Anti-GM1 Antibodies Do Not Predict Positive Response to IVIG in Lower Motor Neuron Disease
Source: van den Berg LH, et al. Intravenous immunoglobulin treatment in lower motor neuron disease associated with highly raised anti-GM1 antibodies. J Neurol Neurosurg Psychiatry 1997;63:674-677.
Multifocal motor neuropathy with conduction block and distal lower motor neuron syndrome with high-titer serum IgM anti-GM1 antibodies respond to immunosuppressive treatment modalities including plasma exchange and cyclophosphamide (Pestronk A, et al. Ann Neurol 1988;24:73-78; Pestronk A, et al. Neurology 1994;44:2027-2031). Intravenous immunoglobulin (IVIG) is similarly beneficial in the former but not, it would appear, in the latter. Five patients with lower motor neuron disease and raised serum anti-GM1 antibodies, but without evidence of conduction block on nerve conduction studies, received 0.4 g/kg IVIG for five consecutive days in an open-label study. One patient alone responded, and this was confirmed in a subsequent double-blind, placebo-controlled study. Sadly, despite continued treatment, muscle strength deteriorated within six months to below pre-treatment levels. IVIG appears to be ineffective for purely lower motor neuron syndromes in the absence of conduction block, and the presence of high titer anti-GM1 antibodies does not alter responsivity. mr
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