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PubMed1992

Therapeutic trials on progressive muscular dystrophy.

The special medical care in the National Sanatorium prolonged the life span of the patients with progressive muscular dystrophy from 15.8 years to 20.4 years over the last 20 years. Various new drug trials for muscular dystrophy have been implemented in the last 12 years in Japan. Bestatin and Loxistatin, protease inhibitors, showed definite improvement on dystrophic mice or hamsters, animal models of muscular dystrophy. However clinical application of these drugs failed to prove the effects on patients with Duchenne muscular dystrophy. The difficulty of clinical evaluation and judgement of effects in progressive neurological diseases is discussed.

Animals↗
PubMed1953

Progressive muscular dystrophy; a preliminary report on treatment with amino acids, folic acid and vitamins.

Ten patients with progressive muscular dystrophy were given daily oral doses of amino acids, folic acid and selected vitamins. At the time of this report they had been treated by this means for periods varying from two months to one year. Only one had other therapy concurrently. Definite and progressive improvement, objective and subjective, occurred in all cases. Among objective changes noted-not all of them in all cases-were return of strength, increase in size and tonus of atrophic muscles, restoration of normal respiratory action and relief of depression. Patients reported a sense of well-being, increase in strength and a feeling of bodily warmth.

Amino Acids↗
PubMed1977

Systolic time intervals in patients with progressive muscular dystrophy of the Duchenne type.

Systolic time intervals (STIs) were measured in 57 patients with progressive muscular dystrophy (PMD) of the Duchenne type, and were correlated with the stages of physical disability. The total electromechanical systole (Q-A2), pre-ejection period (PEP), and left ventricular ejection time (LVET) were corrected for heart rate using the linear regression equations calculated from 91 normal subjects. Each systolic time interval index (STII), i.e. corrected STI, was analyzed. In 15 ambulatory patients (Group P-1), the STIIs did not differ significantly from those in normal controls. In 37 patients who were unable to walk (Group P-2) and 5 patients who were confined to bed (Group P-3), there was a significant increase (p less than 0.001) in the PEP index (PEPI), a decrease (p less than 0.001) in the LVET index (LVETI), and an increase (p less than 0.001) in the PEP/LVET compared with those in controls. The PEPI (p less than 0.001), LVETI (p less than 0.025), and PEP/LVET (p less than 0.001) in Group P-2 differed significantly from those in Group P-1. In Group P-3, the PEPI (p less than 0.005), LVET (p less than 0.001), and PEP/LVET (p less than 0.001) were significantly changed in comparison with those in Group P-2. Remarkable myocardial histopathologic changes suggestive of dystrophy were revealed in autopsies of 3 patients who had been in the severely disabled stage and had shown impaired STIs. It is suggested that cardiac function in patients with moderate to advanced PMD of the Duchenne type is deteriorated in proportion to the disability stages, i.e. the severity of changes in the skeletal muscles.

Adolescent↗
PubMed1985

Compressed heart and mitral valve prolapse in a case of Duchenne's progressive muscular dystrophy with thorax deformity.

We report on a 15 year old patient with Duchenne's progressive muscular dystrophy who demonstrated a narrowing of the left ventricular inflow and outflow tracts due to compression by a highly deformed thoracic spine. A systolic murmur (4/6) with thrill and a diastolic murmur (2/6) were heard, with these murmurs being louder in the expiratory phase as compared with the inspiratory phase. The second heart sound showed a paradoxical splitting. Echocardiograms revealed a compressed and narrowed left ventricle and a prolapsed mitral valve. The intensities of the heart murmurs changed synchronously with the chamber's narrowing due to respiration. A narrowed left ventricle occurring as a result of the compression by the deformed thoracic spine is thought to be the cause of these cardiac findings.

Adolescent↗
PubMed1977

[Specific and aspecific myocardial changes in progressive muscular dystrophy and their importance in rehabilitative treatment].

Clinical and instrumental parameters studied in over 200 progressive muscular dystrophy patients during a period of about 10 yr revealed four different types of cardiopathy in this disease. The most interesting finding was that 38% of patients with Duchènne's dystrophy presented a clinical and ECG picture reminiscent of that of obstructive hypertrophic cardiomyopathy. Some suggestions are made for the pharmacological and rehabilitative management of subjects with myocardiopathy in the course of myodystrophy.

Adolescent↗
PubMed1983

[Features of insulin tolerance to glucose in primary and secondary forms of progressive muscular dystrophy].

Fifty-eight patients, aged 15 to 45 years, suffering from various forms of progressive muscular dystrophies (PMD) (13 patients with Erb-Roth's PMD, 18 with Landouzy-Dejerine's PMD, 15 with Charcot-Marie's PMD, and 1 with Kugelberg-Welander's PMD) were observed to study the initial concentration of immunoreactive insulin and its secretion over time, following a glucose load. Insulin concentration in the blood plasma was determined with the help of radioimmunoassay kits. Patterns of the insulinemic curves in primary and secondary forms of PMDs were demonstrated to be different. Primary forms of PMDs were associated with two types of reaction, namely, plane and torpid. In cases of denervation amyotrophies the character of the insulinemic curve was similar to that in control. The results of the study point to insulin metabolism disorders related to PMDs.

Adolescent↗
PubMed1980

[Interaction of the genes of progressive muscular dystrophy (PMD) and blood groups (ABO)].

Verification of the hypothesis that the inheritance of the Progressive Muscular Dystrophies (PMD) may involve dispersed genes, throiugh the study of a sample of 105 patients revealed that 82% of the Duchenne type belong to blood group O, while 79% of the limb-girdle type belong to group A. The fact that the Duchenne gene is sex-linked while the ABO locus is on chromosome 9 confirms the "dispersed genes" hypothesis, with important implications for further research, for genetic counselin and possibly for prevention.

ABO Blood-Group System↗
PubMed2000

[MRSA infection control in the wards for progressive muscular dystrophy: the effects of encouraged handwashing].

There are currently 27 national hospitals with the wards for progressive muscular dystrophy (PMD) in Japan. Today, most patients in these wards are severely motor handicapped and complicated with respiratory and/or cardiac failure. Malnutrition, dysphagia, insufficient respiratory tract clearance are common problems that cause fragility to infection. Although progress in the treatment of infection have remarkably prolonged their life-span, frequent use of antibiotics is a major factor for occurrence of drug resistant bacteria. Since we had the first case of methicillin resistant Staphylococcus aureus (MRSA) infection in 1994 in our hospital, the number of MRSA carriers increased year by year. To prevent the spread of MRSA, we revised our infection precaution manual and surveyed its consequence. We encouraged handwashing of staffs, introduced green tea in cleansing genital region and abolished the use of a private room except a patient with severe seborrheic eczema. The revision of the manual resulted in decrease of MRSA carriers. The surveillance revealed that many patients had MRSA in genital regions, although there were no relationships between colonization of MRSA and severlity of disability or complications. It was strongly suggested that toilettary care by the hands of nursing staffs was a major factor of transferring MRSA. Our study revealed that encouragement of handwashing is more powerful in preventing the spreading MRSA, and more favorable for quality of lives of PMD patients than isolating the patients.

Adolescent↗
PubMed1981

[Heart sounds and heart murmurs in progressive muscular dystrophy of Duchenne type (author's transl)].

Phonocardiographic and echocardiographic investigation was performed in patients with progressive muscular dystrophy of Duchenne type (PMD). The clinical materials consisted of 90 patients with PMD (aged 8 to 21 yrs, a mean of 14.5), and 90 normal subjects (aged 6 to 19 yrs, a mean of 11.7). The patients with PMD were classified into 8 stages from the mildest, S(1), to the severest, S(8), according to Swinyard-Deaver' criteria. In the 90 normal subjects the diminished first heart sound was noted in 12 cases (13.3%), presystolic murmurs in 4 cases (4.4%), and diastolic rumbles in 9 cases (10%), whereas, in the patients with PMD the diminished first heart sound was noted in 47 cases (52.2%), presystolic murmurs in 41 cases (45.6%), and diastolic rumbles in 44 cases (48.9%). There was a significant difference in the incidence of the above-mentioned three phonocardiographic findings between the PMD patients and the normal subjects. But there was no significant difference in the incidence of a systolic click between these two groups. The incidence of the diminished first heart sound increased with the progress of Swinyard-Deaver' classification. A presystolic murmur was observed with the highest incidence in the stage of S(8). The incidence of a rumble was also augmented with increasing severity of the disorder from the stages of S(1) to S(7), but decreased in S(8). Another attenpt was made to relate the phonocardiographic findings to those of the echocardiogram. In the cases with anterior mitral leaflet fluttering, there were diastolic rumble in 69% whereas 16.7% of the patients without anterior mitral leaflet fluttering had diastolic rumbles. In two-dimensional echocardiography, the anterior and posterior mitral leaflets looked like pennants fluttering in the wind. All these observations positively indicate that anterior mitral leaflet fluttering was closely associated with the genesis of rumbles. Consequently, it can be concluded that the diminished first heart sound, presystolic murmurs and diastolic rumbles might be useful clinical signs in the assessment of the myocardial involvement in PMD.

Adolescent↗
PubMed1983

Prospective study of X-linked progressive muscular dystrophy in Campania.

Within the Campania region of southern Italy a prospective study on X-linked progressive muscular dystrophy was conducted over a period of 12 years from 1969 to 1980, inclusive. The mean incidence rate was 21.7 per 100,000 male livebirths for Duchenne muscular dystrophy (DMD) cases and 3.2 per 100,000 male livebirths for Becker muscular dystrophy (BMD) cases. The familial cases were 38.5% among the DMD patients and 50% among the BMD patients. Myocardial involvement appeared in DMD patients at about 6 years of age in a high percentage of cases and increased progressively until the last years of life, when cardiac damage occurred in 95% of cases. The percentage of myocardial involvement in BMD patients was very low before 13 years of age, but increased progressively until 20 years, when cardiac damage occurred in 80% of cases studied; severe cardiomyopathy did not occur before the age of 21. The data reported also include the effects of age on physical performance, serum creatine kinase activity and serum myoglobin levels, the types of cardiac damage, and the causes of death.

Adolescent↗
PubMed1978

Serum creatine kinase isoenzymes in progressive muscular dystrophy.

Creatine kinase isoenzymes in sera and muscle biopsies obtained from 50 controls, 72 patients with progressive muscular dystrophy (PMD), 68 patients with other neuromuscular disorders, 17 carriers of Duchenne-type PMD and 15 patients with myocardial infarction were studied. MB isoenzyme was detected in the sera of 58 patients with PMD and 56 out of 61 muscle biopsies. The MB activity varied between 4 and 400 IU/1 or 3.4--22% of total activity. The MB activity was demonstrated in a considerably smaller number of cases with polymyositis, dystrophic myotonia and Kugelberg-Welander disease. The MB isoenzyme in sera of PMD persisted for many years. It is admitted that the MB isoenzyme in the serum of patients with PMD originates chiefly from skeletal muscle.

Child↗
PubMed1987

[Congenital progressive muscular dystrophy of Fukuyama type: report of a case].

The authors report the first Fukuyama type congenital progressive muscular dystrophy case described in Brazil, and confirmed through clinical findings and complementary tests. Emphasis is given to the presence of early fibrotendinous retractions and impairment of the central nervous system, which constitute the fundamental characteristics of this affection. This disease is very common in Japan but very seldom described in other countries. Its etiopathogeny has not yet been defined.

Child, Preschool↗
PubMed1992

[The two brothers' case of dilated cardiomyopathy with benign Duchenne type of progressive muscular dystrophy (Becker's type)].

The presence of myocardial involvement is rare in benign Duchenne type of progressive muscular dystrophy (Becker's muscular dystrophy). We describe two brothers suffering from Becker's muscular dystrophy, both of whom presented with dilated cardiomyopathy. The first case is a 39-year-old male who had suffered from gait disturbance from the age of 17. When 37 years old, he was found to have heart disease. When he first came to our hospital, pseudohypertrophy of the calves was present. Chest radiography, electrocardiogram, ultrasonocardiography and clinical feature indicated Becker's muscular dystrophy with dilated cardiomyopathy. The second case is the younger brother of the 37-year old male. He suffered from leg weakness. He came to our hospital with the chief complaint of discomfort of the anterior chest. Pseudohypertrophy of the calves was present. Chest radiography, electrocardiogram, ultrasonogram indicated dilated cardiomyopathy.

Adult↗
PubMed1984

[Secondary changes in the hip joints of children with progressive muscular dystrophy].

The authors state that a marked hypotonia of the lower extremity muscles in children with progressive muscular dystrophy may lead to the development of secondary dislocations and semi-dislocations of the femur, which were detected in 18 of the 32 patients examined. Most of the patients had Werdnig-Hoffmann's spinal amyotrophy which was related to an especially gross muscular hypotonia.

Child↗
PubMed1977

[Particular aspects of cardiomyopathy in Landouzy-Déjérine's progressive muscular dystrophy. Study of a family (author's transl)].

In the study of a family affected by progressive muscular dystrophy (DMP) of facio-scapulo-humeral type (f.s.o.) the myocardial involvement has been found to be constant and of moderate degree. The myocardial component has been found to appear in the adult age with initial and prevalent atrial involvement. In one case the myocardiopathy was not associated with appreciable clinical signs of skeletric myopathy. Possible causes of the pecuationship between the DPM type f.s.o. associated myocardiopathies and familial idiopathic cardiomyopathies are outlined.

Adult↗
PubMed1995

Notched T wave as evidence of autonomic nervous lability in Duchenne progressive muscular dystrophy.

We investigated the significance of notched T waves on the ECG in 30 patients with Duchenne progressive muscular dystrophy (DMD) and 50 age-matched controls using noninvasive cardiovascular examinations and measurement of urinary catecholamines. Notched T waves were more frequently observed in patients with DMD than in control subjects (46.7% vs. 20.0%, p < 0.05). Moreover, their frequency was age-independent in DMD, whereas they decreased with age in controls. Patients with notched T waves showed significantly increased heart rate, prolonged QTc and augmented excretion of urinary adrenaline compared with patients without them. There were no significant differences in casual BP or incidences of characteristic UCG abnormalities, such as mitral valve prolapse, and ECG abnormalities, such as tall R waves in the right precordial leads, between DMD patients with and without notched T waves. These findings suggest that notched T waves are associated with accelerated sympathetic nervous activity rather than progressive cardiac involvement in DMD.

Adolescent↗