Document Type : Poster Presentation

Author

• Mohammad Amin Najafi

Isfahan University of medical sciences, Isfahan, Iran

Abstract

Introduction: Acute disseminated encephalomyelitis (ADEM) is a rare disease of central nervous system with myriads of presentation. We report a case with atypical feature and partial response to treatment.
Case History:   A 39-year-old woman, with no previous relevant medical history, was admitted to the Emergency Department for acute confusional state and gait disorders and urinary retention over the last 48 hours. Two weeks earlier, the patient presented fever, headaches and nausea. On examination, she was drowsy, aphonic, dysphagia, urinary incontinence and flaccid quadriplegia but right-sided dominantly. Deep tendon reflexes were decreased with positive Babinsky sign. Eye movements and fundoscopy was normal. There is no fever, seizures or meningeal irritation. Lumbar puncture was done. Normal CSF analysis and OCBs  was negative. All laboratory tests, including Aquaporin 4 and MOG Antibodies tests were unremarkable.  Brain MRI revealed multiple hyper intense lesions seen in T2-\9+weighted, FLAIR, and PD imaging scans. The lesions may be large and confluent, occupying almost all of the white matter. (Figs.). Cervical MRI seems to be normal.  The diagnosis of ADEM is suggested and treated with high-dose intravenous of methylprednisolone 1 g/d for 5 days. The response was poor. Pulse IV methylprednisolone was repeated again after one week. Despite two courses pulse, there was no change in the patient's symptoms and brain MRI.  So we started plasmapheresis (250 CC/Kg (Every other day for two weeks.  After two months, control MRI before hospital discharge demonstrated partial regression of the brain hyperintensity. The patient recovered partially within six months.
Conclusion: The outcome of ADEM in most cases is good. We report a  rare case with refractory  to treatment. 

Keywords

• Acute disseminated encephalomyelitis
• case report