Clinical and Radiological Profiles, Treatment, and Outcome of Pediatric Acquired Demyelinating Disorders of Central Nervous System

Vykuntaraju K Gowda; Deepthi Shetty; Bhaskar V Madivala; Naveen Benakappa; Asha Benakappa

doi:10.4103/jpn.JPN_62_18

Clinical and Radiological Profiles, Treatment, and Outcome of Pediatric Acquired Demyelinating Disorders of Central Nervous System

J Pediatr Neurosci. 2019 Apr-Jun;14(2):76-81. doi: 10.4103/jpn.JPN_62_18.

Authors

Vykuntaraju K Gowda¹, Deepthi Shetty², Bhaskar V Madivala³, Naveen Benakappa², Asha Benakappa²

Affiliations

¹ Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.
² Department of Pediatrics, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.
³ Department of Radiology, Sparsh Super Speciality Hospital, Bengaluru, Karnataka, India.

Abstract

Introduction: Demyelinating disorders of central nervous system are rare childhood disorders that cause significant physical and cognitive disabilities. Early diagnosis and appropriate treatment determines prognosis and outcome.

Objective: The objective of this work was to study clinical profile, investigative and radiological features, treatment, and outcome of children with demyelinating disorders.

Materials and methods: A retrospective chart review of 32 children with demyelinating disorders admitted in a tertiary-care pediatric hospital from Bangalore between November 2013 and October 2017 was conducted. Sociodemographic data, clinical features, cerebrospinal fluid (CSF) findings, radiological features, treatment received, and outcome were collected and results were analyzed.

Results: Among 32 patients of demyelinating disorders, majority were acute disseminated encephalomyelitis (ADEM) (15, 46.9%), followed by transverse myelitis (9, 28.1%), multiple sclerosis (MS) (3, 9.4%), optic neuritis (3, 9.4%), and neuromyelitis optica (NMO) (2, 6.2%). Mean age of presentation was 7.8 years. Among 32 patients, 15 were male (47%). In patients with ADEM, the mean age of presentation was 5.5 years. Two patients had tumefactive ADEM and 10 recovered after steroid therapy. In patients with MS, the mean age of presentation was 10.6 years; one patient died and the condition of two patients improved. In patients with transverse myelitis, the mean age of presentation was 8.9 years and seven patients recovered. In a patient with NMO, CSF was positive for antibody to aquaporin-4. The child improved with steroids and intravenous immunoglobulin. Three patients with optic neuritis were treated with steroids and they recovered.

Conclusion: Even though demyelinating disorders are rare in pediatric age group, one should be aware of this entity as early diagnosis and treatment improves outcome. Most common among them is ADEM.

Keywords: ADEM; demyelinating disorders; multiple sclerosis; neuromyelitis optica; optic neuritis; transverse myelitis.