Spectrum of pediatric acquired demyelinating syndromes (PADS) of the central nervous system in a tropical developing country: A 10-year retrospective study.

BACKGROUND

Epidemiological data on the pediatric acquired demyelinating syndromes (PADS) in the Philippines has not been described previously in the literature. There may be geographic differences in frequencies of PADS where true disease burden is not known or underestimated in resource-limited settings due to lack of case recognition and/or diagnostic facilities. The purpose of this study was to determine the frequencies and clinical characteristics of the different subtypes of PADS at our institution and compare these with those in published literature.

METHODS

We conducted a retrospective cross-sectional study of children diagnosed with PADS who were admitted at the Philippine General Hospital from January 2009 to December 2018. Charts of these patients were reviewed to determine frequencies, clinical profile, diagnostic findings and outcomes.

RESULTS

A total of 77 patients were identified with PADS using the appropriate diagnostic criteria. The frequencies of the PADS subtypes were the following: transverse myelitis (n = 21; 27.3 %); acute disseminated encephalomyelitis (n = 20, 26.0 %); multiple sclerosis (n = 17, 22.1 %); optic neuritis (n = 13, 16.9 %); clinically isolated syndrome (n = 4, 5.2 %); and neuromyelitis optica spectrum disorder (n = 2, 2.6 %). Overall, the mean age at initial event and at diagnosis were 10.6 ± 4.6 years. Female:male ratio was 1.02:1. On admission, the majority of patients had motor paralysis (n = 49, 63.6 %) while several patients manifested with sensory deficits (n = 31, 40.3 %), visual changes (n = 26, 33.8 %) and brainstem involvement (n = 20, 26.0 %). Nearly all patients had evidence of lesions in magnetic resonance imaging (n = 72, 93.5 %) located in spinal cord (n = 25; 32.5 %), cerebral white matter (n = 24; 31.2 %), and optic nerve (n = 12, 15.6 %). Among patients who underwent cerebrospinal fluid analysis (n = 34), 7 patients had abnormal findings (20.6 %). The most utilized treatment regimens during admission were intravenous methylprednisolone (n = 53, 68.8 %) and oral prednisone (n = 43, 55.8 %). The majority had partial recovery (n = 56, 72.7 %) and 16 experienced full recovery (20.8 %) at discharge. Five patients died (6.5 %).

CONCLUSIONS

Our study provided the first comprehensive summary on the clinical features of children with PADS admitted in a Philippine tertiary hospital with limited resources. Our study highlights the value of using clinical diagnostic criteria in improving case recognition especially in low-and middle-income countries. Regional disparities in disease burden warrant international registries with wider geographic representation in order to come up with diagnostic and management guidelines suitable for various levels of care.