A 36-year-old white patient is described. He received treatment for hypertension and showed slightly increased excretion of 17-OHCS- and 17-ketosteroids but no increase in values for 3-methoxy-4-hydroxymandelic acid in the urine. He was admitted to hospital for a myocardial infarction, which was found to be situated in the anterior wall. During his stay in hospital a sudden increase in blood pressure occurred, together with a typical attach of perspiration, loss of consciousness, and ventricular fibrillation. The assay by 3-methoxy-4-hydroxymandelic acid now showed markedly increased amounts. A phaeochromocytoma was thought to be the most probably diagnosis, but now withstanding therapy the patient died from cerebral lesions. At necropsy a recent anteroseptal myocardial infarction and some minor lesions were found but no tumour and notably no phaechromocytoma, neither in the adrenals nor elsewhere. Using Dobbie's morphometric technique, as described by Munro Neville (1969), changes in the adrenals were demonstrated, which were considered to represent primary adrenal medullary hyperplasia. Criteria for the diagnosis of this syndrome are discussed. Until now it had been presumed to be present in a number of cases but never convincingly demonstrated.
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