Marked Muscle Atrophy Research Articles

A Case Report of Osteoid Osteoma of the Patella with Marked Muscle Atrophy of the Quadriceps

A Case Report of Osteoid Osteoma of the Patella with Marked Muscle Atrophy of the Quadriceps

Canine X-linked muscular dystrophy: An animal model of Duchenne muscular dystrophy: Clinical studies

The progression of clinical disease and serum creatine kinase (CK) levels in canine X-linked muscular dystrophy (CXMD) was studied in 7 dogs from birth to 12–14 months and in 18 dogs at varying intervals from birth to 8 weeks. One affected male was studied from age 3.5 to 6 years, and all pups were descendants of this dog. A lethal neonatal form was recognized in some pups. In the more typical form, clinical signs of stunting, weakness and gait abnormalities were evident by 6–9 weeks and were progressive, leading to marked muscle atrophy, fibrosis and contractures by 6 months. Serum CK levels were markedly elevated, such that affected pups could be identified by 1 week. CK values increased until 6–8 weeks, then plateaued at approx. 100 times normal. Affected females and beagle-cross dogs were less severely affected than large breed-cross dogs. In the 2 adult dogs with cardiac insufficiency CK levels had decreased to 5–15 times normal. These studies show that CXMD and Duchenne muscular dystrophy have striking phenotypic as well as genotypic similarities. In addition, these studies of CXMD suggest that in females and in smaller dogs the same genetic defect results in a less severe clinical disease.

当院における頚椎症性筋萎縮症例の臨床的検討

Among 124 patients with cervical spondylosis who were operated on during the past 6 years at our clinic, cervical spondylotic amyotrophy was noted in 5 patients (4.0%). Severe weakness with marked muscle atrophy in the segmental distribution was a cardinal sign of neurological findings in all cases.But two cases out of five had long tract sign and four cases had minimal sensory disorder. Myelography and selective angiography were performed on all cases.The level of muscle atrophy corresponded well with that of block found in the cervical myelogram. The gap of levels between muscle atrophy and myelogram, however, was observed in one case. The gap of those levels and the findings of angiogram suggested that the circulatory failure of spinal cord, not only compression, perticipates in the mechanism of CSAM.In our opinion, putting the operative findings together, there are 3 types of lesions impaired in CSAM: ventral horn cell, anterior root, and both ventral horn cell and anterior root.

The effect of long-term immobilization on the motor unit population of the cat medial gastrocnemius muscle

The properties of medial gastrocnemius and soleus muscles and of individual medial gastrocnemius motor units were evaluated after chronic (3–29 weeks) unilateral immobilization of ankle and knee joints in 10 adult female cats. Joint fixation markedly impaired the mobility of the pinned limb and, when prolonged (> 17 weeks; 8 animals), produced marked muscle atrophy and some shortening of whole muscle isometric twitch contraction times (mean contraction time of pinned medial gastrocnemius was 83.3 percent of the contralateral muscle). However, the immobilized muscles were not truly disused, since the patterns of medial gastrocnemius and soleus electromyographic activity during posture and locomotion were qualitatively normal in the 4 animals examined. Despite severe whole muscle atrophy, all but 3 of the 239 individual medial gastrocnemius motor units studied were readily classified into one of the 4 motor unit types (types FF, F(int), FR and S) found in the normal muscle. The histochemical mosaic of immobilized muscles was also the same as in contralateral unoperated muscles. When compared to data from normal cats, there was a highly significant loss of maximum twitch and tetanic force output in all unit types after long-term immobilization (17–29 weeks; N = 187), most marked among the fatigue-resistant unit groups (types FR and S), and mean isometric twitch contraction times were shorter than expected in all unit type groups. The most surprising finding in the present work was that the mean homonymous (medial gastrocnemius) and heteronymous (lateral gastrocnemius-soleus) group Ia excitatory postsynaptic potentials were significantly smaller than expected in all motor unit groups in both short-term and long-term immobilization. The mean axonal conduction velocity of medial gastrocnemius motor axons was unchanged from the normal sample. These results indicate that the essential features that define the motor unit types in normal medial gastrocnemius muscles are resistant to change despite long-term immobilization. We conclude that the motor units types found in normal muscles are robust and essentially unaltered despite severe muscle atrophy. The effects on group Ia excitatory postsynaptic potentials suggest that limb immobilization produces altered patterns of impulse traffic in group Ia afferents that induce significant decreases in synaptic efficacy, although it is unclear whether or not Ia traffic is increased or decreased by immobilization.

Muscle Atrophy in a Case of Acute Disseminated Encephalomyelitis

A case of acute disseminated encephalomyelitis (ADEM) accompanied by muscle atrophy is reported. A man of 30 was admitted with acute onset of clouded consciousness, fever, paraplegia and sensory loss, following an upper respiratory tract infection. A week later, marked muscle atrophy was noticed in both legs. A biopsy of the quadriceps femoris muscle showed groups of small angular fibres indicating neuropathic change, and also fibres with round contour and varying diameter suggestive of myopathic change. Some fibres contained vacuoles, basophilic material and or eosinophilic mass. Histochemical examinations suggested that the basophilic material contained mitochondria, lipid and glycogen, while the eosinophilic mass was regarded as hyaline degeneration. Cellular infitration was not observed in the interstitium. Electron micrographs showed disarray and loss of myofibrils, abundant mitochondria and glycogen granules, myelin figures and honeycomb structures. The cause of the muscle changes is uncertain. However, as conceivable causal mechanisms, the possibility of polymyositis is discussed as well as the effect of immobilization and probable involvement of the nerve roots and peripheral nerves.

A Patient with Cronkhite‐Canada Syndrome, Myxedema and Muscle Atrophy

A case of Cronkhite-Canada syndrome is presented. The patient had alopecia, onychodystrophy and gastrointestinal polyposis, mainly in the stomach and duodenum, with transient diarrhea and hypoproteinemia. Marked atrophy and weakness of the shoulder girdle muscles due to myopathy were also present. In addition she had primary hypothyroidism. The outcome of the disease is usually fatal within months, but so far our patient is alive four years after the onset of symptoms. The pathological changes, pathophysiology, symptoms, course and treatment of this rare disorder of unknown etiology are discussed.

神経損傷後の腓腹筋およびヒラメ筋におけるCholinesterase活性の変化と，その変化に及ぼすビタミンB群の影響

In an analysis of the gastrocnemius muscle, the microsomal fraction showed the highest cholinesterase (ChE) activity. The ChE activity of all fractions decreased to a greater extent after strong nerve crushing than after weak crushing. This change in the activity in the microsomal fraction was the most marked change observed. Although in the analysis of the soleus muscle the ChE activity was measured only in the homogenate and in the microsomal fraction, the results were the same as those obtained with the gastrocnemius. A preparation of vitamins B1, B2, and B12 (B complex) had little effect on the ChE activity in the gastrocnemius muscle. In the soleus muscle on the lesion side, the B complex increased the ChE activity to some extent after nerve crushing, but such was not significant. However, the B complex signifiicantly increased this activity in the soleus on the intact side. In the soleus muscle, strong nerve crushing induced more marked muscle atrophy than weak crushing. On the other hand, no significant difference was found in the gastrocnemius. Effects of the B complex on muscle atrophy were found in the soleus, but not in the gastrocnemius. These results suggest that the ChE activity in the microsomal fraction containing sarcoplasmic reticulum reflects the nervous disorders clearly, and that the B complex increases the ChE activity and muscle weight in the soleus, but not in the gastrocnemius muscle.

Electrophysiological and contractile properties of the levator ani muscle after castration and testosterone administration.

Electrical and contractile properties of the levator ani muscle were studied in normal rats, in castrated rats and in castrated rats treated with testosterone. 2. No significant changes in the frequency of miniature end-plate potentials were found 6 months after castration. The frequency increased already 6 h after testosterone treatment; an increase of about 100% was observed after 7 days of testosterone treatment. 3. Castration led to a 2-fold increase of the input resistance of the muscle fibres. After 7 days of testosterone treatment the input resistance was only slightly higher than normal. 4. The weight of the muscle was decreased to 18% of the control value after 6 months castration. It increased to 46% after 7 days of testosterone treatment. 5. The muscles of castrated animals revealed a prolongation of contraction time and marked changes in maximal rate of tension development and half relaxation time. Partial recovery of these parameters was found after 7 days of testosterone treatment. 6. Long-term castration did not induce any denervation-like changes of action potential parameters, and no tetrodotoxin resistance was found in spite of marked muscle atrophy.

Preliminary neurological evaluation of generalized weakness in zinc pyrithione-treated rats

Male rats were fed ad lib. a diet containing 250 ppm zinc pyrithione for 10 days and were then given the basic, untreated, diet for the rest of the study. Initial clinical signs of locomotor abnormalities were observed after the rats had been on the treated diet for 8–10 days. Progressive hind-limb weakness with accompanying muscle atrophy was a constant finding. Muscles of the forelegs and the chest appeared normal and showed almost normal function. The back muscles seemed involved, since kyphosis developed without skeletal abnormality. Weight loss was significantly greater and recovery significantly slower in treated rats than in matched pair-fed controls. Neurophysiological studies of sciatic-nerve conduction velocities failed to show differences between treated and control animals. A significant difference in contraction was noted between normal and affected muscles in that treated animals with marked muscle atrophy had a weaker contractile response to a greater electrical sciaticnerve stimulus than had the controls. This study suggests that the lesion is at the subcellular level and the muscle atrophy may be caused by a basic abnormality in neural conduction, by dysfunction at the myoneural junction or in the muscle-fibre membrane or by a change at the molecular level in the muscle fibres.

Dystrophia myotonica: Peripheral nerve involvement and pathogenetic implications

A comparative electrophysiological study of patients with dystrophia myotonica and control subjects is presented. The study includes estimation of the number of motor axons innervating the extensor digitorum brevis muscle and measurements of the conduction of M- and F-waves along the deep peroneal nerve. There is unequivocal electrophysiological evidence of nerve involvement in the disease. This was indicated by (a) prolongation of the terminal latencies and slowing of the motor conduction of the deep peroneal nerve ( P < 0.001), (b) delayed conduction of the F-wave along the proximal segments of the nerves ( P < 0.02-0.01), (c) reduced number of motor axons innervating the extensor digitorum brevis muscle ( P < 0.001), (d) high amplitude motor unit potentials and discrete EMG activity on the maximal volitional contraction of minimally affected muscles in one patient. However, if the muscle changes were secondary to the nerve involvement occurring in the disease, one would expect that the electrophysiological findings of neuropathy would become more prominent in patients with severe muscle wasting and weakness. Our results showed that in some patients with marked muscle atrophy and weakness there was no evidence of nerve involvement while in other patients with slight to moderate degree of muscle weakness the electrophysiological studies indicated peripheral neuropathy. Therefore, it appears that both the nerves and the muscles are independently affected by the pleiotropic action of the responsible gene and the evidence does not suggest that the muscle atrophy of patients with dystrophia myotonica is entirely neural.

Osteoid Osteoma with Marked Muscle Atrophy: A case report

Osteoid Osteoma with Marked Muscle Atrophy: A case report

Effects of the type A toxin from Clostridium botulinum on the development of skeletal muscles and of their innervation in chick embryo

Chronic injections of high doses of the type A toxin from Clostridium botulinum in the yolk sac of chick embryos cause a marked atrophy of skeletal muscles. However, in contrast with what was previously observed after injection of Naja nigricollis α-toxin, the motor innervation shows little regression. Endplates and motor nerve fibers are numerous and the total content of the muscle in choline acetyltransferase does not change. Ultrastructural studies show that in the muscles of embryos treated by botulinum toxin many endplates are established between motor nerve fibers and highly immature contractile elements such as myoblasts and myotubes.

Axonal filamentous spheroids associated with cardiomyopathy with "targetoid fibers". I. Clinical, histologic, and electron microscopic studies.

A 13-year-old boy had cardiomyopathy, marked peripheral muscle atrophy and weakness, mild peripheral neuropathy, and mild cerebellar signs. At autopsy, axonal spheroids made up of aggregates of neurofilaments were found in the dorsal gray horns and dorsal columns of the spinal cord, in the tegmentum of the medulla, and in the dorsal spinal roots. A cardiomyopathy was present with targetlike (“targetoid”) fibers consisting ultrastructurally of electrondense material intermingled with fine fibrillary elements. Similar but less extensive changes were seen in the skeletal muscles. This case may represent a new disease entity resulting from an unknown metabolic defect that affects both the neural axons and the muscular tissue at a common basic level and produces abnormalities in the structure and physiology of the contractile elements of the affected tissue.

Pneumatosis and pseudo-obstruction in scleroderma.

Two cases of pneumatosis cystoides intestinalis in scleroderma have been recently published (1, 2). Pseudo-obstruction in this disease is a better known phenomenon (9–12). The simultaneous occurrence of these findings has not yet been reported. During the years 1964 to 1967, three unusual cases of generalized scleroderma with both pneumatosis and pseudo-obstruction were seen at the University of Colorado Medical Center. The classical gastrointestinal changes in scleroderma (3–9) consist of a dilated, atonic esophagus with decreased peristalsis in the lower two-thirds, delayed gastric emptying, decreased motility of the gut with malabsorption patterns and findings of intestinal obstruction, and colonic atony with sacculation or pseudodiverticulum formation. The roentgenologic findings of pneumatosis cystoides intestinalis (PCI) are also well known (1, 2, 13–15). Multiple radiolucent cysts or linear streaks of gas are seen within the bowel wall or mesentery. Sometimes this condition is associated with a pneumoperitoneum which is usually of long duration and nearly always asymptomatic. Multiple theories on the etiology of PCI ((2, 16–19, 21–23, 29) have been discussed at great length in the recent literature and will not be argued or amplified here. Case Reports Case I: L. C., a 58-year-old white female, had suffered from Raynaud's phenomenon since 1959, In 1962 she noticed definite hardening of the skin of the hands, arms, and face. Dysphagia, nausea and vomiting, constipation, and severe abdomina distension led to her admission at another hospital for exploratory laparotomy. Filmy adhesions were noted in the right upper quadrant, secondary to a previous cholecystectomy. The surgical conclusion was “a small bowel obstruction probably caused by paralytic ileus.” The patient presented at University of Colorado Medical Center (UCMC) on Aug. 20, 1964, with obvious clinical scleroderma. Films of the hands, esophagus, small bowel, and colon confirmed clinical findings. She became progressively more cachectic and intermittently and chronically obstructed. Laparotomy revealed an “ileal adhesion.” However, she showed no objective improvement and eventually required long tube intestinal decompression for the eight months prior to her death. Pneumatosis intestinalis without pneumoperitoneum was first observed in January 1965 and became more marked two weeks before death. Autopsy in April 1965 revealed severe cutaneous and subcutaneous sclerosis. There was marked gastrointestinal smooth muscle atrophy with replacement by fibrous connective tissue. Most of the small bowel serosa was distended with small air bubbles. The lungs demonstrated delicate alveolar walls with only scattered minimal perivascular fibrosis. Bronchopneumonia was thought the terminal event.

A spinal muscular atrophy with scapuloperoneal distribution.

PROGRESSIVE muscular atrophy with scapuloperoneal distribution is unusual. Kaeser indicated the diverse neuromuscular etiologies in his summary of previously reported cases, and described the first patient with proven anterior horn cell degeneration. 1 In the present paper a sporadic case is reported which differs from earlier cases and illustrates the difficulties that may be encountered in distinguishing disorders of the anterior horn cell from those of the peripheral nerve. Report of a Case A Negro girl was born after an uncomplicated full-term pregnancy, labor, and delivery, weighing over 2.7 kg (6 lb) at birth. She sat alone shortly before 1 year and walked at 14 months. Abnormal gait, with toe-walking, was evident at age 4. At age 6, in another hospital, severe bilateral equinovarus deformities of the feet, marked atrophy of the peroneal muscles, and mild weakness about the shoulder girdle were reported. Other muscles were normal. Laboratory tests

CONGENITAL DISLOCATION OF THE HIP

HomeRadiologyVol. 42, No. 2 PreviousNext EditorialCongenital Dislocation of the HipPublished Online:Feb 1 1944https://doi.org/10.1148/42.2.188MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In AbstractThere has never been a very general agreement as to the exact mechanism of congenital dislocation of the hip. It has been variously ascribed, to several developmental changes. One school holds that it is primarily a bone defect, the result of deficient development of the acetabulum with poor formation of the posterior rim. Others believe that it is due to a relaxation of the capsule and ligamentous structures, with secondary bone changes resulting from the lack of articulation. It is of interest, therefore, to find a new etiological concept described in a recent communication by Badgley (1). He is unable to correlate the clinical, embryological, and anatomical facts with a theory based on a primary fault inherent in the development of the acetabulum. He cannot understand how, if such a theory were correct, so large a percentage of patients with preluxation could go on to normal development after treatment. In a series of 478 cases of preluxation of the hip, treated in the first year of life by Putti's method of abduction and internal rotation of the hip, excellent results were obtained in 94 per cent.Badgley's contention is that the deformity is secondary to anteversion of the head and neck of the femur with a resultant secondary hypoplasia of the acetabulum. The chief evidence available of congenital dislocation of the hip in early embryonic life is to be found in arthrogryposis multiplex congenita, and for this reason a special study was made of that syndrome. The conclusion is reached that the characteristic posture of the limbs in arthrogryposis is definitely that of an arrest in development, with failure of normal rotation of the limb bud. The extrinsic and intrinsic factors producing this arrest must be assumed to be the marked muscle atrophy and degeneration and the rigidity of joints characteristic of this condition. The “secondary acetabulum” as described in these cases is not a true secondary acetabulum but actually represents a failure of development of the anterior margin of the ilium from pressure by the femoral head. Hypoplasia of the posterior superior margin of the acetabulum, which has been supposed to be the inherent acetabular defect in congenital dislocation, may well be due to similar pressure produced by anteversion of the head of the femur rotating the neck against the posterior acetabular rim. In further support of his theory, the author calls attention to a series of 500 cases of clubfoot, with evidence of dislocation of the hip in 39, and cites Böhm's theory of the embryological origin of clubfoot as an arrest of development of the hind foot which prevents its normal rotation.Badgley feels that there is an intrinsic design for the form of the acetabulum but that extrinsic forces play an increasing part in the later stages of development.Article HistoryPublished in print: Feb 1944 FiguresReferencesRelatedDetailsRecommended Articles RSNA Education Exhibits RSNA Case Collection Vol. 42, No. 2 Metrics Altmetric Score PDF download