Life-saving decompressive craniectomy for acute disseminated encephalomyelitis in a child: A case report

摘要

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating immune-mediated disorder which is more common in the pediatric population. It is often associated with a viral or bacterial infection or as a complication after vaccination. The pathophysiology involves transient autoimmune response directed at myelin or other self-antigens, possibly by molecular mimicry or by nonspecific activation of autoreactive T cell clones [2, 10]. Acute clinical presentation is characterized by a rapid onset of encephalopathy, multifocal neurological features, convulsions, and impaired consciousness with fever. There are no biological markers of the disease. Brain and spinal MRI is the imaging modality of choice to point out white matter lesion in ADEM. Mortality is actually lower than 5 % in children [2, 14]. Prognosis is more serious in patients hospitalized in the intensive care unit: mortality can reach 25 % [13].