Three women with lupus transverse myelitis: Case reports and differential diagnosis

Inslicht, David V; Stein, Adam; Pomerantz, Ferne; Ragnarsson, Kristjan T.

doi:10.1016/s0003-9993(98)90150-2

Cited by 27 publications

(16 citation statements)

References 10 publications

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“…Firstly, there are striking similarities between NMO and a variant of MOG-induced experimental allergic encephalomyelitis (EAE) in Brown Norway rats which develop a marked antibody response associated with pronounced demyelination mainly affecting the optic nerves and the spinal cord 93 . Secondly, NMO patients have a number of circulating autoantibodies and many of them have autoimmune co-morbidities such as Sjögren syndrome, systemic lupus erythematosus and mixed connective tissue disease [94][95][96][97][98][99][100][101][102][103] . Finally, some treatment peculiarities such as improvement of corticosteroid-refractory acute attacks following plasma exchange 104,105 and a better response with general immunossupression than with standard MS immunomodulatory drugs 106,107 strongly support the role of autoantibodies in the disease process.…”

Section: Pathology and Immunopathogenesismentioning

confidence: 99%

Devic’s neuromyelitis optica: a critical review

Lana-Peixoto

2008

Arq. Neuro-Psiquiatr.

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-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.

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Section: Pathology and Immunopathogenesismentioning

confidence: 99%

Devic’s neuromyelitis optica: a critical review

Lana-Peixoto

2008

Arq. Neuro-Psiquiatr.

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“…Hydroxychloroquine and non-steroidal anti-inflammatory drugs are used for milder disease, whereas corticosteroids and immunosuppressive therapies are reserved for major organ involvement 2. Even though the prognosis for patients with lupus TM is usually not good, recent reports of better outcomes have emphasised early recognition and treatment with high-dose corticosteroids, cyclophosphamide and other immunosuppressive agents;23 this combination therapy is also thought to improve the prognosis of renal disease associated with SLE 24…”

Section: Outcome and Follow-upmentioning

confidence: 99%

Lupus or syphilis? That is the question!

et al. 2015

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A 47-year-old man presented with fever, a maculopapular rash of the palms and soles, muscular weakness, weight loss, faecal incontinence, urinary retention and mental confusion with 1 month of evolution. Neurological examination revealed paraparesis and tactile hypoesthesia with distal predominance, and no sensory level. Laboratory investigations revealed a venereal disease research laboratory (VDRL) titre of 1/4 and Treponema pallidum haemagluttin antigen (TPHA) of 1/640, positive anti-nuclear antibodies of 1/640 and nephrotic proteinuria (3.6 g/24 h). Lumbar puncture excluded neurosyphilis, due to the absence of TPHA and VDRL. The diagnosis of systemic lupus erythematosus (SLE) was established and even though transverse myelitis as a rare presentation of SLE has a poor outcome, the patient improved with cyclophosphamide, high-dose corticosteroids and hydroxychloroquine. A diagnosis of secondary syphilis was also established and the patient was treated with intramuscular benzathine penicillin G.

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“…1 TM is a rare (0.4%) manifestation of APS, either primary or secondary to SLE. [43][44][45][46] The association of TM and APS is not well established and the exact mechanisms are not clearly known. It is not yet clear if aPLs have a pathogenic role in TM.…”

Section: Antiphospholipid Syndromementioning

confidence: 99%

“…In recent years, pulses of MP in combination with immunosuppressive drugs, such as CP or plasmapheresis, have been used. [44][45][46][47] Other therapies include anticoagulation. 31 44 46 AON was first associated with aCL in Kupersmith's series 48 and confirmed by several other reports, 49 50 without demonstration of full blown APS in the vast majority of patients.…”

Section: Antiphospholipid Syndromementioning

confidence: 99%

Demyelination in rheumatic diseases

Theodoridou

Settas

2008

Postgraduate Medical Journal

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Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system (CNS) characterised by multifocal areas of demyelination in the white matter of the brain and spinal cord. Autoantibodies, for example antinuclear antibodies, can also be present. MS and other demyelinating processes, such as transverse myelitis and optic neuritis (which may be clinically isolated cases or be part of the clinical spectrum of MS), are sometimes difficult to differentiate from CNS involvement in systemic autoimmune diseases like systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), Sjoegren's syndrome (SS), and Adamantiades-Behcet disease (BD). An acute isolated neurological syndrome presents the biggest diagnostic problem, since it is common in MS, but can also be the only feature or first manifestation in SLE, APS, SS, and BD. Indeed, the clinical presentation and lesions evidenced by magnetic resonance imaging may be similar.

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Three women with lupus transverse myelitis: Case reports and differential diagnosis

Cited by 27 publications

References 10 publications

Devic’s neuromyelitis optica: a critical review

Devic’s neuromyelitis optica: a critical review

Lupus or syphilis? That is the question!

Demyelination in rheumatic diseases

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