Phrenic nerve conduction studies as a biomarker of respiratory insufficiency in amyotrophic lateral sclerosis

Jenkins, Julie; Sakamuri, Sarada; Katz, Jonathan; Forshew, Dallas; Guion, Lee; Moore, Dan H.; Miller, Robert G.

doi:10.3109/21678421.2015.1112406

Cited by 24 publications

(24 citation statements)

References 33 publications

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“…The phrenic nerve assessment proved to be a reliable means of predicting respiratory failure, as previously demonstrated in ALS patients (33)(34)(35), particularly when spirometry is not possible owing to limitations due to the illness (36). The chest X-ray and subsequent restrictive failure confirmed the diaphragmatic weakness.…”

Section: Discussionsupporting

confidence: 57%

64. Acute flaccid paralysis by Enterovirus D68 infection: First Italian description on adult patient and role of electrophysiology

Ceccanti¹,

Rubino²,

Romanzi³

et al. 2017

Clinical Neurophysiology

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A Peruvian woman was admitted to the Emergency Department, due to an acute flaccid paralysis (AFP) of the upper limbs that progressively involved also lower limbs and respiratory muscles. She previously suffered from non-Hodgkin's lymphoma and had to undergo hematopoietic stem cell transplantation. A magnetic resonance imaging showed a T2 hyperintensity in the anterior and central region of the cervical segment with an elective involvement of gray matter. This finding, combined with other clinical, laboratory, and electrophysiological data, led to a diagnosis of AFP. Enterovirus D68 was isolated in the patient's cerebrospinal fluid, plasma, and throat swab. To our knowledge, this is the first Italian case of AFP by Enterovirus D68 infection in an adult. The diagnostic assessment and management of AFP by Enterovirus D68 are discussed.

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Section: Discussionsupporting

confidence: 57%

64. Acute flaccid paralysis by Enterovirus D68 infection: First Italian description on adult patient and role of electrophysiology

Ceccanti¹,

Rubino²,

Romanzi³

et al. 2017

Clinical Neurophysiology

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“…These findings are (10,20), but because this was not measured in the current study we are unable to comment on its value in comparison with measurement of force as Pdi. We also acknowledge that our analysis is strictly confined to indices of respiratory muscle function.…”

Section: Critique Of the Methodsmentioning

confidence: 64%

“…Therefore, the current study assessed the value of noninvasive and invasive respiratory muscle strength assessments, at different time points up to 3 years, for the prediction of death or the initiation of NIV in a large cohort of patients with ALS (n = 78) (10,(19)(20)(21). Sn Pdi and Twi Pdi were the best performing tests for prediction of ventilator-free survival, but SNIP also had excellent predictive power.…”

Section: Significance Of the Findingsmentioning

confidence: 99%

Respiratory Muscle Strength as a Predictive Biomarker for Survival in Amyotrophic Lateral Sclerosis

Polkey

Lyall

Yang

et al. 2017

Am J Respir Crit Care Med

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Rationale: Biomarkers for survival in amyotrophic lateral sclerosis (ALS) would facilitate the development of novel drugs. Although respiratory muscle weakness is a known predictor of poor prognosis, a comprehensive comparison of different tests is lacking.Objectives: To compare the predictive power of invasive and noninvasive respiratory muscle strength assessments for survival or ventilator-free survival, up to 3 years.Methods: From a previously published report respiratory muscle strength measurements were available for 78 patients with ALS. Time to death and/or ventilation were ascertained. Receiver operating characteristic analysis was used to determine the cutoff point of each parameter.Measurements and Main Results: Each respiratory muscle strength assessment individually achieved statistical significance for prediction of survival or ventilator-free survival. In multivariate analysis sniff trans-diaphragmatic and esophageal pressure, twitch trans-diaphragmatic pressure (Tw Pdi), age, and maximal static expiratory mouth pressure were significant predictors of ventilationfree survival and Tw Pdi and maximal static expiratory mouth pressure for absolute survival. Although all measures had good specificity, there were differing sensitivities. All cutoff points for the VC were greater than 80% of normal, except for prediction of 3-month outcomes. Sequential data showed a linear decline for direct measures of respiratory muscle strength, whereas VC showed little to no decline until 12 months before death/ventilation. Conclusions:The most powerful biomarker for mortality stratification was Tw Pdi, but the predictive power of sniff nasal inspiratory pressure was also excellent. A VC within normal range suggested a good prognosis at 3 months but was of little other value.Keywords: amyotrophic lateral sclerosis; survival; diaphragm; maximal inspiratory pressure; sniff nasal inspiratory pressure Author Contributions: All authors contributed to the concept of the study, critically reviewed the manuscript, and approved this submission for publication. R.A.L. made the original measurements and reviewed the source data to create the material for the present analysis. K.Y. and E.J. undertook statistical analysis of the survival data to provide the analysis results for this paper. M.I.P. reviewed the source data with R.A.L. to create the material for the present analysis and prepared the first and subsequent draft of the manuscript. P.N.L. and J.M. were supervisors for the neurologic and respiratory muscle aspects, respectively, of the original data collection.

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“…Билатеральная патология диафрагмального нерва приводит к снижению переносимости физической нагрузки, диспноэ, ортопноэ или одышке в положении лежа, гиперкапнической дыхательной недостаточности, выраженной утомляемости и повышению артериального давления. В ряде случаев развиваются серьезные жизнеугрожающие состояния [2][3][4]. Новорожденным с двустронним поражением диафрагмального нерва требуется искусственная вентиляция легких.…”

Section: оригинальные исследованияunclassified

Conduction studies of phrenic nerve in healthy children

Войтенков¹,

Команцев²,

Скрипченко³

et al. 2018

Neuromuscular Diseases

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Background. Unlike parameters of phrenic nerve conduction in healthy adults characterized by stability, in children variations with age are observed. The objective is to investigate the M-wave latency and amplitude in electroneurographic (ENG) examination of the phrenic nerve in healthy children of different ages for development of a normative database of neurophysiological data.Materials and methods. 48 healthy children (28 girls and 20 boys) were examined. Mean age was 9.19 ± 5.43 years (1–18 years). ENG examination of the phrenic nerve was performed with the modified standard procedure of stimulation at the level of the outer margin of the lower third of the sternocleidomastoid muscle with registration of M-wave from standard diaphragm point and parallel registration of the muscle activity from m.deltoideus and m.serratus anterior.Results. Mean values of the M-response latency were 5.64 ± 1.25 ms, amplitude – 0.66 ± 0.34 mV. For age-differentiated subgroups 1–2 years (n = 7), 3–5 years (n = 9), 6–12 years (n = 15), and 13–18 years (n = 17), the latency was 4.96 ± 1.94; 5.01 ± 1.13; 5.42 ± 0.84, and 6.44 ± 1.43 ms, respectively; the amplitude was 1.01 ± 0.37; 0.87 ± 0.31; 0.61 ± 0.24, and 0.45 ± 0.21 mV, respectively. The M-response amplitude values in children aged 1–2 years significantly differed from the values in children aged 6–12 and 13–18 years.Conclusion. ENG examination of the phrenic nerve is a technically uncomplicated procedure, and the obtained data is easy to interpret. During phrenic nerve ENG in children, it is necessary to take age variability of the M-wave latency and amplitude into account. The M-wave amplitude in healthy toddlers (1–2 years old) was significantly lower than in children aged 6–18 years.

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Phrenic nerve conduction studies as a biomarker of respiratory insufficiency in amyotrophic lateral sclerosis

Cited by 24 publications

References 33 publications

64. Acute flaccid paralysis by Enterovirus D68 infection: First Italian description on adult patient and role of electrophysiology

64. Acute flaccid paralysis by Enterovirus D68 infection: First Italian description on adult patient and role of electrophysiology

Respiratory Muscle Strength as a Predictive Biomarker for Survival in Amyotrophic Lateral Sclerosis

Conduction studies of phrenic nerve in healthy children

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