Longitudinally Extensive Transverse Myelitis: One Disease, Variable Outcomes-A Case Series

Area of Science:

• Neurology
• Pediatrics
• Immunology

Background:

• Longitudinal extensive transverse myelitis (LETM) is a rare, severe spinal cord inflammation.
• LETM presents acutely with sensory, motor, and autonomic dysfunction, often with poor prognosis.
• Pediatric LETM cases exhibit diverse clinical presentations and etiologies.

Purpose of the Study:

• To present a case series of three children diagnosed with LETM.
• To highlight the varied clinical manifestations and diagnostic challenges of pediatric LETM.
• To discuss the role of immunomodulatory therapies in managing LETM.

Main Methods:

• Case series analysis of three pediatric patients (ages 4-13) with LETM.
• Clinical assessment, neurological examination, and contrast-enhanced MRI spine were performed.
• Treatment involved pulsed dose methylprednisolone, intravenous immunoglobulin, and oral prednisolone.

Main Results:

• Patients presented with diverse symptoms including vision loss, seizures, abdominal pain, urinary retention, constipation, and limb weakness.
• Spinal MRI revealed long segment T2 hyperintensity in all cases, with some brain involvement.
• Two of three children showed faster recovery with early immunomodulatory treatment.

Conclusions:

• LETM in children has varied presentations and etiologies.
• Testing for Aquaporin-4 IgG and anti-myelin oligodendrocyte glycoprotein antibodies is recommended.
• Prompt and aggressive immunomodulatory therapy can facilitate recovery in pediatric LETM.