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At least 217 records · Page 12Linked to original sources
PubMed2000

The strength and functional performance in patients with facioscapulohumeral muscular dystrophy.

Facioscapulohumeral muscular dystrophy (FSHD) is a slowly progressive myopathy with autosomal dominant inheritance remarkable for its early involvement of facial musculature. The purpose of our study was to assess the rate of strength deterioration, functional condition and performance of activity of daily living of patients with FSHD in Taiwan. Twenty patients diagnosed with FSHD were included in this study. Manual muscle testing (MMT) was used to evaluate muscle strength. The Brooke and Vignos scales were used to assess upper and lower extremity function respectively, and the capability of the activity of daily living was measured by Barthel index. The result of the strength testing was characterized by the presence of a progressive asymmetrical muscular weakness in patients with FSHD. The mean muscular strength of the right extremity was weaker than its left counterparts (p < 0.05) and the shoulder muscle group was the weakest. According to the Brooke functional scale, 20% of our patients were graded as 1, 30% as grade 2, and 50% as grade 3. On the Vignos functional scale, 50% of patients fell into grade 1, 10% in grade 2, and 40% in grades 3-5. Vignos scale was significantly correlated with mean muscle strength (p < 0.05). The average value of Barthel index was 97.8 +/- 4.7. The muscle strength decline in this Taiwanese of FSHD population was more severe in shoulder girdle area. The mean muscle strength of the right extremity was weaker than the left. Most of our patients suffered from mild or moderate physical disability. Finding of these Taiwanese FSHD population is similar to those reported elsewhere in the world.

Activities of Daily Living↗
PubMed2003

[Cutaneous necrosis is predictive of cancer in adult dermatomyositis].

INTRODUCTION: Adult dermatomyositis is associated with cancer in 15 p. 100 to 50 p. 100 of cases and, hence, investigations should be systematically performed to search for cancer. A number of predictive factors have been reported. The aim of our study was to search for predictive factors of cancer, among adults with dermatomyositis. METHODS: We prospectively assessed 26 adults presenting with dermatomyositis, hospitalised in our department of dermatology from January 1993 to June 2000. The parameters assessed were: association with a cancer, age, gender, cutaneous necrosis, muscular weakness, electromyographic abnormalities, erythrocyte sedimentation rate, and muscular enzyme levels. RESULTS: Mean age was of 52 years and sex ratio (M/F) was of 0.53. Cancers were diagnosed in eight cases (31 p. 100) (mean age: 59.5 years; sex ratio=1; cancer localization: lung (2), breast (2), ovary, endometrium, bladder, and melanoma). Five patients in the cancer group had cutaneous necrosis and only 2 in the without cancer (p=0.01; PPV=71.4 p.100). Elevation of muscular enzyme was also associated with cancer. CONCLUSION: Our report demonstrates that cutaneous necrosis is closely associated with cancer and it suggests that in selected patients with dermatomyositis and cutaneous necrosis, more exhaustive and repeated investigations should be performed to search for cancer. The interest of elevation in muscular enzyme as a predictive factor of cancer is discussed.

Adult↗
PubMed1992

[A case of syringomyelia with proximal dominant muscle weakness and without superficial sensory disturbance].

A 43-year-old woman had noticed muscular weakness in her arms for four years before her admission. Muscle weakness and atrophy were prominent in the bilateral deltoid muscles, but muscular strength was almost unimpaired in the bilateral forearms and intrinsic muscles. There was no sign of sensory impairment except vibratory sensation. EMG revealed neuropathic NMU. X-P of the cervical spine showed enlargement of the spinal canal diameter, and MRI of the spinal cord revealed a large syrinx. On the basis of metrizamide CT and cranial MRI, a diagnosis of syringomyelia with Chiari malformation (type I) was made. Despite the presence of a large syrinx extending from C 1 to Th 11, the only detectable neurological sign was proximal weakness of the upper extremities simulating myopathy.

Adult↗
PubMed1981

Infant botulism type B in central Europe.

A case of moderately severe botulism was diagnosed in a 4 weeks old white female. Clostridium botulinum toxin was identified repeatedly in the infant's faeces by means of the mouse protection assay. Clostridium botulinum was isolated in pure culture from faecal material. Both the organism and the toxin were type B. The onset of illness was characterized by mild constipation, apathy, weak sucking and difficulty with swallowing. Incipient, probably aspiration, pneumonia was diagnosed at the same time. Further signs of botulism developed during hospitalization, viz. loss of head control, pooled oral secretion, weak cry, mild ptosis, reduced facial expression, generalized muscular weakness and reduced spontaneous activity. A nasogastric feeding tube was needed because the ability to suck and swallow was impaired. Immediately on admission of the infant to hospital emergency treatment was started with ampicillin, which was followed by penicillin injections. The infant recovered in 60 days. Subsequent medical examinations demonstrated that the recovery was complete and the development normal. The case represents the first instance of infant botulism detected on the European Continent.

Botulinum Toxins↗
PubMed2004

Rocking bed and prolonged independence from nocturnal non-invasive ventilation in neurogenic respiratory failure associated with limb weakness.

A 40 year old mother of three with autosomal dominant scapuloperoneal muscular dystrophy presented with severe neurogenic respiratory failure requiring nocturnal non-invasive ventilation (NIV). Because of the development of profound proximal muscular weakness as a consequence of the progressive nature of her neurological disease, she eventually was unable to apply and remove the facial interface to set up her NIV circuit. She therefore became dependent on her children and carers to start and stop NIV during the night. A rocking bed was successfully employed as an alternative to nocturnal NIV. Ventilation was facilitated by the passive movement of the diaphragm as a consequence of the movement of the abdominal contents under the effect of gravity. Benefit was demonstrated objectively by pulse oximetry and subjectively by the improvement in the patient's symptomatology and continued independence at night. The ease of use of a rocking bed should be borne in mind when the necessity for nocturnal ventilatory support in neuromuscular disease results in the potential loss of independence for a patient.

Adult↗
PubMedDate not supplied

[A case with severe respiratory muscle weakness due to chronic myositis associated with PBC].

We report a 37-year-old woman with slowly developing muscular weakness for 2 years following insidious stiffness of calf muscle. Serum CK was elevated up to 4,207 IU/l. She presented sleepiness, weakness of proximal and truncal muscles and systemic muscular atrophy. While she had not experienced dyspnea, her arterial blood gas analysis revealed extreme hypoxia and hypercapnea due to weakness of respiratory muscles. Echocardiogram showed thinness and hypokinesis of left ventricular wall, and arrhythmia was pointed out by holter ECG. Needle elctromyogram of the proximal muscles exhibited polyphasic units with low amplitude. Muscle biopsy showed degeneration and necrosis of muscle fibers as well as regeneration. Mild infiltration of inflammatory cells was shown. Serological examination showed positive antimitochondrial M2 antibody, especially specific for primary biliary chirrhosis (PBC). She was diagnosed as chronic myositis associated with PBC. Four cases of idiopathic myositis with severe weakness of respiratory muscle, associated with PBC had been reported. These cases and our present case share the similar feature in respect of insidious or chronic course and resistance to therapy. In our present patient, respiration had been supported by BiPAP and she has been successfully improving slowly by oral steroid following 4 courses of methylprednisolone pulse therapy.

Adult↗
PubMed1990

[Effect of splenectomy for management of thrombocytopenia associated with systemic lupus erythematosus: a case report].

A 51-year-old female with systemic lupus erythematosus (SLE) was admitted in November 1987 because of general fatigue and muscular weakness. She was treated with prednisolone (PSL) 30 mg and azathioprine (AZP) 50 mg after failure in the management of thrombocytopenia by PSL 15 mg. She exhibited no splenomegaly. Muscular atrophy and weakness were seen in the proximal muscles. Her platelet count was 44,000/microliters. A bone marrow aspiration revealed an increase in megakaryocytes. The blood chemistry revealed a normal CPK level and an elevated LDH level, indicating a presence of steroid myopathy. A splenectomy was performed after an increase of platelet count by giving gamma-globulin 400 mg/kg for 5 days. The platelet count rose to 368,000/microliters on the 46th postoperative day. She was treated with PSL 5 mg and AZP 50 mg as postsplenectomy therapy. The splenectomy did not adversely affect other aspects of SLE, in particular, renal function. She had no major complications in the postoperative period. Her platelet count reached a plateau 4 months later and revealed 115,000/microliters 18 months postoperatively.

Female↗
PubMed1993

Molecular analysis of the Duchenne muscular dystrophy gene in patients with Becker muscular dystrophy presenting with dilated cardiomyopathy.

Molecular analysis of the Duchenne muscular dystrophy (DMD) gene was performed on 4 unrelated patients with Becker muscular dystrophy (BMD) presenting with dilated cardiomyopathy. Two patients with a deletion involving exon 1 were quite unique in that they developed fatal myocardial involvement in their teens, despite the absence of significant muscular weakness. The deletion found in these patients comprised the 3'-end of exon 1 and the greater part of intron 1. Two other patients with a deletion of exon 47 showed progressive muscular atrophy and weakness; they were considered to be typical BMD in both clinical features and the type of gene deletion. We speculate that a deletion around exon 1 may severely damage the expression and/or the function of dystrophin selectively in cardiac muscle, but not in skeletal muscle.

Adolescent↗
PubMed1998

Decreased expression of brain beta-dystroglycan in Duchenne muscular dystrophy but not in the mdx animal model.

Abnormalities in the muscle dystrophin-glycoprotein complex are implicated in the molecular pathogenesis of various neuromuscular disorders. Weakening of the trans-sarcolemmal linkage between the actin membrane-cytoskeleton and the extracellular matrix appears to trigger destabilization of the muscle cell periphery. In addition to muscular weakness, one-third of patients suffering from Duchenne muscular dystrophy exhibit mental retardation. Since little is known about the pathophysiology of brain abnormalities in these patients, we investigated the fate of the most abundant dystrophin-associated protein, beta-dystroglycan, in the central nervous system. It was found to be present throughout all normal brain regions studied. In contrast, this glycoprotein was greatly reduced in brain microsomes derived from Duchenne specimens, while it is of normal abundance in the brain from the dystrophic animal model mdx. Deficiency in brain beta-dystroglycan might render nervous tissue more susceptible to cellular disturbances and this may result in cognitive impairment in some Duchenne patients.

Animals↗
PubMed1988

Thyrotoxic periodic paralysis.

We present the case of a 21-year-old man who presented to the emergency department with an episode of profound weakness due to thyrotoxic periodic paralysis, a syndrome of muscular weakness occurring in patients with hyperthyroidism. Prior to the diagnosis, the patient was treated with a parenteral tranquilizer. When hypokalemia was discovered, potassium was administered, resulting in the development of hyperkalemia. Episodes of thyrotoxic periodic paralysis are usually self limited, and recovery of motor strength is complete. However, potassium is frequently administered to hasten recovery and prevent cardiac arrhythmias and respiratory arrest. Serum potassium must, therefore, be monitored carefully in these patients during treatment.

Adult↗
PubMed2005

[Analysis of the expression of collagen VI in congenital muscular dystrophy].

Congenital muscular dystrophy (CMD) composes a group of disorders characterized by hypotonia and muscular weakness noticed in the first year of life. The Ullrich's form is characterized by proximal joint contractures and distal hiperextensibility. About 40% of these patients present mutations in one of the genes that codify the sub-units of the collagen VI protein (COL6), producing total or partial deficiency of the protein expression. We analyzed, through immunohistochemistry, the expression of COL6 in muscle fragments of 50 patients with CMD; 20 of them presented merosin expression deficiency. We identified 4 cases with total COL6 deficiency (8% of the total), representing 13% of the cases with normal merosin expression. The histological findings of patients with deficiency of COL6 were indistinguishable from other forms of CMD, but milder than that abnormalities observed in merosin deficient patients. In three COL6 deficient patients were observed hypotonia and weakness in the neonatal period, delayed of motor milestones, muscular retractions of knees and elbows, distal joint hiperextensibility and congenital hip dislocation (two patients). One patient lost the ability to walk; and one died due to respiratory problems. The analysis of COL6 expression, as well as merosin expression, in the muscle tissue from CMD patients, can be important for identification and phenotypic characterization of different CMD subtypes.

Biomarkers↗
PubMed1983

[Centronuclear myopathy. Apropos of a new case].

The observation of a thirteen-year-old boy who presented with motor developmental delay, muscular weakness and a waddling gait is reported. Moderately increased muscular enzyme activities and electromyographic findings suggested muscular disease. Histological and ultrastructural examinations of muscle biopsy specimens showed both hypotrophia of type 1 fibers and characteristic distribution of the nuclei within the sarcomere in a chainlike pattern in the center of the fiber surrounded by a space devoid of myofibrils. A few aspects of myophagia were found explaining why muscular enzyme activities were increased. The mother was clinically normal with slightly increased muscular enzyme activities and normal muscle biopsy specimens. The maternal grandmother had delayed gait abnormalities with normal muscular enzyme activities. Approximately one-hundred cases such as ours, isolated within a family, have been published. The course and the mode of inheritance suggest heterogeneity.

Adolescent↗
PubMed1987

[Thyrotoxic myopathy (clinical picture, diagnosis and principles of treatment)].

A series of 263 patients with thyrotoxic myopathy (TM) (47 males and 216 females) were studied. A total of 151 patients were investigated by the author and 112 case reports were taken from the literature. New clinical peculiarities of TM were detected. The author presented a quantitative assessment of the incidence, degree of weakness and atrophy of individual muscles, described the topography of muscular lesions at different phases of the development of thyrotoxicosis and myopathy. He also proposed an original formula of muscular weakness and atrophy which makes it possible to identify myopathy in its initial stage even when there are no evident clinical manifestations of thyrotoxicosis. Thus, a new method of early diagnosis of TM on the basis of topography of muscular lesions and other clinical characteristics is proposed. This method provides reliable evidence for the preliminary diagnosis of thyrotoxicosis. In addition to thyrotoxicosis correction the question is raised about the pathogenetic treatment of TM.

Adult↗
PubMed1986

Progressive muscular dystrophy with congenital adrenal hypoplasia: an unusual autopsy case.

A 3 1/2-year-old child with progressive muscular dystrophy (PMD) and congenital adrenal hypoplasia (CAH) is described. Symptoms and signs of adrenocortical insufficiency appeared shortly after birth. Despite corticosteroid therapy, the muscular weakness and elevated CK level continued. A diagnosis of Duchenne muscular dystrophy was made on the basis of clinical signs and characteristic muscle biopsy. The affection of his older brother suggests an X-linked recessive inheritance. The autopsy revealed a very rare combination of cytomegalic type CAH and PMD. This combination suggests that a small deletion of X-chromosome might be responsible for the two disorders.

Adrenal Glands↗
PubMed1999

[A case of acute renal failure with rhabdomyolysis caused by the interaction of theophylline and clarithromycin].

Theophylline toxicity has been recognized since its introduction into clinical medicine. Clarithromycin is a new oral macrolide antibiotic with excellent antibacterial activity and rare adverse effect. Patients with upper respiratory infection are often treated with theophylline and clarithromycin concurrently. We report a case of acute renal failure due to acute rhabdomyolysis caused by the interaction of theophylline and clarithromycin. A 72-year-old man visited our hospital because of coughing and a sore throat continuing for 1 week. He was diagnosed as having the common cold with a bronchial asthmatic symptom and was prescribed 200 mg/day of sustained-release theophylline for the treatment of asthma for 7 days. One week later, he visited our hospital again. Radiographic study of the chest revealed mild interstitial pneumonia and 200 mg/day of sustained-release theophylline and 400 mg/day of clarithromycin were administrated concomitantly. Five days after the second visit, the patient was admitted to our hospital because of generalized twitching, muscular weakness, high fever and serious general condition. He experienced generalized muscular twitching and tremor. Blood urea nitrogen was 106.1 mg/dl, serum creatinine was 7.4 mg/dl, serum creatinine kinase (CK) was 36,000 IU/l (normal 15-130 IU/l), CK isozyme revealed the following ratio: BB 0%, MB 1% and MM 99%. He was diagnosed as having acute renal failure with rhabdomyolysis caused by the interaction of theophylline and clarithromycin. Hemodialysis therapy was started. After 5 weeks, his serum creatinine was markedly decreased. It is well-known that clarithromycin enhances the serum concentration of theophylline by inhibition of the cytochrome P450-dependent pathway in hepatocytes. Theophylline toxicity may be enhanced when clarithromycin is administrated concomitantly, especially to elderly patients with dehydration.

Acute Kidney Injury↗
PubMed1994

An unusual case of muscular limb-girdle dystrophy and mitochondrial myopathy.

Limb-girdle muscular weakness and wasting could be caused by different diseases (inflammatory and hereditary myopathies, muscular dystrophies and neurogenic atrophies). Among these, Limb-Girdle Muscular Dystrophy (LGMD) is an heterogeneous group of pathologies that have progressive proximal limbs and girdle weakness, with some dystrophic features by the muscle biopsy. We studied a case of LGMD in an adult man with a typical histological and histochemical profile, associated to a mitochondrial deficit characterized by presence of ragged-red fibers, a histochemical Cytochrome Oxidase deficiency and abnormal mitochondria by ultrastructure.

Adult↗
PubMed1988

Soccer injuries and their relation to physical maturity.

A prospective study of injuries encountered during participation in a summer soccer camp for youths aged 6 through 17 years revealed an injury incidence of 10.6 per 1000 hours for girls (107 injuries in 458 girls) and 7.3 per 1000 hours for boys (109 injuries in 681 boys). For both sexes, the incidence of injury increased at age 14 years. One certified athletic trainer (W.D.C.) assessed and documented all injuries during the course of the study. Seventy percent of injuries involved the lower extremities. Contusions represented 35.2%, strains 27.8%, sprains 19.4%, and fractures and dislocations 2% of all reported injuries. The ankle was the most frequent site of injury in both sexes. Quadriceps strain was a common injury in boys. The boys with the highest incidence of injury were tall (greater than 165 cm) and had a weak grip (less than 25 kg), suggesting that skeletally mature but muscularly weak boys may be more susceptible to injury while playing soccer with peers of the same chronological age.

Adolescent↗
PubMed1985

Neonatal spinal muscular atrophy presenting as respiratory distress: a clinical variant.

Respiratory distress from diaphragmatic and intercostal muscular weakness was the presenting feature of two neonates with progressive spinal muscular atrophy, leading to death in the first 2 months of life. This atypical feature provides evidence of heterogeneity within the commonly used clarification of type 1 spinal muscular atrophy.

Diagnosis, Differential↗