Chest
Volume 107, Issue 5, May 1995, Pages 1309-1316
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Clinical Investigations: Pulmonary Function
Dyspnea in Dystonia: A Functional Evaluation

https://doi.org/10.1378/chest.107.5.1309Get rights and content

Background

Dystonia consists of action-triggered sustained focal muscle contractions, worsened by effort, and resulting in voice changes, abnormal posturing, and dyspnea. The cause of dyspnea, previously unexplained, is the basis of this report.

Methods

Since the maximal efforts required to perform pulmonary function testing (PFT) could worsen the muscular contractions in dystonic patients, we used several tests to identify possible causes of dyspnea. These included spirometry with flow volume loops (FVL), tidal volume breathing, maximum voluntary ventilation (MVV), and inspiratory and expiratory muscle pressures (PImax, PEmax), sitting and supine. We used cycle ergometry with arterial blood gas (ABG) values to detect cardiac/pulmonary limitations and respiratory inductive plethysmography (RIP) to assess chest wall/abdominal movements for synchrony. Dynamic videofluoroscopy (VF) assessed and recorded the action-triggered muscle activity of the upper airways and the diaphragm during quiet breathing, speech, swallowing, and maximal respiratory maneuvers similar to the efforts required during PFT.

Results

Twenty-six dystonic patients, 12 women and 14 men, ages 14 to 70 years (mean age, 52.3 years) were evaluated. Their neurologic classification included 22 primary (idiopathic) and 4 secondary (2 postneuroleptic use, 2 posttraumatic). Four patients originally classified as having focal dystonia had dyspnea and were found to have diaphragmatic and/or upper airway dysfunction too. The PFTs showed abnormal FVL and/or tidal volume breathing patterns, with intermittent interruptions of air flow during inspiration or expiration in 20 of 24 patients. The VF was abnormal in 24 of 26 patients: 19 patients had combined upper airway (UA) and diaphragmatic dysfunction (DD); 1 patient had UA dysfunction alone, and 4 patients had DD alone. Except for poor effort and/or dystonic movements, cycle ergometry was normal in 18 of 21 patients. The ABG values and/or pulse oximetry were normal in 19 of 22 patients.

Conclusion

Dyspnea in dystonia appears to be due to excessive and/or dysynchronized contractions of the upper airways and/or diaphragm, with usually normal gas exchange. These spasmodic and irregular muscular contractions during speech and daily activities are associated with the sensation of excessive effort to overcome the spasms. Excessive spasms can be triggered during PFT and are best detected on FVL patterns coupled with dynamic VF.

Section snippets

Methods

Twenty-six dystonic patients with dysphonia (strangled quality of speech, with interruptions) and dyspnea were referred for respiratory assessment from the Movement Disorders Center of the Neurological Institute of Columbia Presbyterian Medical Center, New York. Dystonia was primary (idiopathic) in 22 patients and secondary in 4 patients (Table 2). All described an awareness of or an increased effort for breathing. They were assigned a dyspnea score, depending on their perception of difficulty

Results

There were 12 women and 14 men (Table 2). Their ages ranged from 14 to 70 years (mean, 52.3 ± 13.8). They were grouped into three categories, based on the results of their spirometry and lung volumes: group 1 with normal PFT (n=19), group 2 with obstructive PFT (n=3), and group 3 with restrictive PFT (n=4). Flow volume loops were assessed for interruptions and/or irregularities and noted separately for each group. The etiology and classification of dystonia was determined by the referring

Discussion

Dyspnea in dystonia appears to be associated with spasmodic contractions of the upper airways and/or the diaphragm in most patients. Since maximum efforts are necessary for optimal performance of PFT, and increased efforts tend to worsen any volitional muscular contractions causing spasms, these efforts appear to be perceived as discomfort or dyspnea. Although patients showed a desire to comply with testing, extra efforts were necessary to overcome the spasmodic muscular contractions to produce

Acknowledgments

The authors gratefully acknowledge Dr. Joseph Ghassibi's help with exercise testing, and Beverly Cayetano's assistance with manuscript preparation.

References (21)

There are more references available in the full text version of this article.

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