Biomedical subjects
Publications and source records attributed to W J Cumming.
The case of a patient with leptospirosis (treated with trimethoprim) with late neurological complications manifesting as a bilateral plexus syndrome is described. The probable reasons for the continued weakness, in this patient, of the muscles supplied by the anterior interosseus nerve, despite improvement in the proximal muscles, are briefly discussed.
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Three cases of a painful, inflammatory, nodular myopathy in young and middle-aged adult males are described. In two of the cases the nodular process gradually evolved into a diffuse proximal myopathy accompanied by dysphagia, and manifesting the so-called "facioscapulohumeral syndrome". The electromyogram (EMG) in all three cases was "myopathic" in type and in two cases abnormalities consistent with an inflammatory muscle disorder were seen. Muscle biopsy revealed a consistent pattern of intense, predominantly interstitial, pleomorphic infiltration of muscle with scattered foci of muscle fibre destruction and regeneration, which resembled muscle infarcts. It is suggested that this syndrome, whilst readily recognizable from both the clinical and pathological standpoints, is not a nosological entity in its own right but rather an unusual presentation of polymyositis.
An increasing number of cases of myopathy due to disordered lipid metabolism have recently been recognised and these appear to fall into two contrasting clinical and biochemical categories. Some patients present with steadily prograssive proximal weakness which sometimes responds to steroid therapy and which is due to carnitine deficiency in skeletal muscle. In the second category, patients usually present with muscle cramps on exertion then followed by myoglobinuria without established proximal myopathy and their symptoms are associated with deficiency of carnitine palmityl transferase in skeletal muscle. In this paper we report a patient who has well-documented carnitine palmityl transferase deficiency, whose symptoms were triggered by violent exercise after fasting and in whom there was variable histochemical and ultrastructural evidence of lipid accumulation in muscle biopsy samples. Development of the patient's symptoms was suppressed by a high carbohydrate diet.
The case of a previously health 17-year-old youth who presented a fatal cerebral infarct is described. A localized defect in the anterior left ventricular myocardium with overlying thrombus, and left ventricular hypertrophy were found at necropsy. An old infarct was found in the left kidney. There were no signs of ischaemic or inflammatory heart disease. The possible origins of the lesion are discussed.
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Rats were subjected to 3,500 r of X-irradiation in a single dose while breathing oxygen at 1 ATM pressure. Comparison was made between the delayed effects of irradiating thoracic, lumbar, and the cauda equina fields. The lumbar field involved the alpha-motoneurons and spinal roots supplying the sciatic nerve, while the cauda equina field involved these spinal roots but spared the alpha-motoneurons in the spinal cord. Thoracic irradiation produced paraplegia after an interval of 127-150 days. In the irradiated zone, the spinal cord was severely damaged, but the thoracic spinal roots were spared. Lumbar irradiation produced paraplegia after an interval of 83-211 days. In the irradiated zone, the alpha-motoneurons were largely spared, the spinal cord showed mild to moderate white matter damage, but the most severe damage was of the lumbosacral spinal roots. The posterior roots were more affected than the anterior. In longer interval cases the degeneration of the roots appeared to be due to focal devitalization. Evidence is advanced that root degeneration had been progressing for at least 4 weeks before the onset of paraplegia. In the cauda equina series the lumbosacral spinal root changes were similar to those in the lumbar series. This study indicates that different levels of the neuraxis have different degrees of susceptibility to X-irradiation. The thoracic cord appears more susceptible than the lumbosacral; the lumbosacral roots appear more susceptible than the thoracic; the posterior roots are more susceptible than the anterior. These findings may have relevance to the study of radiation damage in man, even though the dose schedule used in this experimental study differs greatly from that used for radiotherapy.
A middle-aged man presented with a thirty-year history of progressive, asymmetrical limb-girdle weakness. The muscle biopsy revealed a vacuolar myopathy. The vacuoles which did not disrupt the fibre outline, lay in a subsarcolemmal position. They were PAS-positive and the material was partially resistant to diastase digestion. Electron microscopy showed the vacuoles to contain free unbound glycogen with filamentous material. Leucocyte brancher enzyme activity was normal but the muscle activity was less than half the control value. Histochemical and ultrastructural characteristics of the storage material resemble the amylopectin polysaccharide deposits seen in childhood Type IV glycogenosis.
It has previously been suggested that the pathological abnormalities seen in muscle biopsies from patients with motor neuron disease (MND) are of predictive value in relation to the rate of progression of the disease. In this study, quadriceps muscle biopsies from 19 patients with MND and 20 age matched controls were prepared for histochemistry and analysed morphometrically. Pathological features of denervation and reinnervation were observed in all MND patients although considerable variation between patients was noted. Motor neuron disease biopsies also showed increased connective tissue, an increased variation in fibre size, and a random fibre type distribution. Several of these abnormalities were more severe in female patients. Many of these 'abnormalities' were also frequent, albeit to a milder degree, in control biopsies and emphasize the need for age matched controls. The morphometric data was not related to the age of the patient, disease duration, type of MND or muscle strength, thus suggesting that the progression and severity of MND and its prognosis cannot be judged on the basis of quadriceps muscle pathology alone.