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At least 973 records · Page 54Linked to original sources

PubMed1984

A case of hypokalemic myopathy associated with transient hypothyroidism.

A case of transient hypothyroidism in the course of hypokalemic myopathy is reported. A 69-year-old woman had severe muscle weakness and marked potassium deficiency associated with alkalosis during treatment with thiazide diuretics. The cause of muscle weakness proved to be hypokalemic myopathy confirmed by clinical findings and muscle biopsy. After the episode of hypokalemic myopathy, serum levels of thyroid hormone were lowered (T4; 3.8 micrograms/dl, T3; 54 ng/dl) and that of TSH was elevated (25.1 microU/ml). Antithyroid microsomal antibody was positive (1:25600) and anti-thyroglobulin antibody was negative. About one month after potassium supplement, her thyroid functions returned to normal, along with normalization of serum potassium level. This is the first documented case report of hypokalemic myopathy accompanied by transient hypothyroidism in a patient with autoimmune thyroiditis. We suggest that this transient hypothyroidism might be induced by hypokalemia during the course of autoimmune thyroiditis.

Aged↗

PubMed1995

Musculoskeletal disorders in juvenile onset mixed connective tissue disease.

OBJECTIVE: We studied the occurrence of musculoskeletal involvement in patients with juvenile onset mixed connective tissue disease (MCTD) and their functional ability. METHODS: Thirteen patients who fulfilled the Kasukawa criteria of MCTD, were subjected to a general orthopedic assessment, as well as to a specific examination of tenderness and swelling of joints, muscle strength, joint motion and alignment, radiographical evaluation, and on disability and discomfort. RESULTS: Low indices were found on the modified Ritchie Articular Index (RI) (0.07) and the Fuchs Swelling Index (0.13). Mild to moderate indices were measured on the Joint Alignment and Motion Scale (JAM) (0.52) and on the Larsen Radiographic Evaluation Scales for the wrist, metacarpal, proximal and distal interphalangeal joints (L-RES) (1.40, 0.62, 0.31, 0.31). The mean pulp-thickness ratio was 22%, which is an indication for sclerodermic changes in the hands. Proximal muscle weakness was found in 10/13 patients (mainly in the upper extremity), distal muscle weakness was found in 12/13 patients. On the pediatric version of the Stanford Health Assessment Questionnaire (C-HAQ), pain and discomfort scored 0.04, disability scored 0.46; those scores were considered to below. Between L-RES and JAM a significant correlation was found (0.74, 0.74 and 0.73; p < 0.01). Another significant correlation was found between the RI and the disability score of the HAQ (0.58; p < 0.05). CONCLUSION: Sclerodermic changes as well as arthritic changes are of a benign character in our population of patients with juvenile onset MCTD.

Adolescent↗

PubMed1996

AAEM case report #30: multifocal motor neuropathy.

A 73-year-old man with a 16-year history of fasciculations and 15 years of weakness in his right arm was diagnosed with focal motor neuron disease. After 10 years of purely motor symptoms, he developed mild parasthesias although his sensory examination remained normal. Reflexes were reduced or absent in the weak muscles but were normal elsewhere. Nerve conduction was studied in nerves innervating weak muscles and showed severe motor conduction block. Sensory nerve conduction studies were minimally abnormal, showing reduced amplitudes with normal velocities. Based on the clinical picture and the presence of severe motor conduction block, the patient was diagnosed as multifocal motor neuropathy. Treatment with high-dose intravenous immunoglobulin was given with significant improvement in strength and partial resolution of the conduction block. As this case demonstrates, this treatable disorder may occasionally be mistaken for motor neuron disease although the resemblance is only superficial, and it should never be mistaken for amyotrophic lateral sclerosis. Multifocal motor neuropathy is an inflammatory, demyelinating neuropathy which, like chronic inflammatory demyelinating polyneuropathy (CIDP), is probably immune-mediated. It differs from typical CIDP by virtue of a marked predilection for motor axons, a strikingly restricted distribution, and a protracted course. Treatment with high-dose intravenous immunoglobulin is frequently helpful, but other forms of immune manipulation are less effective.

Demyelinating Diseases↗

PubMed1994

Myotonic dystrophy with no trinucleotide repeat expansion.

We report 3 patients from 2 families with myotonic dystrophy who do not show an abnormal expansion of CTG trinucleotide repeats within the myotonic dystrophy gene. Characteristic features of myotonic dystrophy in these patients were frontal balding, cataracts, cardiac conduction abnormalities, and testicular atrophy with myotonia and muscle weakness. Results of muscle histopathology were consistent with myotonic dystrophy. Genetic analysis of leukocyte and muscle DNA showed a normal number of CTG repeats. The demonstration of normal CTG repeat number for the myotonic dystrophy gene does not exclude the diagnosis of myotonic dystrophy.

Adult↗

PubMed1990

McArdle's disease: two clinical expressions in the same pedigree.

Two patients with McArdle's disease within the same pedigree and with two different clinical forms are presented. The first patient suffered from progressive muscle weakness and atrophy. Muscle morphology was that of myopathy. Residual activity of phosphorylase was 28% and sodium dodecyl sulphate electrophoresis showed decreased protein. The second case was typical of McArdle's disease, clinically and biochemically. It was concluded that the first patient was a heterozygote (residual activity 28% of normal) and the second was a homozygote, the genetic transmission being autosomal recessive.

Adult↗

PubMed1986

Progressive muscular dystrophy in a golden retriever dog: light microscope and ultrastructural features at 4 and 8 months.

The clinical and morphological features of a congenital myopathy in a young male golden retriever dog were studied. Muscle biopsies at 4 and 8 months of age were examined with light and electron microscopy. Clinical features included early onset of generalized muscle weakness with selective muscle atrophy and hypertrophy, splaying of the limbs, stiff gait, and marked elevation of serum creatine kinase (CK). An electromyograph revealed spontaneous electrical activity characterized by sustained high-frequency activity, which was not abolished by neuromuscular blockade. Morphologically there was marked hypercontraction and segmental necrosis of muscle fibers with phagocytosis and regeneration. Ultrastructurally, dilatation of sarcoplasmic reticulum was the most consistent feature associated with early fiber degeneration. No abnormalities were noted in the central or peripheral nervous system. Progression of the disease was evident at 8 months. It was concluded that the findings are consistent with a dystrophic process of primary muscle origin. The probable genetics and comparison to other animal models of muscular dystrophy and to Duchenne dystrophy are discussed.

Animals↗

PubMed2000

Different effects of corticosteroid-induced muscle wasting compared with undernutrition on rat diaphragm energy metabolism.

An important adverse effect of corticosteroid treatment is respiratory muscle weakness with diaphragm muscle wasting, but little is known about the underlying pathophysiological processes involved. In order to differentiate between the effects of nutrition depletion and corticosteroids on diaphragm muscle metabolism, a study was performed to investigate the effects of triamcinolone (TR) for 2 weeks and of chronic undernutrition in a pair-weight (PW) group on the structure and energy metabolism of the diaphragm in male Wistar rats compared with a free-fed (FF) group. Diaphragm mass was reduced in TR and PW rats to a similar degree, but the extent of type-IIx/b atrophy was more pronounced in TR rats than in PW rats. No myopathic features were observed after either treatment. ATP in absolute terms as well as the ATP/ADP ratio, total adenine nucleotides, the phosphocreatine (PCr) level and the ratio between PCr and creatine (PCr/Cr) were decreased in the diaphragm of both TR and PW rats. In contrast to the PW group, the total Cr pool was reduced and pyruvate and lactate levels were elevated in the diaphragm of the TR group compared with the FF group. In conclusion, the results of this study indicate that severe undernutrition causes a decrease in muscle energy status resulting in a new metabolic equilibrium, while chronic low-dose TR treatment (0.25 mg/kg per day i.m.) causes a decrease in muscle energy status together with a mismatch between glycolysis and oxidative metabolism.

Adrenal Cortex Hormones↗

PubMed1988

Familial bulbo-spinal muscular atrophy associated with testicular atrophy and sensory neuropathy (Kennedy-Alter-Sung syndrome). Autopsy case report of two brothers.

Autopsy cases of two brothers with bulbo-spinal muscular atrophy associated with gynecomastia, testicular atrophy and sensory neuropathy are reported. The disease started with finger tremor, proximal muscle weakness and facial muscle twitching at the second and fourth decades, accompanied by bulbar signs and glove-stocking type sensory disturbance. Systemic neurogenic patterns and diminished sensory nerve action potential amplitudes were recorded by electrophysiological studies. A marked loss of myelinated fibers was noticed upon sural nerve biopsy. Gonadal hormone values were normal, except for elevated urinary estrogen. Postmortem examinations revealed a remarkable degeneration of the facial and hypoglossal nuclei, and the spinal cord motoneurons. The skeletal muscles and the tongue showed neurogenic muscular atrophy with fatty replacement. Testicular atrophy was prominent showing hyalinized seminiferous tubuli with nodular and diffuse Leydig cell hyperplasia, containing estrogen immunoreactive substance. These clinical and histological features seemed to be highly compatible with those of Kennedy-Alter-Sung type bulbo-spinal muscular atrophy. The involvement of sensory peripheral nerves, however, was a distinct feature of this family.

Aged↗

PubMedDate not supplied

Myopathy with abnormal distribution of dystrophin, growth retardation, mental retardation, and hypospadia.

A 9-year-old boy with severe growth retardation, mild mental retardation, and hypospadia had a high serum CK level without muscle weakness and atrophy. Muscle biopsy revealed a moderate variation in fiber size with a few necrotic and scattered regenerating fibers. Although muscle membranes were clearly stained by immunostaining with antibody to dystrophin, N-terminal region (2-5E2), fibers in groups revealed striking, intense staining with the other antibody, C-terminal region (4C5), suggesting some aberration of the dystrophin gene near the C-terminal area. His unique clinical features, as well as myopathy, are reported, although further study is necessary to clarify the relationship between the anomalous conditions and dystrophin abnormalities.

Adrenal Insufficiency↗

PubMed2006

Novel beta subunit mutation causes a slow-channel syndrome by enhancing activation and decreasing the rate of agonist dissociation.

We traced the cause of a slow-channel syndrome (SCS) in a patient with progressive muscle weakness, repetitive compound muscle action potential and prolonged low amplitude synaptic currents to a V --> F substitution in the M1 domain of the beta subunit (betaV229F) of the muscle acetylcholine receptor (AChR). In vitro expression studies in Xenopus oocytes indicated that the novel mutation betaV229F expressed normal amounts of AChRs and decreased the ACh EC50 by 10-fold compared to wild type. Kinetic analysis indicated that the mutation displayed prolonged mean open duration and repeated openings during activation. Prolonged openings caused by the betaV229F mutation were due to a reduction in the channel closing rate and an increase in the effective channel opening rate. Repeated openings of the channel during activation were caused by a significant reduction in the agonist dissociation constant. In addition, the betaV229F mutation produced an increase in calcium permeability. The kinetic and permeation studies presented in this work are sufficient to explain the consequences of the betaV229F mutation on the miniature endplate currents and thus are direct evidence that the betaV229F mutation is responsible for compromising the safety margin of neuromuscular transmission in the patient.

Acetylcholine↗

PubMed2004

Follow-up of nemaline myopathy in two patients with novel mutations in the skeletal muscle alpha-actin gene (ACTA1).

Nemaline myopathy has been associated with mutations in five different genes, which all encode protein components of the sarcomeric thin filaments. We report follow-up studies in two children with mutations not previously described in skeletal muscle alpha-actin (ACTA1). Case 1 was a male patient who after birth suffered from pronounced muscle weakness and hypotonia. Muscle biopsy showed small fibers with numerous rods. He failed to achieve any motor milestones. At the age of 17 he required 24 h ventilator support. He could not lift his arms against gravity, but he could use his hands to control his electric wheelchair. The muscle biopsy showed marked replacement of muscle tissue by fat and connective tissue. Only few fibers showed nemaline rods. He had a de novo, heterozygous mutation, G268D in ACTA1. Case 2 was a female patient with feeding difficulties and mild hypotonia in the neonatal period. Muscle biopsy showed hypoplastic muscle fibers and numerous rods. At 11 years of age she walked and moved unhindered and could run fairly well. She had a de novo, heterozygous mutation, K373E, in ACTA1. These two patients illustrate the marked variability in the clinical features of nemaline myopathy in spite of similar muscle pathology in early childhood. The severe muscle atrophy with replacement of fat and connective tissue in case 1 demonstrates the progressive nature of nemaline myopathy in some cases. The described two mutations add to the previously reported mutations in ACTA1 associated with nemaline myopathy.

Actins↗

PubMed1983

Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification.

The medical records of 47 children with dermatomyositis who were seen in the pediatric rheumatology clinic at the University of Michigan between 1964 and 1982 were reviewed. Although most children with dermatomyositis have a good prognosis, the best predictor of both good functional recovery and minimal calcinosis is early treatment after the onset of symptoms, using high doses of prednisone for an adequate length of time. Of the children given such treatment, 78% had good functional outcomes, and disabling calcinosis was seen in 20% or less. Children given treatment late in the course of disease and with low doses of steroids are more likely to be functionally limited and have a greater amount of dystrophic calcium salt deposition. In our study, only 33% of patients given such treatment had a mild disease course with good functional outcome. We have identified a subgroup of children with dermatomyositis who appear to do poorly despite optimal therapeutic regimens. These patients are distinguished by a severe disease course responding minimally to corticosteroid therapy and manifested by persistent muscle weakness, elevations of muscle enzyme activity, and severe generalized cutaneous vasculitis. These children are at high risk for the development of exoskeleton-like calcification; consideration should be given to combined immunosuppressive therapy early in the course of disease.

Adolescent↗

PubMed1984

Polymyositis associated with monoclonal gammopathy.

Three patients presented with gradual onset of proximal muscle weakness. Electromyography and muscle biopsy were abnormal in each patient and typical of myositis. All three had an IgG kappa monoclonal paraprotein in the serum. Examination of muscle biopsy specimens by direct immunofluorescence revealed linear deposits of IgG kappa along the sarcolemmal basement membrane of individual fibres. Stains for lambda light chains, other immunoglobulins, and complement components were negative. Plasmapheresis and immunosuppressive drugs produced clinical improvement in all three patients. Muscle biopsies were taken from two of the patients after plasma exchange and drug therapy and showed no immune deposits. These observations suggest a possible cause-and-effect relation between the serum paraprotein and the inflammatory myopathy in these patients.

Aged↗

PubMed1997

Neonatal paralysis of the rat soleus muscle selectively affects motoneurones from more caudal segments of the spinal cord.

Transient paralysis of the rat soleus muscle shortly after birth leads to permanent muscle weakness, loss of muscle fibres and the death of motoneurones. The soleus muscle receives its innervation from motoneurones whose axons exit the spinal cord either via the L4 ventral ramus or in the more caudal part of the cord via the L5 ventral ramus. Whether both populations of motoneurones are equally affected by neonatal paralysis was studied here. In soleus muscles paralysed with alpha-bungarotoxin shortly after birth and examined 8-10 weeks later, there is no loss of force or muscle fibres in the part of the muscle supplied by axons in the L4 ventral ramus. Loss of force and muscle fibre numbers occurs only in the part of the muscle supplied by axons in the L5 ventral ramus. In a normal adult soleus 30.3 +/- 2.4% of muscle force is produced by stimulating the L4 ventral ramus and 69.0 +/- 5.5% by stimulating the L5 ventral ramus. In soleus muscles treated with alpha-bungarotoxin 28 +/- 1.4% of the force produced by the contralateral control soleus was generated by axons in the L4 ventral ramus and only 20.3 +/- 5.6% by stimulating the L5 spinal nerve. The number of muscle fibres supplied by either ventral ramus in control and experimental muscles confirmed that the decrease of force after treatment with alpha-bungarotoxin can be accounted for by loss of muscle fibres supplied by axons in the L5 ventral ramus. The reduced force production and muscle fibre numbers was due to a selective loss of motoneurones that had their axons in the L5 ventral ramus. The number of axons to soleus in the L4 ventral ramus was 9.3 +/- 0.7 in controls and 10.3 +/- 0.9 in the experimental animals, whereas the L5 ventral ramus contained 17.2 +/- 0.7 in controls and only 4.7 +/- 1.7 in the experimental animals. Thus paralysis of the soleus muscle at birth selectively affects motoneurones in the more caudal part of the spinal cord, suggesting that the more cranial motoneurones are more mature and less likely to be influenced by lack of neuromuscular interaction at the time of birth.

Acute Disease↗

PubMed1980

Myasthenia gravis.

Myasthenia gravis is characterized by muscle weakness, which is alleviated by rest and by anticholinesterase drugs. There are two forms of the disease in the dog, acquired and congenital. The acquired form occurs either in young adults, or in older animals that have developed mediastinal tumors. Clinically, there is weakness of the muscles of the limbs, neck, and head, together with dilatation of the esophagus. In some cases, circulating antibody to acetylcholine receptor is present and the amount of receptor in the end-plates is decreased. It is thought to be an autoimmune disease. The congenital form of canine myasthenia gravis occurs most frequently in Jack Russell terriers from six to eight weeks of age. The clinical manifestations are similar to those accompanying acquired myasthenia, although dilatation of the esophagus is not a feature. Raised antibody levels to acetylcholine receptor have not been noted, although the amount of receptor in the end-plates is decreased. Thus, the congenital form does not appear to be an autoimmune disease and the pathophysiology has yet to be established. Both forms of canine myasthenia gravis provide useful models analogous to the disease in man.

Animals↗

PubMed1999

Stabilizing neuromuscular contacts reduces motoneuron death caused by paralysis of muscles in neonatal rats.

Transient paralysis of the soleus muscle in neonatal rats leads to permanent muscle weakness, loss of muscle fibres and motoneuron death. Application of leupeptin, an inhibitor of a calcium-activated neutral protease, to the neuromuscular junction is known to enhance the maintenance of neuromuscular contacts during development and axonal sprouting. Here, we show that treatment of soleus muscles with leupeptin as they recover from a period of paralysis rescues motoneurons that would otherwise die. The number of motoneurons to the soleus muscle was established by retrograde labelling with horseradish peroxidase eight to 10 weeks after recovery from paralysis. There were only 38.4 (+/-2.8 S.E.M., n=5) motoneurons innervating the soleus muscle that had been paralysed with alpha-bungarotoxin, compared to 58.2 (+/-3.1 S.E.M., n=5) to the control untreated soleus. Thus, the number of motoneurons to the soleus muscle on the alpha-bungarotoxin-treated side was 66.9% (+/-6.2% S.E.M., n=5) of the control side. In those animals where paralysis of the soleus muscle was followed three days later by treatment with leupeptin, the number of labelled motoneurons on the treated side of the spinal cord was 61.5 (+/-4.6 S.E.M., n=4) and that on the contralateral untreated control side was 59 (+/-3.8 S.E.M., n=4). This improvement in motoneuron survival in the leupeptin-treated animals is also confirmed by counts of the number of motor units in the soleus muscle obtained by recording muscle tension. In animals that had their soleus muscles paralysed at birth, only 21 (+/-0.7 S.E.M., n=5) motor units were present, compared to 30 motor units in control muscles. When the paralysed soleus muscle was subsequently treated with leupeptin, the number of remaining motor units in the muscle was 29.8 (+/- 1.0 S.E.M., n=5). In addition, the force output of the soleus muscles that had undergone a period of neonatal paralysis was calculated for both the NaCl- and leupeptin-treated animals. The results showed that paralysis at birth results in a reduction in weight and force output of the soleus muscle, which is not improved following treatment with leupeptin. This study shows that application of leupeptin to the soleus muscle after alpha-bungarotoxin-induced paralysis rescues motoneurons to the soleus that would otherwise die. This effect is most likely due to stabilization of their neuromuscularjunctions.

Animals↗

PubMed1996

Comprehensive gait analysis in posterior-stabilized knee arthroplasty.

Sixteen patients implanted with a posterior-stabilized prosthesis (Insall-Burstein PS II, Zimmer, Warsaw, IN) and 32 age-matched control subjects were evaluated by isokinetic muscle testing and comprehensive gait analysis at a mean 46 months following the index arthroplasty. The contralateral knee was normal in 13 patients and an asymptomatic total knee arthroplasty in 3 patients. No significant differences (P > .05) were found between the study and control groups in spatiotemporal gait parameters nor were there isokinetic strength deficits. A prolonged firing of the vastus medialis was observed in some patients. Knee range of motion during level walking and stair descent was decreased significantly (P < .05) in the study group. There was no significant difference for knee range of motion between the study and control groups during stair ascent. Spatiotemporal gait parameters in the total knee arthroplasty patients show no significant differences from the control subject at nearly 4 years after surgery. However, other gait abnormalities are present and cannot be accounted for on the basis of muscle weakness, abnormal phasic muscle activity, or inadequate knee range of motion. With reference to historic control subjects, the persistent gait abnormalities of patients implanted with posterior-stabilized prostheses (posterior cruciate substituting) are comparable to those of patients implanted with cruciate-retaining prostheses and superior to cruciate-sacrificing prostheses.

Adult↗

PubMed2002

Congenital muscular dystrophy with adducted thumbs, ptosis, external ophthalmoplegia, mental retardation and cerebellar hypoplasia: a novel form of CMD.

At least six different forms of congenital muscular dystrophy are associated with structural changes of the central nervous system, and three of these have been mapped: merosin-deficient congenital muscular dystrophy on chromosome 6q2, Fukuyama congenital muscular dystrophy on chromosome 9q31, and muscle eye brain disease on chromosome 1p32. Walker-Warburg syndrome, congenital muscular dystrophy with calf hypertrophy, pontocerebellar hypoplasia, and normal eyes, and congenital muscular dystrophy with severe mental retardation and cerebellar cysts are nosologically distinct and have been excluded from the known congenital muscular dystrophy loci with structural changes of the central nervous system. Here, we describe a novel congenital muscular dystrophy syndrome which is phenotypically distinct from the recognized forms of congenital muscular dystrophy with brain involvement. Two siblings, a boy and a girl, were born to consanguineous parents from Sicily. Both children were born with adducted thumbs and toe contractures. They were floppy from birth, walked late, showed profound generalized muscle weakness including facial muscles, elevated creatine kinase levels of 200-700U/l, and histological changes compatible with muscular dystrophy. In addition, both showed ptosis, external ophthalmoplegia, mild mental retardation, and mild cerebellar hypoplasia on MRI. Immunocytochemistry showed normal expression of muscle membrane proteins including laminin alpha 2, laminin beta 2, and alpha-dystroglycan. Linkage analysis excluded the candidate loci on chromosomes 6q2, 9q31, and 1q32. The gene locus for congenital muscular dystrophy 1B, MDC 1B, on chromosome 1q42 was also excluded. Adducted thumbs are a distinct clinical sign that has not been reported in congenital muscular dystrophy before and should facilitate recognition of further patients with this disorder.

Blepharoptosis↗