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PubMedDate not supplied

Muscle weakness and congenital contractures in a case of congenital myasthenia.

A case of nonautoimmune, prenatal-onset, congenital myasthenia with congenital contractures including camptodactyly is presented. The clinical features re identical to those in three previously reported cases. In this form of congenital myasthenia, the muscle weakness and congenital contractures may resolve completely after conservative treatment, including anticholinesterase medication. Diagnosis can be made by the characteristic clinical history, neurological assessment, and electromyography with repetitive nerve stimulation.

Contracture↗
PubMed2004

Nerve root distribution of deltoid and biceps brachii muscle in cervical spondylotic myelopathy: a potential risk factor for postoperative shoulder muscle weakness after posterior decompression.

To investigate the nerve root distribution of deltoid and biceps brachii muscle, compound muscle action potentials (CMAPs) were recorded intraoperatively following nerve root stimulation in cervical spondylotic myelopathy. A total of 19 upper limbs in 12 patients aged 55-72 years (mean, 65.5 years) with cervical spondylotic myelopathy were examined. CMAPs were recorded from deltoid and biceps brachii muscle following C5 and C6 root stimulation. Although both C5 and C6 roots were innervated for deltoid and biceps brachii muscle in all subjects, the amplitude ratio of CMAPs (C5/C6) differed individually depending on the symptomatic intervertebral levels of the spinal cord. The C5 root predominantly innervated both deltoid and biceps brachii in patients with symptomatic cord lesions at the C4-C5 intervertebral level compared to patients with symptomatic cord lesions at the C5-C6 intervertebral level. Although no patients sustained postoperative radiculopathy in our study, severe weakness and unfavorable recovery are expected when the C5 root in patients with C4-C5 myelopathy is damaged. From the electrophysiological aspect, C4-C5 cord lesions are likely to be a potential risk factor for postoperative shoulder muscle weakness in patients with compressive cervical myelopathy.

Action Potentials↗
PubMed2005

Hip muscle weakness and overuse injuries in recreational runners.

OBJECTIVE: To test for differences in strength of 6 muscle groups of the hip on the involved leg in recreational runners with injuries compared with the uninvolved leg and a control group of noninjured runners. DESIGN: Descriptive analysis. SETTING: : Three outpatient physical therapy clinics in the Minneapolis/St. Paul metropolitan area. PARTICIPANTS: Thirty recreational runners (17 female, 13 male) experiencing a single leg overuse injury that presented for treatment between June and September 2002. Thirty noninjured runners (16 female, 14 male) randomly selected from a pool of 46 volunteers from a distance running club served as controls. MAIN OUTCOME MEASURES: Self-report demographic information on running habits, leg dominance demonstrated by preferred kicking leg, and injury information. Muscle strength of the 6 major muscle groups of the hip was recorded using a hand-held dynamometer. The highest value of 2 trials was used, and strength values were normalized to body mass(2/3). RESULTS: Results comparing the injured and noninjured groups showed that leg dominance did not influence the leg of injury (chi(2)(1) = 0.134; P = 0.71). Correlations for internal reliability of muscle measurements between trials 1 and 2 with the hand-held dynamometer ranged from 0.80 to 0.90 for the 6 muscle groups measured, and all P values were less than 0.0001. No significant side-to-side differences in hip group muscle strength were found in the noninjured runners (P = 0.62-0.93). Among the injured runners, the injured side hip abductor (P = 0.0003) and flexor muscle groups (P = 0.026) were significantly weaker than the noninjured side. In addition, the injured side hip adductor muscle group was significantly stronger (P = 0.010) than the noninjured side. Duration of symptoms was not a contributing factor to the extent of injury as measured by muscle strength imbalance between injured and uninjured sides. CONCLUSIONS: Although no cause-and-effect relationship has been established, this is the first study to show an association between hip abductor, adductor, and flexor muscle group strength imbalance and lower extremity overuse injuries in runners. Because most running injuries are multifaceted in nature, areas secondary to the site of pain, such as hip muscle groups exhibiting strength imbalances, must also be considered to gain favorable outcomes for injured runners. The addition of strengthening exercises to specifically identified weak hip muscles may offer better treatment results in patients with running injuries.

Adolescent↗
PubMed1990

Permanent muscle weakness in familial hypokalaemic periodic paralysis. Clinical, radiological and pathological aspects.

Myopathy accompanying familial hypokalaemic periodic paralysis (HPP) is much less well documented than the paralytic attacks from which the disease derives its name. Eleven affected members of a large kinship with HPP were studied clinically and radiologically for the presence of myopathy. In 4 patients muscle biopsies were also performed and in 1 of them the histological findings obtained at autopsy were compared with the CT scans of various muscles. In another patient not previously biopsied, the specimens of both amputated legs were examined histologically. The age of the studied individuals ranged from 33 to 74 yrs. The 4 youngest patients showed no clinical signs of myopathy. However, in 2 of them CT scans demonstrated discrete hypodense lesions in the leg muscles, whereas in the other 2, muscle biopsies showed a vacuolar myopathy. The other 7 patients, all older than 50 yrs, presented both clinical and CT evidence of myopathy of proximal and distal muscles ranging from very mild to very severe, males being slightly more affected than females. In all 11 patients a mean CT grading was made that was based on the abnormalities found in the different muscle groups. The myopathy appeared to be unrelated to the history of paralytic attacks, but a strong correlation was found between age and mean CT grading. It was concluded that HPP is a myopathy with permanent muscle weakness of late onset in all the patients. The expression of the paralytic attacks is variable.

Adult↗
PubMed2001

[Muscle weakness or rigidity due to hereditary ion channel diseases].

Three men, aged 36, 16 and 66 years, had suffered for several years from muscular weakness; after a low serum potassium level had been established, supplementary examination revealed hypokalaemic periodic paralysis. A woman aged 25 had suffered since youth from muscular stiffness on sudden movements; she suffered from hereditary myotonia of the recessive type (Becker's disease). Both rare skeletal muscle ion channel diseases are characterized by the fact that the variable clinical expression complicates making the diagnosis. Since the causal mutations are known, genetic analysis is an essential step in confirming the diagnosis. Additional EMG procedures may be of diagnostic value, even in cases that cannot be clarified genetically.

Adolescent↗
PubMed2001

[Proximal muscle weakness, depressed tendon reflexes and autonomic dysfunction: the Lambert-Eaton myasthenic syndrome].

Three patients with Lambert-Eaton myasthenic syndrome (LEMS), two men aged 61 and 64 and a woman aged 55 years, all developed proximal weakness, depressed tendon reflexes and autonomic dysfunction. Although this clinical triad is highly suggestive for LEMS, the disorder had not been recognized initially. The woman had a small-cell bronchial carcinoma, treated successfully by chemotherapy, whereafter the LEMS symptoms gradually disappeared. The first man was treated with 3,4-diaminopyridine and azathioprine, whereupon his symptoms diminished. The other man had only slight complaints and refused drug treatment. The three cases illustrate that presentation and course of LEMS can vary between patients. Furthermore, clinical and electrophysiological features can suggest myasthenia gravis, myopathy or axonal polyneuropathy. Therapeutic options and the risk of underlying malignancy make early diagnosis important. In conclusion, in every patient presenting with unexplained proximal weakness, LEMS should be considered, especially if depressed tendon reflexes and autonomic dysfunction are found as well.

Autonomic Nervous System↗
PubMed2001

[Adult form of acid maltase deficiency presenting with pattern of muscle weakness resembling facioscapulohumeral dystrophy].

We report a 61-year-old female patient with adult form of acid maltase deficiency showing many clinical similarities to facioscapulohumeral muscular dystrophy (FSHD). She developed difficulty in raising her right arm in her thirties followed by leg weakness. She had the typical features of FSHD, including bilateral scapular winging sparing the levator scapulae and deltoid muscles, and Beevor's sign. Muscle involvement was asymmetrical. Facial muscles were not affected, while the neck flexor was weak. No muscle shortening or joint contracture was observed. On muscle CT, the lumbar paravertebral, gluteal and thigh muscles were replaced by adipose tissue, while the rectus femoris, gracilis, and sartorius muscles were spared. Serum creatine kinase level was not elevated. Muscle biopsy showed some vacuoles and many granular inclusions with high acid phosphatase activity. Acid maltase activity was very low in both muscle and cultured skin fibroblasts. Absence of shortening of affected muscles appears to be the characteristic finding suggesting metabolic myopathies with minimal fibrosis, rather than FSHD.

Diagnosis, Differential↗
PubMed1982

Muscle weakness following dynamic exercise in humans.

Electrical stimulation of the triceps surae in five healthy male subjects showed that following 1-2 h level running and uphill walking, at submaximal voltages of stimulation, exercise enhanced the twitch and tetanic responses, but the supramaximal time to peak tension (TPT), twitch (Pto) and tetanic tensions (Po) at 10 and 20 Hz were reduced by 16 ms (-12.6%), 11 (-8.9%), 163 (-17.5%), and 230 N (-18.1%), respectively. High-frequency (50 and 100 Hz) tetanic stimulation produced qualitatively similar changes to the 20-Hz response, but the stimulus response curve for the two frequencies was different and the ratio of 20- to 50-Hz response (20/50) (cf. Edwards et al., J. Physiol, London 272: 769-778, 1977) was voltage dependent. The reduction in Po at 100 Hz was associated with a decrease in maximal voluntary contraction (MVC). The effects of exercise on Pto and Po at 10, 20, 50, and 100 Hz were short lived and recovered within approximately 2 h. In contrast box-stepping produced a greater fall in Pto and Po at 10 and 20 Hz, which was long lasting (at least (22 h), and there was a consistent fall in the 20/50 ratio. a 2-min "fatigue" test showed that the muscles were weaker but not more fatigable after exercise. Our results seriously question the validity of using submaximal stimulation voltages and ratios for testing human muscle function and suggest that long-lasting muscle weakness is not associated with recovery from prolonged walking, running, and only observed after box-stepping exercise.

Adult↗
PubMed1985

Muscle weakness following sustained and rhythmic isometric contractions in man.

The effects of sustained and rhythmically performed isometric contractions on electrically evoked twitch and tetanic force generation of the triceps surae have been investigated in 4 healthy male subjects. The isometric contractions were performed separately and on different occasions at 30%, 60% and 100% of the force of maximal voluntary contraction (MVC). The area under the maximal voluntary contraction (MVC) force/time curve during the rhythmic and sustained contractions was the same for each experiment. The results showed that following rhythmic isometric exercise there was a small decrease in low (10 and 20 Hz) and high (40 Hz) frequency tetanic tension which was associated with % MVC. However, there was no change in the 20/40 ratio of tetanic forces, MVC or the contraction times and force of the maximal twitch. In contrast, following sustained isometric exercise tetanic forces were markedly reduced, particularly at low frequencies of stimulation. The 20/40 ratio decreased and the induced muscle weakness was greater at 30% than 60% or 100% MVC. The performance of sustained isometric contractions also effected a decrease in contraction time of the twitch and MVC. The results are in accord with previous findings for dynamic work (Davies and White 1982), and show that if isometric exercise is performed rhythmically the effect on tetanic tensions is small and there is no evidence of a preferential loss of electrically evoked force at either high or low frequencies of stimulation following the contractions. For sustained contractions, however, the opposite is true, the ratio of 20/40 Hz forces is markedly reduced and following 30% sustained MVC there is a significant (p less than 0.05) change in the time to peak tension (TPT) of the maximal twitch.

Adult↗
PubMed1997

Muscle weakness is related to utilization of health care resources in COPD patients.

The factors determining utilization of health care resources in patients with chronic obstructive pulmonary disease (COPD) are poorly understood. In order to obtain insight into these factors, we studied the utilization of health care resources in 57 stable COPD patients with a forced expiratory volume in one second (FEV1) of 36 +/- 9% predicted. Patients were divided into two groups: admitted at least twice in the last year (high medical consumption; n = 23) or not admitted in the last year (low medical consumption; n = 34). Other variables related to utilization of health care resources studied were; the number of hospital days; the number of out-patient visits to a pulmonary department in the last year; and the average daily dose (ADD) of corticosteroids taken in the last 6 months. The actual cost of utilization of health care resources, however, was not studied. In addition, pulmonary function, serum electrolytes, blood gas values, 6 min walking distance, respiratory and peripheral muscle force, and appraisal of self-care agency (ASA score) were studied. Pulmonary function, serum electrolytes, blood gas values, ASA score and walking distance were not different between the two groups (e.g. FEV1 36 +/- 8 vs 36 +/- 10% pred). Respiratory muscle forces tended to be lower in the high medical consumption group, this tendency almost reaching statistical significance for maximal expiratory pressure (PE,max) (p = 0.08). Peripheral muscle force, however, was clearly reduced in the high medical consumption group (quadriceps force 63 +/- 20 vs 82 +/- 26% pred; p < 0.05). The number of admissions, the number of hospital days, the number of out-patient visits, and ADD were interrelated and also related to ventilatory and peripheral muscle force (r -0.18 to -0.38). This relationship was statistically significant for PE,max, whilst a similar tendency was present for maximal inspiratory pressure (PI,max). In stepwise multiple regression analysis, only quadriceps force was a significant determinant of utilization of health care services. We conclude that utilization of health care services in patients with chronic obstructive pulmonary disease is related to ventilatory and peripheral muscle force. Whether or not reduced muscle force is simply an expression of disease severity remains to be determined.

Activities of Daily Living↗
PubMed2001

Patterns of extraocular muscle weakness in vasculopathic pupil-sparing, incomplete third nerve palsy.

OBJECTIVE: To determine the pattern of extraocular muscle (EOM) paresis in incomplete vasculopathic third nerve palsies (3NP) that have normal pupillary function. METHODS: A retrospective study in a private practice and academic neuro-ophthalmic practice. Patients diagnosed with vasculopathic 3NP within 4 weeks of symptom onset were identified. The chart of each patient was reviewed to determine pupillary function and the pattern and degree of EOM and levator palpebrae paresis at the time of presentation. RESULTS: Of 55 patients with vasculopathic 3NP, 42 (76%) had normal pupillary function. Of these 42, 23 (55%) demonstrated an incomplete EOM palsy, defined as partially reduced ductions affecting all third nerve-innervated EOMs and levator (diffuse pattern) or partially reduced ductions that involved only some third nerve-innervated EOMs and levator (focal pattern). Twenty (87%) of these 23 patients showed a diffuse pattern of paresis; only three (13%) showed a focal pattern of paresis, one that affected only the superior rectus and levator muscles (superior division weakness). CONCLUSIONS: Based on our series, most patients with EOM/levator involvement in pupil-sparing, incomplete 3NP of vasculopathic origin have a diffuse pattern of paresis. In contrast, our review of the literature suggests that pupil-sparing 3NP of aneurysmal origin usually have a focal pattern of paresis. We propose that distinguishing these two patterns of EOM paresis may be helpful in differentiating between vasculopathic and aneurysmal 3NP. Future studies will be needed to confirm the clinical utility of this hypothesis.

Adult↗