Disseminated acute encephalomyelitis with fatal evolution: clinicopathological study
*Correspondencia: Dra. Arantxa Ortega-Aznar. Servicio de Neuropatolo- gía. Departamento de Anatomía Patológica. Hospital Vall d’Hebron. Pas- seig de la Vall d’Hebron, 119-129. E-08035 Barcelona.
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Introduction. Disseminated acute encephalomyelitis is a monophasic demyelinating disease which progresses rapidly and is often fatal. It is an autoimmune condition, mediated by T lymphocytes, in which the immune response is directed against the myelin antigens. Clinical case. We describe the clinical, radiological and neuropathological findings in the case of a 31 year old woman who, ten days after complaining of a clinical condition of upper respiratory tract inflammation, presented with unilateral focal neurological signs, subsequent rapid deterioration of consciousness and death. On autopsy the neuropathological characteristics of disseminated acute encephalomyelitis were seen. Conclusions. The relative rarity of this condition at the present time makes clinical diagnosis difficult. The differential diagnosis with other conditions may be difficult also. Thus, this disease often leads to a neuropathological diagnosis.
Conclusiones La relativa poca frecuencia de esta enfermedad en la actualidad y el hecho de que el antecedente infeccioso puede pasar desapercibido, dificultan el diagnóstico clínico. Junto a esto cabe tener en cuenta que el diagnóstico diferencial con otras entidades puede ser difícil. Todo ello hace que esta enfermedad represente con frecuencia un diagnóstico neuropatológico.
