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Biomedical subjects

W J Cumming

Publications and source records attributed to W J Cumming.

At least 19 recordsLinked to original sources
PubMed1992

Myelitis and toxic, inflammatory and infectious disorders.

Developments in our understanding of the anatomical-clinical and pathological correlates of spinal cord disorders have greatly enhanced our understanding of the clinical presentation. The impact of viral disease on the spinal cord is reviewed. The value of magnetic resonance (MR) scanning of the brain and spinal cord in the evaluation, both in the short and long term, of patients with myelopathy is underlined.

HIV Infections↗
PubMed1990

Histomorphometric and histopathological study of the human cricopharyngeus muscle: in health and in motor neuron disease.

Abnormalities in muscle histology have been reported frequently for the cricopharyngeus muscle of patients with oculopharyngeal muscular dystrophy, motor neuron disease and other neurological disorders in which dysphagia is a common clinical sign. However, there are few detailed reports of the normal structure of this muscle nor quantitative baseline data with which to compare the diseased state. In this study, cricopharyngeus muscles from 21 healthy individuals and four patients with motor neuron disease underwent quantitative histological and histochemical examination. In addition to the extensive connective tissue content (40%), comprising abundant elastic fibres, cricopharyngeus muscles from normal individuals possessed small calibre striated muscle fibres (mean narrow diameter 30 microns) of widely varying size (coefficient of variation 41%). The majority of fibres were histochemically type I (82%) and highly oxidative. All muscles comprised numerous muscle fibres with aberrant histological and histochemical features (internalized nuclei, 'ragged red' crescents, splits, degenerating fibres, 'moth-eaten' fibres, or nemaline rods.) The histomorphometric and histopathological features were similar in males and females and some showed a correlation with age. There were increases in fibre size and roundedness and decreases in the numerical density and percentage of type I and split fibres in the specimens from older individuals. Cricopharyngeus muscles from patients with motor neuron disease were not significantly different from the controls for most parameters. It is therefore suggested that previous descriptions of specific cricopharyngeal pathology accompanying neuromuscular disease or dysphagia be interpreted with caution. The importance of obtaining normal structural, morphometric and histopathological data from muscles other than the usually biopsied limb muscles, is emphasized.

Adult↗
PubMed1990

A clinicopathological study of the paraneoplastic neuromuscular syndromes associated with lung cancer.

The highest incidence of remote neuromuscular disorders in cancer has previously been reported in lung carcinoma. The clinical incidence of neuromuscular disorder was estimated and correlated with muscle histology and the histological type of lung tumour in 100 patients with lung carcinoma who were studied prospectively. Thirty-five patients had small cell carcinoma and 65 patients non-small cell lung cancer. Clinically, 33 patients had a polymyopathy, of whom 18 had a cachectic myopathy and 15 had a proximal myopathy (two patients had Lambert-Eaton myasthenic syndrome, one presented with dermatomyositis and one had evidence of ectopic ACTH production). Cachexia was more common in non-small cell cancer; proximal myopathy was more common in small cell cancer. Ninety-nine patients had abnormal muscle histology; 74 had type II atrophy, 12 had type I and II atrophy, one had type I atrophy and 12 had necrosis. The majority of patients were affected sub-clinically and the clinical entities of cachectic and proximal myopathy did not correspond to previous pathological classifications. Atrophy was not related to the duration of tumour symptoms, ageing, clinical type of myopathy or histological type of lung tumour, and was statistically different from that seen in controls. Qualitatively, the presence of weight loss, muscle wasting and metastatic disease were not factors in the development of atrophy. Similarly, necrosis was not related to the type of lung tumour, the presence of metastases, ageing, weight loss, muscle wasting, duration of tumour symptoms or the clinical form of myopathy. This study demonstrates that lung carcinoma has a direct effect on the motor unit, including atrophy, a necrobiotic myopathy and Lambert-Eaton myasthenic syndrome. Clinical assessment does not accurately assess the 'remote' neuromuscular effects of cancer on the motor unit.

Adult↗
PubMed1989

Familial thrombocytopenia and myopathy.

We report a family in which a combination of myopathy and thrombocytopenia is transmitted as an autosomal dominant characteristic through three generations. The myopathy has unusual histological features with type II fibre atrophy and vacuolation while the thrombocytopenia appears to be due to a defect in megakaryocyte maturation, platelet morphology and function being normal. The haematological findings in such a family have not been described previously.

Adult↗
PubMed1989

Linkage analysis in the spinal muscular atrophy type of facioscapulohumeral disease.

Facioscapulohumeral disease is probably a heterogeneous disorder. We have ascertained and sampled two multigeneration families with the neurogenic form of this disorder, considered to be a type of spinal muscular atrophy (FSHSMA). The two families have 36 affected members. Linkage studies with 10 expressed and seven DNA restriction fragment length polymorphism (RFLP) markers failed to show significant linkage (Zmax greater than or equal to 3.00). However, two areas of probable linkage were defined on chromosomes 1p and 4q with the markers MNS (Zmax = 1.47 at theta max = 0.10) and PGM1 (Zmax = 0.94 at theta max = 0.001) respectively. We are using additional RFLPs from these and other areas of the human genome to screen these families for linkage to FSHSMA.

Chromosome Mapping↗
PubMed1989

An unusual familial muscle disorder.

Autosomal dominant inheritance of an unusual muscle disease is reported in a family. The pathological appearance, of regularly arranged markedly atrophic muscle fibres without other evidence of disturbed innervation, are similar in each case. However, the onset of the disease, its distribution and its progression has varied within the family.

Adolescent↗
PubMed1988

CT muscle scanning in the evaluation of patients with spinal muscular atrophy (SMA).

One hundred patients with spinal muscular atrophy (SMA) were assessed by CT scanning using a standardised technique. The spectrum of CT abnormality occurring in SMA was observed and by overall analysis the patients were divided into 4 groups. While the CT appearances of these groups correlated well with clinical assessment of severity of disease, the disease process was usually much more widespread than clinical examination suggested. CT abnormality was first observed in the leg and gluteal muscles, progressing to the posterior spinal, thigh, shoulder girdle and sternomastoid muscles. Hypertrophy of sartorius and gracilis was observed in a significant number of patients. Fascial planes were preserved in involved muscles in over half of the patients, even in late-stage disease. Asymmetrical muscle involvement was seen with increasing frequency as the disease process increased in extent as evaluated by CT scanning. There was no discernible difference in the CT appearances in those patients who clinically had limb-girdle, facioscapulohumeral or scapuloperoneal distribution of weakness.

Adolescent↗
PubMed1988

Familial myopathy associated with thrombocytopenia: a clinical and histomorphometric study.

We describe an unusual vacuolar myopathy with tubular aggregates in a mother and son from a family presenting with a slowly progressive, predominantly limb girdle, weakness and distal upper limb weakness in association with reduced blood clotting ability. To our knowledge this is the first report of a familial clinical defect of both muscle and platelets in the same individuals. The possibility that the primary defect may be due to an abnormality of the tubular intramembranous systems in muscle cells and platelet precursors is discussed.

Adolescent↗
PubMed1987

Review of coracoclavicular ligament reconstruction using Dacron graft material.

Operative management of Grade III acromioclavicular dislocations in young, active patients is a controversial issue with regard to operative management. Nine patients with complete dislocation (Allman Grade III) of the acromioclavicular joint who had undergone reconstruction of the coracoclavicular ligament with woven Dacron arterial grafts were reviewed clinically, radiologically and functionally utilizing an isokinetic dynamometer. Average follow-up was 2 years and 7 months. All results were good or excellent in terms of function, range of movement and patient acceptance. One patient developed a transient brachial plexus lesion due to compression caused by exuberant heterotopic calcification in the region of the synthetic ligament. One other exceptional patient continued to get more than occasional discomfort. It is concluded that Dacron loop repair is a relatively simple surgical technique with minimal morbidity and high efficacy in restoring stability and function in high demand patients.

Acromioclavicular Joint↗
PubMed1987

Subacute myelopathy caused by spinal venous infarction.

A 44 year old female presented with a subacute myelopathy in association with pelvic venous thrombosis. It is inferred from the temporal relationship of these events that the patient suffered a subacute spinal venous infarction. This is discussed along with the aetiology, anatomical distribution and management of the condition.

Adult↗
PubMed1979

Acute intoxication with sodium valproate.

Two epileptic patients developed an acute toxic encephalopathy consisting of altered behavior, deteriorating seizure control, and confusion while taking sodium valproate alone. Serum valproate levels were greater than 100 microgram/ml in both. Toxic symptoms resolved when the dose of valproate was reduced, with a consequent fall in serum concentration of the drug.

Acute Disease↗