Abstract
Objective: We report a case of Unusual presentation of acute hemorrhagic leukoencephalitis with autoimmune myopathy, this association has never been reported in the literature.Patient responded well with Steroid and plamapheresis and made a complete recovery. Background Acute hemorrhagic leukoencephalitis(AHLE) is a rare acute inflammatory myelinopathy of the central nervous system (CNS) with high mortality, its association with autoimmune myopathy has not been reported before .This provide a strong evidence of CNS & peripheral nervous system involvement. Design/Methods: A 24 yr old female admitted with generalized seizure. She was lethargic, but moving all four extremities, without any focal neurological signs. Her Serum chemistries were all normal. MRI of Brain demonstrated mass lesion with abnormal signal & multiple small hemorrhages.Pathology showed white matter demyelinating lesions with necrotizing destruction of small vessels and acute inflammation. Later on pt developed severe paraplegia. EMG was consistent with demyelinating diffuse polyneuropathy &myopathy.Pathology of muscle showed myopathic changes suggestive of autimmune myopathy. Results: Pt was initially treated with Dexamethasone, Keppra, Antibiotics & Acyclovir. But when she developed diffuse polyneuropathy & myopathy, she was given Plasmapheresis. The patient responded to treatment and made a full recovery. Conclusions: The etiology of AHLE remains unclear ,with a prevailing theory that infectious agents trigger inflammatory cascade targeting myelin basic protein.Usually the antigen target Central nervous system myelin and spare the peripheral and vice versa in this case there was diffuse involvement of central/peripheral myelin and muscle,this require further investigation in autoimmune process and consistent with peripheral demylination some time seen in Multiple Sclerosis. Disclosure: Dr. Ahmed has nothing to disclose. Dr. Ahmed has nothing to disclose.
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