Acute transverse myelitis in childhood: A single centre experience from North India

Abstract

Background: Acute transvers myelitis (ATM) is a rare and disabling condition in childhood. There are only few reports of clinical profile, prognosis and predictors of ATM from developing countries.

Objective: To study the clinical profile of children with ATM and predictors of its outcome.

Method: Retrospective analysis of children <12 years of age diagnosed with ATM over a period of 6 years from a tertiary care institute.

Results: Thirty six children (21 boys, median age-7.5 years) were diagnosed with ATM. Weakness was symmetrical at onset in 27 (75%) children with progression over a median of 2 days (IQR 1-5 days). Severe weakness at onset with lower limb power ≤ 1/5 on MRC scale was present in 27 (75%), a sensory level in 25(69.4%) and bladder dysfunction in 31(86.1%) children. MRI showed longitudinal extensive myelitis (LETM) in 27 (75%) children and the thoracic cord was most commonly affected [18 (50%)]. On a median follow up of 35 months (range IQR 11-57 months); 15 (41.7%) were non ambulatory or required assistance to walk. Severe weakness at onset with power ≤ 1 on MRC scale, spinal shock, respiratory muscle weakness, mechanical ventilation, greater mean time to diagnosis and treatment was associated with bad outcome. ATM was a monophasic illness in all, except in 3 children; all with neuromyelitis optica spectrum disorder. Progression to multiple sclerosis was not seen in any child in our cohort.

Conclusion: In this series of childhood ATM from North India, the disease was severe, monophasic and involved long segments (≥ 3) of cord in majority. Nearly half the children remain dependent on follow up. Delayed diagnosis and delayed initiation of steroid therapy was associated with poor outcome.

Keywords: Acute transvers myelitis (ATM); Children; Longitudinal extensive transverse myelitis (LETM); Neuromyelitis optica spectrum disorders (NMODS); Outcome.