CASSAVA CYANOGENS AND KONZO, AN UPPER MOTONEURON DISEASE FOUND IN AFRICA

Konzo is a distinct form of tropical myelopathy characterised by abrupt onset of spastic paraparesis. Epidemics in East Africa have been attributed to dietary cyanide exposure from insufficiently processed cassava but a study done in Zaire disputed such an aetiology. We investigated a konzo-affected population in rural Zaire and measured the cyanogen content of cassava flour, determined urinary thiocyanate as an indicator of cyanide intake, and compared blood cyanide concentrations in cases and controls. The affected population consumed flour made from short-soaked (one day) cassava roots and thus had high dietary cyanide exposure (urinary thiocyanate in 31 children = 757-mu-mol/l) compared with the unaffected population (urinary thiocyanate in 46 children = 50-mu-mol/l) that ate cassava that had been soaked for three days before consumption. 3 konzo patients, but only 2 of 23 controls, had blood cyanide concentrations above 4-mu-mol/l (p < 0.01), although serum thiocyanate concentrations were similar. Our findings indicate a causal role in konzo of sustained high blood cyanide concentrations maintained by a deficient sulphur intake impairing cyanide to thiocyanate conversion. The underlying causes of konzo are poverty and food shortage, but a minor improvement of food processing may, as in beri-beri, be preventive.