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Acute transverse myelitis in childhood: A single centre experience from North India
Renu Suthar1, Naveen Sankhyan1, Jitendra K Sahu1
1Unit of Pediatric Neurology and Neurodevelopment, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
Insights
Acute Transverse Myelitis (ATM) in children is often severe and impacts long spinal cord segments. Delayed diagnosis and treatment are linked to poor outcomes, with nearly half of affected children requiring ongoing assistance.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Clinical Neuroscience
Background:
- Acute Transverse Myelitis (ATM) is a rare and debilitating neurological condition affecting children.
- Limited data exists on the clinical characteristics, prognosis, and predictive factors of ATM in developing nations.
Purpose of the Study:
- To elucidate the clinical profile of children diagnosed with ATM.
- To identify predictors associated with the outcome of ATM in pediatric patients.
Main Methods:
- A retrospective analysis was conducted on pediatric patients (<12 years) diagnosed with ATM.
- Data was collected over a six-year period from a tertiary care institution.
- Clinical presentation, MRI findings, and treatment outcomes were assessed.
Main Results:
- Thirty-six children were diagnosed with ATM, with a median age of 7.5 years.
- The majority presented with severe weakness, sensory level, and bladder dysfunction.
- Longitudinal Extensive Myelitis (LETM) was observed in 75% of cases, predominantly affecting the thoracic cord.
- At follow-up (median 35 months), 41.7% of children had persistent ambulation deficits.
- Severe onset weakness, spinal shock, respiratory compromise, delayed diagnosis, and treatment initiation were associated with poor outcomes.
- ATM was typically monophasic, with no progression to multiple sclerosis observed.
Conclusions:
- Childhood ATM in North India is characterized by severity, monophasic course, and extensive cord involvement.
- A significant proportion of children experience long-term functional dependence.
- Timely diagnosis and prompt initiation of steroid therapy are crucial for improving outcomes in pediatric ATM.
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.