Devic’s neuromyelitis optica: a critical review

Abstract

-Devic's neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating and necrotizing disease characterized by predominant involvement of the optic nerves and spinal cord. In Asian countries relapsing NMO has been known as opticospinal multiple sclerosis. It has long been debated if NMO is a variant of multiple sclerosis (MS) or a distinct disease. Recent studies have shown that NMO has more frequently a relapsing course, and results from attack to aquaporin-4 which is the dominant water channel in the central nervous system, located in foot processes of the astrocytes. Distinctive pathological features of NMO include perivascular deposition of IgG and complement in the perivascular space, granulocyte and eosinophil infiltrates and hyalinization of the vascular walls. These features distinguish NMO from other demyelinating diseases such as MS and acute demyelinating encephalomyelopathy. An IgG-antibody that binds to aquaporin-4, named NMO-IgG has high sensitivity and specificity. Magnetic resonance imaging (MRI) studies have revealed that more frequently there is a long spinal cord lesion that extends through three or more vertebral segments in length. Brain MRI lesions atypical for MS are found in the majority of cases. Treatment in the acute phase includes intravenous steroids and plasma exchange therapy. Immunosupressive agents are recommended for prophylaxis of relapses.KEY WORS: Devic's neuromyelitis optica, opticospinal multiple sclerosis, recurring optic neuritis, transverse myelitis, aquaporin-4, NMO-IgG antibody. Neuromielite óptica de Devic: revisão críticaResumo -Neuromielite óptica ou doença de Devic (NMO) é uma doença inflamatória com desmielinização e necrose envolvendo preferencialmente os nervos ópticos e a medula espinal. Desde sua descrição inicial tem havido controvérsia se a NMO é uma variante da esclerose múltipla (EM) ou se é uma entidade independente. Na Ásia a doença é conhecida como esclerose múltipla óptico-espinal. Recentes avanços tem demonstrado que na maioria dos casos a NMO é recorrente e resulta de alterações inflamatórias por ataque à aquaporina-4, uma proteína localizada nos pés dos astrócitos na barreira hemato-encefálica. Patologicamente a NMO difere da EM pela presença de necrose e cavitação no centro da medula, deposição perivascular de IgG e complemento, infiltração de neutrófilos e eosinófilos, assim como por hiperplasia e hialinização dos vasos. O anticorpo contra a aquaporina-4 (IgG-NMO), detectado no soro dos pacientes, tem alta sensibilidade e especificidade. Imagem por ressonância magnética demonstra lesão medular que se estende três ou mais segmentos vertebrais. Na maioria dos casos há lesões cerebrais atípicas para EM. Corticosteróide venoso em altas doses e plasmaférese são usados no tratamento das fases agudas, enquanto os imunossupressores devem ser usados na profilaxia das recorrências. PALAVRAS-CHAVE: neuromielite óptica, doença de Devic, esclerose múltipla óptico-espinal, neurite óptica recorrente, mielite transversa, aquaporina-4, IgG-NMO.

Combined Screening for Serum Anti-Nuclear and Anti-Aquaporin-4 Antibodies Improves Diagnostic Accuracy for Distinguishing Neuromyelitis Optica from Multiple Sclerosis

“…Neuromyelitis optica (NMO) is an inflammatory disease of the central nervous system with many clinical features resembling multiple sclerosis (MS) [1]. Once considered a subtype of MS [2], recent clinical, epidemiological, and immunological data indicate that NMO is a distinct clinical entity [1,3].…”

“…Once considered a subtype of MS [2], recent clinical, epidemiological, and immunological data indicate that NMO is a distinct clinical entity [1,3]. It commonly presents as optic neuritis and acute longitudinally extensive transverse myelitis (LETM), either simultaneously or separately, with occasional involvement of other CNS areas (often as asymptomatic lesions).…”

Seropositive Neuromyelitis Optica (Devic’s Disease): A Rare Case Report

“…5 In rare cases viral illnesses or immunizations precede the clinical onset. 6 In our case patient had no history of preceding vaccinations or viral infections.…”

Sero-Negative Neuromyelitis Optica

“…The first description of a co-existence between acute myelitis and optic nerve involvement was reported in 1870 by Albutt [7]. Eugène Devic in 1884, reported a 45-year-old woman with symptoms of headache and malaise followed by development of urinary retention, complete paraplegia and blindness.…”

“…Devic named the syndrome "neuromyélite optique". This led to discovery of a new disease entity which almost 30 years later was named after him as "Devic's disease" [7].…”