Sorenson, Eric J.

ABSTRACTIntroduction: Valosin containing protein (VCP) mutations cause a rare disorder characterized by hereditary inclusion body myopathy, Paget disease of bone (PDB), and frontotemporal dementia (FTD) with variable penetrance. VCP mutations have also been linked to amyotrophic lateral sclerosis and Charcot‐Marie‐Tooth disease type 2. Methods: Review of clinical, serological, electrophysiological, and myopathological findings of 6 VCP‐opathy patients from 4 unrelated families. Results: Patients manifested muscle weakness between ages 40 and 53 years and developed predominant asymmetric limb girdle weakness. One patient had distal weakness at onset and co‐existing peripheral neuropathy. Another patient had PDB, 1 had mild cognitive deficits, and 1 had FTD. All patients had myopathic and neurogenic electromyographic findings with predominant neurogenic changes in 2. Rimmed vacuoles were infrequent, while neurogenic changes were prominent in muscle biopsies. Conclusions: VCP‐opathy is a multifaceted disorder in which myopathy and peripheral neuropathy can coexist. The electrophysiological and pathological neurogenic changes raise the possibility of coexisting motor neuron involvement. Muscle Nerve, 2015 Muscle Nerve 54: 94–99, 2016 Muscle Nerve 54: 94–99, 2016

ABSTRACTIntroductionPneumothorax is a potentially serious complication of electromyography (EMG). Data on the frequency of pneumothorax after EMG are lacking. The purpose of this study was to determine the frequency, timing, and risk factors for iatrogenic pneumothorax after EMG. Methods: Cases of pneumothorax after EMG were reviewed for clinical, electrophysiological, and radiological data. Results: Of 64,490 EMG studies, 7 patients had an association between the EMG and pneumothorax. All patients were symptomatic and presented within 24 hours of EMG. Sampling of serratus anterior and diaphragm was causative in 1 patient each. In 5 patients, multiple high‐risk muscles were sampled. The highest frequency of pneumothorax was observed with examination of serratus anterior (0.445%) and diaphragm (0.149%). Conclusions: The frequency of symptomatic iatrogenic pneumothorax after EMG appears to be low, and examinations of serratus anterior and diaphragm carry the highest risk. Electromyographers should be aware of the risk of pneumothorax and should counsel patients accordingly. Muscle Nerve 53: 518–521, 2016

BACKGROUND: Poliomyelitis is a viral infectious disease caused by 1 of the 3 strains of poliovirus. The World Health Organization launched an eradication campaign in 1988. Although the number of cases of poliomyelitis has drastically declined, eradication has not yet been achieved, and there are a substantial number of survivors of the disease. Survivors of poliomyelitis present a unique set of challenges to the anesthesiologist. The scientific literature regarding the anesthetic management of survivors of poliomyelitis, however, is limited and primarily experiential in nature. Using a retrospective, matched cohort study, we sought to more precisely characterize the anesthetic implications of poliomyelitis and to determine what risks, if any, may be present for patients with a history of the disease. METHODS: Using the Mayo Clinic Life Sciences System Data Discovery and Query Builder, study subjects were identified as those with a history of paralytic poliomyelitis who had undergone major surgery at Mayo Clinic Rochester between 2005 and 2009. For each case, 2 sex- and age-matched controls that underwent the same surgical procedure during the study period were randomly selected from a pool of possible controls. Medical records were manually interrogated with respect to demographic variables, comorbid conditions, operative and anesthetic course, and postoperative course. RESULTS: We analyzed 100 cases with 2:1 matched controls and found that the peri- and postoperative courses were very similar for both groups of patients. Pain scores, postanesthesia care unit admission, length of postanesthesia care unit stay, intensive care unit admission, length of intensive care unit stay, and initial extubation location were not significantly different between the 2 groups. Looking at pulmonary complications in our primary outcome, there was no significant difference between the 2 groups (17% vs 14% for polio versus control, respectively; conditional logistic regression odds ratio = 1.5; 95% confidence interval, 0.7–3.3; P = 0.33). In addition, no difference was noted in those requiring a code or rapid response team intervention (4% vs 3% for polio versus control; P = 0.46) and the 30-day mortality rate was also not significantly different, with 2% of polio patients dying compared with 3% of controls ( P = 0.79). The analysis of the primary outcome was repeated for the subset of patients with a history of poliomyelitis who had persistent neurologic deficits preoperatively ( n = 36) and their matched controls ( n = 72). In this subset analysis, there were 4 (11%) polio patients and 8 (11%) control patients who experienced pulmonary complications (conditional logistic regression odds ratio = 1.00; 95% confidence interval, 0.27–3.72; P = 1.00). The percentage of patients experiencing specific pulmonary complications of interest was similar between groups (postoperative mechanical ventilation: 6% vs 8% for polio and control patients, respectively; prolonged mechanical ventilation: 0% vs 1%; reintubation: 8% vs 4%; pulmonary infection: 6% vs 6%; and aspiration: 0% vs 1%). CONCLUSIONS: This study suggests that patients with a history of poliomyelitis do not seem to have an increased risk of pulmonary complications in the perioperative period. However, an odds ratio as great as 3.3-fold may be present.

ABSTRACTIntroduction: In acid maltase deficiency (AMD), electrical myotonia (EM) may be restricted to paraspinal muscles. A comprehensive description of the electromyographic (EMG) findings in AMD is lacking. The purpose of this study is to describe the EMG features in adult‐onset AMD, focusing on the distribution of EM. Methods: A retrospective chart review of AMD patients diagnosed at Mayo Clinic over age 18 years. Results: Thirty‐seven patients were included. Twenty‐eight (76%) had EM in at least 1 muscle, and EM was more common in paraspinal and proximal limb muscles. The tensor fasciae latae (TFL) was equally sensitive to the paraspinals for EM. Three of 4 patients had EM identified in the diaphragm. Conclusions: Approximately three‐quarters of adult‐onset AMD patients display EM on EMG. The paraspinal muscles and TFL are the most likely to demonstrate EM, and EM can be detected in the diaphragm of adult onset AMD patients. Muscle Nerve, 2015 Muscle Nerve 51:759–761, 2015