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[Multifocal motor neuropathies with conduction block: long-term follow-up of ten patients treated with IVIg]
Rev Neurol (Paris). 2006 Jun;162 Spec No 1:3S46-3S50.
[Article in French]
Cros D, Drake K.
Massachusetts General Hospital, Boston, USA.
Multifocal
motor neuropathy (MMN) with conduction block responds to high-dose i.v.
polyvalent immunoglobulins (IVIg) over the short term, but several
studies have demonstrated a long-term increase in the degree of axonal
degeneration and the number of conduction blocks, factors indicating a
poor prognosis. The objective of this study was to evaluate the
long-term effect of IVIg on clinical and neurophysiological parameters.
METHODS: We reviewed the records of ten patients who had initially
responded well to IVIg and received regular, long-term treatment. The
parameters studied were muscular strength, motor function status
(modified Rankin scale), as well as the number and progression of
conduction blocks and the degree of axonal degeneration. Patients were
followed up for a mean of 7.25 years (range, 3.5-12). They were all
initially treated with 2 g IgIV/kg in 5 days every 4 weeks for 3
months. Maintenance therapy was administered every 4 weeks with dose
adjustment to prevent muscular strength deterioration. RESULTS: We
noted a significant and persistent improvement in muscular strength and
in the Rankin motor function score over the long term, with no escape
phenomenon. The number of conduction blocks and the degree of axonal
degeneration decreased markedly. CONCLUSION: IVIg treatment remains
effective over the long term in MMN. These conclusions differ from
those of other authors in earlier studies, but our patients were
treated with significantly higher doses of IVIg. These results have
important implications for long-term treatment of patients with MMN.
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