Cranial dystonia (Meige syndrome) in postencephalitic parkinsonism.
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Biomedical subjects
Publications and source records attributed to V H Patterson.
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In a one year prospective study, 19% of 925 adult medical admissions to a district general hospital (the Downe Hospital, Downpatrick, Northern Ireland) were due primarily to a neurological complaint. A further 19 cases had an active neurological disorder which contributed to their hospital admission. Most were acute admissions via the General Practitioner or through the Accident and Emergency Department, a situation different from the practice of most regional neurological centres. Only 15 patients (8% of the neurological cases) were referred to a specialist centre.
T lymphocyte control of Epstein-Barr virus (EBV) infection of autologous B lymphocytes was examined in parallel to the enumeration of subpopulations of mononuclear cells in 22 multiple sclerosis (MS) patients and in 22 healthy individuals. All were seropositive for EBV. The incidence of lack of T cell control was significantly higher in patients than in controls, confirming previous published work. In the present study, we have shown in addition a significantly reduced proportion of OKT8+ cells and a significantly increased ratio of OKT4/OKT8 cells in the group of patients with lack of control. The findings point to abnormal immunoregulation in MS.
The results of 14 years' experience in the surgical treatment of myasthenia gravis are reported. Twenty-one patients (14 female, 7 male) underwent thymectomy for myasthenia gravis between 1971 and 1984. The mean age of the patients was 33 years (range 14 - 57 years). The median duration of symptoms prior to surgery was 18 months (range 5 months to 35 years). The mean follow-up was 5.3 years. There were no post-operative deaths: 76% obtained benefit from thymectomy. The patients' age, sex, duration of symptoms and histology of the thymus gland did not correlate with the result of treatment. This series suggests that, while thymectomy is often beneficial in the treatment of myasthenia gravis, there are no accurate predictors of the outcome following surgery.
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The failure of forearm exercise to increase plasma hypoxanthine in subjects with adenylate deaminase deficiency confirms this enzyme's role in hypoxanthine production by normal forearm exercise. The conversion of adenosine monophosphate (AMP) to hypoxanthine may reflect an alternative method of adenosine triphosphate (ATP) regeneration in working muscle.
Visual thresholds and perceptual latencies were determined in patients with multiple sclerosis (MS) and in normal control subjects. Measurements were made under light- and dark-adapted conditions, with stimuli chosen to stimulate rod and cone receptors selectively. More abnormalities in perceptual latency and luminance threshold were recorded in the light-adapted condition than in the dark-adapted condition, but this result was not specific to the rod or cone systems. Possible underlying pathophysiological processes are discussed, and it is suggested that reduced conduction velocity in the demyelinated visual pathway is the most likely explanation of the observed perceptual delays and that there is no evident retinal contribution.
Plasma hypoxanthine was measured in three normal subjects during aerobic forearm exercise. The comparative increase of hypoxanthine greatly exceeded that of ammonia or lactate. It is proposed that hypoxanthine production reflects ATP breakdown in muscle. The test may prove useful in the investigation of patients with metabolic muscle disease.
Luminance threshold, perceptual latency, double-flash resolution, and critical flicker frequency were examined in patients with multiple sclerosis (MS) and in normal control subjects. The intensities of the stimuli used to test the temporal properties of vision were equalized with respect to individual luminance thresholds. In eight patients with MS, all the properties tested showed abnormality, double-flash resolution being most commonly affected. Retinal sites were not however, uniformly abnormal according to these measures. We conclude that abnormal temporal properties of vision in patients with MS are not a simple functional consequence of altered luminance thresholds.
Visual thresholds were measured at four different background luminance levels in patients with multiple sclerosis (MS) and in control subjects by means of frequency-of-seeing curves. Results were examined by probit analysis and measures of threshold and threshold variability were obtained. Comparison of patient and control groups showed that the patient's threshold was significantly raised only at the highest background luminance level, but that threshold variability was greater at all three non-zero background luminance levels tested. In addition, threshold variability increased with background luminance in the MS patients but not in the control subjects. Possible underlying pathophysiological mechanisms are discussed, and it is suggested that this luminance-dependent variability in visual threshold shown by patients with multiple sclerosis may be due to intermittent conduction block or ephaptic transmission occurring within the demyelinated visual pathway.
Visual fields were examined with a tangent screen in 54 patients with multiple sclerosis (MS) or optic neuritis (ON). Visual fields were abnormal in all patients with definite MS, 94% with probable MS and 81% with possible MS. Three-quarters of the MS patients with no history of visual symptoms had abnormal fields. The commonest defect found was an arcuate scotoma. As a diagnostic test of visual pathway involvement in MS, tangent screen examination compares favourably with more sophisticated methods.
A family is described whose members showed different clinical presentations of central core disease. In 4 members the myopathy was congenital and nonprogressive, but in 2, muscle weakness first developed in adult life. Two further members had raised CPK levels without muscle weakness. Muscle weakness in one of the late-onset cases increased over eighteen months. This was associated with evidence of loss of type 2 fibres and the appearance of type 3 fibres on muscle biopsy. It is suggested that central core disease involves reinnervation of type 2 fibres by type 1 axons, and that the clinical manifestation of the disease depends on the age of onset and rate of progression of this process.
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Forearm exercise in a patient with myophosphorylase deficiency resulted in abnormally high levels of hypoxanthine in the venous blood. The post-exercise hypoxanthine response may reflect ATP depletion in the muscle and provides a useful screening test in muscle diseases with abnormal energy metabolism.