K. Hoangxuan et al., TREATMENT OF IMMUNE-MEDIATED NEUROPATHIES WITH INTRAVENOUS HUMAN IMMUNE GLOBULIN - OPEN STUDY IN 16 CASES, Revue neurologique, 149(6-7), 1993, pp. 385-392
Between June 1989 and February 1992 in an open controlled study 16 pat
ients with various types of polyneuropathy were treated with high-dose
intravenous immunoglobulins (IgIV). Every month during 3 months, each
patient received three courses of IgIV in doses of 0.4 g/kg/day durin
g 5 successive days. The trial was discontinued in case of no response
or if the neuropathy was considered as being in remission. In the oth
er cases, at most one course of IgIV was given once a month if there w
as significant improvement (assessed by previously published clinical
functional scales, electrophysiological examinations and titers of spe
cific antibodies), or spaced at intervals which varied according to ea
ch patient, and sometimes in low doses. Results : 1) A first group of
6 patients had chronic demyelinating polyneuropathy with severe motor
disability. The first infusions of IgIV resulted, in 4/6 cases, in a d
ramatic improvement which lasted under regularly spaced courses in low
er doses. 2) Four patients had chronic neuropathy associated with mono
clonal IgM gammopathy of undertermined significance (3 had anti-MAG an
d anti-SPG antibodies, and 1 had anti-GD1a and GD1b antibodies) and ha
d not been improved by the usual immunosuppressive treatments. In 1 ca
se the IgIV treatment had to be discontinued because of skin allergy.
In the remaining 3 patients the clinical disorders (mainly the sensory
ones) were reduced, but no significant improvement of neurophysiologi
cal or immunological data was observed 3) Three patients had a purely
multifocal motor neuropathy with persistent conduction blocks at EMG a
nd high titers of anti-GM1 antibodies in 2/3 cases. IgIV therapy resul
ted in improvement of the motor functional scale in all cases, marked
in 2/3; there was no significant improvement of the neurophysiological
and immunological data in these 3 cases. 4) In the last group of 3 pa
tients with severe axonopathy associated with signs of mild systemic d
isease, IgIV therapy was ineffective. The effectiveness and mechanism
of action of IgIV in immune-mediated polyneuropathies are discussed.