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[Raxone in the Leber optical neuropathy: Parisian experience].
J Fr Ophtalmol 2019; 42(3):269-275JF

Abstract

INTRODUCTION

Leber's Hereditary Optic Neuropathy (LHON) causes a rapid and severe decrease in visual acuity. Raxone® (Idebenone, Santhera) is the only drug to have a European Marketing Authorization for the treatment of this optic neuropathy. It can be proposed in the first months after the onset of this optic neuropathy, according to an international consensus meeting.

PATIENTS AND METHODS

Retrospective study of the efficacy of Raxone® on the visual acuity of patients with genetically confirmed LHON who were followed in four Parisian hospitals. The primary endpoint is the best recovery of LogMar visual acuity between baseline and the end of follow-up. The secondary endpoints are the evolution of LogMar visual acuity of the best eye at baseline and change in LogMar visual acuity for each eye considered separately.

RESULTS

Seventeen patients, three women and 14 men, mean age 34.2 years, naive to treatment with Raxone® were included in this study. The mean duration of treatment was 11.0±6.6 months. A mitochondrial DNA mutation was found in all patients. Only 2 had the 14484 mutation. A recovery of better LogMar visual acuity was found at the end of the treatment for 4 eyes (23.5 %), and a deterioration was observed for 8 (47.0 %). Only 2 eyes (11.7 %) with the best visual acuity at baseline improved. On the other hand, 17.6 % of the eyes considered separately had an improvement in their LogMar visual acuity at the end of the treatment.

CONCLUSION

The results confirm the trend of Raxone® treatment to improve patients' visual acuity. Given the recommendations of a consensus conference, this treatment should be started early after the onset of LHON. It is therefore important to look for this diagnosis in the presence of any hereditary optic neuropathy, in order to be able to initiate this treatment.

Authors+Show Affiliations

UF d'ophtalmologie, CRMR Ophtara, HUPO/HEGP, AP-HP, 20, rue Leblanc, 75015 Paris, France. Electronic address: christophe.orssaud@aphp.fr.Fondation ophtalmologique A-de-Rothschild, 75019 Paris, France; Service d'ophtalmologie, CRMR Ophtara, hôpital de la Pitiè-Salpétrière, AP-HP, 75013 Paris, France.Fondation ophtalmologique A-de-Rothschild, 75019 Paris, France.Service d'ophtalmologie, CRMR Ophtara, hôpital Necker-Enfants-Malades, AP-HP, 75015 Paris, France.Service d'ophtalmologie, CRMR Ophtara, hôpital de la Pitiè-Salpétrière, AP-HP, 75013 Paris, France.Fondation ophtalmologique A-de-Rothschild, 75019 Paris, France; CRMR maladies neuro rétiniennes, Centre nationale d'ophtalmologie des Quinze-Vingts, 75012 Paris, France.

Pub Type(s)

Journal Article

Language

fre

PubMed ID

30712826

Citation

Orssaud, C, et al. "[Raxone in the Leber Optical Neuropathy: Parisian Experience]." Journal Francais D'ophtalmologie, vol. 42, no. 3, 2019, pp. 269-275.
Orssaud C, Bidot S, Lamirel C, et al. [Raxone in the Leber optical neuropathy: Parisian experience]. J Fr Ophtalmol. 2019;42(3):269-275.
Orssaud, C., Bidot, S., Lamirel, C., Brémond Gignac, D., Touitou, V., & Vignal, C. (2019). [Raxone in the Leber optical neuropathy: Parisian experience]. Journal Francais D'ophtalmologie, 42(3), pp. 269-275. doi:10.1016/j.jfo.2018.06.010.
Orssaud C, et al. [Raxone in the Leber Optical Neuropathy: Parisian Experience]. J Fr Ophtalmol. 2019;42(3):269-275. PubMed PMID: 30712826.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Raxone in the Leber optical neuropathy: Parisian experience]. AU - Orssaud,C, AU - Bidot,S, AU - Lamirel,C, AU - Brémond Gignac,D, AU - Touitou,V, AU - Vignal,C, Y1 - 2019/02/01/ PY - 2018/03/15/received PY - 2018/06/19/revised PY - 2018/06/27/accepted PY - 2019/2/5/pubmed PY - 2019/9/17/medline PY - 2019/2/5/entrez KW - Acuité visuelle KW - Idebenone KW - Idébénone KW - Leber's hereditary optic neuropathy KW - Neuropathie optique héréditaire de Leber KW - Traitement KW - Treatment KW - Visual acuity SP - 269 EP - 275 JF - Journal francais d'ophtalmologie JO - J Fr Ophtalmol VL - 42 IS - 3 N2 - INTRODUCTION: Leber's Hereditary Optic Neuropathy (LHON) causes a rapid and severe decrease in visual acuity. Raxone® (Idebenone, Santhera) is the only drug to have a European Marketing Authorization for the treatment of this optic neuropathy. It can be proposed in the first months after the onset of this optic neuropathy, according to an international consensus meeting. PATIENTS AND METHODS: Retrospective study of the efficacy of Raxone® on the visual acuity of patients with genetically confirmed LHON who were followed in four Parisian hospitals. The primary endpoint is the best recovery of LogMar visual acuity between baseline and the end of follow-up. The secondary endpoints are the evolution of LogMar visual acuity of the best eye at baseline and change in LogMar visual acuity for each eye considered separately. RESULTS: Seventeen patients, three women and 14 men, mean age 34.2 years, naive to treatment with Raxone® were included in this study. The mean duration of treatment was 11.0±6.6 months. A mitochondrial DNA mutation was found in all patients. Only 2 had the 14484 mutation. A recovery of better LogMar visual acuity was found at the end of the treatment for 4 eyes (23.5 %), and a deterioration was observed for 8 (47.0 %). Only 2 eyes (11.7 %) with the best visual acuity at baseline improved. On the other hand, 17.6 % of the eyes considered separately had an improvement in their LogMar visual acuity at the end of the treatment. CONCLUSION: The results confirm the trend of Raxone® treatment to improve patients' visual acuity. Given the recommendations of a consensus conference, this treatment should be started early after the onset of LHON. It is therefore important to look for this diagnosis in the presence of any hereditary optic neuropathy, in order to be able to initiate this treatment. SN - 1773-0597 UR - https://www.unboundmedicine.com/medline/citation/30712826/[Raxone_in_the_Leber_optical_neuropathy:_Parisian_experience]_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0181-5512(18)30539-4 DB - PRIME DP - Unbound Medicine ER -