Neuromuscular Respiratory Failure Research Articles

Sensitivity and specificity of diagnostic ultrasound in the diagnosis of phrenic neuropathy.

To determine the sensitivity and specificity of B-mode ultrasound in the diagnosis of neuromuscular diaphragmatic dysfunction, including phrenic neuropathy. A prospective study of patients with dyspnea referred to the EMG laboratory over a 2-year time frame for evaluation of neuromuscular respiratory failure who were recruited consecutively and examined with ultrasound for possible diaphragm dysfunction. Sonographic outcome measures were absolute thickness of the diaphragm and degree of increased thickness with maximal inspiration. The comparison standard for diagnosis of diaphragm dysfunction was the final clinical diagnosis of clinicians blinded to the diaphragm ultrasound results, but taking into account other diagnostic workup, including chest radiographs, fluoroscopy, phrenic nerve conduction studies, diaphragm EMG, and/or pulmonary function tests. Of 82 patients recruited over a 2-year period, 66 were enrolled in the study. Sixteen patients were excluded because of inconclusive or insufficient reference testing. One hemidiaphragm could not be adequately visualized; therefore, hemidiaphragm assessment was conducted in a total of 131 hemidiaphragms in 66 patients. Of the 82 abnormal hemidiaphragms, 76 had abnormal sonographic findings (atrophy or decreased contractility). Of the 49 normal hemidiaphragms, none had a false-positive ultrasound. Diaphragmatic ultrasound was 93% sensitive and 100% specific for the diagnosis of neuromuscular diaphragmatic dysfunction. B-mode ultrasound imaging of the diaphragm is a highly sensitive and specific tool for diagnosis of neuromuscular diaphragm dysfunction. This study provides Class II evidence that diaphragmatic ultrasound performed by well-trained individuals accurately identifies patients with neuromuscular diaphragmatic respiratory failure (sensitivity 93%; specificity 100%).

Clinical and laboratory findings of 21 patients with radiation-induced myopathy

BackgroundLimited data are available on radiation-induced myopathy (RIM) in adult cancer survivors.MethodsWe retrospectively reviewed the clinical, electrophysiological, serological, radiological and pathological findings of patients with RIM seen in the neurology...

Community-acquired pneumonia in pediatric patients with acute neuromuscular respiratory failure: A microbiologic perspective

Pediatric PulmonologyVolume 49, Issue 8 p. 827-828 Response to Letter Community-acquired pneumonia in pediatric patients with acute neuromuscular respiratory failure: A microbiologic perspective Jong-Hau Hsu MD, Jong-Hau Hsu MD Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Faculty of Medicine, Department of Pediatrics, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, TaiwanSearch for more papers by this authorTai-Heng Chen MD, Tai-Heng Chen MD Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Division of Pediatric Emergency, Department of Emergency, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, TaiwanSearch for more papers by this authorYuh-Jyh Jong MD, DMSci, Corresponding Author Yuh-Jyh Jong MD, DMSci Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Department of Laboratory Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Department of Biological Science and Technology, National Chiao Tung University, Hsinchu, TaiwanCorrespondence to: Prof. Y.-J. Jong, MD, DMSci, Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University No. 100, Shih-Chuan 1st Road, Kaohsiung 80708, Taiwan. E-mail: [email protected]Search for more papers by this author Jong-Hau Hsu MD, Jong-Hau Hsu MD Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Faculty of Medicine, Department of Pediatrics, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, TaiwanSearch for more papers by this authorTai-Heng Chen MD, Tai-Heng Chen MD Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Division of Pediatric Emergency, Department of Emergency, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, TaiwanSearch for more papers by this authorYuh-Jyh Jong MD, DMSci, Corresponding Author Yuh-Jyh Jong MD, DMSci Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Department of Laboratory Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Department of Biological Science and Technology, National Chiao Tung University, Hsinchu, TaiwanCorrespondence to: Prof. Y.-J. Jong, MD, DMSci, Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University No. 100, Shih-Chuan 1st Road, Kaohsiung 80708, Taiwan. E-mail: [email protected]Search for more papers by this author First published: 09 April 2014 https://doi.org/10.1002/ppul.22959 Conflict of interest: None. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Volume49, Issue8August 2014Pages 827-828 RelatedInformation

Acute neuromuscular respiratory failure: a population-based study of aetiology and outcome in Northern Ireland

BackgroundAcute neuromuscular respiratory failure (NMRF) is a life-threatening feature of a variety of neurological conditions that can present in extremis prior to the establishment of a definitive diagnosis, so early...

Pneumonia in pediatric acute neuromuscular respiratory failure

Pediatric PulmonologyVolume 49, Issue 8 p. 825-826 Letter to the Editor Pneumonia in pediatric acute neuromuscular respiratory failure Massimiliano Don MD, PhD, Corresponding Author Massimiliano Don MD, PhD Pediatric Care Unit, “Sant'Antonio” General Hospital, San Daniele del Friuli, Udine, ItalyCorrespondence to: Massimiliano Don, MD, PhD, Pediatric Care Unit, “Sant'Antonio” General Hospital, Viale Trento Trieste, 33, 33038 San Daniele del Friuli, Udine, Italy. E-mail: max.don@libero.itSearch for more papers by this author Massimiliano Don MD, PhD, Corresponding Author Massimiliano Don MD, PhD Pediatric Care Unit, “Sant'Antonio” General Hospital, San Daniele del Friuli, Udine, ItalyCorrespondence to: Massimiliano Don, MD, PhD, Pediatric Care Unit, “Sant'Antonio” General Hospital, Viale Trento Trieste, 33, 33038 San Daniele del Friuli, Udine, Italy. E-mail: max.don@libero.itSearch for more papers by this author First published: 11 December 2013 https://doi.org/10.1002/ppul.22961Citations: 1 The present Letter to Editor has been done without any grant or financial support and there are not potential conflicts of interest. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume49, Issue8August 2014Pages 825-826 RelatedInformation

A NOVEL TAUOPATHY PRESENTING WITH A MOTOR NEURONE DISEASE PHENOTYPE

We report a case of subacute progressive walking difficulty, dysarthria and dysphagia, which clinically resembled motor neurone disease (MND) but which proved on post mortem examination to be a novel...

Changing trends in the management of end-stage neuromuscular respiratory muscle failure: Recommendations of an international consensus

Changing trends in the management of end-stage neuromuscular respiratory muscle failure: Recommendations of an international consensus

Prolonged ventilatory failure and flaccid quadriparesis after ingestion of poison hemlock

A 28-year-old man presented with acute flaccid paralysis and respiratory failure that persisted for 2 weeks after suicidal ingestion of unknown substances. Extensive clinical, nerve, laboratory, and neuroimaging testing excluded alternative causes of this neuromuscular syndrome. Prompted by clues provided by family members, liquid chromatography time-of-flight mass spectrometry was used to investigate for the presence of poison hemlock. Testing of the residue in a jar used for the ingestion of a poisonous concoction confirmed the presence of the nicotinic alkaloid coniine. Analysis of patient serum suggested the presence of conhydrine. Concentrations of amitriptyline and diazepam were also found to be supratherapeutic, but only through the first few days of hospitalization. Herein we describe a case of reversible coma, flaccid quadriparesis, and neuromuscular respiratory failure caused by intentional ingestion of poison hemlock.

Combined noninvasive ventilation and mechanical in‐exsufflator in the treatment of pediatric acute neuromuscular respiratory failure

The present study aims to evaluate the efficacy and complications of combined noninvasive ventilation (NIV) and assisted coughing by mechanical in-exsufflator (MIE) for acute respiratory failure (ARF) in children with neuromuscular disease (NMD). A prospective study was conducted in the pediatric intensive care unit. Children with NMD and ARF treated by combined NIV and MIE were included. Treatment success was defined as freedom from tracheal intubation during the hospital stay. Physiologic indices including PaO2 , PaCO2 , pH, and PaO2 /FiO2 were recorded before and 12, 24 hr after the use of NIV/MIE. Combined NIV/MIE was used in 15 NMD children (mean: 8.1 years, range: 3 months to 18 years) with 16 cases of ARF. There was no mortality in this cohort. Treatment success was achieved in 12 cases (75%), including six cases (38%) demanding "Do Not Intubate." ARF was due to pneumonia, with a mean baseline PaCO2 of 73.2 ± 19.0 mmHg. In the success group, hypercarbia and acidosis improved after use of NIV/MIE for 24 hr (PaCO2 : 71.7 ± 18.6 mmHg vs. 55.8 ± 11.6 mmHg, P < 0.01; pH: 7.29 ± 0.07 vs. 7.38 ± 0.05, P < 0.01). All patients tolerated NIV/MIE well despite transient skin pressure sores in five cases. Combined NIV/MIE is a safe and effective approach to rapidly improve physiologic indices and decrease the need for intubation in NMD children with ARF. NIV/MIE provides a good alternative for those refusing intubation.

Effect of α7 nicotinic acetylcholine receptor agonists and antagonists on motor function in mice

Nicotinic acetylcholine receptors (nAChRs) are ligand-gated cation channels found throughout the body, and serve to mediate diverse physiological functions. Muscle-type nAChRs located in the motor endplate region of muscle fibers play an integral role in muscle contraction and thus motor function. The toxicity and teratogenicity of many plants (which results in millions of dollars in losses annually to the livestock industry) are due to various toxins that bind to nAChRs including deltaline and methyllycaconitine (MLA) from larkspur (Delphinium) species, and nicotine and anabasine from tobacco (Nicotiana) species. The primary result of the actions of these alkaloids at nAChRs is neuromuscular paralysis and respiratory failure. The objective of this study was to further characterize the motor coordination deficiencies that occur upon exposure to a non-lethal dose of nAChR antagonists MLA and deltaline as well as nAChR agonists nicotine and anabasine. We evaluated the effect of nAChR agonists and antagonists on the motor function and coordination in mice using a balance beam, grip strength meter, rotarod, open field analysis and tremor monitor. These analyses demonstrated that within seconds after treatment the mice had significant loss of motor function and coordination that lasted up to 1min, followed by a short period of quiescence. Recovery to normal muscle coordination was rapid, typically within approximately 10min post-dosing. However, mice treated with the nAChR agonist nicotine and anabasine required a slightly longer time to recover some aspects of normal muscle function in comparison to mice treated with the nAChR antagonist MLA or deltaline.

CLINICAL EVALUATION OF TRANSCUTANEOUS CARBON DIOXIDE MONITOR (TOSCA) IN PATIENTS WITH MOTOR NEURONE DISEASE

BackgroundMotor Neurone Disease (MND) causes progressive disability and neuromuscular respiratory failure is the commonest cause of death. Detection of respiratory failure (PCO2>6 kPa by definition) and treatment with non-invasive ventilation,...

Home-Based Overnight Transcutaneous Capnography/Pulse Oximetry for Diagnosing Nocturnal Hypoventilation Associated With Neuromuscular Disorders

ObjectiveTo determine the utility of home-based, unsupervised transcutaneous partial pressure of carbon dioxide (tc-Pco2) monitoring/oxygen saturation by pulse oximetry (Spo2) for detecting nocturnal hypoventilation (NH) in individuals with neuromuscular disorders. DesignRetrospective case series analyzed consecutively. SettingMultidisciplinary neuromuscular respiratory failure (NMRF) clinic at an academic institution. ParticipantsSubjects (N=35, 68.6% men; mean age, 46.9y) with spinal cord injury (45.7%) or other neuromuscular disorders underwent overnight tests with tc-Pco2/Spo2 monitoring. Fifteen (42.9%) were using nocturnal ventilatory support, either bilevel positive airway pressure (BiPAP) or tracheostomy ventilation (TV). InterventionsA respiratory therapist brought a calibrated tc-Pco2/Spo2 monitor to the patient’s home and provided instructions for data collection during the subject’s normal sleep period. Forced vital capacity (FVC), body mass index (BMI), and exhaled end-tidal Pco2 (ET-Pco2) were recorded at a clinic visit before monitoring. Main Outcome MeasuresDetection of NH (tc-Pco2 ≥50mmHg for ≥5% of monitoring time). Data were also analyzed to determine whether nocturnal oxygen desaturation (Spo2 ≤88% for ≥5% of monitoring time), FVC, BMI, or daytime ET-Pco2 could predict the presence of NH. ResultsNH was detected in 18 subjects (51.4%), including 53.3% of those using BiPAP or TV. NH was detected in 43.8% of ventilator-independent subjects with normal daytime ET-Pco2 (present for 49.4%±31.5% [mean ± SD] of the study period), and in 75% of subjects with an elevated daytime ET-Pco2 (present for 92.3%±8.7% of the study period). Oxygen desaturation, BMI, and FVC were poor predictors of NH. Only 3 attempted monitoring studies failed to produce acceptable results. ConclusionsHome-based, unsupervised monitoring with tc-Pco2/Spo2 is a useful method for diagnosing NH in NMRF.

Nocturnal Oximetry and Transcutaneous Carbon Dioxide in Home-Ventilated Neuromuscular Patients

Pulse oximetry alone has been suggested to determine which patients on home mechanical ventilation (MV) require further investigation of nocturnal gas exchange. In patients with neuromuscular diseases, alveolar hypoventilation (AH) is rarely accompanied with ventilation-perfusion ratio heterogeneity, and, therefore, oximetry may be less sensitive for detecting AH than in patients with lung disease. To determine whether pulse oximetry (S(pO(2))) and transcutaneous carbon dioxide (P(tcCO(2))) during the same night were interchangeable or complementary for assessing home MV efficiency in patients with neuromuscular diseases. Data were collected retrospectively from the charts of 58 patients with chronic neuromuscular respiratory failure receiving follow-up at a home MV unit. S(pO(2)) and P(tcCO(2)) were recorded during a 1-night hospital stay as part of standard patient care. We compared AH detection rates by P(tcCO(2)), S(pO(2)), and both. AH was detected based on P(tcCO(2)) alone in 24 (41%) patients, and based on S(pO(2)) alone with 3 different cutoffs in 3 (5%), 8 (14%), and 13 (22%) patients, respectively. Using both P(tcCO(2)) and S(pO(2)) showed AH in 25 (43%) patients. Pulse oximetry alone is not sufficient to exclude AH when assessing home MV efficiency in patients with neuromuscular diseases. Both P(tcCO(2)) and S(pO(2)) should be recorded overnight as the first-line investigation in this population.

Eelco Wijdicks: a neurocritical appraisal

Eelco Wijdicks: a neurocritical appraisal

New hopes and better treatment prospects for patients with neuromuscular pathologies and respiratory failure?*

New hopes and better treatment prospects for patients with neuromuscular pathologies and respiratory failure?*

Usefulness of pulmonary function tests and blood gases in acute neuromuscular respiratory failure

Define the usefulness of pulmonary function tests (PFT) and arterial blood gases (ABG) in patients admitted to the ICU with acute neuromuscular respiratory failure (NMRF). We reviewed 76 patients admitted to an ICU at Mayo Clinic (Rochester) between 2003 and 2009 with acute NMRF defined as need for mechanical ventilation (MV) because of primary impairment of the peripheral nervous system. Poor functional outcome was defined as a modified Rankin score >3. Median age was 65years. The most frequent diagnosis was myasthenia gravis (25 patients); 54% of patients had no known neuromuscular diagnosis before admission, and 11% had no specific diagnosis at discharge. Median MV time was 16days; 14% of patients died during hospitalization, and 63% were severely disabled at discharge. Maximal expiratory pressure ≤30cm H(2) O and maximal inspiratory pressure (MIP) worse than -28cm H2 O before MV were associated with need for invasive MV for longer than 7days (P=0.02). Indicators of chronic respiratory acidosis (low pH, high pCO2 , and high HCO3 ) before MV were associated with in-hospital death and poor functional outcome, but mostly in patients with progressive, untreatable neuromuscular diagnoses. In patients with primary acute NMRF, bedside PFT and ABG before MV can be used to predict evolution and outcome. Lower MIP and MEP portend prolonged MV and are more useful than forced vital capacity. Presentation with chronic respiratory acidosis is associated with high risk of in-hospital mortality and severe disability, especially in patients without treatable diagnoses.

Home-Based Diagnosis of Nocturnal Hypoventilation in Neuromuscular Disorders

PURPOSE: Neuromuscular respiratory failure (NMRF) often begins with nocturnal hypoventilation, but early diagnosis is limited because facility-based polysomnograms can be difficult to obtain for severely disabled patients. To address this problem, we have employed in-home monitoring with pulse oximety/transcutaneous capnography. This study describes our experience with this approach.

Survival and CTG repeat expansion in adults with myotonic dystrophy type 1

An association is observed between the severity of myotonic dystrophy type 1 (DM1) and the genetic abnormality of cytosine-thymine-guanine (CTG) repeat expansion. It is unknown whether an association exists between survival and CTG repeat expansion. In an adult 406-patient DM1 cohort, the phenotype, including survival age, was evaluated in relation to CTG repeat expansion. At study entry, age was 42 ± 12 (range 18-78) years, with a CTG repeat length of 629 ± 386 (range 54-1965). An inverse correlation was observed between CTG repeat length and the age at onset and younger DM1 phenotype. Over a follow-up of 9.2 ± 3.1 years, 118 (29.1%) patients died, including 60 of neuromuscular respiratory failure, 41 of cardiac causes, and 17 of non-neuromuscular, non-cardiac causes. There was an inverse relationship between all-cause survival and CTG length (relative risk 5.4 per log repeat, 95% confidence interval 2.9-10.2, P < 0.001). The data demonstrate a genotype-mortality association in DM1.

Causes and Outcomes of Acute Neuromuscular Respiratory Failure

Causes and Outcomes of Acute Neuromuscular Respiratory Failure

Causes and Outcomes of Acute Neuromuscular Respiratory Failure

Causes and Outcomes of Acute Neuromuscular Respiratory Failure