ABSTRACT
Acute disseminated encephalomyelitis (ADEM) is classified in the group of inflammatory demyelinating diseases of the central nervous system (CNS), and it is generally considered a complication of infections or vaccinations. The outcome can be favorable with spontaneous recovery, but pharmacological treatment is required in most cases. The best therapeutic option has not yet been established by controlled trials1,2, but high-dose steroids are usually employed, resembling treatment of multiple sclerosis relapse. In a few cases the disease is particularly aggressive, leading to severe disability or death, in spite of early steroid administration. Although ADEM is classically considered a monophasic disease of the CNS, it is now accepted that both disseminated and site-restricted ADEM variants3 could show a multiphasic or chronic course4,5, and could be complicated by severe impairment of the peripheral nervous system. In these cases a chronic or ‘pulsed’ pharmacological treatment could be required, but treatment choice is difficult as chronic steroids are often contraindicated and other immunosuppressive drugs, such as azathioprine and cyclophosphamide, are not advisable owing to severe toxicity and poor effectiveness. Further options are, at present, not available. During the past decade some authors have reported positive results following intravenous immunoglobulin (IVIG) administration as a second-line treatment in both pediatric and adult series of steroid-resistant ADEM cases6-12.
