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Konzo: a distinct disease entity with selective upper motor neuron damage
T Tylleskär1, W P Howlett, H T Rwiza
1Department of Pediatrics, University Hospital, Uppsala, Sweden.
Journal of Neurology, Neurosurgery, and Psychiatry
|June 1, 1993
Summary
Konzo, a neurological disorder, caused severe spastic paraparesis in two Tanzanian patients due to cyanide exposure from bitter cassava. This distinct disease entity selectively damages upper motor neurons.
Area of Science:
- Neurology
- Toxicology
- Nutritional Science
Background:
- Konzo is a distinct neurological disease characterized by spastic paraparesis.
- Outbreaks have been linked to cyanide exposure from improperly processed cassava.
Observation:
- Two Tanzanian patients presented with severe, non-progressive spastic paraparesis after a konzo epidemic.
- High dietary cyanide intake from bitter cassava consumption preceded symptom onset.
Findings:
- Magnetic brain stimulation revealed absent motor evoked potentials, even in mildly affected limbs.
- Brain and spinal cord MRI scans were normal, indicating a specific type of upper motor neuron damage.
- Central visual field defects were observed in the more severely affected patient.
Implications:
- Konzo represents a unique neurological condition with selective upper motor neuron damage.
- Findings underscore the critical need for proper cassava processing to prevent cyanide toxicity.
- This research contributes to understanding the pathogenesis of konzo and its neurotoxic effects.