R O McKeran

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A case of type 1 (adult) Gaucher's disease with a late onset tapeto-retinal degeneration and an initially dopamine responsive extrapyramidal syndrome is described. The literature reporting neurological involvement in type 1 Gaucher's disease is reviewed, and it is concluded that the absence of symptoms and signs of nervous system involvement cannot be used(More)
Nine patients with myxoedema and carpal tunnel syndrome have been studied clinically and electrophysiologically to determine the presence or absence of the tarsal tunnel syndrome. Four patients had electrophysiological evidence of the tarsal tunnel syndrome, three of whom were mildly symptomatic. This would suggest that the tarsal tunnel syndrome is(More)
Myofibrillar protein catabolic rate was calculated in seven patients with Duchenne muscular dystrophy from the amount of 3-methylhistidine excreted in the urine, and found to be over three times that found in a control series when expresses as the percentage of myofibrillar protein catabolised per day. It is suggested that measurement of myofibrillar(More)
A 30-year-old patient with cerebrotendinous xanthomatosis was studied over a 6-year period. The clinical manifestations were cataracts, intellectual deterioration, ataxia, palatal and pharyngeal myoclonus, corticospinal tract damage and an electrophysiologically demonstrated sensorimotor peripheral neuropathy. Peripheral motor and sensory nerve conduction(More)
A review of the United Kingdom (UK) multiple sclerosis (MS) literature suggests that over the last three decades prevalence and estimated incidence rates have increased, while mortality rates have been declining. UK mortality data over a 30 year period have been studied to examine temporal and geographical variations, to estimate changes in survival, and to(More)
Serial percutaneous needle muscle biopsies of vastus lateralis were studied in 10 patients who had varying degrees of hypothyroid myopathy. The biopsies were taken before and during treatment with l-thyroxine. Before treatment the most severely clinically affected patients showed type II muscle fibre atrophy and loss, together with increased central nuclear(More)
After intravenous injection of therapeutic doses of bleomycin only small amounts could be measured in glioma tissue obtained at operation in patients with malignant gliomas and the drug was rapidly cleared from the blood (T1/2 = 2 hrs). Negatively charged liposomes injected through Ommaya reservoirs into the glioma tumour bed were tolerated without(More)
Myofibrillar protein catabolism has been calculated in a variety of neuromuscular diseases from the amount of 3-methylhistidine excreted in the urine. It was found to be significantly raised in Duchenne type muscular dystrophy, motor neurone disease, polymyositis, and thyrotoxic myopathy. In Becker type muscular dystrophy the level was slightly raised. It(More)