Trastorno del espectro de la neuromielitis óptica / Neuromyelitis Optica Spectrum Disorder: case report / Distúrbio do spectro da neuromilite óptica

El síndrome de Devic, también conocido como trastorno del espectro de la neuromielitis óptica (NMOSD, por sus sigla en inglés), es considerado una enfermedad inflamatoria, desmielinizante y autoinmune del sistema nervioso central que afecta en su mayoría los nervios ópticos, el quiasma óptico y la médula espinal. Si bien en algunos casos se puede simular un cuadro clínico similar a esclerosis múltiple, hoy en día se conocen aspectos imagenológicos, inmunológicos y patológicos que permiten establecer las diferencias entre estas dos entidades. Se presenta el caso de una mujer adulta mayor con antecedente de síndrome de Sjögren en manejo ambulatorio con corticoide y azatioprina, quien ingresa por cuadro clínico de mes y medio de evolución consistente en disestesias de predominio en miembros inferiores, alteración de la marcha y compromiso visual. Ante el compromiso neurológico, se decide realizar imágenes diagnósticas y perfil inmunológico, con reportes de resonancia magnética nuclear cervical y torácica con contraste que evidencian mielitis multifocal por compromiso cervical y torácico a nivel de C4 en T7-T8 con hiperdensidad centromedular sin realce, además de autoanticuerpos séricos dirigidos contra el canal acuaporínico (AQP4) positivos, dando así el diagnóstico de NMOSD.Se inició manejo con pulsos de metilprednisolona concomitantemente con plasmaféresis completando cinco sesiones. Sin embargo, ante la persistencia del cuadro clínico se inició manejo con un agente biológico selectivo que bloquea la actividad de los linfocitos B tipo rituximab con resolución parcial de los síntomas. Se discute, además, la evolución clínica e imagenológica de este caso ejemplar, así como los avances más notables en el diagnóstico y manejo

Devic's Syndrome, known as Optic Neuromyelitis Spectrum Disorder (NMOSD), is considered an inflammatory, demyelinating and autoimmune disease of the central nervous system (CNS) that mainly affects the optic nerves, the optic chiasm and the spinal cord. Although the said syndrome can sometimes simulate multiple sclerosis (MS), nowadays there are imagining, immunological and pathological aspects that allow to establish the differences between these two entities. In the present paper, the case an older adult woman with a history of Sjögren's syndrome in ambulatory care with corticosteroid and azathioprine, who is admitted for a month and a half of evolution consisting of dysesthesias of predominance in the lower limbs, ambulation impairment and visual compromise is presented . In view of the neurological compromise, it was decided to perform diagnostic imaging and immunological profile. The imaging suggested a report of cervical and thoracic MRI with contrast with evidence of multifocal myelitis due to cervical and thoracic involvement at the level of C4 and T7-T8, with a report of serum autoantibodies directed against the positive aquaporic channel (AQP4). All of the above is consistent with the diagnosis of Devic Syndrome or NMOSD. Treatment was implemented through methylprednisolone pulses alternated with plasmapheresis. Five sessions of the said treatment were completed. Given the persistence of the clinical picture, treatment with rituximab was initiated resulting in partial improvement of the symptoms. The clinical and imaging evolution of this case is also discussed, as well as breakingthrough advan-ces in its diagnosis and management

O síndrome de Devic, convencido cómo Transtorno do espectro da neuromielite ótica ou NMOSD pelo Nome em ingles é considerado uma doença inflamatória, desmielinizante e autoimune do sistema nervioso central (SNC) que afeta principalmente os nervos óticos, o quiasma ótico e a mêdula espinhal; embora em alguns casos pode se apresentar como sendo un caso clínico de esclerosis múltipla, hoje existem aspectos imagemológicos, inmunológicos e patológicos para diferenciar entre as duas condições. Apresentam-se o caso de una mulher idosa com antecedente de Síndrome de Sjögren com tratamento ambulatorio de corticoides, ingresada por quadro clínico de un mês e meio de evoluçao más disestêsias nos miembros inferiores com alteraçao para caminhada e comprometimento visual. Perante essa alteraçao neurológica, decide-se realizar imagens diagnósticas e perfil inmunológico com reporte de RMN cervical e torácica com contaste evidencia de mielitis multifocal por compromiso cervical e torácico a nivel de C4 e T7- T8 com hiperdensidade centromedular sem realce, reporte de autoanticuerpos séricos dirigidos contra o canal acuaporínico positivos, o diagnóstico foi Síndrome de Devic ou NMOSD. Perante esse scenário inicoou-se tratamento com pulsos de metlprednisolona junto con plasmaférese, por cinco sessões. No entanto, perante persistência do quadro clínico, inicoouse tratamento com agente biológico selectivo que bloqueia a atividade dos linfositos B tipo rituximab com resoluçao parcial dos síntomas. Discute-se a evoluçao clínica deste caso exemplar, mesmo cómo os avanços mais notáveis no diagnóstico e tratamento.IntroducciónEl síndrome de Devic, ahora conocido como trastor-no del espectro de la neuromielitis óptica (NMOSD, por su sigla en inglés), es una enfermedad inflamato-ria, desmielinizante y autoinmune del sistema nervioso central (SNC) que en su mayoría y de forma simultá-nea afecta los nervios ópticos, el quiasma óptico y la médula espinal (1). En los hallazgos imagenológicos en resonancia magnética nuclear (RMN) cerebral se evi-dencian lesiones mielínicas trasversas extensas longi-tudinales en tres o más segmentos vertebrales (2).El NMOSD fue reportado por primera vez en el siglo XIX, hacia 1894, luego de un reporte de caso; en esa ocasión se nombró síndrome de Devic y se describió como una variante de esclerosis múltiple (EM) (3,4). Keywords: Devic syndrome. Optic neuromyelitis, multiple sclerosis, antiacuaporin 4 antibodies, methylprednisolone, corticoid, plasmapheresis, rituximab Palavras Chave: sindrome de Devic, neuromielite óptica, esclerosis múltipla, anticorpos antiacuaporina 4, metlprednisolona, corticoides, plasmaférese, rituximab.

A Case of Neuromyelitis Optica with Paraneoplastic Syndrome in Hepatocellular Carcinoma

PURPOSE: We report a case of neuromyelitis optica (Devic's syndrome) with hepatocellular carcinoma. CASE SUMMARY: A 70-year-old male with hepatocellular carcinoma presented with bilateral visual loss. A relative afferent pupillary defect was not observed in either eye due to bilateral mydriasis. On brain MRI, there was no specific finding, however, on spine MRI, multiple and severe myelopathies were observed. After high-dose methylprednisolone pulse therapy, the visual acuity was 0.03 in the right eye and counting fingers at 30 cm in the left eye. CONCLUSIONS: We encountered a case of neuromyelitis optica involving bilateral optic neuropathy in hepatocelluar carcinoma. Therefore, in patients with an optic neuropathy of uncertain etiology, clinicians should consider performing a systemic evaluation.

A Case of Coexisting Neuromyelitis Optica in Systemic Lupus Erythematosus

PURPOSE: We present a case of a patient with coexisting neuromyelitis optica and systemic lupus erythematosus (SLE). CASE SUMMARY: A 26-year-old female was hospitalized in our medical center due to decreased visual acuity in her left eye; she had a history of gastric ulcers and herpes zoster infection. Steroid treatment was started under suspicion of optic neuritis, and she was diagnosed with SLE. After treatment, her vision improved, but eleven months later she was hospitalized with paresthesia on the abdomen and left flank progressing to the lower extremities. Spinal MRI showed transverse myelitis, suggesting multiple sclerosis. Fifteen months later, the patient was hospitalized due to decreased visual acuity and ocular pain in the right eye. Her vision was improved by steroid therapy. However, optic neuritis recurred in the right eye after five weeks, thus azathioprine was added to the treatment. Anti-aquaporin-4 Ab test was conducted based on the suspicion of neuromyelitis optica, and the serum was positive for anti-aquaporin-4 Ab (NMO-IgG). The patient was hospitalized again due to paraplegia after three months. Coexistence of neuromyelitis optica was verified because spinal MRI showed longitudinally extensive transverse myelitis. The symptoms were improved by high doses of steroids, a series of plasmaphereses, and rituximab. Optic neuritis was repeated in the right eye and the symptoms were improved with high doses of steroids. Myelitis recurred later and the symptoms improved with high doses of steroids and a series of plasmaphereses. CONCLUSIONS: Coexisting neuromyelitis optica should be considered in cases with relapsing events which have transverse myelitis without cranial lesions in autoimmune diseases such as SLE.

Neuromielitis Óptica: patología, diagnóstico y tratamiento en el siglo XXI / Optic Neuromyelitis: pathology, diagnosis and therapy in the XXI century

La neuromielitis óptica (NMO) o enfermedad de Devic, pertenece al grupo de las enfermedades desmielinizantes del sistema nervioso central, afecta de manera significativa los nervios ópticos y la médula espinal. Desde el siglo XIX cuando Eugene Devic dio a conocer una serie de casos en los que existía asociación de lesiones en los nervios ópticos y la medula espinal, la relación de la neuromielitis óptica (NMO) y la Esclerosis Múltiple (EM) ha sido controversial, considerándose una variante de esta última; datos recientes muestran que puede ser distinguida de la Esclerosis Múltiple (EM). Los síntomas de la NMO son por lo general más agudos y severos y la presencia de un autoanticuerpo específico en sangre llamado NMO-IgG tipo acuoporina-4 (AQP4) desempeña un rol muy importante en la patogenia de esta enfermedad,asimismo se encuentran varias características entre ellas de tipo clínico, de laboratorio, neuroimágenes y en la anatomía patológica que diferencian la esclerosis múltiple (EM) de esta enfermedad. La proporción entre mujeres y hombres es mayor de 4 a 1.La presentación clínica y el curso de la enfermedad puede ser con recaídas en 80-90% y en un 10-20% curso monofásico. La manifestación distintiva de la entidad NMO es la ocurrencia ya sea consecutiva o simultánea de NO (unilateral o bilateral) y la presencia de mielitis longitudinal extensa (Mle). La terapia con corticosteroides intravenosos normalmente es el tratamiento inicial para los ataques agudos de neuritis óptica o mielitis. La plasmaféresis es la terapia de rescate cuando no hay respuesta a los esteroides durante los ataques de neuromielitis óptica. Los agentes inmunosupresores se usan para la prevención de las recaídas.

Optic Neuromyelitis (NMO) or Devic’s disease, is a demyelinating disease of central nervous system, affects the optic nerves and spinal cord. Since the nineteenth century when Eugene Devic unveiled a series of cases in which there was an association of lesions in the optic nerves and spinal cord with relation of NMO and Multiple Sclerosis (MS) has been controversial, in some cases described like a variant of the latter but recent data show that can be distinguished from multiple sclerosis (MS). Symptoms of NMO are usually more acute and severe and then we can find a presence of a specific autoantibody called NMO-IgG, it is a blood type acuoporina-4 (AQP4) and plays an important role in the pathogenesis of this disease. Also in the new millennium are various types of clinical, laboratory, neuroimaging and pathological anatomy then differentiates multiple sclerosis (MS) of this disease. In the epidemiology the ratio of women to men is greater than 4 to 1.The clinical presentation and course of the disease can be relapsing by 80-90% and 10-20% monophasic course. The distinctive manifestation is the occurrence NMO entity either consecutively or simultaneously not (unilateral or bilateral) and the presence of extensive longitudinal myelitis. Intravenous corticosteroid therapy is usually the initial therapy for acute attacks of optic neuritis or myelitis. Plasmapheresis therapy is used when the steroids therapy no work during acute attacks of NMO. Immunosuppressive agents used to prevent relapses.

A Case of Neuromyelitis Optica(Devic's Syndrome) with Acute Bilateral Central Retinal Artery Occlusion / 대한소아신경학회지

Neuromyelitis optica(NMO) or Devic's syndrome is an uncommon clinical syndrome associating with unilateral or bilateral optic neuritis and transverse myelitis. It is rarely found in children and usually reported in adults with serious neurologic manifestations. We report a case of an 8-year-old girl with neuromyelitis optica whose first clinical manifestation was acute visual loss of both eyes. Initially the patient had been diagnosed with central retinal artery occlusion and optic neuritis by ophthalmologic examination, a brain magnetic resonance imaging, and cerebrospinal fluid findings. She was treated with intravenous methylprednisolone pulse therapy and heparinization. Then the treatments were replaced with oral prednisolone and warfarin. At the fifteenth day after the start of oral prednisolone tapering, she visited our emergency room for voiding difficulty and paresthesia on both legs. A spinal magnetic resonance imaging revealed increased signal intensity in T2-weighted images from cervical to lumbar level, and neuromyelitis optica- IgG(NMO-IgG) was detected in the patient's serum. After we diagnosed her as having neuromyelitis optica, intravenous methylprednisolone and nine courses of daily plasmapheresis were tried. However, the patient still had visual loss, pain, and sensory loss below the sixth thoracic dermatome, and we tried maintenance therapy with intravenous rituximab. We report our case with reviews of the related literatures.

A Case of Systemic Lupus Erythematous Associated with Neuromyelitis Optica (Devic's Syndrome) / 대한류마티스학회지

Neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating disease, characterized by optic neuritis and myelitis. NMO is a very uncommon and serious neurologic manifestation of systemic lupus erythematous (SLE). We report a 28-year-old man with NMO as neuropsychiatric manifestation of SLE. He was diagnosed as lupus nephritis at 16-year-old. He had optic neuritis at three years and seven months ago. Oral prednisolone was tapered off according to the improved eye symptoms. Two months later, he visited rheumatology clinics for urinary disturbance and paresthesia on both feet. A spinal magnetic resonance imaging revealed increased signal intensity in T2-weighted images from second to sixth cervical level and from eleventh to twelfth thoracic level. We diagnosed neuromyelitis optica and treated with intravenous cyclophosphamide therapy monthly for three times. He was discharged without any neurological deficits and has been followed up.