Childhood CNS inflammatory demyelinating diseases

CNS inflammatory demyelinating diseases (CIDD) are rare. At first presentation children are diagnosed with acute disseminated encephalomyelitis (ADEM) or a clinically isolated syndrome (CIS) such as transverse myelitis (TM) or optic neuritis (ON). Many of these disorders are monophasic, however a small number of children relapse and are diagnosed with multiple sclerosis (MS) or neuromyelitis optica (NMO). Paediatric onset Multiple Sclerosis (POMS) is a chronic inflammatory neurodegenerative demyelinating disease of the CNS that is usually relapsing-remitting at onset. There has been significant recent interest and progress in these disorders. Encephalopathy (behavioural change or altered consciousness) distinguishes ADEM from other demyelinating conditions. A high index of suspicion for CIDD is required in children presenting with neurological deficits, encephalopathy or status epilepticus. Several UK and international studies are underway to further our understanding of these diseases. There has been significant development of new treatments for MS and NMO. A national service for these rare disorders will enable better management of these well deserving patients. In this review we describe current understanding of the epidemiology, pathogenesis, clinical features, outcome and management of childhood CIDD.