Pediatric Inflammatory Diseases

Abstract

Acute post-infectious immune disorders include Acute Disseminated Encephalomyelitis (ADEM) and its variants such as Acute Hemorrhagic Encephalomyelitis (AHEM), acute necrotizing hemorrhagic leukoencephalitis (ANHLE) of Weston Hurst, multiphasic and recurrent ADEM. Acute Necrotizing Encephalopathy of Childhood (ANE or ANEC) represents a dramatic event, consequent to viral infections, especially Influenza-A, and is now considered different from ADEM. ADEM and variants are classically described as uniphasic syndrome occurring in association with an immunization or vaccination (postvaccine encephalomyelitis) or systemic viral infection (parainfectious encephalomyelitis). However, multiphasic forms are not rare. Pathologically, there is perivascular inflammation, edema, and demyelination within the CNS. Clinical features are focal or multifocal neurologic disorder following exposure to virus or receipt of vaccine. The onset of the CNS disorder is usually rapid and include encephalopathy ranging from lethargy to coma, seizures, and focal and multifocal signs reflecting cerebral and spinal cord involvement. The mortality rate is estimated at 10 to 30 percent, with complete recovery rates of 50 percent cited. Poor prognosis is correlated with severity and abruptness of onset of the clinical syndrome. Multifocal CNS lesions are generally evident on MRI that can be similar from those observed in MS.