Respiration in Dystrophia Myotonica

Gillam, P. M. S.; Heaf, P. J. D.; Kaufman, Léon; Lucas, B. G. B.

doi:10.1136/thx.19.2.112

Cited by 65 publications

(33 citation statements)

References 17 publications

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“…Irregu larity may then be considered to depend, to a large extent, on influences from the higher centers during conscious ness [20], not sufficiently corrected by the chemical con trol mechanism. This hypothesis is supported by Gillam et al [6], who showed irregularity to increase in MD in cases of depression of CO2 sensitivity by anesthetic agents. Similarly, in an earlier investigation [5], we found irregularity decreased with CO2 stimulus caused by an external dead space.…”

Section: Discussionsupporting

confidence: 76%

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Breathing Pattern Awake and Asleep in Myotonic Dystrophy

Ververs

Meché²,

Verbraak³

et al. 1996

Respiration

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Because myotonic dystrophy patients show marked irregularities of breathing both awake and asleep, variables related to breathing pattern under both conditions were measured in 11 patients, together with pulmonary function indices, ventilatory CO₂ response and maximal mouth pressures. The aim of the study was to detect and explain a possible interrelationship between daytime and nocturnal irregularity. Awake, patients demonstrated significantly more variability in tidal volume and respiratory cycle time than controls. Asleep, periodic breathing occurred during up to 100% of the time spent in light sleep, but not during deep sleep. A strong correlation was found with age (r = 0.73, p = 0.01). No relationship was found between disturbed breathing awake and asleep. There was a tendency for increased variability of tidal volume awake in cases with a decreased ventilatory CO₂ response (p = 0.1). The results indicate that different mechanisms may be involved in daytime and nocturnal irregularity. It is hypothesized that brain stem integrative functions may be impaired in myotonic dystrophy.

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Section: Discussionsupporting

confidence: 76%

“…It has been de scribed as oscillations in breathing amplitude, regularly decreasing and increasing. Moreover, irregular breathing during wakefulness has also been reported in MD patients [5][6][7].…”

Section: Introductionmentioning

confidence: 99%

Breathing Pattern Awake and Asleep in Myotonic Dystrophy

Ververs

Meché²,

Verbraak³

et al. 1996

Respiration

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“…These impulses have a stimulatory effect on alertness as well as on the respiratory centre (Kilburn et al, 1959a;Gillam et al, 1964). The hypothesis of a primary central disturbance of sleep regulation would also serve to clarify some anomalies in sleep structure, such as the abundance and precocity of the REM stages which we discovered in our case and which were probably present in the patient of Kilburn et al (1959a) who reported frequent dreams and episodes of sleep paralysis.…”

Section: Discussionmentioning

confidence: 50%

“…However, the first detailed description of the clinical picture of alveolar hypoventilation in a patient with hypofunction and myotonia of the respiratory muscles was provided by Benaim and Worster-Drought in 1954. Some other similar cases have subsequently been described (Bashour et al, 1955;Kilburn et al, 1959a;Cannon, 1962;Gillam et al, 1964;Kohn et al, 1964;Lee and Hughes, 1964).…”

mentioning

confidence: 73%

Alveolar hypoventilation and hyperosmnia in myotonic dystrophy.

Coccagna¹,

Mantovani²,

Parchi³

et al. 1975

Journal of Neurology, Neurosurgery & Psychiatry

101

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SYNOPSIS A case of myotonic dystrophy accompanied by alveolar hypoventilation and hypersomnia is presented. Radiological studies and EMG examination of the intercostal muscles demonstrated that the respiratory muscles were affected by the disease, while polygraphic recordings showed that the alveolar hypoventilation and pulmonary hypertension worsened during sleep. The hypersomnia preceded the appearance of clinical signs of the muscular disease by many years and persisted even after treatment when the blood gas analysis values were greatly improved. During both diurnal and nocturnal sleep, the patient frequently fell asleep directly into a REM stage. The possibility is discussed that, concomitant with the respiratory musculature involvement, there is an alteration in the central nervous system in myotonic dystrophy which is at least partially responsible for both the alveolar hypoventilation and the hypersomnia.

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“…The severity of the muscular dis ease has been classified into four grades [6]: disease mild (grade 1), disease moderately severe but patient still able to do light work (grade II), patient severely incapacitated (grade III) and patient bedridden (grade IV). Quinidine, Dilantin and procainamide used to treat MD and other drugs were not administered or discontinued 10 days before the study.…”

Section: Methodsmentioning

confidence: 99%

Ambulatory Electrocardiographic Monitoring in Myotonic Dystrophy (Steinert’s Disease)

Fragola¹,

Ruscitti²,

Autore³

et al. 1987

Cardiology

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Ambulatory electrocardiographic monitoring (AEM) was performed in 22 patients (range 13–62 years; mean age 38.2 ± 12.7) with grades I, II and III of myotonic dystrophy in order to evaluate the occurrence of potentially dangerous cardiac arrhythmias and conduction disturbances. All patients had previously undergone echocardiographic examination to determine whether structure and function abnormalities were present. In 6 patients with normal resting electrocardiogram, AEM revealed: first degree A-V block (4 cases), class IVa Lown ventricular arrhythmias (3 cases) and episodes of atrial fibrillation (4 cases). In 2 of 3 cases with abnormal scalar electrocardiogram new abnormalities (first degree A-V block and further prolongation of P-R interval) were demonstrated by AEM. Only 1 patient had mild signs of left ventricular dysfunction at echo. Disorders of cardiac conduction and rhythm are characteristic of myotonic dystrophy and can predispose to severe cardiac events. In this respect AEM is shown to be an early and sensitive tool in identifying patients at risk.

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Respiration in Dystrophia Myotonica

Cited by 65 publications

References 17 publications

Breathing Pattern Awake and Asleep in Myotonic Dystrophy

Breathing Pattern Awake and Asleep in Myotonic Dystrophy

Alveolar hypoventilation and hyperosmnia in myotonic dystrophy.

Ambulatory Electrocardiographic Monitoring in Myotonic Dystrophy (Steinert’s Disease)

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Respiration in Dystrophia Myotonica

Cited by 65 publications

References 17 publications

Breathing Pattern Awake and Asleep in Myotonic Dystrophy

Breathing Pattern Awake and Asleep in Myotonic Dystrophy

Alveolar hypoventilation and hyperosmnia in myotonic dystrophy.

Ambulatory Electrocardiographic Monitoring in Myotonic Dystrophy (Steinert&rsquo;s Disease)

Contact Info

Product

Resources

About

Ambulatory Electrocardiographic Monitoring in Myotonic Dystrophy (Steinert’s Disease)