Inflammatory demyelinating diseases comprise a series of autoimmune disorders affecting myelin at the level of the central nervous system (CNS) and peripheral nervous system. They can be monophasic, multiphasic, progressive, monofocal or multifocal, and their diagnosis is usually of exclusion. We report the case of a 15-year-old female patient with headache, ataxia, hemiparesis, ophthalmoplegia and altered consciousness. Magnetic resonance imaging showed lesions compatible with demyelinating disease. Acute disseminated encephalomyelitis was diagnosed. The patient responded to treatment with corticosteroids.
Acute disseminated encephalomyelitis
Desmielinización del Sistema Nervioso Central Asociado a Encefalomielitis Diseminada Aguda: Reporte de un Caso. Demyelination Of Central Nervous System Associated With Acute Disseminated Encephalomyelitis: Case Report.
Palabras clave: encefalomielitis diseminada aguda,
Enfermedad desmielinizante,
Encefalomielitis Aguda Diseminada: Formas de Presentación y Espectro Clínico. Serie de Casos.
Acute disseminated encephalomyelitis is an inflammatory demyelinating disease of the central nervous system. Although it often follows an infection or immunization, there are some cases without previous history. It develops usually as a monophasic disease, however recurrent and multiphasic cases have been reported. Its diagnosis is suggested based on clinical features, magnetic resonance imaging and negative complementary test results. We report 5 cases of acute disseminated encephalomyelitis illustrating its wide clinical and prognostic variety. Both mild and significant cases are reported. A previous history of infection was present only in one case. No patient developed symptoms suggesting multiple sclerosis during an 18 to 48 months follow-up. Conclusions: Acute disseminated encephalomyelitis is an uncommon condition that presents with a variety of symptoms. The major differential diagnosis is MS; however other demyelinating and infectious processes must be included. Treatment options include intravenous corticoids, immunoglobulins or plasmapheresis. The patient’s response to treatment is proportional to the prognosis.
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Palabras clave: Encefalomielitis aguda diseminada,
esclerosis múltiple,
Encefalomielitis Aguda Diseminada: Reporte de un Caso con Afectación Selectiva de Tallo Cerebral.
Introduction: Acute Disseminated Encephalomyelitis (ADEM) is an autoimmune demyelinating disease of the central nervous system´s white matter; it usually has a monophasic course with an idiopathic cause or following infection or immunization processes.
Objective: This article has the purpose to present a clinical case with selective involvement of brainstem. We describe a 28 year old woman who developed ADEM. Her clinical presentation, neuroradiological findings and treatment are reported.
Conclusions: ADEM is an infrequent disease which can be fatal. MRI findings confirm the diagnosis and steroid therapy appears to be the most effective treatment, although the disease may spontaneously improve.
