Acute idiopathic transverse myelitis in children: Early predictors of relapse and disability

Deiva et al. published a study in 2014 describing predictors of relapse and disability in a pediatric sample of transverse myelitis. They included children who were 16 years or younger who had a first episode of acute idiopathic TM. Children were from 12 neuropediatric centers in France and from 3 centers in the United Kingdom who had presented between January 2004 and December 2011a. They also defined cases as having definite or probable TMb. At the last follow-up with the patients, children were assessed as either having a monophasic or relapsing disease course. Children were also rated as having a poor outcome if their ASIA score was less than D, or their Expanded Disability Status Score was greater than or equal to 4.

108 children were identified for this study, but 13 were excluded because they either had normal CSF or MRI or their data was missing, leaving 95 children in the sample. 67 of these children had definite TM, while 28 had probable TM. Of these 95 children, 16 ended up having relapsing disease (13 with MS, 3 with AQP4-positive NMO), and their relapses occurred relatively quickly after the first attack (median 3.5 months). There was a greater percentage of children who reached ultimate disability in under 24 hours in the monophasic group than in the relapsing group. Also, the percentage of children who had brain lesions at onset was greater in the relapsing group than in the monophasic group. Furthermore, 30% of the children had a poor outcome, and this was associated with time to ultimate disability of less than 24 hours, and sphincter dysfunction.

They found two risk factors for relapsing disease: abnormal brain MRI at onset (those with an abnormal brain MRI were almost 14 times more likely to have relapsing disease than those with a normal brain MRI) and female sex (females were 3 times more likely to have relapsing disease than males). They also found 5 risk factors for poor outcome, which included a severe ASIA score at onset, spinal lesion with gadolinium enhancement, female sex, CSF pleocytosis <10 cells/mm, and no cervical or cervico-thoracic lesion.

Relapsing Disease

Monophasic TM

Poor Outcome

Good Outcome

The authors do note that the follow-up time for the monophasic group was significantly shorter than the follow-up time for the relapsing group, so it is possible that more children in the monophasic group would have eventually been diagnosed with relapsing disease. Though the authors believe that the follow-up time for the monophasic group was long enough to monitor most of the relapsing cases.

a They included cases if they fit these criteria: (1) sensory, motor, or autonomic dysfunction attributable to the spinal cord (2) bilateral signs or symptoms but not necessarily symmetric (3) progression to most disability less than 21 days following the onset of symptoms and (4) absence of symptoms from to an acquired brain demyelinating syndrome.

b Definite TM included cases that had either (1) CSF pleocytosis greater than or equal to 10 leukocytes/mm3 (2) elevated immunoglobulin G index or presence of intrathecal oligoclonal bands or (3) MRI spinal gadolinium enhancement. Cases were considered probable TM if they did not meet the above criteria or if they did not have a lumbar puncture or spinal gadolinium MRI.

Original research: Deiva K et al. Acute idiopathic transverse myelitis in children: Early predictors of relapse and disability. Neurology. 2014;84:1-9.