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PubMed2004

[Muscular dystrophy: advances in research works and therapeutic trials].

Muscular dystrophy is defined as "a group of hereditary disorders with the major symptom of progressive muscle weakness due to muscle fiber degeneration and necrosis". After the discovery of the dystrophin gene and the gene product for Duchenne muscular dystrophy in 1986, there has been remarkable progress in the differential diagnosis and in understanding the pathogenetic mechanism of muscle fiber necrosis. With discoveries of genes responsible for many other disorders, the classification of muscular dystrophy has become more complicated; for instance, there are at least 15 diseases in the limb-girdle muscular dystrophy (LGMD) group, including the autosomal dominant forms, LGMD1A-1E and the recessive forms, LGMD2A-2I. Among them, gene defects in the sarcoglycan complex (sarcoglycanopathy) have been added to LGMD2C-2F. Sarcoglycanopathy seems to be rare in Japan since only 6-7% of LGMD patients had this defect. There are two major possible strategies in treating these patients. One is gene therapy, which is recently being investigated in the mdx mouse by using adenovirus-associated virus (AAV) vector inserted with a microdystrophin gene. Dr Takeda has reported favorable results in mdx mouse muscle with this method. Another is regeneration therapy using stem cells. There are many barriers to overcome to treat patients with stem cells isolated from bone marrow. The most difficult problem to solve is how to culture the stem cells to increase their numbers for application and how to introduce the normal dystrophin gene into these cells.

Animals↗

PubMed2005

[Recent advances in congenital muscular dystrophy research].

Congenital muscular dystrophy (CMD) is a group of heterogeneous disorders characterized clinically by delayed milestones due to generalized muscle weakness and dystrophic muscle pathology. The discovery of fukutin, responsible gene for Fukuyama CMD (FCMD) and defective glycosylation in its muscle biopsy has lead significant advances in CMD researches, especially disorders with glycosylation defects to a dystroglycan (alphaDG). The highly glycosylated a DG is one of the major dystrophin-associated proteins anchored a basement membrane protein, laminin 2 to the dystrophin molecule. The disorders with the defective glycosylation are now categorized as a dystroglycanopathies which include FCMD, muscle-eye-brain (MEB) disease, Walker-Warburg syndrome (WWS) and diseases with mutations in fukutin-related protein (FKRP) and LARGE genes. Among them, MEB and WWS were proven to have mutations in the glycosyltransferase genes, POMGnT1 (protein O-mannose beta 1,2-N-acetylglucosaminyl/transferase 1) and POMT1 (protein O-mannosyltransferase 1), respectively, though others are still unknown how the glycosylation defect is induced. Although the disease with FKRP mutation has variable phenotypes from CMD to limb-girdle muscular dystrophy, others with defective to decreased a DG show CMD, central nervous system involvement with migration disorder (polymicrogyria) and ocular abnormalities.

Humans↗

PubMed2006

The gene expression and activity of calpains and the muscle wasting-associated ubiquitin ligases, atrogin-1 and MuRF1, are not altered in patients with primary hyperparathyroidism.

Hyperparathyroidism (HPT) can be associated with muscle atrophy and weakness. Muscle atrophy is typically caused by increased muscle protein breakdown. The influence of HPT on calpains and the ubiquitin-proteasome pathway, which are important regulators of muscle proteolysis, is not yet known. We examined the expression in skeletal muscle of mu- and m-calpain and the ubiquitin ligases, atrogin-1 and MuRF1, in patients with primary HPT. A biopsy was obtained from the sternohyoid muscle in patients undergoing surgery for primary HPT (n=8) and in normocalcemic control patients undergoing thyroid surgery (n=11). mRNA levels for atrogin-1, MuRF1 and the calcium-regulated proteases, mu- and m-calpain, were determined by real-time PCR. Calpain activity was measured using the calpain-specific substrate, BODIPY-FL-casein, and by zymography. Serum calcium was 11.4+/-0.46 and 9.5+/-0.10 mg/dl in HPT and control patients, respectively (p<0.01). The corresponding phosphate levels were 2.7+/-0.2 and 3.6+/-0.1 mg/dl (p<0.05). Parathyroid hormone serum concentration was 286+/-103 pg/ml (range, 77-946 pg/ml) in patients with HPT and was not measured in control patients. There were no significant differences in mRNA levels for atrogin-1, MuRF1, mu- or m-calpain and in calpain activity between HPT and control patients. The results suggest that the ubiquitin-proteasome and calpain systems are not activated in skeletal muscle in patients with primary HPT, at least not in patients with moderate hypercalcemia.

Adult↗

PubMed1991

Severe hemolytic uremic syndrome associated with rhabdomyolysis and insulin-dependent diabetes mellitus.

Hemolytic uremic syndrome has been associated with insulin-dependent diabetes mellitus eight times in the past, while the hemolytic uremic syndrome and rhabdomyolysis association has been described once. The occurrence of both conditions in a patient with the hemolytic uremic syndrome has not been reported. In this report, we described a 28-month-old girl who presented with severe hemolytic uremic syndrome muscle weakness and elevated muscle enzymes. Later, she developed hyperglycemia and ketosis requiring initiation of insulin therapy. The current literature was reviewed, and a hypothesis for the mechanism of injury and the multisystemic nature of this syndrome is presented. In patients with the hemolytic uremic syndrome, identification of potential extrarenal involvement is important as it may determine the final outcome of this disease.

Child, Preschool↗

PubMed1990

[Progressive systemic sclerosis and polymyositis overlap syndrome: a report of 40 cases].

We studied 40 patients who had clinical features of both typical progressive systemic sclerosis (PSS) and polymyositis (PM). The indurated skin extended over the metacarpophalangeal joints in all patients. The sclerotic changes, diffuse in type, involved the whole body, including the face, neck, extremities and trunk in most cases. All showed Raynaud's phenomenon. All patients had muscle weakness, elevated skeletal muscle enzymes and myogenic disorders in electromyograms, and biopsy of muscles showed myositic changes. The symptoms involved multiple systems, particularly the gastrointestinal and circulatory systems, presenting features of both PSS and PM. Serologic examinations often revealed auto-antibodies in these patients. Treatment with small doses of corticosteroid and traditional chinese medicines usually had good results. We found that either PSS or PM might appear first, or they might appear simultaneously. It is our opinion that as long as we keep alert and carefully observe the features and their development, we can easily diagnose the disease early.

Adolescent↗

PubMed1990

Maximal isometric torque of knee extension as a function of muscle length in subjects of advancing age.

Many neuromuscular diseases are associated with muscle weakness. Assessment of this weakness by manual muscle testing or with hand-held equipment has been criticized. Furthermore, muscle length influences peak force development. One hundred fifty-three female and 116 male asymptomatic subjects between the ages of 20 and 80 (approximately 20 per decade) were studied. Maximal strength of the quadriceps group was determined isometrically, on a specially designed bench, at 3 hip angles (45 degrees, 90 degrees, and 180 degrees of extension), which represent three rectus femoris muscle lengths. Maximal strength was observed at a hip angle of 180 degrees and did not decrease significantly from 20 to 50 years of age, although the values for the male and female subjects over 50 years were significantly decreased (approximately 15% per decade). The increase in strength as the muscle was lengthened from 45 degrees to 180 degrees hip angle was about 80% (of the force at 45 degrees) in 20-year-olds; strength decreased progressively with age to a value of approximately 50% in the 70-year-olds. This greater reduction in maximal strength at longer quadriceps muscle lengths has functional significance in rising from a chair, climbing stairs, and eventually, walking and standing.

Adult↗

PubMed1984

[Clinical effects of total-body hyperthermia combined with anticancer chemotherapy for far-advanced cancer].

We treated 27 patients with far-advanced cancer with extracorporeally induced total-body hyperthermia combined with anticancer chemotherapy (TBHC). All of them under went unsuccessful anticancer chemotherapy. Excluding 7 patients who could not be evaluated, a partial response was obtained in 7 of 20 patients (35%). Considering that the patients in our series were in the terminal stage of cancer, our results are encouraging. However, despite the regression of the mass in some patients, the survival time after TBHC was not always prolonged. A characteristic complication of TBHC was the weakness of the muscles in the lower extremities. The occurrence of muscle weakness could be prevented by administration of phosphate. To improve the therapeutic effects of TBHC, there are some problems to be solved. In combined anticancer chemotherapy, the selection of anticancer drugs and the timing of their administration are of importance. Thus, the optimal heating method and the combined anticancer chemotherapy are the areas requiring further study to determine the clinical efficacy of TBHC in patients.

Animals↗

PubMed1995

[A case of radiation neuropathy following radiation therapy for metastasis of breast cancer].

We report a 49-year-old woman with radiation neuropathy, which occurred 7 years after radiation therapy for supraclavicular lymph node metastasis of breast cancer. The patient had noticed a hard induration in the right breast twelve years previously and had a radical operation for right breast cancer. After surgery, she remained well until 7 years ago when she noted a right supraclavicular mass. Based on a diagnosis of metastasis of breast cancer at the right supraclavicular lymph node, she received radiation therapy at 50 Gy in 25 fractions to the lesion. Seven years after radiation therapy, the patient noted muscle weakness of II-V fingers of the right hand, followed by muscle atrophy of the right forearm. We diagnosed her condition as radiation neuropathy based on the absence of a mass lesion in the right supraclavicular region, no RI deposits in that region on Ga and bone scintigraphy, findings supportive of a lesion in the right brachial plexus on EMG and the history of radiation therapy for supraclavicular lymph node metastasis of breast cancer 7 years previously. We treated the patient with pulse therapy with high-dose oral prednisolone. Subsequently, the muscle weakness and atrophy of the right forearm and fingers have improved gradually. We suggest that pulse therapy with high-dose oral prednisolone for radiation neuropathy should be evaluated in a clinical trial, since few therapies are available for this condition.

Breast Neoplasms↗

PubMed1993

Gait and posture changes in the Duchenne muscular dystrophy child.

As the child with Duchenne muscular dystrophy (DMD) grows older, gait and posture changes occur. This is attributable to the natural progress of muscle weakness secondary to muscle tissue destruction. Bracing improves body alignment and maintains limb stability passively. Surgical correction of contractures allows for bracing and maintenance function and is recommended as an adjunct to the overall care of the ambulatory DMD patient.

Braces↗

PubMed1997

[Chronic respiratory failure in a case with juvenile-onset acid alpha-glucosidase deficiency; successful therapy with nasal intermittent positive pressure ventilation (NIPPV)].

A 13-year-old boy with juvenile-onset acid alpha glucosidase deficiency was reported. Proximal muscle weakness including respiratory muscles and scoliosis progressed since nine year of age. He developed nocturnal dyspnea and daytime somnolence at age 13. His arterial blood gas analysis showed hypoxemia (PO2 54.1 mmHg) and hypercapnia (PCO2 72.3 mmHg), and spirometry showed significantly decreased vital capacity (% VC 21%). He was treated with nocturnal NIPPV employing a device for delivering bilevel positive airway pressure (Bi-PAP). Nocturnal dyspnea and daytime somnolence rapidly disappeared with nocturnal ventilatory support. Daytime arterial PO2 and PCO2 improved after the therapy, namely 74.8 mmHg and 64.1 mmHg respectively. We conclude that NIPPV is a noninvasive and effective therapy for respiratory failure in patients with chronic progressive neuromuscular disorder including acid alpha glucosidase deficiency.

Adolescent↗

PubMed1997

[Vitamin-resistant rickets cured by removal of a bone tumor. Review of the literature].

PURPOSE OF THE STUDY: Rickets secondary to bone or soft tissue tumors are rare in children. Majority of the reported cases occurred in adults older than thirty. This entity can be cured after tumor removal. The authors present a case in a ten year boy and literature review. MATERIAL: A ten year boy complained of diffuse bone and muscle weakness for two years. A diagnosis of arthritis was made but the patient continued to complain. Serum calcium level was normal (2.33 mmol/l), phosphorus was very low (0.43 mmol/l), serum alkaline phosphatase was high, parathyroid hormone and vitamin D level were normal. Urinalysis showed abnormal phosphate excretion. METHODS: The absence of malabsorption, no family history of rickets or hypophosphatermy presence of a marked excess of urinary phosphate, very low serum phosphate and normal serum calcium, vitamin D and parathyroid hormone levels led us to consider a diagnosis of tumor induced osteomalacia. Radiographs showed a large round radiolucent lesion in the left superior pubic ramus and generalized demineralisation. RESULTS: We performed a complete tumor resection and the space was filled with bone graft. On histopathologic examination it was a benign mesenchymal tumor. Rapid reversal of biochemical anomalies, radiographs anomalies and clinical manifestation were observed after complete tumor resection. DISCUSSION: The authors have described the tumor, the osteomalacia and the pathogenesis of tumor rickets. Histologically the most common causative tumors were vascular tumors, mesenchymal tumors and non ossifying tumors. The tumor were of bone or soft tissue origin. Clinical symptoms were muscular weakness, bone and muscle pain. Biochemically there is a very low phosphate level, a normal serum calcium level as well as a normal vitamin D and PTH level. There is a significant high level of urinal phosphate. The mechanism proposed to explain oncogenic osteomalacia includes tumor secretion of phosphaturic substance other than PTH and calcitonin. Another hypothesis is a substance interfering with normal vitamin D metabolism. The pathogenesis is not clearly defined. CONCLUSION: Regardless to the mechanism of osteomalacia, complete removal of the tumor will cure the patient. A diligent search for tumors should be done in patients with vitamin D resistant rickets.

Bone Neoplasms↗

PubMed1998

[T 0 N 2 M 0 small cell lung cancer in a patient with Lambert-Eaton myasthenic syndrome].

We report an unusual case of T 0 N 2 M 0 small cell lung cancer in a patient with Lambert-Eaton myasthenic syndrome (LEMS). A 52-year-old man began to notice muscle weakness in a left limb in January 1996, which was followed by muscle weakness in his left arm and fingers, appetite loss, and general fatigue. An electromyogram (EMG) showed the waxing phenomenon in response to high-frequency repetitive stimulation. Lambest-Eaton myasthenic syndrome was diagnosed, based on his symptoms and EMG findings. Chest computed tomography (CT) was done, and left paratracheal, tracheobronchial, subaortic, and hilar lymphadenopathy were found. No mass was seen in either lung field. Cytologic examination of the sputum and bronchial lavage fluid were done, but no malignant cells were found Small cell lung cancer was diagnosed after thoracoscopic resection of the subaortic lymph nodes. No metastases were detected by bone scintigraphy, abdominal CT, or magnetic resonance imaging of the brain. Complete response and resolution of symptoms were obtained by chemotherapy and irradiation.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1970

Myasthenia gravis and the myasthenic syndrome.

Two disorders of neuromuscular transmission producing muscle weakness and easy fatigability which may confront the physician are myasthenia gravis and the myasthenic syndrome. The former has early symptoms and signs of oculobulbar and then extremity weakness with rapid decline of action potential and contractile strength with repetitive use and nerve-muscle stimulation. Anticholinesterases improve strength. The myasthenic syndrome has early symptoms and signs of pelvic girdle, pectoral girdle and proximal limb muscle weakness. This is worst when first starting to use or carry out nerve muscle stimulation in the rested muscles. It improves significantly for a time with use or on rapid stimulation, and then declines with continued activation. Deep tendon reflexes are sluggish or absent. Small cell carcinoma of the lung is often associated. Guanidine improves the strength. Other features and possible underlying mechanisms of the two disorders help to differentiate and treat them.

Adult↗

PubMed1997

Herpes zoster polyradiculopathy.

Herpes zoster infection, resulting from reactivation of the dormant varicella zoster virus in the dorsal root ganglia, usually causes a painful dermatomal vesicular rash. Rarely, associated peripheral motor weakness is present, the mechanism of which is unclear. Three patients are reported who had focal limb muscle weakness associated with zoster infection. Physical and occupational therapy played a key role in motor function recovery of the patients, yet emphasis on the rehabilitation of postherpetic motor weakness is lacking in the literature. Physiatrists evaluating patients with limb muscle weakness following herpes zoster infection should be alert to this condition. The clinical syndrome of herpes zoster radiculopathy and the rehabilitation of these patients are discussed.

Adult↗

PubMed1984

[2 autopsy cases of congenital muscular dystrophy of Fukuyama type--a typical and an atypical cases].

Two autopsy cases of congenital muscular dystrophy of Fukuyama type (F-CMD) were described. The first case was diagnosed clinically and pathologically as its typical case. Neither his family history nor the history of his prenatal period were contributory. He had suffered from muscle weakness and atrophy since his birth. Serum CPK was markedly elevated. EMG and muscle biopsy proved dystrophic changes of the skeletal muscles. In addition, he manifested mental retardation and attacks of convulsion. EEG failed to elicit remarkable changes, but PEG represented ventricular dilatation. He died of respiratory insufficiency at age 12. His postmortem examination showed variegated anomalies in the nervous system. Extensive micropolygyria was present in the cerebrum and cerebellum accompanied by adhesions between the bilateral cerebral hemispheres. Assymmetry of the longitudinal fibers was pointed out in the pontine base. Anterior horn cells were atrophic and moderately depopulated. On the other hand, the second patient was an atypical F-CMD case in symptoms, signs and pathology. His grand-mothers on both father's and mother's sides wee first cousins. His three siblings showed no similar disorders. His mother developed slight gestational toxicosis in the sixth and seventh months of pregnancy. His muscle weakness, contracture of the bilateral hip-joints and clubfoot had been observed since his birth. Physical and neurological examinations at age 6 showed deformity of the skull, myopathic face, macroglossia, high-arched palate, pigeon chest, scoliosis of the thoracic spine. In addition, generalized muscular atrophy, hypotonia and areflexia were recognized. Pseudohypertrophy of the muscles was absent. Sensation was intact to all modalities. Serum CPK and LDH were moderately increased.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMedDate not supplied

Myasthenia gravis in conjunction with Graves' disease: a diagnostic challenge.

OBJECTIVE: To describe an association between Graves' disease and myasthenia gravis and discuss the clinical features and laboratory tests that may help distinguish these two diseases. METHODS: The clinical, laboratory, and electrophysiologic findings in a patient with Graves' disease and myasthenia gravis are presented. RESULTS: A 28-year-old African American man was admitted to the University of Louisville Hospital with generalized muscle weakness, exophthalmos, diplopia, weight loss, and mild dysphagia. The diagnosis of Graves' disease with ophthalmologic involvement was suspected clinically and confirmed by an undetectable thyrotropin level (<0.03 mIU/mL), high total thyroxine (20.5 mg/dL), and increased homogeneous 123I thyroid uptake. Because of the generalized muscle weakness and mild dysphagia, assessment was done by a neurology team, and severe thyrotoxic myopathy was diagnosed. He was treated with 131I and b-adrenergic blocking agents and scheduled for follow-up as an outpatient. Two weeks later, the patient presented in acute respiratory failure. The neurology team was reconsulted because of suspected myasthenic crisis. Anti-acetylcholine receptor antibodies were undetectable in the serum, and computed tomography of the chest showed no thymic enlargement. Repetitive nerve stimulation testing, however, showed findings consistent with an abnormality of the neuromuscular junction. The patient responded dramatically to an anticholinesterase agent and corticosteroids. CONCLUSION: The overlapping clinical features may cause diagnostic confusion when myasthenia gravis and Graves' disease coexist, and numerous tests may be needed to distinguish these two conditions, which have differing treatments and prognoses.

Acetylcholinesterase↗

PubMed2001

Knee muscle isometric strength, voluntary activation and antagonist co-contraction in the first six months after stroke.

PURPOSE: Muscle weakness may contribute to functional problems after stroke, but is rarely addressed during rehabilitation. Functional problems are commonly thought to be caused by abnormal movement patterns or possibly disuse atrophy. We investigated voluntary isometric strength, activation and the extent of co-contraction in the knee muscles during the first six months during stroke. METHODS: Twelve stroke patients (58 +/- 3 years, mean+/- SEM, 7 female) were studied bilaterally on admission for rehabilitation (21 +/- 1 days after stroke) and then at 1, 2, 3, and 6 months. Twenty healthy controls (61 +/- 5 years, 17 female) were tested once on their preferred leg. Subjects performed maximal voluntary contractions of the quadriceps and hamstring muscles. Simultaneous measurements were made of agonist force and surface EMG from agonist and antagonist muscles. Voluntary activation was estimated using the twitch superimposition technique. RESULTS: Both paretic muscles showed lower (p = 0.01-0.0005) voluntary strength than both non-paretic and control muscles until three months after stroke. Co-contraction of antagonists was similar in all groups and greater during knee extension than flexion. Stroke patients showed considerable bilateral voluntary activation failure (25-40%, p = 0.01-0.001) throughout the study while most control subjects did not (group mean 7%). CONCLUSIONS: The muscle weakness and bilateral activation failure in the stroke patients was not explained by either excessive antagonist activity or disuse atrophy. They had potential for increased voluntary strength and if this were addressed during rehabilitation, then the rate and extent of functional recovery might be enhanced.

Case-Control Studies↗

PubMed1999

[Various manifestations of the A8344G mtDNA heteroplasmic mutation in 4 families with the MERRF syndrome].

BACKGROUND: The most frequent manifestation of mitochondrial DNA (mtDNA) mutation 8344 A-->G is MERRF syndrome (Myoclonic Epilepsy and Myopathy with Ragged Red Fibres). Less frequent symptoms include ataxia, perceptive type of deafness, cardiomyopathy or external ophthalmoplegia and mental and motor retardation in children. We describe heterogeneity of clinical symptoms and results of biochemical and molecular investigations in four families with the heteroplasmic mutation 8344 A-->G in mtDNA. METHODS AND RESULTS: In co-operation with paediatric, neurological and genetic specialists from the Czech and Slovak Republic we found in 1993-1998 at the enzymatic or molecular level more than 90 children and adults with impaired mitochondrial energy metabolism. Heteroplasmic mutation 8344 A-->G in mtDNA was found in four families. Ataxia and progressive muscle weakness appeared in the first proband with 50% of mutated copies of mtDNA in muscle at the age of 30 years. The second proband with 95% of mutated mtDNA had his first clinical symptoms--muscle hypotonia, cardiomyopathy and mental and motor retardation--in infancy while his four relatives with 25-50% mutated mtDNA lack so far clinical symptoms. In a female from the third family with 50% mutated mtDNA in muscle the disease manifested at the age of 42 years with progressive external ophthalmoplegia (PEO) and muscle weakness. In the fourth proband with 50% of mutated mtDNA in blood the disease started in infancy with spastic quadruparesis and arrested mental and motor development. Enzymatic and histochemical investigation in muscle biopsy in two probands revealed lower cytochrom c oxidase activity. Ragged-red fibres were found only in one adult patient. CONCLUSIONS: MtDNA mutation 8344 A-->G can manifest by heterogeneous symptoms. A higher percentage of mutated mtDNA is usually associated with more serious forms of the disease, but there is not always a correlation between the degree of heteroplasmy and severity of the disease or the age of the first clinical symptoms.

Adult↗