Severe vasculitic neuropathy in systemic lupus erythematosus and response to cyclophosphamide.
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Adult↗
Biomedical subjects
C M Wiles
Publications and source records attributed to C M Wiles.
Growth hormone treatment in growth hormone-deficient adults. I. Effects on muscle mass and strength.
The effect of recombinant DNA human growth hormone (rhGH) treatment in adults with growth hormone (GH) deficiency was studied in 24 patients in a double-blind placebo-controlled trial. The dose was 0.07 U/kg body wt daily. After 6 mo of treatment, significant increases were noted in the rhGH group for total cross-sectional area of thigh muscle (+11.2 +/- 3.1 vs. -0.5 +/- 3.0 cm2; P = 0.015 vs. placebo) and quadriceps muscle (+4.1 +/- 0.8 vs. +0.4 +/- 1.2 cm2; P = 0.031) measured by computerized tomography. Strong correlations were noted between lean body mass (measured as total body potassium) and total thigh muscle area in normal and GH-deficient adults both before and after rhGH treatment. Strength of hip flexors (+1.25 +/- 0.27 vs. +0.25 +/- 0.12 z-scores; P = 0.004) and limb girdle muscles increased (P = 0.02) in the rhGH group. We conclude that 1) rhGH increases lean tissue and skeletal muscle mass in adults with human GH deficiency, 2) this suggests a role for GH in the regulation of body composition of adult humans, 3) the increase in strength of limb girdle muscles after rhGH treatment suggests that adults with GH deficiency may have a proximal myopathy, and 4) the failure to demonstrate an increase in strength in other muscle groups may require the study of larger numbers of patients.
Adolescent↗
Growth hormone treatment in growth hormone-deficient adults. II. Effects on exercise performance.
Growth hormone (GH) treatment in adults with GH deficiency increases lean body mass and thigh muscle cross-sectional area. The functional significance of this was examined by incremental cycle ergometry in 24 GH-deficient adults treated in a double-blind placebo-controlled trial with recombinant DNA human GH (rhGH) for 6 mo (0.07 U/kg body wt daily). Compared with placebo, the rhGH group increased mean maximal O2 uptake (VO2max) (+406 +/- 71 vs. +133 +/- 84 ml/min; P = 0.016) and maximal power output (+24.6 +/- 4.3 vs. +9.7 +/- 4.8 W; P = 0.047), without differences in maximal heart rate or ventilation. Forced expiratory volume in 1 s, vital capacity, and corrected CO gas transfer were within normal limits and did not change with treatment. Mean predicted VO2max, based on height and age, increased from 78.9 to 96.0% in the rhGH group (compared with 78.5 and 85.0% for placebo; P = 0.036). The anaerobic ventilatory threshold increased in the rhGH group (+159 +/- 39 vs. +1 +/- 51 ml/min; P = 0.02). The improvement in VO2max was noted when expressed per kilogram body weight but not lean body mass or thigh muscle area. We conclude that rhGH treatment in adults with GH deficiency improves and normalizes maximal exercise performance and improves submaximal exercise performance and that these changes are related to increases in lean body mass and muscle mass. Improved cardiac output may also contribute to the effect of rhGH on exercise performance.
Adolescent↗
Acute parkinsonism associated with flurbibrofen.
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Acute Disease↗
Laboratory testing of muscle function in the management of neuromuscular disease.
A laboratory was set up to assess muscle weakness and physical disability in patients with peripheral neuromuscular diseases. Muscle strength was mainly measured with a hand held dynamometer and results were expressed in relationship to a "guideline" range for sex: disability was expressed in terms of ordinal data with a performance score and as interval data with simple timed tests. Of 17 patients with polymyositis on immunosuppressive medication followed for a mean of 2.9 years (range 1.1 - 4.7 years) only eight became stronger one of whom died. Assessment of weakness (the major impairment), disability, body weight, creatine kinase and the patient's subjective view of their state were essential to obtaining a clear view of progress.
Adult↗
Skeletal muscle performance in adults with growth hormone deficiency.
Skeletal muscle mass and function were assessed in 24 adults (16 males, 8 females) with severe, long-standing GH deficiency. Compared to 41 untrained controls (26 males, 15 females), adults with GH deficiency had reduced cross-sectional area of thigh muscle/body weight (p = 0.01), reduced quadriceps force/weight (males: p = 0.002; females: p less than 0.0001), and reduced quadriceps force/muscle area (males: p = 0.005). This suggests (a) that adults with GH deficiency have reduced skeletal muscle mass due, in part, to the absence of the anabolic effects of GH, and (b) that skeletal muscle force may be reduced due to altered muscle mass, contractile elements/muscle fibre, anaerobic energy supply, neural recruitment of fibres, or a combination of these.
Adult↗
Slow recovery from severe foodborne botulism.
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Botulism↗
Respiratory complications and their management in motor neuron disease.
Although respiratory insufficiency is common in the advanced stages of motor neuron disease, some patients may develop distressing respiratory symptoms early in the course of the disease or even present with respiratory failure or arrest. We describe 14 patients with motor neuron disease who were considered for respiratory support; 11 received such support and all derived significant symptomatic improvement without distressing prolongation of life. Of the 8 patients with typical features of amyotrophic lateral sclerosis, 7 had predominant diaphragm weakness and 1 generalized respiratory muscle weakness; 7 received negative pressure ventilation by cuirass which improved both the quality of sleep and exercise tolerance. Three patients with predominantly bulbar disease had nocturnal apnoea or hypoventilation. Two received no support. One, who also developed diaphragm weakness, was treated by a cuirass, continuous positive airway pressure (CPAP), and later nocturnal intermittent positive pressure ventilation (IPPV). Three patients with progressive muscular atrophy had predominant diaphragm weakness or nocturnal apnoea. These patients received nocturnal CPAP, cuirass or IPPV with symptomatic benefit. This series shows that some patients with motor neuron disease, mainly those with symptoms due to respiratory muscle weakness in the absence of severe bulbar impairment, derive symptomatic benefit from supported ventilation.
Adult↗
The effect of a high protein diet on leucine and alanine turnover in acid maltase deficiency.
Leucine and alanine production rate was measured in 5 patients with acid maltase deficiency in the postabsorptive state, following 6 months on a normal diet with placebo and 6 months on an isocaloric high protein diet (16-22% protein). Whole body leucine production rate, a measure of protein degradation, expressed in terms of lean body mass was significantly greater than in five control subjects. Following the high protein diet, leucine production rate was decreased in four of the five patients but this was not statistically significant. Alanine production rate expressed in terms of lean body mass was significantly greater than in control subjects. After the high protein diet, alanine production rate and concentration were significantly decreased (p less than 0.05). There were no significant improvements in any of the clinically relevant variables measured in these patients. It is possible that a larger increase in protein intake over a longer time period may have a clinical effect.
Adult↗
MRI appearances of the CNS manifestations of Mycoplasma pneumoniae: a report of two cases.
Two patients are reported with Mycoplasma pneumoniae-related cervical myelitis. Magnetic resonance imaging in each case demonstrated clinically silent lesions suggesting more extensive neurological involvement. This supports the concept of widespread immunologically mediated disease occurring as a remote effect of initial M. pneumoniae respiratory infection. Differences from the MRI appearances of a patient with mycoplasma-related Guillian-Barré syndrome imply that more than one antigenic determinant is involved.
Adolescent↗
Respiratory arrest: a complication of cerebellar ectopia in adults.
Two adult patients are described with cerebellar ectopia whose presentation at diagnosis was respiratory arrest. Following surgical decompression both patients become self ventilating and now lead independent lives. Cerebellar ectopia is a potentially remediable condition and should be considered in patients with unexplained respiratory arrest.
Arnold-Chiari Malformation↗
The late sequelae of poliomyelitis.
Progressive functional deterioration following poliomyelitis has been reported after a prolonged period of stability. We present follow up data on 209 patients; the period from the original illness to December 1985 or death was between two and 73 years (mean 33.9). One hundred and sixty-three (78 per cent) patients developed late functional deterioration. This was due to purely respiratory factors in 99 cases, new neurological signs in 20 cases, and orthopaedic problems in 17 cases; 31 patients deteriorated due to a combination of factors. The commonest cause of respiratory deterioration was the development of nocturnal alveolar hypoventilation, sometimes associated with late progressive scoliosis. Eighty-six patients needed respiratory support beginning between one year and 66 years (mean 28.5) after the acute illness. New neurological signs had a clearly defined cause in each of 20 patients. No cases of motor neurone disease or post-poliomyelitis muscular atrophy (PPMA) were identified. This series shows that late sequelae following poliomyelitis are common. There were no patients with functional deterioration after poliomyelitis in whom a clear underlying cause could not be shown. The major causes of deterioration may be treatable or avoidable, and in particular, those who develop chest infections or new respiratory symptoms should be treated with caution as respiratory failure may rapidly supervene.
Adolescent↗
Shoulder abduction fatiguability.
Shoulder abduction fatiguability has been measured using a hand held myometer in normal subjects and patients with peripheral neuromuscular diseases. An index of fatiguability was based on the decline in force over a series of ten maximum voluntary contractions performed in under a minute. The technique was repeatable and well tolerated. Patients with myasthenia, mitochondrial myopathy and motor neuron disease tended to show excess fatiguability independent of muscle strength. Serial measurements demonstrated alterations in fatiguability but not necessarily strength, associated with changes in symptoms.
Adult↗
Not tarsal tunnel syndrome: a malignant "Triton" tumour of the tibial nerve.
A 23 year old male patient presented with a venous thrombosis in the right calf. This was followed by symptoms, signs and electromyographic findings suggestive of a tarsal tunnel syndrome. Symptoms were briefly relieved by surgical division of the flexor retinaculum. Subsequently, patient developed swelling in the calf and was found to have a malignant ("Triton") tumour of the tibial nerve and required above knee amputation. In the absence of obvious foot or ankle deformity, caution should be exercised in making the diagnosis of tarsal tunnel syndrome.
Adult↗
Protein turnover in acid maltase deficiency before and after treatment with a high protein diet.
A patient with acid maltase deficiency was treated with a high protein diet for 7 months. Protein turnover expressed in terms of lean body mass was shown to be increased in this patient before the diet but was markedly reduced following the diet. The patient improved clinically whilst on the diet both subjectively and in terms of mobility, breathing and reduced peripheral cyanosis at rest.
Adult↗
Hyperbaric oxygen in multiple sclerosis: a double blind trial.
Eighty four patients with multiple sclerosis were treated in monoplace chambers with either hyperbaric oxygen at 2 atmospheres absolute or placebo. Comprehensive double blind assessment was carried out before, immediately after, and one month after treatment. There was no clinically important or significant benefit in any of the four major criteria of outcome--namely, the patient's subjective opinion, the examiner's opinion, the score on the Kurtzke disability status scale, or the time taken to walk 50 m. Out of 40 other clinical variables assessed, two (the sensory scale and timed writing with the left hand) showed a significant improvement without any subjective clinical correlate or change in any of seven other tests of left hand function. No group of symptoms was perceived by the patients as having improved more after treatment with hyperbaric oxygen than placebo. It is concluded that there is no basis for recommending hyperbaric oxygen in the treatment of multiple sclerosis.
Adult↗
A tonic pupil with Horner's syndrome.
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Aged↗
Life-threatening respiratory failure due to a previously undescribed myopathy.
The case of a young man with a previously undescribed myopathy associated with polydactyly is reported. Although both limb girdles were affected, the major effect of the disease was upon the respiratory muscles leading to his presentation with life-threatening respiratory failure. A further feature was pronounced stiffness of the vertebral column and limb girdles, similar in some respects to the 'rigid spine syndrome'. Muscle biopsy appearances were unique but showed some similarities to both nemaline myopathy and myotonic dystrophy. Ventilatory assistance at night using a rocking bed led to a marked improvement and has enabled the patient to return to full-time employment.
Adult↗