With increasing age, peripheral neuropathy becomes more common in multiple symmetric lipomatosis (MSL) and the principal cause of severe disability. High alcohol consumption is frequently associated and the peripheral neuropathy of MSL is often attributed to alcoholism. In this study, sural nerve biopsies from MSL patients revealed an absence of acute axonal degeneration, a significant shift to the left of myelinated fibre diameter distributions, reduced indices of axonal and nerve fibre circularity, and an increase in myelin periodicity. This pathology supports the view that the neuropathy of MSL is not alcohol-induced but that a chronic distal axonopathy is an integral part of the MSL syndrome. Biochemical observations suggest a defect in catecholamine-stimulated lipolysis in MSL at a membrane level, possibly in the amount or function of Gs membrane protein or in the catalytic unit of adenylate cyclase. Evidence is presented that the frequent association of MSL with alcoholism is on the basis of an additional ethanol-induced membrane lesion involving beta-adrenergic receptors.
Two patients presented with abdominal pain, recurrent vomiting, weight loss, and constipation secondary to intestinal pseudo-obstruction. Both patients had symptoms and signs of myasthenia gravis, acetylcholine receptor antibodies, and thymoma. In one patient inflammatory cell infiltrates and occasional degenerate neurons were found in the myenteric plexus. The gastrointestinal symptoms resolved during treatment with pyridostigmine. The close temporal relationship between the onset of the gastrointestinal symptoms and the detection of myasthenia gravis and thymoma suggests that intestinal pseudo-obstruction can be a paraneoplastic syndrome associated with thymoma.
Hypercalcaemia is associated with granulomatous disorders, including sarcoidosis' and tuberculosis.2'-The exact incidence of hypercalcaemia in tuberculosis is not known. We report a case of miliary tuberculosis and severe hypercalcaemia in which measurements of serum vitamin D3 concentrations were made. The probable mechanism of the hypercalcaemia is discussed. Case reportA 44 year old man, a Cook Islander and a non-smoker, presented with an eight month history of lethargy and malaise, a three month history of ascites, and a one month history of polyuria and polydipsia. Abdominal paracentesis had been performed at another hospital but the results of this were not available. Histological examination of a right cervical lymph node showed caseating granulomas typical of tuberculosis. Subsequent cultures of the lymph node, sputum, resting gastric juices, and early morning urine samples grew Mycobacterium tuberculosis, sensitive in vitro to isoniazid, rifampicin, and pyrazinamide.Initial biochemical investigation of the blood revealed hyponatraemia, hypercalcaemia (figure), and no evidence of abnormal renal function. An adrenocorticotrophic hormone stimulation test yielded normal results, as did tests of thyroid function. The serum parathyroid hormone concentration was 20-6 pmol/l, measured in a mid molecule radioimmunoassay (normal 6-0-315 pmol/l). Alkaline phosphatase activity was normal and a technetium-99m bone scan showed nothing to suggest malignant disease. A chest radiograph showed miliary shadowing with no focal changes. A computed tomographic scan of the abdomen revealed mesenteric lymphadenopathy, thickened omentum, and the presence of ascites. The liver, spleen, adrenal glands, and kidneys were unremarkable. An intravenous urogram was normal. There was no evidence of lymphoreticular malignancy.Vitamin D studies (figure) before lymph node biopsy showed depression of 25-hydroxy vitamin D3 (25-OHD3) concentrations with raised 1,25-dihydroxy vitamin D3 (1,25(OH)2D3) concentrations. Before lymph node biopsy he received an infusion of the diphosphonate APD (3-amino-l-hydroxy propylidene-1,I-biphosphonate) 0-3 mg/kg on two consecutive days, which produced a prompt fall in serum calcium concentration. After the diagnosis of Address for reprint requests: Professor I
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