Spinal Cord Involvement in Children with Acquired Demyelinating Syndromes: A Single Centre Study

Abstract

Abstract Background Myelitis may be the first presentation of relapsing acquired demyelinating syndromes (ADS) such as neuromyelitis optica (NMO) or multiple sclerosis (MS) and in association with connective tissue diseases. It may also occur in the context of acute disseminated encephalomyelitis (ADEM). Early recognition of myelitis in these disorders will allow better management plan including theprognosis. Objective To evaluate the frequency of myelitis among children with acquired demyelinating neurological diseases, and the variability of clinical features, neuroimaging findings and treatments given. Methods A pilot observational study with both retrospective and prospective components was conducted on children and adolescents fulfilling the diagnostic criteria for any of the acquired demyelination syndromes admitted inChildren’s Hospital, Ain Shams University whose ages were below 18 years during the period from July 2020 to February 2022. Results Forty-three children were included in our study with mean (SD)age of onset 7.63 (4.28) years. The most common diagnosis was ADEM (n (%) =20 (46.5%), followed by transverse myelitis (TM) (n (%) =11 (25.6%) and NMO (n (%) =7(16.3%). Progression to develop encephalopathy was recorded in 55.8%of cases (100% of ADEM and autoimmune encephalitis (AE) cases). Seizures developed in 23.3% of cases (35% of ADEM, 50% of AE and 14.29% of NMO cases). MRI brain abnormalities were observed in 76.7% of cases (100% of ADEM, NMO, AE and MS cases), and abnormalities of MRI spine were seen in 59% of cases (100% of TM and MS cases, 85.7% of NMO, 33.3% of AE and 23.5% of ADEM cases). Spinal cord affection was most commonly cervical(86.9%) and mostly short segment. All cases received pulsed methylprednisolone therapy, while intravenous immunoglobulins (IVIG) were used in 32.5% of cases and plasmapheresis in 13.9% of cases as a stepwise approach. Nearly half of the cases (53.5%) had monophasic illnesses without the need for long term therapy. Conclusion Spinal cord involvement is frequently observed in childhood acute demyelinating syndromes, and is most commonly cervical and short-segment. MRI spine should be regarded as a sensitive tool for diagnosis of myelitis in the context of ADEM and MS. MRI Brain is a sensitive tool accounting forfindings in 100% of ADEM, NMO, AE and MS cases. Patients with ADEM and AE has higher risk to develop encephalopathy and seizures unlike TM, NMO and MS patients.