Intravenous gammaglobulin therapy in recurrent acute disseminated encephalomyelitis

Abstract

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder of the CNS that seems to be immune mediated. [1,2] It is usually preceded by a viral syndrome and presents with acute neurologic signs. [1] ADEM is usually monophasic, but recurrent episodes may occur. [3] When ADEM is recurrent, the distinction from MS becomes difficult. [2] Corticosteroids are commonly used in ADEM and are thought to shorten the duration of the neurologic symptoms. [4] We report on the use of intravenous gammaglobulin (IVIG) therapy for ADEM after failure of corticosteroids. An 8-year-old previously healthy boy developed headaches and fever 2 weeks before admission to Packard Children's Hospital at Stanford. He was admitted to a community hospital. Vital signs were normal except for a temperature of 100.9 degrees F. There were no signs of meningeal irritation. LP showed 23 WBC/mm3 (34% polymorphonuclear cells, 45% lymphocytes, 21% monocytes), 0 RBC/mm3, with a protein of 23 mg/dL and a glucose of 63 mg/dL. MRI showed prominent leptomeningeal enhancement but no other lesions. He was diagnosed with viral meningitis and treated with IV acylovir for 9 days. Headaches worsened despite analgesics, and he was transferred to the Children's Hospital. On examination, he was awake but in significant distress from a severe headache. His speech and comprehension were normal. Vital signs were normal except for a temperature of 100.7 degrees F. The general examination was unremarkable. There were several beats of ankle clonus, and the plantar responses were extensor bilaterally. The …