Neuromielitis óptica de Devic: análisis de siete casos
Summary. Introduction. Devic’s optic neuromyelitis is an unusual condition characterized by the association of unilateral or bilateral optic neuritis and myelitis, both of which are relapsing. Prognosis is usually poor, both for the optic nerve and for the spinal medulla which becomes cavitated. This leads to severe handicap and deterioration in quality of life, except in cases presenting as children. Patients and methods. We studied seven cases of this condition and describe their clinical and neuroimaging features, cerebrospinal fluid (CSF), evaluation, complications, prognosis and treatment. This data was compared with that in the literature. Results and conclusions. 1. It is an unusual condition –only 7 cases have been seen at our centre– making up approximately 0.1% of the inflammatory pathology of the spinal cord seen. 2. During the first few hours or days the clinical and radiological findings may not correspond. There may be marked deterioration of CNS function but with normal neuroimaging findings. This may lead to serious problems in diagnosis, which can usually be resolved by repeating the investigation. 3. Differential diagnosis should be made with intramedullary tumors when on neuroimaging there is increased spinal cord diameter with uptake of contrast, and psychiatric disorders when this is normal. 4. The condition usually deteriorates leading to severe disability, since loss of visual acuity is added to the severe spinal cord lesion. 5. Diagnosis should be made by application of the criteria of clinical evolution, imaging and biochemistry defined in the literature, although firm diagnosis can only be made on anatomopathological studies, usually at autopsy;
Pacientes y métodos Se estudian siete casos de esta entidad, describiéndose sus características clínicas, de neuroimagen, líquido cefalorraquídeo (LCR), curso evolutivo, complicaciones, pronóstico y tratamiento. Se comparan los datos obtenidos con los de la literatura. Resultados y conclusiones. 1. Es una entidad poco frecuente –sólo 7 casos recogidos en nuestro centro–, lo que constituye aproximadamente un 0,1% de nuestra patología inflamatoria medular; 2. En las primeras horas o días puede no haber correspondencia clinicorradiológica, coexistiendo un grave deterioro funcional del SNC con neuroimagen normal, lo que puede generar dudas diagnósticas que se resolverán habitualmente al repetir la prueba; 3. Es preciso establecer el diagnóstico diferencial con tumores intramedulares cuando en la neuroimagen se visualice un aumento del diámetro medular que capta contraste, y con cuadros psicógenos si ésta es normal; 4. El curso suele ser progresivo hacia una gran discapacidad, pues a la gravedad de la lesión medular se le suma la derivada de la pérdida de agudeza visual; 5. El diagnóstico debe realizarse aplicando los criterios clinicoevolutivos, iconográficos y bioquímicos definidos en la literatura, aunque la certeza sólo se consigue mediante estudio anatomopatológico, generalmente con autopsia
