KONZO - A DISTINCT DISEASE ENTITY WITH SELECTIVE UPPER MOTOR-NEURON DAMAGE

Two Tanzanian patients with konzo were severely disabled by a non-prog ressive spastic paraparesis, since the sudden onset during an epidemic six years earlier. At the time of onset they had a high dietary intak e of cyanide from exclusive consumption of insufficiently processed bi tter cassava roots. MRI of brain and spinal cord were normal but motor evoked potentials on magnetic brain stimulation were absent, even in the only slightly affected upper limbs. Other neurophysiological inves tigations were largely normal but the more affected patient had centra l visual field defects. Konzo is a distinct disease entity with select ive type upper motor neuron damage.