Clinical and brain bioenergetics improvement with idebenone in a patient with Leber's hereditary optic neuropathy: a clinical and 31P-MRS study

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 st...

Full description

Saved in:
Bibliographic Details
Published in:Journal of the neurological sciences 1997-05, Vol.148 (1), p.25-31
Main Authors: Cortelli, Pietro, Montagna, Pasquale, Pierangeli, Giulia, Lodi, Raffaele, Barboni, Piero, Liguori, Rocco, Carelli, Valerio, Iotti, Stefano, Zaniol, Paolo, Lugaresi, Elio, Barbiroli, Bruno
Format: Article
Language:English
Subjects:
31-Phosphorus magnetic resonance spectroscopy ( 31P-MRS)
Adult
AIDS/HIV
Benzoquinones - administration & dosage
Biological and medical sciences
Brain - metabolism
Brain - physiopathology
Energy Metabolism - physiology
Follow-Up Studies
Humans
Hydrogen-Ion Concentration
Idebenone
Leber's hereditary optic neuropathy
Magnetic Resonance Spectroscopy
Male
Medical sciences
Mitochondria, Muscle - drug effects
Mitochondrion
Muscle, Skeletal - metabolism
Muscle, Skeletal - physiopathology
Neurologic Examination
Neuropharmacology
Optic Atrophies, Hereditary - drug therapy
Optic Atrophies, Hereditary - metabolism
Paraparesis
Paraparesis, Tropical Spastic - drug therapy
Paraparesis, Tropical Spastic - metabolism
Pharmacology. Drug treatments
Phosphorus Isotopes
Psychoanaleptics: cns stimulant, antidepressant agent, nootropic agent, mood stabilizer..., (alzheimer disease)
Psychology. Psychoanalysis. Psychiatry
Psychopharmacology
Ubiquinone - analogs & derivatives
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0022-510X
1878-5883
DOI:10.1016/S0022-510X(96)00311-5