AANEM survey of training in electrodiagnostic medicine in U.S. residency programs.

BACKGROUND: Little literature exists describing resident training in peripheral electrodiagnosis (EDX). METHODS: U.S. residency programs in neurology and physical medicine and rehabilitation (PM&R) were surveyed by the AANEM (American Association of Neuromuscular and Electrodiagnostic Medicine) on specific features of EDX training. RESULTS: Ninety-seven programs responded to the survey. Training duration was 4-8 weeks in most neurology programs; training averaged 22 weeks in PM&R programs. EDX experience was required in all PM&R and in 90% of neurology programs. Results varied greatly for the residency years of training, pulling of residents for other responsibilities, participation in continuity clinics, number of teaching physicians, number of needle examinations performed, organization of nerve conduction training, written/oral examinations, muscle/nerve biopsy reviews, and training materials. CONCLUSIONS: This survey demonstrated large variability in training of neurology and PM&R residents in peripheral EDX.

Guillain-Barré Syndrome.

PURPOSE OF REVIEW: This article reviews the current state of Guillain-Barré syndrome (GBS), including its clinical presentation, evaluation, pathophysiology, and treatment. RECENT FINDINGS: GBS is an acute/subacute-onset polyradiculoneuropathy typically presenting with sensory symptoms and weakness over several days, often leading to quadriparesis. Approximately 70% of patients report a recent preceding upper or lower respiratory tract infection or gastrointestinal illness. Approximately 30% of patients require intubation and ventilation because of respiratory failure. Nerve conduction studies in the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) form of GBS typically show evidence for a multifocal demyelinating process, including conduction block or temporal dispersion in motor nerves. Sural sparing is a common phenomenon when testing sensory nerves. CSF analysis commonly shows an elevated protein, but this elevation may not be present until the third week of the illness. Patients with AIDP are treated with best medical management and either IV immunoglobulin (IVIg) or plasma exchange. SUMMARY: GBS is a common form of acute quadriparesis; a high level of suspicion is needed for early diagnosis. With appropriate therapy, most patients make a very good to complete recovery.

How neurologists are paid: Part 3: Hospital support, Veterans Administration, and neurohospitalists.

Part 1 of this series focused on factors influencing payment for patient care services and Part 2 described compensation plans for neurologists in private practice and in academic medicine. In Part 3, we review how hospital salary support and appointments to Veterans Administration hospitals contribute to the salary structure of neurologists. We also discuss neurohospitalist care and ways neurologists can potentially increase compensation from on-call pay, telemedicine, and the use of new transitional care and complex chronic care codes. We conclude with an emphasis on the important role of neurologists as team players in a health care system that will rely on efficient coordination of care among many health care workers.

How neurologists are paid: Part 1: The Medicare payment system.

Neurologists are facing yearly reductions in reimbursement for rendered services. These reductions arise from changes by Medicare, Medicaid, and third-party payers to achieve cost savings. In Part 1, we discuss reimbursement for office visits and procedures, the relative value scale, the conversion factor used by Medicare to transform work into payments, and the recently repealed sustainable growth rate. The establishment of new codes for transitional care and chronic care management may augment the salaries of neurologists who care for patients with chronic conditions. Medicare's recent elimination of payment for consultations and the bundling of nerve conduction studies have dramatically affected reimbursement. Large discrepancies remain between compensation for procedures and office visits.

How neurologists are paid: Part 2: Private practice, research grants, academic and nonclinical activities.

Part 1 of this series focused on factors influencing payment for patient care services. In Part 2, we review compensation models for nonpatient activity such as medical legal reviews, committee participation, and collaboration with the pharmaceutical industry. Compensation to neurologists in private practice is commonly in the form of guaranteed salary and bonuses. Salary for neurologists in academic medicine has changed considerably over the past 3 decades, from small departments with faculty supported by grants and volunteer faculty, to large departments with faculty split between those with research grant support and those focusing on patient care and teaching. Compensation models in academic medicine range from straight salary without bonus to straight salary with personal or shared bonus and salary based on relative value units.

Follow-up nerve conduction studies in CIDP after treatment with IGIV-C: Comparison of patients with and without subsequent relapse.

INTRODUCTION: Electrodiagnostic studies (EDX) are not performed routinely before treatment suspension in CIDP, and no data exist regarding their value in predicting clinical relapse. METHODS: Serial EDX (baseline and after IGIV-C therapy) were analyzed from subjects in the ICE clinical trial who responded to IGIV-C treatment and were subsequently re-randomized to placebo in an extension phase. Comparisons were made between subjects who relapsed and those who did not. RESULTS: A total of 55% (6/11) of the Relapse group had an increase in total number of demyelinating findings (DF) versus 8% (1/13) in the No Relapse group (P = 0.023). In the Relapse group, 100% had ≥ 1 new DF and 73% (8/11) had ≥ 4 new DF versus 60% (8/13) and 8% (1/13), respectively, in the No Relapse group. CONCLUSIONS: An increased total number of DF or the occurrence of ≥ 4 new DF may indicate a higher risk of clinical relapse after treatment cessation in IGIV-C-responsive patients.

Supply and demand analysis of the current and future US neurology workforce.

OBJECTIVE: This study estimates current and projects future neurologist supply and demand under alternative scenarios nationally and by state from 2012 through 2025. METHODS: A microsimulation supply model simulates likely career choices of individual neurologists, taking into account the number of new neurologists trained each year and changing demographics of the neurology workforce. A microsimulation demand model simulates utilization of neurology services for each individual in a representative sample of the population in each state and for the United States as a whole. Demand projections reflect increased prevalence of neurologic conditions associated with population growth and aging, and expanded coverage under health care reform. RESULTS: The estimated active supply of 16,366 neurologists in 2012 is projected to increase to 18,060 by 2025. Long wait times for patients to see a neurologist, difficulty hiring new neurologists, and large numbers of neurologists who do not accept new Medicaid patients are consistent with a current national shortfall of neurologists. Demand for neurologists is projected to increase from ∼18,180 in 2012 (11% shortfall) to 21,440 by 2025 (19% shortfall). This includes an increased demand of 520 full-time equivalent neurologists starting in 2014 from expanded medical insurance coverage associated with the Patient Protection and Affordable Care Act. CONCLUSIONS: In the absence of efforts to increase the number of neurology professionals and retain the existing workforce, current national and geographic shortfalls of neurologists are likely to worsen, exacerbating long wait times and reducing access to care for Medicaid beneficiaries. Current geographic differences in adequacy of supply likely will persist into the future.

Clinical assessment of serious adverse events in children receiving 2009 H1N1 vaccination.

BACKGROUND: Monovalent 2009 H1N1 influenza vaccines were licensed and administered in the United States during the H1N1 influenza pandemic between 2009 and 2013. METHODS: Vaccine Adverse Event Reporting System received reports of adverse events following immunization (AEFI) after H1N1 vaccination. Selected reports were referred to the Centers for Disease Control and Prevention's Clinical Immunization Safety Assessment network for additional review. We assessed causality using modified World Health Organization criteria. RESULTS: There were 3,928 reports of AEFI in children younger than age 18 years after 2009 H1N1 vaccination received by January 31, 2010. Of these, 214 (5.4%) were classified as serious nonfatal and 109 were referred to Clinical Immunization Safety Assessment for further evaluation. Ninety-nine (91%) had sufficient initial information to begin investigation and are described here. The mean age was 8 years (range, 6 months-17 years) and 38% were female. Median number of days between vaccination and symptom onset was 2 (range, -11 days to +41 days). Receipt of inactivated, live attenuated, or unknown type of 2009 H1N1 vaccines was reported by 68, 26 and 5 cases, respectively. Serious AEFI were categorized as neurologic events in 47 cases, as hypersensitivity in 15 cases and as respiratory events in 10 cases. At the time of evaluation, recovery was described as complete (61), partial (16), no improvement (1), or unknown (21). Causality assessment yielded the following likelihood of association with 2009 H1N1 vaccination: 8 definitely; 8 probably; 21 possibly; 43 unlikely; 17 unrelated; and 2 unclassifiable. CONCLUSIONS: Most AEFI in children evaluated were not causally related to vaccine and resolved without sequelae. Detailed clinical assessment of individual serious AEFI can provide reassurance of vaccine safety.

Chronic inflammatory demyelinating polyradiculoneuropathy: from bench to bedside.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common treatable chronic autoimmune neuropathy. Multiple diagnostic criteria have been established, with the primary goal of identifying neurophysiologic hallmarks of acquired demyelination. Treatment modalities have expanded to include numerous immunomodulatory therapies, although the best evidence continues to be for corticosteroids, plasma exchange, and intravenous immunoglobulin (IVIg). This review describes the pathology, epidemiology, pathogenesis, diagnosis, and treatment of CIDP.

Coding issues.

Accurate coding is an important function of neurologic practice. This section of CONTINUUM, contributed by members of the AAN Medical Economics and Management Committee, includes helpful coding information and examples related to the issue topic. This section may include diagnosis coding, evaluation and management coding, procedure coding, or a combination, depending on which is most useful for the subject area of the issue.

Enteroviral meningitis and concurrent peripheral motor axonal polyneuropathy.

Enteroviral infections can cause acute flaccid paralysis resulting from anterior myelitis, but the occurrence of axonal polyneuropathy is not well described. We report an 8-year-old boy who presented with symmetric, ascending flaccid paralysis and was diagnosed with concurrent echovirus type 9 viral meningitis.

Causality assessment of serious neurologic adverse events following 2009 H1N1 vaccination.

BACKGROUND: Adverse events occurring after vaccination are routinely reported to the Vaccine Adverse Event Reporting System (VAERS). We studied serious adverse events (SAEs) of a neurologic nature reported after receipt of influenza A (H1N1) 2009 monovalent vaccine during the 2009-2010 influenza season. Investigators in the Clinical Immunization Safety Assessment (CISA) network sought to characterize these SAEs and to assess their possible causal relationship to vaccination. METHODS: Centers for Disease Control and Prevention (CDC) and Food and Drug Administration (FDA) physicians reviewed all SAE reports (as defined by the Code of Federal Regulations, 21CFR§314.80) after receipt of H1N1 vaccine reported to VAERS between October 1, 2009 and March 31, 2010. Non-fatal SAE reports with neurologic presentation were referred to CISA investigators, who requested and reviewed additional medical records and clinical information as available. CISA investigators assessed the causal relationship between vaccination and the event using modified WHO criteria as defined. RESULTS: 212 VAERS reports of non-fatal serious neurological events were referred for CISA review. Case reports were equally distributed by gender (50.9% female) with an age range of 6 months to 83 years (median 38 years). The most frequent diagnoses reviewed were: Guillain-Barré Syndrome (37.3%), seizures (10.8%), cranial neuropathy (5.7%), and acute disseminated encephalomyelitis (3.8%). Causality assessment resulted in classification of 72 events as "possibly" related (33%), 108 as "unlikely" related (51%), and 20 as "unrelated" (9%) to H1N1 vaccination; none were classified as "probable" or "definite" and 12 were unclassifiable (6%). CONCLUSION: The absence of a specific test to indicate whether a vaccine component contributes to the pathogenesis of an event occurring within a biologically plausible time period makes assessing causality difficult. The development of standardized protocols for providers to use in evaluation of adverse events following immunization, and rapid identification and follow-up of VAERS reports could improve causality assessment.

Muscle ultrasound quantifies the rate of reduction of muscle thickness in amyotrophic lateral sclerosis.

Sensitive biomarkers are lacking in amyotrophic lateral sclerosis (ALS). Muscle ultrasound (MUS) can quantify muscle thickness and echointensity (EI). We evaluated the rate of muscle atrophy in ALS using MUS. Ten patients had serial unilateral MUS examination of biceps, wrist flexors, and tibialis anterior over 6 months. The rates of change of muscle thickness and EI were determined. Muscle thickness declined at a mean rate of -0.663 mm/month (P = 0.0014), greatest in biceps. Muscle thickness correlated moderately with ALSFRS-R, grip dynamometry, and body weight. There was no change in EI. MUS can quantify the rate of reduction in muscle thickness in ALS patients. The lack of strong correlation between muscle thickness and standard ALS measures may reflect limited sensitivity in these conventional measures. The rate of change of muscle thickness merits further study as a potential biomarker in ALS, particularly when considering biceps brachii as a candidate.

Treatment of chronic inflammatory demyelinating polyneuropathy.

OPINION STATEMENT: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, immune-mediated, non-length-dependent polyradiculoneuropathy that is progressive or relapsing over a period of at least 8 weeks, often evolving over time to a relatively symmetric pattern. Although the exact pathogenesis is unclear, it is thought to be mediated by both cellular and humoral reaction to the peripheral nerve myelin sheath involving nerve roots and proximal and distal nerves. Early medical treatment of CIDP is important to prevent axonal loss occurring as a secondary effect of progressive demyelination. Only three treatments for CIDP have demonstrated benefit in randomized controlled studies: corticosteroids, plasma exchange, and intravenous immunoglobulin. About 25% of patients fail to respond to these treatments or respond inadequately. These treatments have similar efficacy but differ significantly in cost and adverse effects. These factors are considered in treatment selection.

Safety and tolerability of immune globulin intravenous in chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a common inflammatory neuropathy that can be progressive, stepwise progressive, or relapsing and remitting. OBJECTIVES: To further evaluate the long-term safety and tolerability of immune globulin intravenous, 10% caprylate-chromatography purified immune globulin intravenous in CIDP. DESIGN: Randomized multicenter trial. SETTING: Hospitals and outpatient clinics. PATIENTS: Adults with CIDP (n = 117) [corrected]. INTERVENTIONS: Immune globulin intravenous, 10% caprylate-chromatography purified (2 g/kg of body weight) or placebo was infused as a baseline loading dose, followed by a maintenance dose (1 g/kg) every 3 weeks for up to 24 weeks. PATIENTS who responded were rerandomized into a double-blind extension phase of immune globulin intravenous, 10% caprylate-chromatography purified (1 g/kg) or placebo every 3 weeks for up to 24 weeks. PATIENTS who relapsed during the extension phase were withdrawn from the study. MAIN OUTCOME MEASURES: Additional analyses of safety and tolerability. RESULTS: Overall, 113 patients and 95 patients were exposed to immune globulin intravenous, 10% caprylate-chromatography purified and placebo, respectively. Exposure to immune globulin intravenous, 10% caprylate-chromatography purified was approximately twice that of placebo (1096 vs 575 infusions). Most maintenance dose courses were administered over 1 day in the immune globulin intravenous, 10% caprylate-chromatography purified (89.1% of 783 dose courses) and placebo (91.1% of 359 dose courses) groups. The most common drug-related adverse events (AEs) with immune globulin intravenous, 10% caprylate-chromatography purified were headache (4.0 per 100 infusions) and pyrexia (2.4 per 100 infusions). Five drug-related serious AEs (pulmonary embolism, pyrexia, vomiting, and 2 headache events) were reported in 3 patients (2.7%) exposed to immune globulin intravenous, 10% caprylate-chromatography purified. The incidence of drug-related serious AEs was higher after loading dose infusions than after maintenance dose infusions (4 AEs vs 1 AE). Age, weight, CIDP severity, and previous immune globulin intravenous exposure had no substantial effect on the percentage of patients with AEs, including serious AEs. CONCLUSION: Data support a favorable safety and tolerability profile for administration of immune globulin intravenous, 10% caprylate-chromatography purified as CIDP maintenance therapy. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00220740.