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Limb-girdle muscular dystrophy

Limb-girdle muscular dystrophy or Erb's muscular dystrophy is a type of muscular dystrophy that includes Duchenne muscular dystrophy, Becker's muscular dystrophy, and a large number of rarer disorders. more...

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The term "limb-girdle" is used to describe these disorders because the muscles most severely affected are generally those of the hips and shoulders -- the limb girdle muscles.

Common symptoms of limb-girdle muscular distrophy are muscle weakness, myoglobinuria, pain, myotonia, cardiomyopathy, elevated serum CK, and rippling muscles.

The muscle weakness is generally symmetric, proximal, and slowly progressive.

Generally pain is not present with LGMD, and mental function is not affected.

LGMD can begin in childhood, adolescence, young adulthood or even later. The age of onset is usually between 10 and 30. Both genders are affected equally. When limb-girdle muscular dystrophy begins in childhood the progression appears to be faster and the disease more disabling. When the disorder begins in adolescence or adulthood the disease is generally not as severe and progresses more slowly.

The distal muscles are affected late in LGMD, if at all. Over time (usually many years), the person with LGMD loses muscle bulk and strength. Eventually, he may need a power wheelchair or scooter, especially for long distances.

While LGMD isn't a fatal disease, it may eventually weaken the heart and lung muscles, leading to illness or death due to secondary disorders.

LGMD is typically an inherited disorder, though it may be inherited as a dominant, recessive, or X-linked genetic defect. The result of the defect is that the muscles cannot properly form the proteins needed for normal muscle function. Several different proteins can be affected, and the specific protein that is absent or defective identifies the specific type of muscular distrophy.

Treatment for LGMD is primarily supportive. Exercise and physical therapy are advised to maintain as much muscle strength and joint flexibility as possible. Assistive devices may be used to maintain mobility and quality of life. Careful attention to lung and heart health is also required.


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Respiratory function, electrocardiography and quality of life in individuals with muscular dystrophy
From CHEST, 7/1/94 by Gerd Ahlstrom

All individuals in a Swedish county afflicted with any type of hereditary muscular dystrophy (MD) were identified and 57 (85 percent) of eligible individuals in the age range 16 to 64 were included in the study. Respiratory disturbances were estimated by means of spirometry and analysis of arterial blood gases, and 58 percent yielded abnormal results on at least one of these examinations. Elevated [Pco.sub.2] was found more commonly than reduced forced vital capacity (FVC) and there was a moderate association between these parameters. Respiratory symptoms, most commonly breathlessness, were encountered in 79 percent. Pathologic ECG recordings were found in 21 individuals (37 percent). Conduction disturbances and affection of the myocard were most frequent in myotonic dystrophy. Quality of life was assessed by means of the Sickness Impact Profile instrument and the Kaasa test. The results showed that quality of life was significantly related to FVC and to the symptom of abnormal fatigue. Respiratory and cardiac parameters showed a greater number of significant correlations with measures of functional ability than with subjective well-being.

(Chest 1994; 106:173-79)

ANOVA=analysis of variance;

df=degrees of freedom;

F=variance ratio;

[FEV.sub.1]=forced expiratory volume at 1 s;

FEV%=[FEV.sub.1] expressed as a percentage of FVC;

FVC=forced vital capacity;

MD=muscular dystrophy;

MDTH=myopathia distalis tarda hereditaria;

MRF=myopathy with respiratory failure;

rho=Spearman Rank Correlations;

SIP=Sickness Impact Profile

Key words: muscular dystrophy; myotonic dystrophy; quality of life; respiration

The muscular dystrophies (MD) comprise several groups of inherited disorders with a wide variation between types in the distribution(1) and severity of muscle involvement. The respiratory muscles are often affected.(2)(3) A severe restrictive respiratory failure is often found in the Duchenne MD(3)(4) variation and in a recently described type of myopathy in Sweden.(5) In some patients with limb-girdle MD, severe weakness of the diaphragm has been reported to cause restrictive respiratory failure.(2) Respiratory insufficiency has been shown in patients afflicted with myotonic dystrophy, secondary to weakness of the respiratory muscles causing a restrictive failure,(6)(7) but central mechanisms are probably involved here.(8)(9)

Cardiac abnormalities are frequent in patients afflicted with myotonic dystrophy,(10)(11)(12)(13) Duchenne MD(14) and Emery-Dreifuss MD.(15) In myotonic dystrophy, the cardiac manifestations are usually asymptomatic even if some of these patients are struck by sudden death caused mainly by cardiac arrhythmia.(12)(13)

In the last decade, questionnaires for the measurement of quality of life have been developed and validated. They have mainly been used to evaluate medical care.(16)(17)(18) A study on 82 ventilator-assisted boys with Duchenne MD indicated that their subjective assessment of life satisfaction did not correlate well with healthcare professionals' estimated quality of life for the boys.(19) Studies on respiratory disorders have shown a weak association between the measured quality of life and ventilatory capacity as measured by spirometry,(20)(21)(22)(23) but a strong association between dyspnea and reduced quality of life.(22)(23)(24)

Respiratory or cardiac failure often causes premature death in individuals with MD, even if most patients lack overt symptoms until late in life. However, earlier in life, these factors might imply a reduced quality of life.

In a recent study,(1) we identified all individuals afflicted with different types of myotonic disorders and other MD in the county of Orebro, Sweden, and the prevalence was 39 per 100,000 population in January 1988.(1) The aim of the present study is to gain knowledge about the respiratory and heart functions in MD patients and to assess the extent to which these factors are associated with quality of life.


The Study Group

In a previous study,(1) involving the total population of the county of Orebro, Sweden, we identified 107 individuals (age range 5 to 79) afflicted with different types MD. The present study group comprised those 76 individuals in the age range 16 to 64 years who were alive during the autumn of 1990. Excluded were four individuals whose MD diagnosis could not be sufficiently certified and five individuals with mental retardation or mental disorders because they were not expected to be able to participate in the examinations and interviews. The remaining 67 individuals were informed about the study and asked to participate. Informed consent was obtained from 57 individuals. The distribution and the characteristics of the study group are shown in Table 1. Patients with MDTH were significantly older than those in the remaining groups (F=11.2, df=2/54, p<0.001) and showed a later onset (F=15.1, df=2/54, p<0.001). The 19 individuals (drop-outs excluded) did not differ substantially from the study group (mean age 43 years, age range 24 to 60).

Table 1--The Distribution of the Study Group by Number, Diagnosis, Sex,

Age, and Duration of Disease

Out of the 57 individuals, 45 (79 percent) were ambulatory but 21 of them had difficulties climbing stairs. Out of the 12 individuals who used a wheel-chair, seven were able to move short distances indoors without it. Three individuals were heavy smokers (more than 10 pack-years: one pack-year=20 cigarettes/day during 1 year), five were moderate, and seven were exsmokers. Three of the exsmokers had previously been heavy smokers. Three individuals afflicted with MRF used a ventilator at night, and one, with severe limb-girdle MD, used a ventilator nearly 24 h/d. Three had tracheostomas and one had a nasal mask. They had used ventilatory assistance for 1, 5, 9, and 10 years, respectively.


Pulmonary Function Tests: The FVC and forced expiratory volume at one second ([FEV.sub.1]) were measured in the sitting and supine positions with a bellows spirometer (Vitalograph Ltd, Buckingham, United Kingdom). One person could not perform spirometry because of leakage through his tracheostoma. The [FEV.sub.1] expressed as a percentage of FVC (FEV percent) was calculated. All values were compared with previously published normal values.(25) Radial artery blood samples were obtained in the sitting position and immediately analyzed in an ABL 2 (Radio-meter, Copenhagen, Denmark). The distributions of pulmonary function parameters were classified in the following categories in an effort to obtain approximate equal group sizes. The FVC: moderately reduced, 50 to 70 percent pred value; severely reduced, <50 percent pred; and [Pco.sub.2]: moderately elevated, 5.7 to 6.0 kPa (43 to 45 mm Hg); severely elevated [greater than or equal to] 6.1 kPa ([greater than or equal to] 46 mm Hg).

All participants replied to a brief questionnaire regarding pulmonary or heart disorders, smoking habits, and respiratory symptoms, ie, breathlessness, fatigue, sleep-related symptoms, and morning headache.

Cardiac Function Test: Standard 12-lead electrocardiograms (ECG) were recorded and classified according to a computerized ECG interpreting system (Hewlett Packard) modified in accordance with the standards of the department of Clinical Physiology, Orebro Medical Center Hospital. An experienced clinical physiologist scrutinized, verified, and classified the abnormalities according to these standards. The ECG abnormalities were classified with regard to the following: (1) rhythm abnormalities including significant sinus arrhythmias, atrial flutter, or fibrillation; (2) conduction disturbances including first degree AV-block and intraventricular blocks; (3) hypertrophy of atria or ventricles; and (4) signs of affection of the myocard, such as significant Q-waves or ST-T abnormalities.

Quality of Life Measurements: Two questionnaires were used for the assessment of quality of life, a test of psychosocial well-being (the Kaasa test)(26)(27) and the Sickness Impact Profile (SIP).(28) In both, a higher score means a lower quality of life.

The Kaasa test estimates the psychosocial well-being and the individual's global evaluation of quality of life. The psychosocial index consists of ten items with five positive and five negative statements to avoid agreement independent of their contents. The items are scored 1 to 5. The global evaluation includes two items/indices (happiness and satisfaction) scored 1 to 7.(26)(27)

The SIP is designated to assess sickness-related behavioral dysfunctions and consists of 136 items. Affirmative answers are weighted(29) and provide summary scores for physical, psychosocial, and overall behavioral dysfunction, as well as separate scores for 12 categories of activity, namely sleep and rest, emotional behavior, body care and movement, home management, mobility, social interaction, ambulation, alertness behavior, communication, employment, recreation and pastime, and eating. All scores express dysfunction in terms of a percentage of the maximum. The physical index combines body care and movement, mobility, and ambulation. The psychosocial index consists of emotional behavior, social interaction, alertness behavior, and communication.(29) The SIP was translated into Swedish in 1981 by three persons each of whom produced an independent version. These persons represented different categories of medical and social expertise. The three versions were pooled and the pooled version was translated back into English for validation.(30) The instrument was used in a pilot study, revised, and then evaluated for Swedish circumstances according to recommended standards for cross-cultural applications.(28)(30)


Participants were interviewed twice in their homes with an interval of 3 months. The interviews concerned experiences from the activities of daily life and coping (data will be presented elsewhere). Before the second interview, they completed the SIP, and at the second interview they completed the Kaasa test, together with a short questionnaire regarding respiratory complaints. Between 2 and 3 months after the second interview, they were examined by a neurologist and lung specialist who confirmed the diagnosis, performed spirometric examination, arterial blood gas analysis, and an ECG. Except for a few cases with myotonic dystrophy, all cases were examined with a muscle biopsy or electromyography (usually both) to verify the diagnosis.

Statistical Analysis

Pearson coefficients of correlation (r) were used on interval-and ratio-scale data. Spearman Rank correlations (rho) were applied to ordinal and nominal data. Analysis of variance (ANOVA) was used to calculate differences between groups.


Results regarding respiratory function are summarized in Table 2. Forced vital capacity was reduced to an average of 2.9 L, compared with an expected average of 4.2 L. Only 22 individuals (39 percent) had normal FVC values and normal [Pco.sub.2]. Fifteen individuals (26 percent) were judged pathologic in both aspects. Another seven individuals had a reduced FVC and a normal [Pco.sub.2]. Furthermore, ten individuals had an elevated [Pco.sub.2] but a normal FVC. Out of the ten individuals who had the highest [Pco.sub.2] ([greater than or equal to]46 mm Hg), nine had an FVC less than 55 percent of the predicted value and one had an FVC 79 percent of the predicted. There was a statistically significant correlation between [Pco.sub.2] and FVC (r=0.6, n=55, p<0.01). Six individuals were slightly hypoxic with a [Po.sub.2] of 63 to 66 mm Hg (8.4 to 8.8 kPa). Four of these had a pathologically reduced FVC, and the two individuals with the most reduced FVC also had an elevated [Pco.sub.2].

Four individuals had an FEV percent below 70 (range 62 to 69). One of them had an FVC below 1 I, which makes the FEV percent inexact. None of the four had reported symptoms of obstructive airways disease. The FVC was pathologically reduced in all individuals with MRF, in 13 (41 percent) of those with myotonic disorders (Table 2), and also in two heavy smokers. All individuals with MRF had a pathologically elevated [Pco.sub.2]. The corresponding value among those with myotonic disorders was 54 percent.

Table 2--Symptoms and Signs Related to Respiratory Dysfunction in Individ

uals With MD

Out of 33 individuals reporting exertional dyspnea (Table 2), 19 had an elevated [Pco.sub.2], and 16 a decreased FVC. Headache was reported by ten individuals (Table 2), and out of these, seven had a reduced FVC and five an elevated [Pco.sub.2]. Twenty-five individuals reported disturbed sleep at night, caused mainly by frequent awakenings, phlegm, and coughing. Fourteen individuals reported that their relatives had complained about snoring or other disturbing noise. Out of 16 individuals who reported abnormal fatigue (Table 2), 8 showed a reduced FVC and 9 had an elevated [Pco.sub.2].

A relatively weak but significant correlation was found between FVC and the duration of the disease, (r=0.33, n=56, p<0.05). No significant association was found between [Pco.sub.2] and duration of disease (r=0.24, n=55, p=0.08).

The ECG was judged to be pathologic for 37 percent of the study group (Table 3). Conduction disturbances and myocard affections were most frequent in myotonic dystrophy and the group of "other" MDs. There were no significant differences in ECG parameters with regard to age or age at onset of the disease (data not shown). There was a fair correlation between the ECG disturbances and duration of the disease (rho=0.34, n=57, p<0.05) as well as between ECG and FVC respectively [Pco.sub.2] (for both, rho=0.4, n=55, p<0.05).


The SIP indicated reductions of quality of life (high scores) for the physical activities ambulation and body care/movement (Fig 1). Other categories indicating low quality of life were home management, employment, sleep/rest, and recreation/pastime. The quality of life (estimated with SIP) was reduced in parallel with the decline of the respiratory function, and the association was statistically significant with regard to FVC (Table 4).



There were no statistically significant differences between the three groups of MD with regard to the mean scores in the Kaasa test; the psychosocial index 2.1, SD 0.7 (variance ratio [F]=0.12, df 2/54), the happiness index 3.1 SD 1.1 (F=0.80), and the index satisfaction 2.5, SD 1.1 (F=0.34). The result was similar with respect to the mean scores, in the three indices of the SIP; the overall index 12.3, SD 12.9 (F=0.18, df 2/54), the physical index 13.5, SD 16.7 (F=1.48), and the psychosocial index 7.1, SD 10.9 (F=0.57).

The Kaasa indices were significantly associated with experienced fatigue (rho=0.42 to 0.52), use of ventilator (rho=0.42 to 0.49) and headache (rho=0.28 to 0.36) but not with dyspnea, respiratory function, and the ECG parameters. In contrast, the SIP indices were related to several parameters (Table 5).


The relative independence of well being (Kaasa) and functional abilities (SIP) is shown by the following pattern of intercorrelations. The physical and psychosocial SIP indices correlated strongly with each other and with SIP overall (r=0.71, 0.94, and 0.88 respectively). The Kaasa indices were strongly interrelated (r=0.72, 0.72, and 0.77) whereas no SIP index correlated higher than 0.28 with any of the Kaasa indices.


The study group comprised 85 percent (57/67) of all eligible adults in the defined age range afflicted with a muscular dystrophy in the county of Orebro identified in a previous study.(1) This permits fairly strong generalizations about conditions for the whole group of individuals with muscular dystrophy, in spite of the fact that the distribution of some of the disorders may vary considerably as a result of an uneven distribution of genes between populations, and the rather small size of the study group in the present study. The local variation is most evident with regard to MDTH and MRF.(1)

More than half of the group presented abnormal values at spirometry or at arterial blood parameters, which confirms that impaired respiratory function is a common feature in MD. The results showed also that spirometry alone is not sufficient for estimating respiratory function in muscular dystrophy.(2)

As many as 26 individuals had pathologically elevated [Pco.sub.2]. Hypercapnia is considered to occur as a late, preterminal event in Duchenne MD,(4)(31) but in various other myopathies such as limb-girdle MD with early involvement of the diaphragm, [CO.sub.2] retention is likely to occur at an earlier stage of the disease.(2) It has previously been established that profound hypoventilation may occur during sleep in patients with MD even if daytime arterial blood gases are normal.(32)(33) Most sleep studies have been performed on Duchenne MD and myotonic dystrophy. In the latter, a complex pattern of sleep related breathing disturbances occur which may partly be of central origin, including apneas both during sleep and at rest while awake.(6)(7)(8)(9) To find out whether the individuals in present study group suffer from hypoventilation during sleep it would be desirable to perform polysomnography or some other kind of sleep monitoring but this was beyond the scope of the present study.

An abnormal ECG is frequently found in myotonic dystrophy. Conduction abnormalities were the most prevalent, encountered in 53 percent of the individuals. This is in the same range as was found in a recent study in Sweden (42 percent)(12) and one in the Netherlands (55 percent).(11) Abnormalities of repolarization and pathologic Q-waves were reported in the Swedish study in 32 and 28 percent respectively, but were less frequent in the present data.

Only 12 individuals (21 percent) used a wheel-chair and were severely disabled. Thus, the high proportion of respiratory and heart dysfunctions cannot be explained by a high degree of handicap in the study group. A similar conclusion has been drawn for patients with myotonic dystrophy and progression of cardiac conduction disease.(13)

The instruments used for the assessment of quality of life have been used in studies of other diseases. Repeated administration of the Kaasa test to patients with lung cancer yielded means and standard deviations within the same range as in the present study.(26) The Swedish version of SIP has been extensively used and validated with individuals with rheumatic disorders,(30)(34)(35)(36)(37) spinal cord injuries,(38)(39)(40) chronic pain, (41) lung cancer,(42)(43) stroke,(44)(45) chronic renal insufficiency,(46) tension headache,(47) osteoarthrosis,(48) intermittent claudication,(49) and respiratory failure in scoliosis and neuromuscular disorders.(50) The results of the SIP from subjects in Swedish population surveys focusing on a progressive chronic disease like rheumatoid arthritis can be compared with the present data. In two surveys on rheumatoid arthritis,(30)(36) the psychosocial index was almost the same (means 7.7, 8.0) as the present results (mean 7.1), but the physical index indicated a better quality of life in rheumatoid arthritis (means 7.7 and 9.8) than for individuals with MD (mean 13.5). In two studies on rheumatoid arthritis,(34)(36) lower total, physical, and psychosocial values for quality of life were indicated. These results were based on data from consecutive outpatients.

Data on persons with neuromuscular disorders (five of them with MD) were described in a recent Swedish study of 39 persons with home mechanical ventilation.(50) The patients in the subgroup neuromuscular disorders (n=10) reported a low quality of life (mean physical index 19.4 and psychosocial index 8.4) compared with scoliosis, postpolio, and healed pulmonary tuberculosis with thoracoplasty.(50) The present results can also be compared with the results from an age-stratified group of 112 healthy Swedish women,(36)(51) who reported markedly better quality of life on both the physical (mean 3.3) and the psychosocial (mean 5.1) indices. A Dutch study of patients with obstructive lung disease in family practice reported better quality of life (mean, physical index 5.7, and psychosocial index 6.5)(23) than for patients with MD in the present study. Thus, individuals with MD have lower quality of life mainly on the physical index, depending on the fact that MD causes disabilities, which may result in restrictions in everyday life. The results of the present study reflect the fact that the data derive from a population-survey. A study on patients only would probably have produced a higher impact of MD on quality of life.

Some studies on quality of life have shown an association between quality of life and dyspnea among patients with lung diseases.(22)(23)(24) In the present study, quality of life showed a stronger relation to fatigue and distrubed sleep comments by relatives.

The present study showed impaired quality of life in individuals with MD irrespective of type of MD, according to both tests used. The Kaasa test assesses quality of life by means of general statements concerning psychosocial well-being, whereas the SIP uses estimates based on statements related to functional ability in 12 categories of activity.(26)(28)(29) In other words, the questionnaires measure different aspects of quality of life. There was a moderate but significant association between respiratory and cardiac parameters and quality of life assessed by the physical index of the SIP. Thus, these factors can be valuable predictors, but not the only ones, with regard to quality of life. The fact that respiratory and cardiac parameters were not related to psychosocial well-being (Kaasa) suggests a relative independence of physical and psychosocial dimensions of quality of life. Previous studies, however, have indicated moderate correlations between functional and psychosocial dimensions.(17)(21) This indicates the necessity of incorporating both dimensions in evaluations of medical care.(17)

The present study is limited by the use of a cross-sectional design, as are most other studies of MDs.(7)(11)(12) Longitudinal studies are usually restricted to a few individuals or only one function.(13) Long-term follow-up of individuals afflicted with MDs are warranted to gather more knowledge about the predictive value of respiratory and heart parameters for future morbidity and the relation of these factors with quality of life.


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(*)From the Orebro College for the Health Professions, Orebro, Sweden (Mrs. Ahlstrom); Department of Neurology (Dr. Gunnarsson), Department of Lung Medicine (Dr. Kihlgren), Department of Clinical Physiology (Dr. Arvill), Orebro Medical Center Hospital, Sweden; and Center for Caring Sciences (Mrs. Ahlstrom and Dr. Sjoden), University of Uppsala, Sweden.

This project was supported by grants from the Orebro County Council Research Committee and the Swedish Association of the Neurologically Disabled.

Manuscript received May 10, 1993; revision accepted November 30.

Reprint requests: Mrs. Ahlstrom, Orebro College of Health Professions, Box 1323, S-701 13 Orebro, Sweden

COPYRIGHT 1994 American College of Chest Physicians
COPYRIGHT 2004 Gale Group

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