Clinical and brain bioenergetics improvement wiuth idebenone in a patient with Leber's hereditary optic neuropathy

A clinical and 31P-MRS study

Pietro Cortelli, Pasquale Montagna, Giulia Pierangeli, Raffaele Lodi, Piero Barboni, Rocco Liguori, Valerio Carelli, Stefano Iotti, Paolo Zaniol, Elio Lugaresi, Bruno Barbiroli

Research output: Contribution to journalArticle

69 Citations (Scopus)

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 11778 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.

Original languageEnglish
Pages (from-to)25-31
Number of pages7
JournalJournal of the Neurological Sciences
Volume148
Issue number1
DOIs
Publication statusPublished - May 1 1997

Fingerprint

Leber's Hereditary Optic Atrophy
Energy Metabolism
Brain
Spastic Paraparesis
Paraparesis
Quinones
Mitochondrial DNA
Neuroimaging
Phosphorus
Lactic Acid
Skeletal Muscle
Magnetic Resonance Spectroscopy
Muscles
Mutation
Therapeutics
Serum
idebenone

Keywords

  • 31-phosphorus magnetic resonance spectroscopy (-P-MRS)
  • idebenone
  • Leber's hereditary optic neuropathy
  • mitochondrion
  • paraparesis

ASJC Scopus subject areas

  • Ageing
  • Clinical Neurology
  • Surgery
  • Developmental Neuroscience
  • Neurology
  • Neuroscience(all)

Cite this

Clinical and brain bioenergetics improvement wiuth idebenone in a patient with Leber's hereditary optic neuropathy : A clinical and 31P-MRS study. / Cortelli, Pietro; Montagna, Pasquale; Pierangeli, Giulia; Lodi, Raffaele; Barboni, Piero; Liguori, Rocco; Carelli, Valerio; Iotti, Stefano; Zaniol, Paolo; Lugaresi, Elio; Barbiroli, Bruno.

In: Journal of the Neurological Sciences, Vol. 148, No. 1, 01.05.1997, p. 25-31.

Research output: Contribution to journalArticle

Cortelli, Pietro ; Montagna, Pasquale ; Pierangeli, Giulia ; Lodi, Raffaele ; Barboni, Piero ; Liguori, Rocco ; Carelli, Valerio ; Iotti, Stefano ; Zaniol, Paolo ; Lugaresi, Elio ; Barbiroli, Bruno. / Clinical and brain bioenergetics improvement wiuth idebenone in a patient with Leber's hereditary optic neuropathy : A clinical and 31P-MRS study. In: Journal of the Neurological Sciences. 1997 ; Vol. 148, No. 1. pp. 25-31.
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