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Test methods to detect hip and knee muscle weakness and gait disturbance in patients with hip osteoarthritis.

OBJECTIVE: To evaluate test methods for hip and knee muscle weakness and gait disturbance. DESIGN: Test-retest. SETTING: Orthopedic university clinic. PARTICIPANTS: Ten young (age, 36+/-6 y) and 13 elderly (age, 69+/-8 y) healthy volunteers and 11 patients (age, 69+/-8 y) with unilateral hip osteoarthritis (OA) were tested for muscular strength. Twenty-five volunteers (age, 42+/-14 y) underwent gait analysis. INTERVENTIONS: A dynamometer assessing maximal voluntary isometric force of hip and knee muscles and an optosensor walkway detecting limp were developed. Tests evaluated reproducibility and tolerance in patients with OA and elderly subjects. MAIN OUTCOME MEASURES: Relative coefficient of variation (CV%) and force (in newtons). RESULTS: CV% for unilateral strength measurements ranged from 7% to 12% for specific muscle groups. CV% for gait parameters ranged from 4% to 8%, except for the double-support phase. Tests were well tolerated, and no patient had to discontinue because of fatigue. Differences related to sex, age, and disease were detected. CONCLUSIONS: Our dynamometer system provides reliable measurements of hip and knee muscle strength in young and old people, and variation is comparable to previous data. Our photocell technique for gait analysis is reliable in people with normal gait. Both methods are attractive because they are affordable, nonstationary, and easy to use.

Adult↗

Creatine supplementation for weak muscles in persons with chronic tetraplegia: a randomized double-blind placebo-controlled crossover trial.

BACKGROUND AND OBJECTIVES: Creatine supplementation improves muscle strength in some patient populations with neurologic disorders. The purpose of this study was to determine whether creatine supplementation improves muscle strength and endurance in weak upper limb muscles in persons with tetraplegia, and whether it improves function. METHODS: Outpatients with tetraplegia and mild wrist extensor weakness were randomized to receive either creatine or placebo in a double-blind crossover design. During creatine supplementation, participants were loaded with 10 g orally twice per day for 6 days, then maintained on 5 g daily until undergoing testing. Main outcome measures, performed at baseline, after placebo, and after creatine supplementation, included maximal voluntary wrist extensor isometric contraction strength (MVC), endurance times for 5 submaximal wrist extensor contractions, and the Grasp and Release Test for hand function. RESULTS: Eight individuals (7 men, 1 woman) with tetraplegia met inclusion criteria and completed all study phases. The mean age of participants was 48 years, and 7 of 8 had C6 motor level injuries. There were no significant differences in MVC, endurance times, or hand function for creatine vs placebo. CONCLUSION: Creatine does not improve MVC and endurance of weak wrist extensors and does not improve hand function in individuals with tetraplegia.

Cervical Vertebrae↗

Hypothyroidism presenting with respiratory muscle weakness.

A 58-yr-old woman presented with recurrent chest infections, breathlessness, and orthopnea. She complained of nonspecific tiredness and aching limbs. A chest radiograph showed an elevated right hemidiaphragm. Thyroid function tests showed her to be severely hypothyroid (T4 = 23 nmol/L; TSH greater than 50 mU/L). Measurement of maximal respiratory mouth pressures (expiratory: 50 cm H2O, normal, 94 +/- 33; inspiratory: 15 cm H2O, normal, 71 +/- 27) suggested global respiratory muscle weakness. Severe bilateral diaphragm weakness was demonstrated by a greatly reduced maximal transdiaphragmatic pressure (Pdi) (Pdi Pimax = 0, normal, 65 +/- 31 cm H2O; sniff Pdi = 25 cm H2O, normal, 121 +/- 25). No Pdi was detectable on stimulation of the right phrenic nerve, whereas, on the left, it was 11 cm H2O (normal 7 to 15 cm H2O). Phrenic nerve conduction time was prolonged to both sides (right, 12 ms, left, 10 ms; normal, less than 9.5 ms). The relaxation rate of Pdi after a maximal sniff and after bilateral phrenic nerve stimulation was abnormally slow (7.4%/10 ms, 6.3%/10 ms, respectively). Three months after starting treatment with thyroxine she had become euthyroid, and phrenic nerve conduction times and Pdi relaxation rates had returned to normal. Maximal respiratory pressures, vital capacity, and maximal voluntary ventilation improved progressively on treatment, although maximal respiratory pressures still had not reached the normal range after six months. We conclude that hypothyroidism may present with breathlessness due to respiratory muscle weakness and/or phrenic nerve neuropathy and is reversible with treatment.

Diaphragm↗

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

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

Adult↗

[A case of familial muscle weakness corrected by exercise (author's transl)].

The symptom described as muscle weakness corrected by exercise appears to be part of the recessive form of congenital myotonia, of which it can constitute the dominant clinical manifestation in some cases. This symptom coincides with decrements of the action of potentials to repetitive stimulations in the electromyogram, which may be particularly severe in these same cases. These observations contribute to the modification and precision of the description of the recessive form of Thomsen's disease, but their pathogenicity remains unclear.

Diagnosis, Differential↗

[Periodic muscle weakness and cervical ventroflexion caused by hypokalemia in a Burmese cat].

A 2-year-old female Burmese cat was referred to the University Hospital of Companion Animals of Utrecht University because of periodic muscle weakness and cervical ventroflexion. Laboratory examinations revealed hypokalemia. The combination of breed, clinical signs and hypokalemia warranted the diagnosis of 'periodic hypokalemic myopathy', a homozygote recessive hereditary disease in Burmese cats. Potassium supplementation resulted in complete disappearance of the signs. Possible causes of hypokalemia in the cat are discussed.

Animals↗

Nausea, emesis, and muscle weakness in a pregnant adolescent.

BACKGROUND: Gitelman syndrome is a rare autosomal recessive disorder that presents in early adulthood with fatigue, muscle cramps and electrolyte abnormalities. CASE: A 17-year-old African-American woman presented at 17 weeks of pregnancy with nausea, emesis, profound lower extremity proximal muscle weakness, hypokalemia, and hypomagnesemia. After a thorough evaluation, Gitelman syndrome was diagnosed. The patient was maintained on high levels of potassium and magnesium supplementation throughout the rest of her pregnancy and delivered a healthy infant. CONCLUSION: In pregnancy, nausea and emesis is most commonly attributed to hyperemesis gravidarum. However, an atypical presentation of these symptoms and/or the coexistence of less common complaints warrant further investigation.

Adolescent↗

[Muscle weakness].

A 31-year old cook suffered from episodes with muscular weakness since 3 weeks. These episodes resolved, at times, spontaneously and completely after a few hours. The weakness affected mainly the muscles of trunk and proximal extremities. Investigation of the patient revealed flaccid tetraparesis with diminished tendon reflexes, however, cranial muscles were not affected. Mental function and sensibility were intact. These findings were consistent with hypopotassemic periodic paralysis rather than a neurological or psychiatric disease. Serum potassium was determined as 1.8 mmol/l. Moreover, the patient suffered from atrial fibrillation. Grave's disease could be identified as the underlying cause of hypopotasemic periodic paralysis and atrial fibrillation. Further manifestations of primary hyperthyroidism were nervousness, diarrhea and weight loss of 5 kg observed during the previous months. The symptoms resolved under treatment with potassium, betablocker and thyrostatic agents. The patient was treated with warfarin because of atrial fibrillation and finally underwent electroconversion.

Adrenergic beta-Antagonists↗

Acetazolamide-induced muscle weakness in hypokalemic periodic paralysis.

A 46-year-old man with hypokalemic periodic paralysis (HypoPP) and diabetes mellitus (DM) had worsened muscle weakness after acetazolamide (ACZ) treatment. During the paralytic episode, serum potassium levels were reduced, and serum chloride and insulin levels were increased. The data suggested proximal renal tubular acidosis due to ACZ. We determined arterial-venous concentrations of potassium, insulin and glucose across the forearm. Venous potassium levels were markedly reduced. ACZ is thought to potentiate potassium uptake into muscles. Hyperinsulinemia and DM could contribute to ACZ-induced exacerbation in our patient. We should pay more attention to ACZ-treated HypoPP patients with hyperinsulinemia and DM.

Acetazolamide↗

Mechanism of quadriceps femoris muscle weakness in patients with anterior cruciate ligament reconstruction.

The purpose of this study was to investigate gamma loop function in the quadriceps femoris muscle in patients who with less than 6 month-history of anterior cruciate ligament (ACL) reconstruction. For this purpose, we compared the response to vibration stimulation in 10 patients with ACL repair and 12 normal healthy subjects, by measuring the maximal voluntary isometric contraction (MVC) and integrated electromyograms (I-EMG) of the quadriceps muscles. Pre-vibration data were obtained from each subject by measuring the MVC of the knee extension and the I-EMG from the vastus medialis, vastus lateralis, and rectus femoris muscles. Vibration stimulation was applied to the infrapatellar tendons, followed immediately by repeating the MVC and I-EMG recording. Prolonged vibration resulted in a significant decrease of both MVC and I-EMG in the control group. In contrast, the same stimulus failed to elicit changes in ACL-repair group. Our results suggest the presence of abnormal gamma loop function in the quadriceps femoris muscle of patients with ACL repair, which may explain the muscle weakness often described in such patients.

Adult↗

[A 63-year-old woman with muscle weakness, myotonia, and parkinsonism].

We report a 63-year-old woman who presented myotonia and parkinsonism. The patient was well until 15 years of the age when she noted that the ring finger of her left hand at times flexed when she did not intend to do so. She noted weakness in her left upper extremity at the age of 40, and difficulty in relaxing her hand grip at 45. She had an onset of tremor in her right foot at age 50, which was followed by difficulty in gait and hand writing. She was admitted to Juntendo University Urayasu Hospital when she was 63-year-old. Her mother, two sisters, and a son were affected with similar muscle weakness and myotonia. Although some of them developed stooped posture in the late stage of the disease, none of them had overt parkinsonism. General physical examination was unremarkable. Neurologic examination revealed an alert and oriented woman with some recent memory loss. She had bilateral ptosis, facial weakness, and a masked face. Myerson's sign was present. Her speech was small and monotonous. The sternocleidomastoid muscles were markedly atrophic and weak. The remaining of the cranial nerves were intact. She walked in small steps with freezing with support. She showed bradykinesia, retropulsion, and resting tremor in her right leg. Slight distal dominant weakness was noted in both upper and lower extremities more on the left. No cerebellar signs were noted. Muscle stretch reflexes were within normal limits in the upper extremities and diminished in the lower limbs. Sensation was intact. Routine laboratory findings were unremarkable. Cranial CT scan and MRI revealed slight cortical atrophy and leukoaraiosis. She responded to levodopa and she became able to walk by herself. She was transferred to another hospital one month after her admission. She had several bouts of airway obstruction with one episode of respiratory arrest. She expired six month after the transfer. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that this patient suffered from myotonic dystrophy and Parkinson's disease which set in later years. Postmortem examination on the iliopsoas muscle revealed uneven muscle fiber diameters, central nuclei, and type 1 fiber predominance; the pathologic finding was consistent with myotonic dystrophy. The substantia nigra showed marked cell loss and Lewy bodies in the remaining neurons. The finding was consistent with Parkinson's disease. In myelin stain, diffuse myelin pallor was noted in the cerebral white matter which was the pathologic substrate of leukoaraiosis in this patient. Combination of these two disorders have never been reported in the literature to our knowledge. It appears to be that the coincidence is just a by-chance phenomenon, but it seems interesting to note that accelerated aging process appears to be present in both myotonic dystrophy and Parkinson's disease.

Diagnosis, Differential↗

Expiratory muscle weakness and assisted cough in ALS.

Elimination of airway secretion is a major issue in the care of patients with ALS. Sufficient cough flows have to be generated by expiratory muscles to allow airway clearance. Bulbar and expiratory muscle weakness are often reasons for failure of non-invasive ventilation (NIV) and may lead to tracheostomy. Expiratory aids may help to overcome these problems, at least for some time. We report a patient with advanced ALS, receiving nocturnal NIV, who gained much benefit from regular use of a mechanical in-exsufflation device.

Amyotrophic Lateral Sclerosis↗

Severe muscle weakness due to hypokalemia as a manifestation of small-cell carcinoma.

We describe the case of a 56-year-old man with severe muscle weakness due to heavy hypokalemia (serum potassium 1.44 mmol/l) associated with inappropriate kaliuria and alkalemia. Subsequent investigation revealed the presence of ectopic ACTH hypersecretion due to a small-cell lung carcinoma. A significant clinical/laboratory improvement was achieved following chemotherapy-induced regression of the primary tumor. The profound hypokalemia was probably the result of cortisol hypersecretion, which in concert with the ACTH-induced decreased 11beta-hydroxysteroid dehydrogenase activity can exhibit an increased mineralocorticoid activity. In addition, other ACTH-dependent mineralocorticoids may play a contributory role in the development of severe hypokalemia.

ACTH Syndrome, Ectopic↗

Muscle weakness and falls in older adults: a systematic review and meta-analysis.

OBJECTIVES: To evaluate and summarize the evidence of muscle weakness as a risk factor for falls in older adults. DESIGN: Random-effects meta-analysis. SETTING: English-language studies indexed in MEDLINE and CINAHL (1985-2002) under the key words aged and accidental falls and risk factors; bibliographies of retrieved papers. PARTICIPANTS: Fifty percent or more subjects in a study were aged 65 and older. Studies of institutionalized and community-dwelling subjects were included. MEASUREMENTS: Prospective cohort studies that included measurement of muscle strength at inception (in isolation or with other factors) with follow-up for occurrence of falls. METHODS: Sample size, population, setting, measure of muscle strength, and length of follow-up, raw data if no risk estimate, odds ratios (ORs), rate ratios, or incidence density ratios. Each study was assessed using the validity criteria: adjustment for confounders, objective definition of fall outcome, reliable method of measuring muscle strength, and blinded outcome measurement. RESULTS: Thirty studies met the selection criteria; data were available from 13. For lower extremity weakness, the combined OR was 1.76 (95% confidence interval (CI)=1.31-2.37) for any fall and 3.06 (95% CI=1.86-5.04) for recurrent falls. For upper extremity weakness the combined OR was 1.53 (95% CI=1.01-2.32) for any fall and 1.41 (95% CI=1.25-1.59) for recurrent falls. CONCLUSION: Muscle strength (especially lower extremity) should be one of the factors that is assessed and treated in older adults at risk for falls. More clinical trials are needed to isolate whether muscle-strengthening exercises are effective in preventing falls.

Accidental Falls↗