Acute disseminated encephalomyelitis is an inflammatory and demyelinating disease of the central nervous system mainly affecting children. In its classical form, it is characterized by the acute or subacute occurrence of multifocal neurological symptoms and encephalopathy secondary to an infection. Although most children have a good prognosis with slow but complete healing, we here describe the case of a 25-month-old girl with a severe form of acute disseminated encephalomyelitis following a primary Epstein-Barr-Virus infection. Her case is particularly unusual, since it was characterized by a rapid and severe extension of the lesions that led to death.
What is already known about the topic?
Acute disseminated encephalomyelitis is a rare disease that usually occurs during childhood and is typically preceded by an infection or vaccination. It combines multifocal neurological symptoms with encephalopathy linked to an inflammatory and demyelinating process of the central nervous system. MRI is the brain imaging gold standard, while corticosteroids seem to be the best therapy. Although diagnosis and treatment are challenging, most children have a good prognosis and obtain a complete cure.
What does this article bring up for us?
The purpose of this article is to draw attention to this rare disease and help clinicians to properly diagnose and treat it. Our case is also atypical, because it was characterized by a severe evolution that led to the child’s death. Its unique presentation enriches the scientific knowledge.
Key words
EBV, infectious mononucleosis, child, demyelinating process, ADEM, lymphocytosis, encephalopathy, neurological deficits, corticosteroids, plasmapheresis