Predicting a positive response to intravenous immunoglobulin in isolated lower motor neuron syndromes

Abstract

Objective: To determine clinically related characteristics in patients with pure lower motor neuron (LMN) syndromes, not fulfilling accepted diagnostic criteria, who were likely to respond to intravenous immunoglobulin (IVIg) treatment.

Methods: Demographic, clinical, laboratory and neurophysiological characteristics were prospectively collected from patients with undifferentiated isolated LMN syndromes who were then treated with IVIg. Patients were classified as either responders or non-responders to therapy with IVIg based on clinical data and the two groups were compared.

Results: From a total cohort of 42 patients (30 males, 12 females, aged 18-83 years), 31 patients responded to IVIg and 11 did not. Compared to patients that developed progressive neurological decline, responders were typically younger (45.8 compared to 56.0 years, P<0.05) and had upper limb (83.9% compared to 63.6%, NS), unilateral (80.6% compared to 45.5%, P<0.05), and isolated distal (54.1% compared to 9.1%, P<0.05) weakness. Patients with predominantly upper limb, asymmetrical, and distal weakness were more likely to respond to IVIg therapy. Of the patients who responded to treatment, only 12.9% had detectable GM(1) antibodies and conduction block (not fulfilling diagnostic criteria) was only identified in 22.6%.

Conclusions: More than 70% of patients with pure LMN syndromes from the present series responded to treatment with IVIg therapy, despite a low prevalence of detectable GM(1) antibodies and conduction block. Patients with isolated LMN presentations, not fulfilling accepted diagnostic criteria, may respond to IVIg therapy, irrespective of the presence of conduction block or GM(1) antibodies, and should be given an empirical trial of IVIg to determine treatment responsiveness.

Figure 1. Responders (white bars) had the typical clinical phenotype of MMN, namely upper limb, unilateral, and distal onset weakness.

Figure 2. A receiver operator curve (ROC) was constructed to identify the fibrillation score cut-off that identified non-responders with the maximal sensitivity while preserving specificity.

Sensitivity is represented on the y axis and (1-specificity) on the x axis. The asterisk (*) indicates a fibrillation score of 0.4. A score>0.4 detected non-responders with moderate sensitivity (64%) and specificity (62%).