Plasmapheresis in the Treatment of Pediatric Patients with Autoimmune Neurological Diseases: 13 Years of Experience

Background: Autoimmune diseases of the nervous system in children comprise a heterogeneous group of rare disorders that can affect the central or peripheral nervous system. Consideration of an autoimmune process as the etiology of neurologic diseases in children is important, for early initiation of immunotherapy (IT) and improvement in long-term neurologic outcomes. If plasmapheresis (PF) is available it should be considered in the early phase of disease, weighed against the severity of illness. The goal of PF is to either remove circulating autoantibodies or toxins. The aim of our study was to determine the outcome of children with severe neurologic symptoms treated with plasmapheresis in our center.

Methods:

Setting: 12 bed interdisciplinary pediatric ICU

Time period: 2008–2021

Patients: 18 patients; 8 male, 10 female; mean age: 13 years (range: 2–17), treatment sessions with PF: 3–13.

Indications for PF by diagnoses: myasthenia gravis (MG), four patients; NMDAR encephalitis, two patients; acute demyelinating encephalomyelitis (ADEM), four patients; autoimmune encephalitis (AIE), three patients with status epilepticus in two of them; encephalitis disseminata (ED), three patients; myelitis, one patient; aseptic meningitis, one patient.

Additional immunosuppression: steroids (PRED) and cyclosporine A (CsA), or mycophenolate mofetil (MMF), or rituximab (RTX).

Results: In all 18 patients, neurological symptoms improved within the first 2 weeks of PF treatment and 10 patients recovered completely. Additional immunosuppression was necessary in all patients for at least 3 months. Four patients with severe MG had complete remission with additional chronic immunosuppression. Two patients with NMDAR encephalitis improved during PF and recovered within 6 weeks with incomplete recovery in one patient with severe chorea following herpes encephalitis. Two patients with ADEM had mild residual symptoms; the other two had complete recovery. One of the three patients with AIE showed full recovery, while only one patient with ED had full recovery. The patient with myelitis had only mild improvement of clinical symptoms, whereas the patient with aseptic meningitis showed full recovery.

Conclusion: In pediatric patients with severe neurologic disease resulting from an immunologically mediated process, the combined treatment with plasmaphereses and immunosuppression seems effective and safe and may accelerate clinical improvement and shorten intensive care as well as periods of immobility.

Publication History

Article published online:
28 October 2021