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PubMed1986

Emery-Dreifuss syndrome.

A young adult male is described with muscular dystrophy of probable X-linked recessive inheritance. An onset of muscle weakness in late adolescence was preceded by contractures of the neck and elbows dating back to childhood. The distribution of muscle weakness was proximal in the upper limbs and both proximal and distal in the lower. The mixed pattern of muscle involvement in the legs favours the view that cases of Emery-Dreifuss muscular dystrophy with proximal weakness in both the upper and lower limbs and X-linked scapuloperoneal muscular dystrophy represent the same disorder. A muscle biopsy in the present case showed unique appearances.

Adult↗

PubMed2001

Does bone mass equate with bone health? An argument for the negative.

This article argues that diagnosing osteopenias and osteoporoses by bone mass Z-scores, and viewing disorders detected in that way as diseases, fostered a belief that those scores evaluate whole-bone strength and bone health equally well (here, mass has its meaning in absorptiometry, not in physics). However, load-bearing bones normally adapt their strength to the past and present voluntary loads on them in ways that make strong muscles make strong bones, and make persistently weak muscles make weak bones. The resulting adaptations also make bones strong enough to keep voluntary mechanical usage from fracturing them. Such observations suggest a new criterion for a bone's health that would depend on the relationship among its strength, the size of the voluntary loads it carries, and any spontaneous fractures caused by those loads. Such a relationship could indicate a bone"s health independently of its mass. For example, mouse and horse femurs differ in strength more than 1000-fold, but they could be equally healthy if they were to satisfy that criterion in the animals from which they come. That idea would distinguish abnormal in a strictly statistical sense (which T- and Z-scores would evaluate) from unhealthy in the sense of both abnormal and diseased (which the above relationship would evaluate). Thus, a bone with a negative Z-score could still be healthy if voluntary loads do not fracture it. These ideas and some of their implications question some long-held views. This article presents the ideas, evidence they stand on, and some of their implications.

Absorptiometry, Photon↗

PubMed2000

Strength and physiological response to exercise in patients with chronic fatigue syndrome.

OBJECTIVE: To measure strength, aerobic exercise capacity and efficiency, and functional incapacity in patients with chronic fatigue syndrome (CFS) who do not have a current psychiatric disorder. METHODS: Sixty six patients with CFS without a current psychiatric disorder, 30 healthy but sedentary controls, and 15 patients with a current major depressive disorder were recruited into the study. Exercise capacity and efficiency were assessed by monitoring peak and submaximal oxygen uptake, heart rate, blood lactate, duration of exercise, and perceived exertion during a treadmill walking test. Strength was measured using twitch interpolated voluntary isometric quadriceps contractions. Symptomatic measures included physical and mental fatigue, mood, sleep, somatic amplification, and functional incapacity. RESULTS: Compared with sedentary controls, patients with CFS were physically weaker, had a significantly reduced exercise capacity, and perceived greater effort during exercise, but were equally unfit. Compared with depressed controls, patients with CFS had significantly higher submaximal oxygen uptakes during exercise, were weaker, and perceived greater physical fatigue and incapacity. Multiple regression models suggested that exercise incapacity in CFS was related to quadriceps muscle weakness, increased cardiovascular response to exercise, and body mass index. The best model of the increased exercise capacity found after graded exercise therapy consisted of a reduction in submaximal heart rate response to exercise. CONCLUSIONS: Patients with CFS were weaker than sedentary and depressed controls and as unfit as sedentary controls. Low exercise capacity in patients with CFS was related to quadriceps muscle weakness, low physical fitness, and a high body mass ratio. Improved physical fitness after treatment was associated with increased exercise capacity. These data imply that physical deconditioning helps to maintain physical disability in CFS and that a treatment designed to reverse deconditioning helps to improve physical function.

Adult↗

PubMed2003

Infantile spinal muscular atrophy with respiratory distress type 1 (SMARD1).

Autosomal recessive spinal muscular atrophy with respiratory distress type 1 (SMARD1) is the second anterior horn cell disease in infants in which the genetic defect has been defined. SMARD1 results from mutations in the gene encoding the immunoglobulin micro-binding protein 2 (IGHMBP2) on chromosome 11q13. Our aim was to review the clinical features of 29 infants affected with SMARD1 and report on 26 novel IGHMBP2 mutations. Intrauterine growth retardation, weak cry, and foot deformities were the earliest symptoms of SMARD1. Most patients presented at the age of 1 to 6 months with respiratory distress due to diaphragmatic paralysis and progressive muscle weakness with predominantly distal lower limb muscle involvement. Sensory and autonomic nerves are also affected. Because of the poor prognosis, there is a demand for prenatal diagnosis, and clear diagnostic criteria for infantile SMARD1 are needed. The diagnosis of SMARD1 should be considered in infants with non-5q spinal muscular atrophy, neuropathy, and muscle weakness and/or respiratory distress of unclear cause. Furthermore, consanguineous parents of a child with sudden infant death syndrome should be examined for IGHMBP2 mutations.

Carrier Proteins↗

PubMed2000

Quantification of upper motor neuron loss in amyotrophic lateral sclerosis.

OBJECTIVE: To quantitatively estimate upper motor neuron (UMN) loss in ALS. METHODS: We used the recently developed triple stimulation technique (TST) to study corticospinal conduction to 86 abductor digiti minimi muscles of 48 ALS patients. This method employs a collision technique to estimate the proportion of motor units activated by a transcranial magnetic stimulus. At the same time, it yields an estimate of lower motor neuron (LMN) integrity. RESULTS: The TST disclosed and quantified central conduction failures attributable to UMN loss in 38 sides of 24 patients (subclinical in 15 sides), whereas conventional motor evoked potentials detected abnormalities in only 18 sides of 12 patients (subclinical in two sides). The increased sensitivity of the TST to detect UMN dysfunction was particularly observed in early cases. Increased central motor conduction times (CMCT) occurred exclusively in sides with conduction failure. In sides with clinical UMN syndromes, the TST response size (but not the CMCT) correlated with the muscle weakness. In sides with clinical LMN syndromes, the size of the peripherally evoked compound muscle action potentials correlated with the muscle weakness. CONCLUSION: The TST is a sensitive method to detect UMN dysfunction in ALS. It allows a quantitative estimate of the UMN loss, which is related to the functional deficit. Therefore, the TST has a considerable impact on diagnostic certainty in many patients. It will be suited to follow the disease progression and therapeutic trials.

Action Potentials↗

PubMed2004

Hepatocellular carcinoma,polymyositis,rhabdomyolysis,and acute renal failure.

A 55 yr-old man presented with progressive muscle weakness and oliguria for 5 days. Laboratory findings suggested rhabdomyolysis complicated with acute renal failure. A diagnosis of polymyositis was based upon the proximal muscle weakness on both upper and lower limbs, elevated muscle enzyme levels, muscle biopsy findings and the needle electromyography findings. The muscle biopsy showed extensive muscle necrosis and calcification. Investigations for underlying malignancy demonstrated hepatocellular carcinoma. The patient was managed with hemodialysis and high dose prednisolone. His renal function was fully recovered and his muscle power did improve slightly, but he died of a rupture of the hepatic tumor. In our view, this is an interesting case in that the hepatocellular carcinoma was associated with polymyositis and fulminant rhabdomyolysis-induced acute renal failure requiring hemodialysis.

Acute Kidney Injury↗

PubMed1990

[A case of suspected allergic granulomatosis and angiitis with a rapid clinical course of paraplegia].

A 68-year-old man with suspected allergic granulomatosis and angiitis is reported. He had received 10 mg of prednisolone daily since July 1988 for asthma. He abruptly developed muscle weakness of the lower extremities, followed two days later by paraplegia. Six days after the onset of the muscle weakness, he was hospitalized. He showed disturbance of recent memory, disorientation, neck rigidity, paraplegia, mild muscle fasciculation and hypesthesia. He also showed paralytic ileus. Laboratory findings showed leukocytosis (24580/mm3), eosinophilia (56% of the peripheral white blood cells and 19% of the cells in the cerebrospinal fluid), on erythrocyte sedimentation rate of 31 mm/h, and the IgE level of 1200 IU/ml. The ECG showed loss of the r-wave in V1 and V2. A granulomatous lesion anterior to the spinal cord was found on myelography and MRI. Prednisolone was given at a dose of 60 mg daily resulting in improvement of the clinical symptoms and eosinophilia. There was disappearance of the granuloma on MRI performed after prednisolone therapy. Despite the severe manifestation of allergic granulomatosis and angiitis, prednisolone therapy had a marked effect in this patient. The granulomatous lesion anterior to the spinal cord shown by MRI suggested an eosinophilic granuloma, and may have been the etiology of some of the neurological symptoms.

Aged↗

PubMed1998

Juvenile dermatomyositis at diagnosis: clinical characteristics of 79 children.

OBJECTIVE: To evaluate demographic and clinical characteristics, duration of time between disease onset (date of first rash and/or weakness), and diagnosis/therapy, as well as socioeconomic status, of children with newly diagnosed juvenile dermatomyositis (JDM). METHODS: Structured telephone interview of families of a cohort of 79 children with JDM: interval between onset of symptoms to diagnosis, median of 3 months (range 0.5-20.0). RESULTS: At diagnosis, all the children had rash (100%) and proximal muscle weakness (100%); 58 (73%) had muscle pain; 51 (65%) fever; 35 (44%) dysphagia; 34 (43%) hoarseness; 29 (37%) abdominal pain; 28 (35%) arthritis; 18 (23%) calcinosis, and 10 (13%) melena. Muscle derived enzymes were normal in 10% of the children. Of the 43 children who had an electromyogram (EMG), 8 (19%) had normal results. Fifty-one children had a muscle biopsy; the results were normal/nondiagnostic in 10 (20%). Median time from disease onset to diagnosis was different between racial groups: Caucasians (n=59) 2.0 months: for minorities (n=20), 6.5 months, (p=0.0008). The median time from disease onset to therapy was: Caucasians. 3.0 months; minorities, 7.2 months (p=0.002). Report of calcinosis was associated with increased time to diagnosis and therapy (p=0.04). In the 33 children whose first symptom occurred in June-September, rash preceded or accompanied onset of muscle weakness in 83% (n=27). Ninety-one percent of the children were given steroid therapy and 9% received methotrexate as well. CONCLUSION: The results of an undirected site for muscle biopsy or EMG may not be diagnostic. Minority children had a longer interval between first JDM symptom and diagnosis/therapy than Caucasian children. Delay in diagnosis/therapy was associated with calcinosis.

Adolescent↗

PubMed2002

Primary carnitine deficiency in a male adult.

The case is described of a 36 year-old man who presented with progressive proximal muscle weakness and weight loss. His serum creatine phosphokinase (CPK) levels were markedly elevated. The muscle biopsy showed lipid storage myopathy. The muscle carnitine concentration was extremely low (5.6% of normal levels), establishing the diagnosis of myopathic carnitine deficiency. The disorder was considered as primary because there were no indications of any other identifiable condition which could result in a secondary carnitine deficiency. The patient was treated with oral L-carnitine (2 g per day) and showed rapid improvement. Primary myopathic carnitine deficiency is a curable disorder and therefore it should always be considered as a potential diagnosis in cases of myopathy in young adults.

Adult↗

PubMed2000

[The heartache of muscular dystrophy].

Duchenne and Becker muscular dystrophy are caused by a mutation in the dystrophin gene, located on the short arm of the X chromosome. Three so called dystrophinopathy patients, a women aged 54 and two men aged 23 and 21 years, suffered from a severe dilated cardiomyopathy. Such a cardiomyopathy can develop in both carriers and patients. In addition, it is often more important for prognosis than muscle weakness. For these two reasons it is important to screen both groups for (early) cardiological abnormalities. If these are present, regular follow-up is necessary to start timely therapy. When cardiological investigations yield normal results, it is advised to screen carriers with a five-year interval. Dystrophinopathy patients should be checked every year, because the cardiomyopathy sometimes develops and deteriorates over a short period of time. Patients with dilated cardiomyopathy and with a positive family history for dilated cardiomyopathy, muscle weakness or high serum creatine kinase activity should be screened for a mutation in the dystrophin gene.

Adult↗

PubMed1976

Clinical observations on thyrotoxicosis coexisting with myotonic dystrophy.

Two cases are presented and a third is cited from the literature in which the onset of hyperthyroidism exacerbated acute weakness in previously undiagnosed myotonic dystrophy. In two of the cases a return to the euthyroid state after treatment was associated with marked clinical improvement in muscle weakness. In the third case, severe bulbar and respiratory muscle weakness, which was apparently aggravated by the thyrotoxicosis, was followed by aspiration pneumonitis and death. Possible mechanisms for the effect of excessive thyroxine on muscle function are reviewed. Early diagnosis and aggressive treatment of the hyperthyroidism is extremely important.

Adult↗

PubMed2005

Na(+), K(+)-ATPase content in skeletal muscle of dogs with pituitary-dependent hyperadrenocorticism.

Several hormones regulate Na(+), K(+)-ATPase content in the muscle cell membrane, which is essential for maintaining muscle cell excitability. Chronic glucocorticoid excess is associated with muscle weakness and reduced endurance. We hypothesized that chronic glucocorticoid excess affects Na(+), K(+)-ATPase content in canine skeletal muscle, and contributes to reduced endurance and muscle weakness associated with pituitary-dependent hyperadrenocorticism (PDH) in dogs. Therefore, Na(+), K(+)-ATPase content in skeletal muscle was evaluated before and after hypophysectomy and hormone replacement (cortisone and l-thyroxin) in dogs with PDH (n=13), and in healthy controls (n=6). In addition, baseline and exercise-induced changes in plasma electrolyte concentrations and acid-base balance were evaluated before and after hypophysectomy in dogs with PDH. Na(+), K(+)-ATPase content of gluteal muscle in dogs with PDH was significantly lower than in control dogs (201+/-13pmol/g versus 260+/-8pmol/g wet weight; P<0.01). Similar differences were found in palatine muscle. After hypophysectomy and on hormone replacement, Na(+), K(+)-ATPase was increased (234+/-7pmol/g wet weight). Both plasma pH and base excess in dogs with PDH (7.44+/-0.01; 1.7+/-0.6mmol/l, respectively) were significantly higher (P<0.05) than after hypophysectomy and hormone replacement (7.41+/-0.01; -0.2+/-0.4mmol/l, respectively). Exercise induced respiratory alkalosis, but did not result in hyperkalemia in dogs with PDH. In conclusion, chronic glucocorticoid excess in dogs with PDH is associated with decreased Na(+), K(+)-ATPase content in skeletal muscle. This may contribute to reduce endurance in canine PDH, although dogs with PDH did not exhibit exercise-induced hyperkalemia. Na(+), K(+)-ATPase content normalized to values statistically not different from healthy controls after hypophysectomy and hormone replacement.

Adrenocortical Hyperfunction↗

PubMed1983

Fetal muscle characteristics in nemaline myopathy.

In addition to the intracytoplasmic rods in approximately 1/4 muscle fibers, there were a large number of fibers with fetal muscle characteristics in a female infant who had severe muscle weakness and hypotonia, and failure to thrive since birth. A histochemical examination disclosed abnormal distribution in muscle fiber types including remarkable type 1 fiber predominance and increased number of type 2 C fibers (11.4%). Scattered throughout were fibers consisting of multiple myocytes enclosed in a single basement membrane, and small-calibered fibers containing abundant intermediate (skeleton) filaments and dispersed microtubules. Both were histologically identical to premature fibers found in the fetal muscle. The existence of an increased number of satellite cells as compared with age-matched controls was also suggestive of delayed or arrested muscle fiber maturation. A certain impaired neural influence upon the developing muscle is probably responsible for producing abnormal fiber type distribution and immature small-calibered fibers which account for small muscle bulk and muscle weakness in nemaline myopathy.

Asphyxia Neonatorum↗

PubMed2002

Osteoporosis with underlying connective tissue disease: an unusual case.

A 44-year-old male was initially seen by dermatologists, who noted an erythematous rash on sun-exposed areas, the back, shoulders, and upper arms. There was associated muscle weakness and significant weight loss. Investigation revealed mildly raised aspartate and alanine transaminases but normal creatine kinase. Inflammatory indices and antinuclear antibodies (ANAs) were normal. Biopsy of the rash was reported as consistent with either dermatomyositis (DM) or acute lupus erythematosus. A diagnosis of DM was made, and prednisolone was given with improvement of the rash but deteriorating myopathy. The patient was referred to the rheumatology department, and further history revealed multiple vertebral fractures after falling from standing height; these had occurred six months prior to starting steroids. Besides smoking he had no other risk factors for osteoporosis. Examination showed normal muscle strength, no muscle tenderness, and no joint abnormality. Repeat muscle enzymes were normal, and ANAs were now 1 : 100, but dsDNA antibodies and extractable nuclear antigens were normal. Investigations for osteoporosis revealed a hypergonadotrophic hypogonadism picture. Further examination indicated scanty pubic and auxiliary hair, small testicles, and mild gynecomastia. He is married, though has no children of his own. The hormonal profile raised the possibility of Klinefelter's syndrome, which was subsequently confirmed with karyotyping of 47 XXY. Hypogonadism has been established as a cause of osteoporosis in males, and in this case would explain the occurrence of fractures in the absence of other major risk factors. Systemic lupus erythematosus has been recognized in association with Klinefelter's syndrome; in view of the normal muscle enzymes, his rash is most likely due to acute discoid lupus with androgen deficiency causing muscle weakness.

2-Pyridinylmethylsulfinylbenzimidazoles↗

PubMed2000

[A case of LGMD2A identified with both western blot analysis and immunostaining of calpain 3 in biopsied muscle].

A 45-year-old housewife had proximal dominant limb muscle weakness from around 25 years of age. Her parents were cousins. None of family members was affected. Progressive muscle weakness and atrophy were prominent at the posterior compartments of legs and trunk. Serum CK was moderately elevated. Muscle pathology revealed variation in fiber size, moderate increase in numbers of internal nuclei and abundant lobulated fibers. On immunostaining using by monoclonal antibody against human calpain 3 (NCL-CALP-2 C4; Novocastra) to the biopsied muscle, calpain 3 was completely absent in the sarcoplasm, while granular debris and in part positive striation were noted in control muscle. By Western blot analysis, a band corresponding to 94 kDa of calpain 3 was not detected. A genetic analysis of calpain 3 revealed homozygous C-565-G mutation (Leu189Val). From the present study. Western blot analysis and immunostaining by using calpain 3 antibody were suggested to be useful to diagnose LGMD2A in LGMD patients.

Biomarkers↗

PubMedDate not supplied

Analysis of 31P MR spectroscopy data using artificial neural networks for longitudinal evaluation of muscle diseases: dermatomyositis.

Classical myopathic dermatomyositis (DM) is a chronic autoimmune disease characterized by an erythematous rash and severe, proximal muscle weakness. A disease variant, amyopathic DM, presents with the typical rash but without clinical evidence of muscle weakness. Prednisone and immunosuppressive drugs alleviate symptoms in many patients. Accurate longitudinal evaluations of patients are important to limit serious side effects of these drugs, including osteoporosis, cataracts, and growth inhibition. Metabolic abnormalities detected with 31P magnetic resonance spectroscopy (MRS) provide the best quantitative data for evaluating these patients. With 31P MRS, the levels of inorganic phosphate (Pi), phosphocreatine (PCr), ATP, and phosphodiesters (PDE) were determined in the quadricep muscles of patients during rest and exercise. Artificial neural network (ANN) analyses of these data were previously used for accurate classification of patients with myopathic or amyopathic DM and normal controls. In the present investigation, an artificial neural network was employed for further analysis of the 31P metabolite levels in quantitative, longitudinal evaluations of the extent (percent) of clinical improvement or deterioration during treatment with prednisone and immunosuppressive drugs. The ANN results showed that adult patients in a severe myopathic state could improve with treatment to a clinical status of amyopathic DM. In contrast, severely weak juvenile patients in the myopathic state recovered to normal status. One juvenile patient did not improve and remained in the myopathic state. Additionally, a serious clinical relapse in an amyopathic patient was predicted with serial ANN analyses well in advance of the actual clinical event. These network analyses show potential utility for clinical applications in muscle diseases.

Adolescent↗

PubMed1999

Distinguishing clinical and electrodiagnostic features of X-linked bulbospinal neuronopathy.

X-linked bulbospinal neuronopathy (XLBSN) or Kennedys disease is a rare inherited neuromuscular disease characterized by adult-onset muscle weakness, usually in a limb-girdle distribution. It is frequently misdiagnosed despite a distinctive clinical presentation, usually due to the absence of a clear family history, and perhaps also due to failure of recognition. Accurate diagnosis is crucial for genetic counseling purposes and because alternative diagnoses usually carry a poorer prognosis. We evaluated 4 patients with XLBSN and one symptomatic female heterozygote patient. Based on our clinical observations in these patients and a systematic review of previously reported cases, the following clinical and electrophysiologic features when present in the setting of adult-onset muscle weakness, are strongly suggestive of the disorder: 1) facial weakness, 2) facial twitching or fasciculations, 3) tongue weakness and atrophy, 4) postural hand tremor, 5) hypo- or areflexia, and 6) absent or low-amplitude sensory nerve action potentials despite clinically normal sensation. We also hypothesize regarding the possibility of partial expression of the abnormal XLBSN gene in a symptomatic heterozygote female patient.

Action Potentials↗

PubMed2004

Impairment and activity limitation associated with epiphyseal dysplasia in children.

OBJECTIVES: To develop a profile of impairment and activity limitation among children with epiphyseal dysplasia (ED) and to identify the relationship between these 2 domains. DESIGN: Cross-sectional study. SETTING: Acute, pediatric academic and health sciences center. PARTICIPANTS: Eleven subjects with multiple epiphyseal dysplasia (MED) and 17 subjects with spondyloepiphyseal dysplasia (SED), with a mean age of 12.9 years. INTERVENTIONS: Not applicable. Main outcome measures Anthropometric indexes of growth and nutrition, joint range of motion (ROM) and alignment, muscle strength, pain, and activity limitation. RESULTS: Subjects with SED had significantly shorter stature than the reference population (P<.01). Seventy-three percent of participants with MED and 77% of those with SED were above average or overweight for their height (P<.01). Both groups presented with moderate to severe joint ROM impairment, with greater lower-extremity involvement. Subjects with SED had worse outcomes with respect to overall ROM and alignment impairments (P<.01), particularly in their upper extremities (P<.01), than subjects with MED. Significant overall muscle weakness was noted in all subjects (z=-1.81; P<.01). Ninety-four percent of subjects with SED reported pain with activity, compared with 64% of those with MED (P=.04), although pain intensity did not differ between groups. Fifty percent of subjects had undergone orthopedic surgery. Mild activity limitation was reported by all subjects (mean score, 87.7+/-18.83). Significant correlations were identified between height for age and strength (r=.50) and pain and activity limitation (r=-.50). CONCLUSIONS: Despite moderate to severe impairments, all subjects reported surprisingly mild activity limitation. A positive correlation was identified between pain and activity limitation. This study also identified and described patterns of muscle weakness, pain, and nutritional concerns not previously reported in the ED literature.

Adolescent↗