Objective: To evaluate the likelihood of response to IV immunoglobulin (IVIg) by studying consecutive patients presenting with progressive, asymmetric, pure lower motor neuron (LMN) limb weakness, and to determine the clinical phenotype of those who respond.Methods: Thirty-one consecutive patients with progressive, focal-onset LMN limb weakness, without evidence of clinical upper motor neuron signs; sensory, respiratory, or bulbar involvement; or evidence of motor nerve conduction block on electrodiagnostic studies, were prospectively included in this study. Each patient underwent treatment with IVIg (2 g/kg) for a minimum of 3 months. Electrodiagnostic studies, a neuromuscular symptom score, and expanded Medical Research Council sum score were documented before and after IVIg treatment. The final diagnosis was determined after prolonged clinical follow-up.Results: Only 3 of 31 patients (10%) responded to IVIg. All responders demonstrated distal upper limb-onset weakness, EMG abnormalities confined to the clinically weak muscles, and a normal creatine kinase. This set of features was also identified in 31% of nonresponders presenting with distal upper limb weakness. Sex, age at onset, number of involved limb regions, and the duration of symptoms before treatment were not significantly different between groups. Conclusion:The findings of the present study do not support uniform use of IVIg in patients presenting with progressive asymmetric LMN limb weakness. It is suggested that IVIg treatment be limited to patients who demonstrate clinical and laboratory features suggestive of multifocal motor neuropathy. Classification of evidence:This study provides Class IV evidence that IVIg will not improve muscle function in 90% of patients with progressive, asymmetric, pure LMN weakness. Neurology ® 2013;81:2116-2120 GLOSSARY ALS 5 amyotrophic lateral sclerosis; CB 5 conduction block; CK 5 creatine kinase; CMAP 5 compound muscle action potential; EMRCSS 5 expanded Medical Research Council sum score; GM1 5 ganglioside M1; HRUS 5 high-resolution ultrasonography; IVIg 5 IV immunoglobulin; LMN 5 lower motor neuron; MMN 5 multifocal motor neuropathy; NCS 5 nerve conduction studies; NSS 5 neuromuscular symptom score; PLMNS 5 progressive lower motor neuron syndrome; PMA 5 progressive muscular atrophy; UL 5 upper limb; UMN 5 upper motor neuron.

Introduction Although intravenous immune globulin (IVIg) is used to treat patients in the outpatient setting, there is limited documentation addressing the safety of this practice. Methods Retrospective analysis of 438 patients with neuromuscular diseases receiving IVIg in an outpatient setting. Results Adverse events (AE) overall occurred in 16.9% of patients. Headache was the most common AE, noted in 11.6% of patients. Serious AEs occurred in 0.91% of patients; aseptic meningitis was the only one noted. Multivariate analyses identified the following risk factors for AEs: first‐lifetime course of IVIg, higher dose per course of IVIg, diagnosis of myasthenia gravis, women, and younger age. Discussion Intravenous immune globulin is generally safe to administer in an outpatient setting. Women, myasthenia gravis patients, and those receiving their first course or a higher total dose of IVIg are at an increased risk of experiencing an AE.

ObjectiveTo compare clinical and economic outcomes of patients who received intravenous immunoglobulin (IVIG) therapies and were managed by a clinical management program vs the outcomes of matched controls using administrative claim data.MethodsThis retrospective cohort study used the PharMetrics Plus™ claim database between September 1, 2011 and June 30, 2014. Patients in the intervention group were from a “high-touch” IVIG clinical management program administered by a home infusion specialty pharmacy. A greedy propensity score matching algorithm was used to identify a control group from non-program patients. Generalized estimating equation models were employed to evaluate differences between cohorts who were followed for 1 year.ResultsClinical outcomes were measured as infections and infusion-related adverse events. The proportion of patients who had serious bacterial infections was significantly lower (4.13% vs 7.75%, P=0.049) in the intervention group (n=242) compared to the control group (n=968). Other clinical outcomes assessed were not different between cohorts (P>0.050). The economic outcomes were measured as healthcare costs. The annual adjusted mean total health care costs of patients in the program were $26,522 lower compared to matched controls, representing a 20% lower cost ($109,476 vs $135,998, P=0.002). A major contribution to this difference ($17,269) was IVIG-related total outpatient cost (intervention vs control groups: $64,080 vs $81,349, P=0.001).ConclusionThe patients in this high-touch IVIG clinical management program appeared to have comparable infections or adverse event rates and significantly lower total health costs compared to their matched controls.

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