Acute hemorrhagic leukoencephalitis associated with autoimmune myopathy

Acute hemorrhagic leukoencephalitis is a rare acute inflammatory myelinopathy of central nervous system with high mortality. We report a case of an unusual presentation of acute hemorrhagic leukoencephalitis with autoimmune myopathy and a complete recovery with steroids and plasmapheresis.

A 24-year-old female admitted with generalized seizure, lethargy, but no focal neurological signs. Head scans revealed right frontal hypodensity with loss of basal cisterns, mild transfalcine shift to the left, a mass lesion with abnormal signal and multiple small hemorrhages. Biopsy pathology showed white matter demyelinating lesions with necrotizing destruction of small vessels and acute inflammation. EMG was consistent with demyelinating diffuse polyneuropathy and myopathy. Pathology of muscle showed myopathic changes suggestive of autoimmune myopathy.

Patient was initially treated with Dexamethasone, Mannitol, Keppra, Antibiotics and Acyclovir. Later when she developed diffuse polyneuropathy and myopathy, she was given plasmapheresis. The patient responded to the treatment and made a full recovery.

Acute hemorrhagic leukoencephalitis is a rare and usually fatal disorder. The etiology of AHLE remains clear; cross-reactivity between human myelin antigens and viral or bacterial antigens is thought to initiate an immune process causing demyelination. Usually the autoimmune process targets CNS myelin and spares the peripheral; however, in this case there was diffuse involvement of central and peripheral myelin and muscle.