Long-term plasmapheresis therapy is effective and safe in children with chronic relapsing dysimmune polyneuropathy.
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.
Beydoun
S R
SR
USC Neuromuscular Center, University of Southern California School of Medicine, Los Angeles 90017.
Engel
W K
WK
Karofsky
P
P
Schwartz
M U
MU
D000328:Adult; D002648:Child; D002908:Chronic Disease; D003711:Demyelinating Diseases / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D006801:Humans; D007154:Immune System Diseases / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D010523:Peripheral Nervous System Diseases / Q000503:physiopathology / Q000628:therapy*; D010956:Plasmapheresis; D011596:Psychomotor Disorders / Q000209:etiology / Q000628:therapy; D012008:Recurrence; D013997:Time Factors