Modeling of Lung Function Recovery in Neuralgic Amyotrophy With Diaphragm Impairment

Rice, Brenda L.; Ashton, Rendell W.; Shook, Steven J.; Mireles-Cabodevila, Eduardo; Aboussouan, Loutfi S.

doi:10.4187/respcare.05568

Cited by 5 publications

(7 citation statements)

References 36 publications

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“…Very slow recovery of respiratory muscle function after control of the causative insult has been well described in other neuro-myopathies affecting respiratory muscles. (39,40) In the case of ICI-myotoxicities, the concomitant use of high-dose corticosteroids favoring sarcopenia may also interfere deleteriously with muscular recovery. (41,42) Several limitations of our analysis need to be recognized.…”

Section: Discussionmentioning

confidence: 99%

Abatacept/Ruxolitinib and Screening for Concomitant Respiratory Muscle Failure to Mitigate Fatality of Immune-Checkpoint Inhibitor Myocarditis

et al. 2023

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Immune-checkpoint-inhibitor (ICI)-associated myotoxicity involves the heart (myocarditis) and skeletal muscles (myositis), which frequently occur concurrently and is highly fatal. We report the results of a strategy that included identification of individuals with severe ICI-myocarditis by also screening for and managing concomitant respiratory muscle involvement with mechanical ventilation, as well as treatment with CTLA4-fusion protein abatacept and the Janus-kinase inhibitor ruxolitinib. Forty cases with definite ICI-myocarditis were included with pathological confirmation of concomitant myositis in the majority of patients. In the first 10 patients, using recommended guidelines, myotoxicity-related fatality occurred in 60%, consistent with historical controls. In the subsequent 30 cases, we instituted systematic screening for respiratory muscle involvement coupled with active ventilation and treatment using ruxolitinib and abatacept. Abatacept dose was adjusted using CD86-receptor occupancy on circulating monocytes. Myotoxicity-related fatality rate was 3.4%(1/30) in these 30 patients vs.60% in 1st quartile(p<0.0001). These clinical results are hypothesis-generating and need further evaluation.

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Section: Discussionmentioning

confidence: 99%

Abatacept/Ruxolitinib and Screening for Concomitant Respiratory Muscle Failure to Mitigate Fatality of Immune-Checkpoint Inhibitor Myocarditis

et al. 2023

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“…They also saw a reduction in PImax to 38 % mean. Patients who subsequently recovered had lower PImax values, both initially and during follow-up, than patients without recovery, but the increase was significant [50].…”

Section: Functional Testing In Diaphragmatic Dysfunctionmentioning

confidence: 78%

Hepatitis E and diaphragmatic dysfunction: Case series and review of the literature

Westhoff,

Arias,

Litterst

2024

Pneumologie

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Introduction The causes of diaphragmatic paresis are manifold. An association between neuralgic amyotrophy (NA) and hepatitis E virus (HEV) infection has been reported. We wondered about the prevalence of diaphragmatic disfunction and hepatitis E infection in our clinic. Methods From July 1st, 2020 to August 31st, 2023, patients presenting with diaphragmatic dysfunction and simultaneous clinical symptoms of an acute NA, or a history of NA, as well as patients with previously unexplained diaphragmatic dysfunction were examined for HEV infection. Results By August 31st, 2023, 13 patients with diaphragmatic dysfunction and HEV infection were diagnosed (4 women, 9 men). Mean age was 59 ± 10 years. Liver values were normal in all patients. The median latency to diagnosis was five months (range: 1–48 months); nine patients, 4 of them with typical symptoms of NA, presented with acute onset three patients showed bilateral diaphragmatic dysfunction. All patients had a positive IgG immunoblot. Seven patients, three with NA, had an elevated hepatitis E IgM titer and six of them also a positive IgM immunoblot. In all cases, O2C hepatitis genotype 3 was identified. In eight cases, all those with a high IgG titer >125, the O2 genotype 1 was also detected. Conclusion NA that shows involvement of the phrenic nerve resulting in diaphragmatic dysfunction and dyspnoea, may be associated with HEV infection. The observation of 13 patients with diaphragmatic dysfunctions and HEV infection within a period of three years indicates a high number of undetected HEV-associated diaphragmatic dysfunction in the population, especially in the absence of NA symptoms. Therefore, even in diaphragmatic dysfunction without NA symptoms and causative damaging event, HEV infection should be considered, as it may represent a subform of NA with only phrenic nerve involvement. Therapy of HEV-associated diaphragmatic dysfunction in the acute phase is an open question. In view of the poor prognosis for recovery, antiviral therapy should be discussed. However, no relevant data are currently available.

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“…Recovery of pulmonary function is varied. In a study of 16 subjects (15 men) with mean follow-up 4 years [2], 31% recovered to a normal FVC, 38% recovered partially and 31% did not recover. When FVC improved, it was rapid initially followed by slowing and plateau.…”

Section: Discussionmentioning

confidence: 99%

“…The current patient experienced partial recovery, similar to the 38% and the 44% of patients in the above-mentioned series. No predictors of recovery, neither complete nor partial, have been determined [2, 3].…”

Section: Discussionmentioning

confidence: 99%

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Use of Noninvasive Ventilation with Volume-Assured Pressure Support in Neuralgic Amyotrophy with Bilateral Diaphragmatic Paralysis

Diaz‐Abad¹,

Porter²,

Zilliox³

et al. 2019

TORMJ

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Neuralgic Amyotrophy (NA) is a rare, acute onset inflammatory brachial plexopathy that frequently presents with acute pain followed by shoulder girdle muscle weakness. Phrenic nerve involvement affecting the diaphragms occurs in 7-10% of cases. We present the case of a 52-year-old man with neuralgic amyotrophy with phrenic nerve involvement and bilateral diaphragmatic paralysis with marked respiratory symptoms and sleep hypoventilation, who was treated with non-invasive ventilation with volume assured pressure support mode. By 21 months post disease onset, the patient had experienced marked improvement in orthopnea, sleep quality and functional status. This is the first reported case of the use of this mode of noninvasive ventilation in neuralgic amyotrophy.

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Modeling of Lung Function Recovery in Neuralgic Amyotrophy With Diaphragm Impairment

Abstract: Sixty-nine percent of subjects with diaphragm impairment from neuralgic amyotrophy experience recovery of lung function and diaphragm strength, but recovery is slow and may be incomplete.

Cited by 5 publications

References 36 publications

Abatacept/Ruxolitinib and Screening for Concomitant Respiratory Muscle Failure to Mitigate Fatality of Immune-Checkpoint Inhibitor Myocarditis

Abatacept/Ruxolitinib and Screening for Concomitant Respiratory Muscle Failure to Mitigate Fatality of Immune-Checkpoint Inhibitor Myocarditis

Hepatitis E and diaphragmatic dysfunction: Case series and review of the literature

Use of Noninvasive Ventilation with Volume-Assured Pressure Support in Neuralgic Amyotrophy with Bilateral Diaphragmatic Paralysis

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