Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr.
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J. Neurol. Neurosurg. Psychiatr. · Aug 2010
Preserved motor axonal membrane potential in mitochondrial disease.
Mitochondrial disorders are characterised by protean neurological manifestations including peripheral neuropathy. The neuropathy is typically an axonal process, with a proposed mechanism being degradation of the membrane potential of the axon due to dysfunction of energy-dependent Na(+)/K(+) ATPase pump. ⋯ Strength--duration time constant, threshold electrotonus, current--threshold relationship and recovery cycle were identical to control values. These findings imply that an alternative mechanism to a change in membrane potential is responsible for neuronal injury in these patients.