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Title: [Encephalopathy and neuromyelitis optica: the importance of recognising atypical symptoms].
Author: Hervás-García JV, Grau-López L, Doménech-Puigcerver S, Ramo-Tello C.
Journal: Rev Neurol; 2014 Jan 01; 58(1):20-4. PubMed ID: 24343537.
Abstract:
INTRODUCTION: Neuromyelitis optica (NMO), or Devic's disease, is an autoimmune, inflammatory and demyelinating disease of the central nervous system which mainly and characteristically involves the optic nerve and the spinal cord. Anti-aquaporin-4 (AQ-4) antibodies are a specific biomarker of the entity and, since their discovery, both the number of symptoms and the radiological data about the disease have progressively increased, and the concept of clinical spectrum of NMO has been defined. CASE REPORT: A 66-year-old female diagnosed with NMO after suffering attacks of optic neuritis and recurrent myelitis, and showing positive for AQ-4 antibodies. The patient presented a diminished level of consciousness, and magnetic resonance imaging of the head revealed a number of lesions in the white matter, without contrast enhancement, which resolved without treatment. One month later, her general state had declined and was accompanied by symptoms of confusion and blindness. A magnetic resonance imaging scan showed new lesions in the white matter and an increase in the size of those already present. The patient was diagnosed as suffering from encephalopathy within the context of NMO and she was treated with intravenous immunoglobulins and corticoids, which resulted in a clinical and radiological improvement. CONCLUSIONS: Since the discovery of AQ-4 antibodies, there has been an increase in the number of clinical and radiological manifestations of NMO beyond involvement of the optic nerve and the spinal cord, including manifestations in the brain. It is important to recognise them in order to make an early diagnosis, to avoid unnecessary complementary tests and to establish the most suitable treatment. TITLE: Encefalopatia y neuromielitis optica: importancia del reconocimiento de la sintomatologia atipica. UNLABELLED: Introduccion. La neuromielitis optica (NMO) o enfermedad de Devic es un trastorno autoinmune, inflamatorio y desmielinizante del sistema nervioso central, que afecta principal y caracteristicamente al nervio optico y a la medula espinal. Los anticuerpos antiacuaporina-4 (AQ-4) son un biomarcador especifico de esta entidad y, desde su descubrimiento, se ha ampliado el numero de sintomas y datos radiologicos de la enfermedad y se ha definido el concepto de espectro clinico de NMO. Caso clinico. Mujer de 66 años diagnosticada de NMO por haber sufrido brotes de neuritis optica y mielitis de repeticion junto con anticuerpos AQ-4 positivos. Presento un cuadro de disminucion del nivel de conciencia, con resonancia magnetica cerebral que mostro multiples lesiones en la sustancia blanca, sin realce de contraste, que se resolvio sin tratamiento. Un mes despues, sufrio empeoramiento del estado general, sindrome confusional y ceguera. En la resonancia magnetica cerebral se observaron nuevas lesiones en la sustancia blanca y aumento del tamaño de otras ya existentes. Se emitio el diagnostico de encefalopatia en el contexto de NMO y se trato a la paciente con corticoides e inmunoglobulinas intravenosas, con lo que se produjo mejoria clinica y radiologica. Conclusiones. Desde el descubrimiento de los anticuerpos AQ-4, ha aumentado el numero de manifestaciones clinicas y radiologicas de la NMO mas alla de la afectacion del nervio optico y de la medula espinal, entre ellas las manifestaciones cerebrales. Reconocerlas es muy importante para hacer un diagnostico precoz, evitar pruebas complementarias no necesarias e instaurar el tratamiento adecuado.

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