[P1-138] How Can We Manage a Child Who Is Diagnosed with Subacute Inflammatory Demyelinating Polyradiculo-neuropathy?
[Intreoduction]: Subacute inflammatory demyelinating polyradiculoneuropathy (SIDP) in children is relatively rare, and treatment is based primarily on intravenous immunoglobulins (IVIG) or corticosteroids. Treatment strategies for rapidly progressive cases, however, are ambiguous. [CASE and RESULT]: We report the case of a 10-year-old girl who first presented with weakness in both lower limbs and walking impairment for one month. Her symptoms rapidly progressed to loss of ambulation. We hospitalized her, and the results of neurological examination, spinal magnetic resonance imaging, nerve conduction study, and cerebrospinal fluid showed that she suffered from SIDP. Her clinical symptoms were not improved by IVIG therapy; rather, they got progressively worse. We administered plasma exchange (PE) therapy four times/two weeks, and from the fifth to seventh PE we added methylprednisolone pulse (m-PLS) therapy (30mg/kg /day, 3 times a week) for three weeks. She showed gradual improvement in muscle strength after those therapies. Thereafter, we changed from m-PLS pulse PE to 40mg oral predonisolone and monthly IVIG (1g/kg/day). She could stand from a prone position by herself sixteen weeks after the start of PE. [CONCLUSION]: Our case showed acute progressive onset. She had refractory to first-line treatment, but showed an early improvement and a favorable outcome after PE and m-PSL therapies. Early recognition of SIDP and an empirical start of treatment are important to avoid potentially irreversible axonal damage and associated disability.