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Long-term plasmapheresis therapy is effective and safe in children with chronic relapsing dysimmune polyneuropathy
S R Beydoun1, W K Engel, P Karofsky
1USC Neuromuscular Center, University of Southern California School of Medicine, Los Angeles 90017.
Insights
Long-term plasmapheresis is effective and safe for managing chronic relapsing dysschwannian neuropathy, even in children. This treatment helps prevent major relapses and improves daily functioning for affected individuals.
Area of Science:
- Neurology
- Immunology
Background:
- Chronic relapsing dysschwannian neuropathy presents with severe limb weakness.
- Patients often experience major relapses requiring significant medical intervention, including ventilatory support.
- Pharmacologic antidysimmune treatments may not be sufficient for disease control.
Observation:
- Two pediatric patients with dysschwannian neuropathy requiring lifelong management were treated with frequent plasmapheresis for over 8 years.
- Despite experiencing major relapses requiring ventilatory support prior to treatment, plasmapheresis was initiated and maintained.
Findings:
- Neither patient experienced major relapses after initiating plasmapheresis, indicating sustained disease control.
- Both patients achieved functional daily living as students, with only slight to moderate residual weakness, demonstrating treatment efficacy.
Implications:
- Long-term plasmapheresis is a safe and effective treatment for chronic relapsing dysschwannian neuropathy in children.
- This approach may offer benefits for other chronic dysimmune neuropathies, warranting further investigation.
Abstract:
Since childhood, two persons, a boy age 12 and a girl age 24 years, with chronic relapsing dysschwannian neuropathy causing severe limb weakness have been maintained on frequent plasmapheresis for more than 8-1/2 and 9 years respectively. They are totally dependent on it. Onset of disease was at ages 2-1/2 and 9 years. Both patients had had major relapses, some requiring ventilatory support, despite continued maximally-tolerated pharmacologic antidysimmune treatment. Plasmapheresis was started in August 1980 and December 1979, respectively. The current schedule is a set of 3 in one week at eight-week intervals for patient 1 and twice every 7-10 days for patient 2. They have had more than 330 and more than 1,100 phereses respectively. Since beginning phereses, neither has had a major relapse, and both are functional in their daily life as students, although with slight and moderate residual weakness respectively. The dependence of these 2 patients on regular pheresis for the last 8-1/2 and 9 years proves its efficacy and safety in children. Long-term maintenance pheresis may be beneficial in patients with other forms of chronic dysimmune neuropathy.
