Beydoun S R, Engel W K, Karofsky P, Schwartz M U
USC Neuromuscular Center, University of Southern California School of Medicine, Los Angeles 90017.
Rev Neurol (Paris). 1990;146(2):123-7.
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.
