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Devic's neuromyelitis optica and HIV-1 infection
  1. P BLANCHE,
  2. E DIAZ,
  3. B GOMBERT,
  4. D SICARD
  1. Service of Internal Medicine 2, Cochin Hospital, René Descartes University, 27 rue du Faubourg Saint-Jacques, 75679 Paris, Cedex 14, France
  2. Service of Ophthalmology
  1. Dr P Blanche, René Descartes University, 27 Rue du Faubourg Saint- Jacques 75679 Paris, Cedex 14, France didiex.sicard{at}cch.ap-hop-paris.fr
  1. O RIVOAL,
  2. A BREZIN
  1. Service of Internal Medicine 2, Cochin Hospital, René Descartes University, 27 rue du Faubourg Saint-Jacques, 75679 Paris, Cedex 14, France
  2. Service of Ophthalmology
  1. Dr P Blanche, René Descartes University, 27 Rue du Faubourg Saint- Jacques 75679 Paris, Cedex 14, France didiex.sicard{at}cch.ap-hop-paris.fr

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Neuromyelitis optica (Devic's syndrome) can be defined as a severe transverse myelitis, an acute unilateral or bilateral optic neuropathy, no clinical involvement beyond the spinal cord or optic nerves, and a monophasic or, rarely, a multiphasic illness.1 Optic neuritis precedes transverse myelitis in 80% of cases, by less than 3 months in most cases. Neuromyelitis optica may occur as a result of demyelination from multiple sclerosis, but many differences have to be noted between neuromyelitis optica and multiple sclerosis. Neuromyelitis optica is not predominantly seen in the white ethnic group. Severe deficits after the acute episode are more frequent in neuromyelitis optica. Multiple sclerosis presenting as transverse myelitis is rare.2 Oligoclonal bands in the CSF and white matter lesions on brain MRI are rare in neuromyelitis optica compared with over 90% of patients with definite multiple sclerosis. These two abnormalities can resolve in neuromyelitis optica but very rarely in multiple sclerosis. The high CSF protein concentration and pleiocytosis seen in 60% of cases are often more important in neuromyelitis optica. Swelling and signal change on MRI extending beyond a single segment are more frequent in neuromyelitis optica and cavitation can be seen. Most authors consider that neuromyelitis optica is often a variant of postviral acute disseminated encephalomyelitis mostly due to varicella zoster virus, but other causes have been identified —for example, systemic lupus erythematosus, antiphospholipid syndrome, and pulmonary tuberculosis. To the best of our knowledge, only one case of neuromyelitis optica (followed by a bilateral acute retinal necrosis) due to varicella zoster virus has been described in a patient with AIDS.3 We report the case of an HIV infected African women developing neuromyelitis optica.

A 41 year old woman from the Congo Democratic Republic was admitted to hospital in March 1999 because of a 10 day progressive blindness of the left eye with optic disc oedema. There were left leg dysaesthesias of 1 month duration and a T6 left sensory level was noted within the next few days. Progressive ascending paralysis of both legs without bowel or bladder dysfunction developed. HIV infection had been diagnosed 11 years earlier. Her CD4 lymphocyte count was 499/mm3 and her plasma viral load was 3.06 log (1150 RNA/ml). She received no treatment. Examination of CSF showed 57 white blood cells/mm3 (94% lymphocytes). Total protein concentration was 48 mg/dl. No oligoclonal bands were detected. Serology for syphilis and Borrelia burgdorferi andCryptococcus neoformans antigen were negative in blood and CSF. Search for herpes simplex virus type 1 and 2, varicella zoster virus, cytomegalovirus, and Epstein-Barr virus by polymerase chain reaction remained negative in CSF. Serology for HTLV1 was negative. Interferon and angiotensin converting enzyme concentrations were normal in blood and CSF. Cultures of CSF were negative. Antinuclear and anticardiolipin antibodies were not detected. Brain MRI with and without gadolinium DPTA was normal. Spinal cord MRI disclosed four high signal intensity lesions on the T2 weighted sequence on T6, T7, T10, and T12 levels. Enhancement over the four segments was seen after intravenous gadolinium DPTA. The patient received 0.5 g/day intravenous methylprednisolone for 3 days. Subsequently, she was maintained on oral prednisone (80 mg/day) for 12 days and treatment with lamivudine (300 mg/day), zidovudine (600 mg/day), and nelfinavir (2250 mg/day) was started. Symptoms dramatically improved. The patient was able to walk alone without help after 10 days. Visual acuity returned to normal. Dysaesthesias were absent 1 month later. Three months later, plasmatic viral load was under 50 RNA/ml. Examination of CSF and MRI of the spinal cord gave normal results. There was no recurrence after a 5 month follow up.

Optic neuritis occurring in patients infected with HIV4 is usually due to syphilis or to opportunistic organisms such as varicella zoster virus1, cytomegalovirus,Cryptococcus,Histoplasma,Bartonella,Toxoplasma, orMycobacterium tuberculosis. The role of HIV itself is now well established. Intramedullar involvement in the course of HIV infection may result from HIV itself (vacuolar myelopathy), coinfection with HTLV1 in endemic areas, or transerse myelitis mostly due to varicella zoster virus, cytomegalovirus, or syphilis. Such infections were excluded in our patients. There are only few reports of multiple sclerosis-like illness occurring with HIV infection.5 Cases of sarcoidosis associated with HIV infection remain exceptional even when highly active antiretroviral therapy (HAART) is used2 (optic neuritis is rare in sarcoidosis and no case of neurosarcoidosis has been described with AIDS). Primary CNS lymphoma associated with HIV is associated with Epstein-Barr virus and may cause optic neuritis or spinal cord involvement. We think that multiple sclerosis, sarcoidosis, and lymphoma were excluded in our patient. Neuromyelitis optica without an identified cause can be seen in the course of HIV infection, even at an early stage of the disease, before immunodepression occurs.

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