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International Health
another two subjects. Six of the patients with abnormal VEPs had normal Concentric needle electromyography (EMG) was normal in all investigated
visual acuity. The mean P100 latency was significantly increased and the patients (n=21), with no neurogenic signs. Muscle biopsies from five
P100 amplitude significantly decreased in konzo patients compared with konzo patients revealed non-specific changes secondary to muscle waste.
healthy controls. The abnormalities in konzo patients do not correlate to
duration or severity of konzo. Comments on the Neurophysiological Findings in Konzo
Although the neurophysiological investigations indicate a more widely
Nerve Conduction Studies, Electromyography and distributed cortical and subcortical dysfunction in konzo, the exact site
Muscle Biopsy of lesion in konzo remains a challenge. The predominant motor
In 1998, the first study of peripheral nerves in konzo subjects was features in konzo indicate a preponderant vulnerability in the
performed (not counting two patients who were investigated at motor cortex pyramidal cells; however, whether the lesion is pre-
the Neurophysiological Laboratory in Uppsala at the beginning of the synaptic, neuronal or post-synaptic, or a combination of these, is
1990s). Twenty-one konzo subjects nine to 49 years of age (median 17 unclear. Abnormal MEPs, SEPs and VEPs have also been described in
years, mean 22 years) participated in the study. Surprisingly, other upper MNDs. Further studies, in regard to both neuropathology,
considering that the konzo subjects were malnourished, no patient effects on cellular level of cyanogenic compounds and their
showed any signs of polyneuropathy.
45
Motor and sensory nerve metabolites, and MEPs of various kinds, are needed to further our
conductions were normal. understanding of the pathogenesis of konzo. ■
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