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Do patients having a decrease in SNAP amplitude during the course of MMN present with a different condition?

Emilien Delmont¹, Charles Benaïm, Mael Launay

¹Centre de référence pour Maladies NeuroMusculaires et SLA, Hôpital Archet 1, CHU de Nice, route saint Antoine de Ginestière, BP3079, 06202 Nice Cedex 3, France. delmont.e@chu-nice.fr

Journal of Neurology

|July 31, 2009

Summary

This summary is machine-generated.

A decrease in sensory nerve action potential (SNAP) amplitude in multifocal motor neuropathy with conduction blocks (MMNCB) indicates a more severe disease and greater axonal loss, requiring further investigation in larger patient groups.

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Area of Science:

Neurology
Neurophysiology

Background:

A decrease in sensory nerve action potential (SNAP) amplitude has been observed in some multifocal motor neuropathy with conduction blocks (MMNCB) patients.
The clinical implications and disease severity associated with reduced SNAP amplitude in MMNCB are not well understood.

Purpose of the Study:

To investigate whether patients with MMNCB and decreased SNAP amplitude exhibit different clinical expressions and disability compared to those with typical MMNCB.
To identify potential correlations between reduced SNAP amplitude and disease severity, axonal loss, and treatment response in MMNCB.

Main Methods:

Clinical, biological, and electrophysiological assessments were conducted on 15 MMNCB patients with initially normal SNAP amplitudes.
Patients were followed for a median of 3 years, with SNAP amplitudes reassessed.
Comparison of clinical and electrophysiological data between patients with normal and decreased SNAP amplitudes at follow-up.

Main Results:

Four out of 15 patients developed decreased SNAP amplitude (below 50% of normal) during follow-up, with no objective sensory loss.
Patients with decreased SNAP amplitude showed significantly worse Overall Neuropathy Limitation Scale (ONLS) scores, more affected nerves and limb regions, and lower compound muscle action potential (CMAP) amplitudes.
These patients also required higher doses of intravenous immunoglobulin (IVIg) therapy.

Conclusions:

A reduction in SNAP amplitude during the course of MMNCB is associated with a more severe disease phenotype and more prominent axonal loss.
This finding suggests that decreased SNAP amplitude may serve as a marker for disease progression and severity in MMNCB.
Further validation in a larger cohort is warranted to confirm these results.