St George Hospital
Publishes on Muscle Physiology and Disorders, Neurogenetic and Muscular Disorders Research, Inflammatory Myopathies and Dermatomyositis. 56 papers and 891 citations.
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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.
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.
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.