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PubMed1976

[Influence of ouabain on the electrophoretic mobility of erythrocytes in 7 patients with Duchenne's muscular dystrophy].

Measurements of electrophoretic mobility of erythrocytes under the influence of ouabain were carried out on seven patients suffering from progressive Duchenne's muscular dystrophy. While ouabain-treated erythrocytes of healthy controls showed a significant decrease (alpha = 0.001) in electrophoretic mobility, an increase was found in five of the patients, and two patients revealed a slight drop in electrophoretic mobility. A significant difference (alpha = 0.001) was obtained in comparing the control group of patients. Initial trial measurements suggested that such a difference is also true for some other forms of muscular dystrophy and Werdnig-Hoffmann's spinal muscular atrophy.

Child↗
PubMed2006

Estrogen induced functional hypertrophy and increased force generation of the female rabbit bladder.

AIMS: Estrogen is essential for physiological maintenance of the female urogenital tract. It is believed that alterations in female sex hormones play a major role in the etiology and response to urinary tract dysfunctions. In animal studies, ovariectomy (Ovx) results in smooth muscle (SM) weakness and atrophy whereas estrogen supplementation reverses these effects. Our study seeks to establish the mechanisms by which estrogen augmentation results in increased contractility. METHODS: Twenty New Zealand White female rabbits were separated into five groups of four each. Group 1 served as control, rabbits of groups 2-5 were ovariectomized, group 2 ovariectomized received no estradiol, groups 3-5 were given 17-beta estradiol (1 mg/kg/day) by subcutaneous slow release tablet implant for 1, 3, and 7 days, respectively, beginning 2 weeks after Ovx. At the end of the experimental period, each rabbit was anesthetized and the urinary bladder was removed for contractile, histological, and biochemical studies. RESULTS: Ovx resulted in significantly decreased bladder contractile function, whereas bladders tested after estradiol administration showed increased contractility. Ovx resulted in a decrease in SM/collagen ratio, whereas estrogen resulted in an increase. The estrogen receptor (ER) density significantly increased following Ovx. After 1 day of estrogen treatment, the ER density decreased significantly below control levels, but rose progressively during the estrogen treatment. CONCLUSION: The present study demonstrates that estrogen supplementation mediates a "functional hypertrophy," that is a hypertrophy characterized by increased contractile responses to all forms of stimulation, and an increased ratio of SM/collagen.

Animals↗
PubMed1981

Muscle adenylate deaminase deficiency. Report of six new cases.

We describe six adult patients (five men and one woman) out of 364 whose muscle biopsy specimens disclosed muscle adenylate deaminase deficiency. Two men had an associated dermatomyositis and another man had an associated progressive systemic sclerosis. Although the patients were different clinically, all complained of muscular weakness or poor exercise tolerance. The occurrence of muscle adenylate deaminase deficiency in both sexes suggests a possible autosomal mode of inheritance.

AMP Deaminase↗
PubMed2001

Epidemiological survey of X-linked bulbar and spinal muscular atrophy, or Kennedy disease, in the province of Reggio Emilia, Italy.

Commencing with the work carried out during the epidemiological survey of amyotrophic lateral sclerosis in the period 1980-1992 and the pathology follow-up, we carried out a perspective incidence, prevalence and mortality survey of X-linked bulbar and spinal muscular atrophy (X-BSMA) in the province of Reggio Emilia in Northern Italy. Based on 11 patients (eight familial and three sporadic cases), the mean incidence per year for the period 1980 through 1997, as evaluated at the onset of symptoms, was 0.09 cases/100,000 for the total population and 0.19 cases/100,000 for the male population. On December 31, 1997, the prevalence rate was 1.6/100,000 for the total population and 3.3/100,000 for the male population. In the 18-year period of 1980-1997, the average yearly mortality rate was: 0.03 cases/100,000 per year for the total population and 0.06 cases/ 100,000 for the male population. The average age at onset was 44.8 +/- 10.1, and the average survival period was 27.3 +/- 2.3 years. The average age of the prevalence day was 58.9 +/- 14.9, and the average age at death was 71.3 +/- 4.7 years. Whereas the incidence rate of X-BSMA in the province of Reggio Emilia is 16 times lower that of amyotrophic lateral sclerosis (ALS), the incidence rate of progressive bulbar palsy in the male population is only slightly higher than X-BSMA; and the prevalence rate of ALS for males is two times the prevalence rate for X-BSMA, with overlapping of confidence intervals. X-BSMA is a rare disease, which is probably under-diagnosed, but due to the long survival period of this disease its frequency is not negligible. Because of the presence of sporadic cases or non-evident familial cases, it is appropriate to consider this diagnostic possibility in making a diagnosis of ALS in patients in whom lower motor neuron dysfunction or bulbar onset predominates.

Adult↗
PubMed1990

Dysarthria of motor neuron disease: clinician judgments of severity.

Motor Neuron Disease (MND) is a terminal, demyelinating disease affecting upper- and lower-motor neurons and producing muscular weakness resulting in a characteristic spastic-flaccid dysarthria of speech. The present study investigates the relationship between the temporal-acoustic parameters of the speech of 15 individuals with MND as they relate to the progression of the disease and clinicians' judgments of dysarthria severity. When temporal-acoustic parameters are used to predict the progression of MND, it becomes apparent that victims provide compensatory gestures to mark voicing distinctions. When the same acoustic parameters are used to predict clinician judgments of severity, it is found that clinicians tend to use the same temporal cues that mark actual disease progression. Differences between the two sets of predictions relate to the linguistic systems of both speaker and judge, and the implications of this are discussed.

Adult↗
PubMed1991

[Clinico-electrophysiological changes in the neuromuscular system of patients with vibration disease].

Neuromuscular alterations associated with vibratory disease related to the pathology of spinal motoneurons, axon (degeneration, demyelinization), synapse, muscle. The site, nature and the intensity of the process depended on the duration of work with vibroinstruments, industrial characteristics. During specialized employment, the patients manifested aggravation of the clinical and neurophysiological parameters of the denervation process. Compensatory innervation of the muscles (branching of axon terminals, establishment of new neuromuscular contacts, ets.) was most effective in polishers and riveters. Foundry cleaners demonstrated a tendency toward progress of neuromuscular pathology. To explain motor disorders in patients with vibratory disease, the mechanisms of microtraumatization of nerve endings followed by involvement of the neurotrophic and neuroreflectory systems are suggested.

Action Potentials↗
PubMed1985

The significance of x-ray examinations of limb musculature in neuromuscular diseases with special regard to childhood diseases.

On the basis of examinations performed on 29 children suffering from various muscular diseases, the authors give a detailed discussion of the application, indication and advantages of muscles x-rays in diagnosing neuromuscular diseases. With the help of x-rays of the musculature an inside view can be obtained in vivo, in a non-invasive manner, of structural changes in the muscles, and the progress of the disease can be followed up objectively. The method opens up new possibilities of diagnosis and promotes a more thorough knowledge of the pathological processes.

Adolescent↗
PubMed1971

Hereditary ptosis.

Weakness of the external eye muscles was believed to be of genetic origin in 94 of 97 patients studied and was familial in 73 patients. Thirty patients had congenital ptosis alone or with weakness of other ocular muscles. The lack of progression and high incidence of other congenital anomalies suggested that congenital ptosis had in most instances a development etiology. Neurogenic congenital ptosis was documented in one instance and was suspected in another patient. The most common causes of progressive ophthalmaplegia with onset after birth was myotonic dystrophy and ocular muscle dystrophy. On clinical and genetic grounds, ptosis associated with retinitis pigmentosa was considered as a distinct disease and there was no conclusive evidence that ocular muscle dystrophy and oculopharyneal dystrophy were separate diseases. The relatively high incidence of familial myasthenia gravis was probably due to the selection of the patients. Two pairs of sibs with "chondrodystrophic myotonia" were included in the study. Progressive external ophthalmoplegia with onset after birth was neurogenic in three instanced. It accompanied motor neuron disease, Kugelberg-Welander disease and an undertermined disease affecting the central and peripheral nervous system, each in one patient.

Blepharoptosis↗
PubMed1990

[Maxillofacial manifestations of systemic scleroderma. Apropos of a case].

The authors relate the case of one patient 54 years old having a progressive systemic sclerosis. It is a rare progressive disease of unknown etiology. The patient was first seen in 1983, with lesions on the face. The following is the report of this patient who demonstrated resorption of the angle of the mandible, atrophy of the zygomatic arches, characteristics facial muscles and cutaneous lesions.

Facial Muscles↗
PubMed1984

Systemic amyloid myopathy--light-microscopic and fine-structural study of the skeletal muscles with histochemical and immunohistochemical study of amyloid.

Studies were made on skeletal muscles obtained by biopsy and autopsy from a 59-year-old man with immunocyte dyscrasia and amyloidosis; he had progressive muscular stiffness with palpable tumors and chi-type Bence-Jones protein, but no overt myeloma. Histologically, much amyloid was seen deposited preferentially in the skeletal muscles, especially in the endo- and perimysia, forming tumors of various sizes and occasionally associating influx of amyloid into some muscle fibers, and in the smaller-sized blood vessel walls, frequently associated with extreme narrowing or occlusion of their lumina. Most muscle fibers were replaced by much amyloid or showed extensive atrophy, having a characteristic circular space under the endomysium, which was concluded to be not due to compression atrophy but to ischemic atrophy. Ischemic neuropathy was observed and seemed to be responsible for the diffuse atrophy without spaces seen exceptionally in some parts. Amyloid was shown histochemically to contain AL as the major protein component, immunohistochemically to have a close relation with chi-type light chain, and fine-structurally to be formed just outside the basement membrane of the blood vessels and deposited in the endo- and perimysia through perivascular infiltration. The clinicopathologic features of nine similar autopsy cases in the literature were compared with those in the present case. The causes of damage in muscle fibers and of preferential deposition of amyloid in the skeletal muscles were discussed.

Amyloid↗
PubMed2001

[Value of training-induced effects on arterial vascular system and skeletal muscles in therapy of NYHA II/III heart failure].

Dynamic muscular exercise performed by healthy subjects leads to a rise in the left ventricular blood ejection with an acute increase in the local wall shear stress on the endothelium of the arterial vessels. These hemodynamic changes results in a release of endothelium-dependent relaxing factors, one of them concerns nitric oxide (NO). Therefore an arterial vasodilatation with an acute increase in the blood flow volume to the exercising muscle groups occurs. If more than 1/6 of the skeletal musculature is involved in exercise and if training duration exceeds 3-5 hours a week the chronically increased blood flow volume in the cardiovascular system triggers structural and functional changes of the heart and the arterial vessels. It develops a functional intact excentric hypertrophy of the myocardium; within the arterial vessels an increase in the diameter of the muscular arteries supplying the trained muscle groups occurs. These training-induced adaptations of the cardiovascular system are adjusted to improve the aerobic skeletal muscle metabolism. In congestive heart failure a pathological excentric myocardial hypertrophy is found. In this case the systolic myocardial function is impaired and the left ventricular ejection fraction is reduced already in early stages, so that the cardiac output can not be sufficiently increased during exercise. In addition a dysfunction of the endothelium of the arterial vessels occurs. As a consequence the endothelium-dependent arterial vasodilation is reduced, so that the peripheral arteries could not supply the muscle groups involved in exercise with enough blood flow volume. Therefore, the acute delivery of the working musculature with oxygen and energy substrates is insufficient, so that premature muscular fatigue occurs. The reduced exercise resistance of the patients leads chronically to a generalized skeletal muscle atrophy. Ultrastructural analysis revealed a decrease of oxidative type 1 muscle fibers with a relative increase of more glycolytic type 2 fibers. In addition, the volume density and the surface area of the cristae of mitochondria are reduced. All these changes results in a decrease of aerobic skeletal muscle metabolism independent of the blood flow volume, so that the physical fitness of the patients progressively decline. On the basis of the training-induced physiological adaptations of the cardiovascular system, a special exercise therapy supervised by a physician was developed for patients with congestive heart failure NYHA II/III. It have been shown that various exercise programs, which are adjusted to the degree of cardiac function impairment are suitable to restore the endothelial dysfunction of the arterial vessels as well as to cure the disturbed skeletal muscle metabolism in these patients independent of an improvement of cardiac function. Therefore in patients with congestive heart failure NYHA II/III who underwent regularly such an exercise therapy, the secondary impaired physical fitness could be rebuild without an excessive risk for an acute exercise-induced cardiovascular emergency.

Cardiac Output↗
PubMed2001

Primary lateral sclerosis: further clarification.

Primary lateral sclerosis (PLS) has been defined as a rare. Non-hereditary disease characterized by progressive spinobulbar spasticity, related to the exclusive involvement of precentral pyramidal neurons, with secondary pyramidal tract degeneration and a preservation of anterior horn motor neurons, the latter allowing PLS to be distinguish from amyotrophic lateral sclerosis (ALS). However, a clear distinction between the two diseases remains a subject of debate. With this in mind, we assessed patients with meeting the previously published criteria for PLS in a prospective, longitudinal study. At regular intervals, we analyzed various clinical and electrophysiological parameters in nine patients with a diagnosis of PLS. We made a deltoid muscle biopsy and PET study.Our results provide evidence that degeneration in PLS is not restricted to the upper motor neurons but also affects the lower motor neurons. The distinction between ALS and PLS is related to the degree and stability of lower motor neuron involvement. In view of the similarities with ALS, we consider that PLS may represent a slowly progressive syndrome closely related to this disease.

Adult↗
PubMed1992

Histochemical and morphometric studies of peripheral muscle in bovine progressive degenerative myeloencephalopathy of brown Swiss cattle.

Histochemical and morphometric analysis of selected skeletal muscles was performed on 14 pure bred, Brown Swiss cattle. Nine cattle were clinically affected with bovine progressive degenerative myeloencephalopathy (BPDME) while five served as controls. Statistically significant trend differences were not observed for the parameters of mean cross sectional area, and mean fiber type percentages for types, I, IIA, and IIB fibers between affected and control test groups. In general, patterns of hypertrophy or atrophy, fiber type grouping, fiber type predominance, or fiber cross sectional profile alteration were not observed in the muscles examined from affected cattle. The findings suggest that BPDME, or weaver syndrome, is not a muscular dystrophy and that muscle pathology is not a primary part of the syndrome nor would muscle pathology be expected to contribute significantly to the clinical signs of the disease.

Animals↗
PubMed1992

Benign monomelic amyotrophy of lower limb: report of three cases.

Three patients with wasting confined to a single lower limb are reported. The characteristic features were: sporadic occurrence, insidious onset with slow progression and in 2 cases arrested course for at least 4 years, wasting out of proportion with disability, absence of sensory, pyramidal tract or bulbar signs. CK, motor and sensory conductions, and lumbar MRI were normal. Muscle CT showed selective loss of muscle tissue and fat replacement in posterior leg muscles. Quantitative electromyography and histologic findings revealed neurogenic features not only in the affected legs, but also in clinically uninvolved limbs. Monomelic amyotrophy of lower limb is a variant of spinal muscular atrophy with a benign course. However, as in the early stages of the disease there are no distinctive clinical or laboratory findings with other motor neuron diseases, the diagnosis of monomelic amyotrophy may be made only retrospectively after a prolonged observation.

Adult↗
PubMed2006

Insights into muscle atrophy and recovery pathway based on genetic models.

PURPOSE OF REVIEW: Muscle atrophy is a pervasive problem that occurs with disuse, aging, and a myriad of disease conditions. The purposes of this review are to describe recent advances in studying muscle atrophy that have elucidated pathways involved at the molecular level; to compare different types of atrophy--primary (e.g. bed rest, immobilization) and secondary (when the atrophy is related to pathology as well as disuse, e.g. injury, sepsis etc.) and their multiple common features; to review progress in studying the recovery process and clinical status. RECENT FINDINGS: Major advances have been made at the molecular level. There are two phenotypes for muscle atrophy--primary, which is mainly related to disuse (e.g. bed), and secondary, when the atrophy is related to pathology as well as disuse. It appears that the two forms have multiple elements in common. Studies on the recovery process reveal a very complex sequence of events that are not the simple reverse of the muscle loss process. In contrast to the progress at the molecular level, progress in treating muscle atrophy or accelerating recovery has been disappointing. SUMMARY: Although nutritional supplementation and pharmacological agents continue to have the potential to minimize muscle atrophy, given its minimal risks, exercise sets a very high standard for treatment options when medically appropriate. Identification of pathways and control points offers the potential for new approaches.

Bed Rest↗
PubMed1981

Rapid atrophy of mouse soleus muscles after tenotomy depends on an intact innervation.

Changes in length and mass of mouse soleus muscles have been determined during the first 14 days after division of the Achilles tendon and/or the tibial nerve. Muscle atrophy and associated histological changes were detectable 24 h after tenotomy, and increased progressively over the first week. These changes were less marked in muscles which had also been denervated, and were rapidly reversed if the tendon became reattached. An attempt is made to distinguish the role of the nerve supply from the effects of reduced longitudinal tension in the production of atrophy after tenotomy.

Achilles Tendon↗
PubMedDate not supplied

Early knee motion after open and arthroscopic anterior cruciate ligament reconstruction.

The hypothesis proposed in this study was that the initiation of active and passive knee motion within 48 hours of major intraarticular knee ligament surgery would not have the deleterious effects of increasing knee effusion, hemarthrosis, periarticular soft tissue edema, and swelling. We conducted a prospective study with randomized assignment of 18 patients into two groups: 9 patients in the "motion" group began 10 hours of daily continuous passive motion (CPM) on the 2nd postoperative day, while the remaining 9 in the "delayed motion" group used a soft hinged knee brace with knee hinges locked at 10 degrees of flexion and entered into the motion program on the 7th postoperative day. All knees were allowed full 0 degrees to 90 degrees of motion except for a total of seven knees with concomitant mensicus repairs and extraarticular reconstructions where 20 degrees to 90 degrees of motion was allowed, limiting the last 20 degrees of knee extension for the first 4 postoperative weeks to protect the repair. In all other respects, the rehabilitation program after surgery was the same for the two groups, including postoperative compression dressings, exercises, and weight-bearing status. Ten of the eighteen patients had acute ACL disruptions and 8 had chronic ACL insufficiencies. There was an even distribution of acute and chronic knee cases and of open and arthroscopic ligament procedures in the early and delayed motion groups. Associated surgery included four meniscus repairs, three medial collateral ligament repairs, and one lateral collateral ligament repair. Special suturing and fixation techniques were used at surgery to maintain the integrity of ligament and meniscus structures, allowing the surgeon to feel safe in subjecting the joint to early postoperative motion. The objective parameters measured were KT-1000 arthrometer measurements, Cybex isokinetic testing, girth measurements at four lower limb locations, range of motion goniometer measurements, postoperative pain medications, and days of hospitalization. Starting intermittent passive motion on the 2nd postoperative day did not increase joint effusion, hemarthrosis, or soft tissue swelling. In both motion groups, postoperative joint effusions were absent after the 14th postoperative day. There was no statistically significant difference in knee extension or flexion limits, pain medication used, or hospital stay in comparing the two knee motion programs. An important finding of this study was the significant decreases in thigh circumference that occurred within the first few weeks of surgery, which progressed despite a closely supervised inpatient and outpatient rehabilitation program.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗