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Biomedical subjects

C M Wiles

Publications and source records attributed to C M Wiles.

At least 73 records · Page 4Linked to original sources
PubMed1994

Total lymphoid irradiation in multiple sclerosis.

Following a report of the efficacy of total lymphoid irradiation (TLI) in the treatment of chronic progressive multiple sclerosis a further randomised double-blind placebo-controlled study was undertaken with the intention of entering 56 patients. In the event it was possible to recruit only 27 patients in a 2.5 year period. Three patients received active treatment openly and 24 were randomised to either active (14) or sham (10) treatment. Treatment was 1980 cGy to the lymphoid system and spleen or sham treatment after full simulation. The primary outcome measure was a comparison of the mean rates of change between treatment groups on the expanded Kurtzke disability scale (EDSS) over the two year follow up period. Patients were also assessed on other clinical outcome measures, psychometry, and serial MRI of the brain. Active treatment resulted in a profound and prolonged fall in T lymphocytes especially those with the CD4 marker and a reversal in CD4:CD8 ratio. No significant benefit was demonstrated on the rate of clinical disease progression (EDSS). A small but significant benefit was found on a score of bladder function. No significant benefit was demonstrated on other clinical or psychometric indices or on subjective visual analogue scales. There was a small but significant difference in the rate of accumulation of lesions on brain MRI favouring the treatment group. The treated group had a higher incidence of clinically relevant side effects, notably amenorrhoea and infections: three deaths (one in the TLI group, two in the sham treated group) occurred. A post hoc calculation indicates that the study had a possible 35% risk of a false negative result using the principal outcome measure. The study fails to confirm the previously reported clinical benefit of TLI although there may be a minor benefit on disease progression as indicated by MRI lesion counts. It is concluded that TLI cannot be recommended for the routine treatment of chronic progressive multiple sclerosis but the beneficial effect on MRI lesions, though modest, suggests that further research into immune modulation of this condition may be worthwhile.

Adult↗
PubMed1994

Severe dysphagia associated with major tranquillizer treatment.

A 56 year old patient with psychiatric complications of systemic lupus erythematosus developed severe dysphagia complicated by weight loss and aspiration. Following investigation it was concluded that the addition of haloperidol to her treatment was the major precipitating cause and withdrawal of the drug was followed by an objective improvement in swallowing. Patients taking major transquillizers may be at increased risk of severe dysphagia; regular observation of swallowing is suggested as a useful addition to the clinical examination of these patients.

Deglutition↗
PubMed1994

Motor neurone disease--a study of prevalence and disability.

We conducted a point prevalence study of motor neurone disease in the counties of South Glamorgan, Mid Glamorgan and Gwent, whose combined population is estimated at 1,394,400. A total of 56 patients were identified, giving a point prevalence for motor neurone disease in this area of 4.02/100,000 on 22/06/92. These cases were reclassified according to the World Federation of Neurology criteria for amyotrophic lateral sclerosis following a further clinical assessment, when disability was also evaluated (in 49 cases). Seven patients were reclassified on review of their medical records alone. Overall, 38 were classified as either definite or probable cases, which gave a point prevalence of 2.73/100,000. Of these patients, 94% (46/49) were living at home, 43% (21/49) scored 15 or less on the Barthel index, 65% (32/49) had some degree of mobility impairment, and 20% (10/49) were wheelchair-dependent. Swallowing speed was reduced (< 10 ml/s) in 67% (33/49), with three requiring feeding through a percutaneous gastrostomy tube. Vital capacity was < 70% of predicted value for age, sex and height in 67% (29/43). One patient was receiving domiciliary ventilation. These results give a quantitative indication of the dependency in a population of patients with motor neurone disease.

Aged↗
PubMed1994

Use of the rocking bed in the treatment of neurogenic respiratory insufficiency.

We describe 53 patients who received ventilatory support with a rocking bed. Diagnoses included previous poliomyelitis (30), muscular dystrophy (12), motor neurone disease (4), adult-onset acid maltase deficiency (4) and a miscellaneous group (3). Patients presented with respiratory insufficiency characterized by diaphragm weakness, progressive nocturnal hypoventilation and/or acute or chronic respiratory failure. Domiciliary rocking beds were used by 43 patients for a mean of 16.0 years (range 1 month to 35 years). Most patients were able to breathe adequately by day when sitting or standing, but needed assistance by rocking bed for 6-11 h when lying down for sleep. The rocking bed was well-tolerated, and associated with both symptomatic relief and amelioration of arterial blood gas abnormalities. Seventeen of these 43 patients discontinued its use, either because of discomfort (9) or increasing respiratory insufficiency (8). The rocking bed is a valuable adjunct in the management of the respiratory insufficiency associated with neuromuscular disease.

Adult↗
PubMed1993

Brown-Vialetto-Van Laere syndrome.

The clinical and pathological findings of a male with the Brown-Vialetto-Van Laere syndrome are described. This rare and fatal affection of the nervous system involves mainly the brain stem with the prominent and early manifestation of sensorineural deafness. Increased awareness and documentation of this disorder has added information on the mode of inheritance.

Adolescent↗
PubMed1993

Respiratory involvement in primary muscle disorders: assessment and management.

Eighty-four patients with primary disorders of muscle were referred for assessment of respiratory insufficiency between 1978 and 1991. The eventual diagnoses were: 'limb girdle syndromes' (18 patients), adult onset acid maltase deficiency (14), dystrophia myotonica (13), inflammatory disorders (10), congenital myopathies (nine), rigid spine syndromes (five), dystrophies (Duchenne (six), facioscapulohumeral (four), Becker (one)) and miscellaneous (four). Presentations were often insidious, with progressive nocturnal hypoventilation culminating in respiratory failure or arrest, recurrent respiratory tract infections, or obstructive sleep apnoea. Respiratory symptoms developed relatively early in patients with acid maltase deficiency and inflammatory disorders but parallelled the development of limb weakness in limb girdle, myotonic and congenital syndromes. Sixty-six patients received respiratory support for a median of 5 years (1-34 years) using various techniques of negative and positive pressure ventilation. Fourteen patients received short-term support for an episode of respiratory failure before being weaned, 51 required nocturnal domiciliary ventilation and one was dependent on continuous domiciliary support. Tracheostomy was performed in 32 patients and used for domiciliary nocturnal ventilation in 25. Twenty-eight patients have subsequently died.

Adult↗
PubMed1992

Measurement of pharyngeal transit time by electrical impedance tomography.

The feasibility of using electrical impedance tomography (EIT) for measuring pharyngeal transit time has been investigated. Imaging sequences were obtained on the neck in a group of eight healthy subjects when swallowing solutions of different electrical conductivities. The solutions used were aqueous Dioralyte (1.5 S m-1) and tap water (0.04 S m-1). The effects of movement upon the images were large compared with the changes due to the solutions, but these were cancelled by a subtraction technique. The mean value of the EIT pharyngeal transit time parameter was 370 +/- 120 ms. EIT has the advantage that repeated measurements could be performed without fear of hazard in contrast to the established technique of video fluoroscopy which involves ionising

Adult↗
PubMed1992

Respiratory involvement in multiple sclerosis.

Respiratory complications occur in advanced multiple sclerosis (MS) but may also complicate acute relapses earlier in the disease. We present 19 patients with MS who developed respiratory complications at a mean of 5.9 (range 1-12) yrs after the onset of neurological symptoms. Fourteen patients developed severe respiratory insufficiency presenting with a combination of reduced forced vital capacity (FVC), hypoxaemia or hypercapnia (12 patients) and respiratory arrest (four patients). Two patients presented with apneustic breathing, one with paroxysmal hyperventilation, one with obstructive sleep apnoea and one with bulbar weakness leading to aspiration pneumonia. Respiratory muscle weakness was a major factor in 14 patients (predominantly diaphragm involvement in six), bulbar weakness in seven patients, impaired voluntary control in three and impaired automatic control in three. Twelve patients received mechanical respiratory support of whom seven have subsequently died. The methods of support used were intermittent positive pressure ventilation (nine patients), iron lung (three), cuirass (two) and rocking bed (one). Six patients were maintained on respiratory support until they died after intervals varying from 24 h to 6 yrs (mean 17.7 mths). Five patients received temporary ventilation for between 6 d and 42 d: of these four remain alive at up to 4 yrs and one died after 16 yrs. One patient remains on domiciliary nasal intermittent positive pressure ventilation (IPPV) after 1 yr.

Adolescent↗
PubMed1992

A timed test of swallowing capacity for neurological patients.

A timed test of swallowing capacity has been designed for use in patients with neurogenic dysphagia. Swallowing speed (ml/s) has been demonstrated to have high intra- and inter- rater and test- retest reliability, and to be essentially independent of flavour or temperature. "Guideline" normal values were established in individuals without a swallowing disorder: swallowing speed was less in females than males and declined in both groups with age. The validity of a swallowing speed less than 10 ml/s as an index of abnormal swallowing was tested by comparison with the complaint of abnormal swallowing in a group of 81 neurological patients. Swallowing speed had a sensitivity of 96% and specificity of 69%: some apparent false positive responses were found in patients with disordered swallowing, mainly due to multiple sclerosis. Using a standard questionnaire and examination a similar pattern of symptoms and signs were statistically associated with both the clinical complaint of abnormal swallowing and swallowing speed. It is concluded that swallowing speed is a reliable and valid index for assessing disordered swallowing in neurological patients and may be of value in monitoring response to therapy.

Adult↗
PubMed1992

Histology of skeletal muscle in adults with GH deficiency: comparison with normal muscle and response to GH treatment.

The histology of needle biopsy specimens of skeletal muscle from the vastus lateralis was quantitatively assessed in a group of adults with growth hormone (GH) deficiency, most of whom had hypopituitarism treated with conventional pituitary hormone replacement. The mean age of the 21 patients (16 males and 5 females) was 39 +/- 2 (SEM). Comparisons were made with age- and sex-matched controls following six months double-blind, placebo-controlled treatment with recombinant human GH (rhGH) in the GH-deficient patients. Before treatment, needle muscle biopsies from patients with GH deficiency showed mean type I and II fibre areas of 5,153 +/- 273 and 4,828 +/- 312 microns 2 respectively, which did not differ from the controls (4,482 +/- 306 and 4,699 +/- 310 microns 2). Percentages of type I fibres were similar in the two groups (47.2 +/- 2.5% in GH deficiency and 45.3 +/- 2.2% in controls). No difference in the variability of type I or II fibre areas was demonstrated between the groups. Correlations between the relative contribution to total fibre area by type I fibres (mean fibre area x percent) and maximal oxygen uptake (p = 0.006), and between type II fibres and quadriceps force (p = 0.035) were noted in GH-deficient adults before treatment. Following rhGH treatment, no change was noted in mean fibre areas, variability of fibre areas, or percentage of either fibre type.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

Cardiovascular effects of growth hormone treatment in growth-hormone-deficient adults: stimulation of the renin-aldosterone system.

1. In adult humans with growth hormone deficiency, treatment with growth hormone has recently been shown to have major anabolic effects and to improve exercise performance. The cardiovascular effects of growth hormone in adults with growth hormone deficiency were examined in 24 patients treated with recombinant human growth hormone (0.07 units/kg at night) in a double-blind, placebo-controlled trial lasting 6 months. 2. Compared with placebo, resting M-mode echocardiography showed increases in left ventricular end-diastolic dimension and stroke volume in the group treated with recombinant human growth hormone. No differences were noted between the groups with respect to left ventricular end-systolic dimension, fractional shortening, wall thicknesses or mean arterial blood pressure. Left ventricular myocardial mass increased in the group given recombinant human growth hormone. 3. The supine plasma renin activity was increased and remained elevated over the 6 months, whereas the plasma aldosterone concentration was unchanged, after treatment with recombinant human growth hormone. Clinical signs of sodium retention were evident during the first 3 months of treatment with recombinant human growth hormone. 4. We conclude that treatment with recombinant human growth hormone in adults with growth hormone deficiency resulted in small increases in left ventricular pre-load, due to the sodium-retaining action of growth hormone. Activation of the renin-aldosterone system was involved in such changes. Myocardial hypertrophy was observed without changes in mean arterial pressure, reflecting the anabolic action of growth hormone.

Adult↗
PubMed1991

3,4-Diaminopyridine in the treatment of congenital (hereditary) myasthenia.

Congenital or hereditary myasthenia describes a heterogeneous group of disorders in which the immune system is not implicated. Treatment has previously depended on anticholinesterase medication. The effectiveness of 3,4-diaminopyridine (3,4-DAP), a preparation that enhances acetylcholine release from motor nerve terminals, has been evaluated using a series of standardised strength measures. Sixteen patients (aged seven to 47 years) were studied in an open prospective trial, and four of them in a double blind crossover trial; existing anticholinesterase medication was continued. For the group as a whole, there was a highly significant increase in muscle strength (p less than 0.001; n = 16). In individual paired comparisons, 13 out of 16 showed significant improvement in the open trial and four out of four in the blind crossover trial. In conclusion, 3,4-DAP, either alone or combined with anticholinesterase medication, may be a useful additional treatment in congenital myasthenia.

4-Aminopyridine↗