Predicting a Positive Response to Intravenous Immunoglobulin in Isolated Lower Motor Neuron SyndromesReport as inadecuate




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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 GM1 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 GM1 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 GM1 antibodies, and should be given an empirical trial of IVIg to determine treatment responsiveness.



Author: James R. Burrell, Con Yiannikas, Dominic Rowe, Matthew C. Kiernan

Source: http://plos.srce.hr/



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