Konzo, an epidemic upper motor neuron disease studied in Tanzania.

An epidemic of spastic paraparesis was studied in a drought-affected rural area of Tarime district in northern Tanzania. The uniform clinical findings in 39 cases, aged 4-46 yrs, indicated abrupt symmetric isolated and permanent but not progressive damage to the upper motor neurons. Due to the failure of other food crops, the diet at onset consisted almost exclusively of bitter cassava roots, a drought-tolerant starchy root crop widely cultivated in Africa. The drought increased the natural occurrence of cyanogenic glucosides in the cassava roots, and the processing procedure normally applied in order to remove cyanide before consumption was shortened because of food shortage. The resulting high dietary cyanide exposure was confirmed by very high serum levels of thiocyanate, the detoxification product of cyanide. Tests for HTLV-1 antibodies were negative and no other findings supported an infectious aetiology. The clinical findings and the associations with cassava toxicity are almost identical to those reported from outbreaks of spastic paraparesis in Mozambique and in Zaire, where this disease was first reported under the name 'konzo'. We thus conclude that konzo constitutes a distinct upper motor neuron disease entity, probably caused by a toxic effect from insufficiently processed cassava under adverse dietary circumstances.