the CSF
The four AQP-4-positive patients we describe above may fall into this category
Summary
Neuromyelitis optica (NMO) or Devic’s disease is a central nervous system inflammatory disorder originally described as a monophasic illness but recurrent cases have been noted.[1] NMO has been described in different ethnic and racial groups worldwide.[2]. The association of the anti-aquaporin-4 (AQP-4) water channel antibody with neuromyelitis optica (NMO) syndrome has been described from various parts of the world. There has been no large study describing this association from southern Africa, an HIV endemic area. HIV patients often present with visual disturbance or features of a myelopathy but seldom both either simultaneously or consecutively. We report our experience of NMO in the era of AQP-4 testing in HIV-positive and HIV-negative patients seen in KwaZulu-Natal, South Africa
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