Clinical and brain
bioenergetics improvement with idebenone in a
patient with Leber's hereditary optic neuropathy: a clinical and 31P-MRS
study.
Cortelli P, Montagna P, Pierangeli G, Lodi R, Barboni P,
Liguori R, Carelli V, Iotti S, Zaniol P, Lugaresi E, Barbiroli B.
Institute of Neurology,
University of Bologna, Italy.
cortelli@unibo.it
J Neurol Sci 1997 May 1;148(1):25-31
Abstract
We used phosphorus magnetic
resonance spectroscopy (31P-MRS) to study in vivo brain and muscle
bioenergetics in a male patient with Leber's hereditary optic neuropathy (LHON)
and mtDNA mutation at 11,778 bp who developed spastic paraparesis with white
matter lesions on brain MR imaging. The study was performed before and during
treatment with idebenone (135 mg t.i.d.) and after withdrawal. Clinical
amelioration and worsening were associated with parallel changes in brain and
skeletal muscle bioenergetics following the administration or withdrawal of
idebenone. Reversal of paraparesis by idebenone was paralleled by
normalization of 31P-MRS, serum lactate and central motor conduction. Extra-ocular neurological dysfunction in LHON may be amenable to treatment by
appropriate quinones.
