6555 An investigation into the assessment and management of pediatric inflammatory myelopathies: single center experience in a tertiary care hospital, Sri Lanka

Objectives

To comprehensively investigate and document the clinical features, etiology, treatment approaches, and outcomes of transverse myelitis (TM) cases within a tertiary care facility.

Methods

This is a descriptive study conducted at Lady Ridgeway Hospital for children, Colombo, Sri Lanka. Data was extracted from clinical records and interviews.

Results

28 children with TM were included and 31 events of TM were analyzed. Most of the children were females (57.1%). The mean age at diagnosis was 8.7 years (SD 4.1 years). At presentation all had lower limb involvement, while long tract signs (71.0%), gastrointestinal symptoms (71.0%), sensory involvement (67.7%), genitourinary symptoms (51.6%), systemic symptoms (48.4%), autonomic features (48.4%), upper limb involvement (41.9%), headache (41.9%), Narcolepsy and sleep disorders (35.5%), and cerebellar involvement (32.3%) were also detected. Males were more prone to have narcolepsy and sleep disorders (p<0.5) and systemic involvement (p<0.05).¹ Myelin Oligodendrocyte Antibody Associated Disease (MOGAD) (21.4%), Neuromyelitis Optica Spectrum Disorder (14.3%), Acute Disseminated Encephalomyelitis (ADEM) (10.7%), Para-Infectious TM (10.7%), Para-Infectious ADEM (7.1%), Multiple sclerosis (7.1%), and paraneoplastic TM (7.1%) were identified as causes while 21.4% were due to idiopathic TM. MOG antibody was positive in 17.8%. At the peak of the disease, 61.3% were of Grade D in the ASIA impairment scale. 40 cerebrospinal fluid (CSF) reports were analyzed. 72.5% had pleocytosis and the mean white blood cell (WBC) count was 32.7 cells/mm³ (SD 53.6). The maximum cell count was 223 cells with 95% being lymphocytes. The mean CSF protein level was 39.6 mg/dL (SD 26.6); however, only 25% had protein elevation above 40 mg/dL with maximum being 120.4 mg/dL and 50% of them were of ASIA A category. Only one report had a sugar difference of more than 50%. 40% had isolated pleocytosis and 2.5% had isolated sugar difference. None had an isolated elevation of protein. 80.5% had undergone MRI brain and 97.2% had undergone MRI of the spinal cord. All patients with para infectious ADEM had medulla oblongata involvement, longitudinally extensive transverse myelitis, and T2/FLAIR hyperintensities whereas 66.6% had leptomeningeal involvement and T1 hypointensities (p<0.05). Cervical cord involvement was noted in 87.1% followed by thoracic (74.2%) and lumbar (29.0%) cord involvement. The highest response rate was seen in plasmapheresis (91.6%) which was undertaken in 12 followed by methylprednisolone 61.3% which was received by all. Among 22 children treated with iv immunoglobulin, 50% responded to treatment. Prednisolone was the most common prophylaxis (90.3%) used and this was combined with Mycophenolate mofetil in 38.7%. Complications were reported in 83.9% including one death in a patient with leukemia due to neutropenic sepsis and 14% of the survivors had residual bladder problems at the time of the study.

Conclusion

MOGAD was the commonest cause of acute TM in our sample. 72.5% had pleocytosis and 25% had protein elevation above 40 mg/dL. Plasma exchange had the highest response rate. Majority had a good outcome except for 1 death and 14% had residual bladder problems.

Reference

Suthar R, et al. 'Acute transverse myelitis in childhood: A single centre experience from North India,' European Journal of Paediatric Neurology 2016.