Polyneuritis in Haemochromatosis.

Case Report A male taxi-driver, aged forty-seven, was admitted to the Dorset County Hospital on 7.9.60. He was a diabetic of five years standing on I8o g. of carbohydrate and 8o units of lente insulin daily. Following an attack of diarrhaea and vomiting he gave a five days' history of increasing weakness in the legs. Examination. He was thin, slightly icteric, with sparse axillary and pubic hair, small atrophic testes and multiple pigmented needle-track marks on the lower abdomen and thighs. The liver was large and hard, and the spleen just palpable. He had a flaccid weakness of both legs with tender muscles and absent knee and ankle jerks. This subsequently developed into a frank paraplegia. There were no sensory changes during the early stages. Investigations. Blood count: Hb 88%, MCHC 36%, PCV 36 mm., ESR 12 mm. (Wintrobe). Icteric Index 20 units, wbc 12, 900 (neutrophils 75%, monocytes 9%, lymphocytes i6%, marked toxic changes in the neutrophils). Prothrombin time 50% of normal. The blood culture grew E. Coli. The serum iron was 440 l±g./ioo ml., the S.G.P.T. was 372 units, and the serum bilirubin 2.9 mg./1oo ml., (direct I.3 mg./ioo ml.). The total protein was 5.7 g./ I00 ml., of which the albumin was markedly decreased and the alpha-2 and gamma globulin increased. The CSF was slightly xanthrochromic; there were 34 wbcs (mainly polymorphonuclears), and the protein was I50 mg./ioo ml. A diagnosis of heemochromatosis and acute polyneuritis was made. Progress and Treatment. His temperature rose to ioz2.5F and his pulse to Is5/min. and there were signs of early meningitis. He was treated with prednisolone in high dosage, chloramphenicol and later tetracycline. His condition deteriorated, and he died on 24.9.60. Autopsy Report. There were bilateral pleural eff-usions. The liver was enlarged and weighed I,652 g.; it was very firm and showed irregular cirrhosis; there were one or two small abscesses. The spleen was enlarged, weighing 364 g. It was firm, and on section showed decreased prominence of the follicles. The pancreas was firm and slatey-grey in colour. There was a turbid ascites present. Histology showed heavy deposits of iron in the cardiac muscle and slight deposition in the zona glomerulosa of the adrenal cortex, the pars anterior ofthe pituitary gland and in the dermis at the site of previous insulin injections. There was no histological abnormality found in the spinal cord,; but the peripheral nerves were not examined. The pathologist's cause of death was:(i) heart failure, (ii) E. coli septicxmia, (iii) hmmochromatosis.