Clinico-radiological profile of acute disseminated encephalomyelitis in the childhood population. A retrospective analysis of a series of 20 patients in a tertiary hospital

Autores de IIS La Fe

• Miguel Tómas Vila

  Autor

• Francisco Menor Serrano

  Autor

• Maria Del Carmen Otero Reigada

  Autor

• Desamparados Perez Tamarit

  Autor

• Montserrat Tellez De Meneses Lorenzo

  Autor

• Inmaculada Pitarch Castellano

  Autor

Abstract

Introduction. Acute disseminated encephalomyelitis (ADE) is an inflammatory disorder of the central nervous system that is mediated immunologically and of unknown pathogenesis. It can present at any age, but is much more frequent in children. ADE has no specific biological marker and diagnosis is based on findings from clinical and neuroimaging studies. Aim. To enhance our knowledge of the clinico-radiological profile of this disease. Patients and methods. This retrospective study involved patients under 14 years of age who were admitted to a tertiary hospital over the last 15 years with a diagnosis of ADE. History, clinical presenting symptoms, lab findings from blood/cerebrospinal fluid analyses and radiological semiology were reviewed. In 16 cases an average follow-up of 25 months was performed. Results. The study examined 20 patients, 70% children, with a mean age of 4.4 years. Forty per cent had a previous febrile episode. Eighty-five per cent presented fever or vomiting, and 70% had altered states of mind. Motor deficits (45%), convulsions (35%) and involvement of the cranial nerves (30%) were predominant. Three children progressed with relapses and three others were left with motor sequelae. Magnetic resonance imaging showed hyperintense lesions in T 2, with a pattern of scarce/no enhancement, which were predominantly located in the thalamus (70%), the spinal cord (67%) and the white matter of the sub-cortex (50%). Haemorrhagic ADE was diagnosed in two patients. Conclusions. ADE is a condition with an important degree of general involvement and neurological repercussions, as well as considerable potential to leave the patient with sequelae. Clinico-analytical data and magnetic resonance scans of the head and spinal cord are relevant for the initial diagnosis and follow-up of patients with ADE.

Keywords

• Acute disseminated encephalomyelitis; Magnetic resonance brain scan; Methylprednisolone; Multiple sclerosis; Recurrence; Sequelae