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Biomedical subjects

E H Denys

Publications and source records attributed to E H Denys.

24 records · Page 2Linked to original sources

Amyotrophic lateral sclerosis. Impairment of neuromuscular transmission.

Neuromuscular transmission was studied in the ulnar-hypothenar group in 55 patients with amyotrophic lateral sclerosis. A decremental response was found in 67.0%. The decrement was larger and present more often in muscles showing atrophy. In addition, muscles with frequent fasciculations showed a larger decrement than the ones with rare fasciculation. A temperature effect similar to that in myasthenia gravis was observed, with a reduction of the decrement following local cooling of the muscles. Administration of edrophonium chloride improved the synaptic defect. Posttetanic exhaustion was observed as well. It is thought that the defect of neuromuscular transmission is due to a decreased trophic function of the neuron followed by morphological changes at the endplate.

Adolescent↗

Plasmapheresis and immunosuppressive drug therapy in myasthenia gravis.

Plasmapheresis combined with prednisone and azathioprine therapy produced striking clinical improvement in five patients with myasthenia gravis who still had moderate to severe disability despite thymectomy, high-dose prednisone therapy and optimal doses of cholinesterase inhibitors. Serial determinations of titers of serum antibody toward the acetylcholine receptor demonstrated a fall to 21 +/- 5 per cent (mean +/- S.D.) of the original levels concurrently with the patients' increasing strength. Clinically improved patients maintained lowered titers, whereas clinical relapses were associated with a rebound in titer. Our results suggest that plasmapheresis will find a place in the management of patients with myasthenia gravis, and they implicate antibodies to acetylcholine receptor as a pathogenic factor in this disease.

Acetylcholine↗