Acute disseminated encephalomyelitis: an acute hit against the brain

Abstract

Capitalizing on experience based on a large body of well characterized patient data collected both cross-sectionally and longitudinally, pharmacotherapy has been improved and mortality and comorbidities due to ADEM have been reduced. Unfortunately, the pathogenic events that trigger the initial clinical attack, and possibly pave the way for ongoing relapsing disease, remain unknown. Clinically applicable diagnostic criteria are still lacking.

Cerebrospinal fluid analysis in the context of CNS demyelinating diseases

“…Usually, ADEM is monophasic and asso ciated with a temporal and probably also with a causative relationship to infection (e.g. measles, herpes, varicella, mumps, Epstein-Barr or influenza) as well as to preventive vaccination, although the development of vaccines that are based on recombinant proteins, which are not contaminated with undue amounts of myelin antigens, has significantly lowered its related incidence 32 . Typically, the clinical syndrome begins with fever, headache, vomitus and meningeal signs, followed by consciousness and behavioral disturbances, seizures and focal neurological signs.…”

“…Acute disseminated encephalomyelitis is an inflammatory demyelination disease of the CNS, encompassing the white and gray matter of the brain and spinal cord, with a distinct tendency to a perivenous localization of pathological chan ges 31,32 . Children are mostly affected, with the median age of 6.5 years 33 .…”

Acute disseminated encephalomyelitis following inactivated influenza vaccination in the Brazilian Amazon: a case report

“…For steroid-unresponsive patients, plasma exchange or intravenous immunoglobulin may be used. Poor outcome of treatment is associated with older age, female sex, degree of functional impairment at clinical onset, CSF protein level, spinal cord involvement, peripheral nervous system damage, and poor response to corticosteroids (5) . A few patients may either have recurrences that respond to glucocorticoid therapy or are at risk for development of multiple sclerosis.…”

“…Acute disseminated encephalomyelitis has no pathognomonic clinical features. However, a combination of neurological symptoms in particular temporally associated with an infection or vaccination should alert the clinician of a possible diagnosis of ADEM (5) . Its clinical presentation includes fever, altered consciousness, and multifocal neurological fi ndings.…”

“…The rate of ADEM cases diagnosed following vaccination in a biologically plausible risk interval is as low as 0.1-0.2 per 100,000 vaccinated individuals of any age (5) . However, it is more common in children.…”