Leber's Hereditary Optic Neuropathy

Changes in Visual Function and Correlations with Inner Retinal Structure in Acute and Chronic Leber’s Hereditary Optic Neuropathy Patients after Treatment with Idebenone

“…Similarly, the mean increase in VA in early chronic patients could also be attributed to a spontaneous recovery, which is seen in some LHON patients within the first 5 years. However, the CRI observed in both m.11778G>A and three of four m.3460G>A patients of the early chronic group indicates a higher recovery rate under treatment than the previously reported spontaneous recovery rates of 4-25% in the m.11778G>A and 11-40% in the m.3460G>A genotypes [2,[19][20][21][22][23]. Finally, a coincidental spontaneous improvement at a rate as observed in six of seven late chronic patients seems even more unlikely.…”

Changes in Visual Function and Correlations with Inner Retinal Structure in Acute and Chronic Leber’s Hereditary Optic Neuropathy Patients after Treatment with Idebenone

“…Similarly, the mean increase in VA in early chronic patients could also be attributed to a spontaneous recovery, which is seen in some LHON patients within the first 5 years. However, the CRI observed in both m.11778G>A and three of four m.3460G>A patients of the early chronic group indicates a higher recovery rate under treatment than the previously reported spontaneous recovery rates of 4-25% in the m.11778G>A and 11-40% in the m.3460G>A genotypes [2,[19][20][21][22][23]. Finally, a coincidental spontaneous improvement at a rate as observed in six of seven late chronic patients seems even more unlikely.…”

Therapeutic benefit of idebenone in patients with Leber hereditary optic neuropathy: The LEROS nonrandomized controlled trial

“…Although spontaneous recovery of vision in NH eyes carrying the m.11778G>A or m.14484T>C mutation was largely consistent with previous data, 3 , 5 , 25 , 34 , 35 it was more frequent in subacute/dynamic (but not chronic) m.3460G>A eyes (61.1% at 24 months vs. 15%–25% in previous reports). 3 , 5 , 34 , 35 , 36 , 37 Spontaneous recovery may be overrepresented in the NH cohort and could partly explain the apparent trend toward a negative treatment effect. It is feasible that this potentially “milder” disease course could also manifest as a reduced rate of CRW; however, this cannot be verified due to the lack of comparable data in the literature.…”

Grand Rounds: Could Occupational Exposure to n -Hexane and Other Solvents Precipitate Visual Failure in Leber Hereditary Optic Neuropathy?

“…Johns et al (1992) reported an isolated case of an LHON patient with occupational exposure to solvent fumes—a firefighter repeatedly exposed to xylene, toluene, and methylethylketone, as well as smoke and other fumes. It is reasonable to suppose that both in the case of this firefighter and in our patient, it may have been an acute exposure to solvents that triggered the onset of LHON.…”

Changes in Visual Function and Correlations with Inner Retinal Structure in Acute and Chronic Leber’s Hereditary Optic Neuropathy Patients after Treatment with Idebenone

“…Similarly, the mean increase in VA in early chronic patients could also be attributed to a spontaneous recovery, which is seen in some LHON patients within the first 5 years. However, the CRI observed in both m.11778G>A and three of four m.3460G>A patients of the early chronic group indicates a higher recovery rate under treatment than the previously reported spontaneous recovery rates of 4-25% in the m.11778G>A and 11-40% in the m.3460G>A genotypes [2,[19][20][21][22][23]. Finally, a coincidental spontaneous improvement at a rate as observed in six of seven late chronic patients seems even more unlikely.…”

Therapeutic benefit of idebenone in patients with Leber hereditary optic neuropathy: The LEROS nonrandomized controlled trial

“…Although spontaneous recovery of vision in NH eyes carrying the m.11778G>A or m.14484T>C mutation was largely consistent with previous data, 3 , 5 , 25 , 34 , 35 it was more frequent in subacute/dynamic (but not chronic) m.3460G>A eyes (61.1% at 24 months vs. 15%–25% in previous reports). 3 , 5 , 34 , 35 , 36 , 37 Spontaneous recovery may be overrepresented in the NH cohort and could partly explain the apparent trend toward a negative treatment effect. It is feasible that this potentially “milder” disease course could also manifest as a reduced rate of CRW; however, this cannot be verified due to the lack of comparable data in the literature.…”

Grand Rounds: Could Occupational Exposure to n -Hexane and Other Solvents Precipitate Visual Failure in Leber Hereditary Optic Neuropathy?

“…Johns et al (1992) reported an isolated case of an LHON patient with occupational exposure to solvent fumes—a firefighter repeatedly exposed to xylene, toluene, and methylethylketone, as well as smoke and other fumes. It is reasonable to suppose that both in the case of this firefighter and in our patient, it may have been an acute exposure to solvents that triggered the onset of LHON.…”

Changes in Visual Function and Correlations with Inner Retinal Structure in Acute and Chronic Leber’s Hereditary Optic Neuropathy Patients after Treatment with Idebenone

“…Similarly, the mean increase in VA in early chronic patients could also be attributed to a spontaneous recovery, which is seen in some LHON patients within the first 5 years. However, the CRI observed in both m.11778G>A and three of four m.3460G>A patients of the early chronic group indicates a higher recovery rate under treatment than the previously reported spontaneous recovery rates of 4-25% in the m.11778G>A and 11-40% in the m.3460G>A genotypes [2,[19][20][21][22][23]. Finally, a coincidental spontaneous improvement at a rate as observed in six of seven late chronic patients seems even more unlikely.…”

Therapeutic benefit of idebenone in patients with Leber hereditary optic neuropathy: The LEROS nonrandomized controlled trial

“…Although spontaneous recovery of vision in NH eyes carrying the m.11778G>A or m.14484T>C mutation was largely consistent with previous data, 3 , 5 , 25 , 34 , 35 it was more frequent in subacute/dynamic (but not chronic) m.3460G>A eyes (61.1% at 24 months vs. 15%–25% in previous reports). 3 , 5 , 34 , 35 , 36 , 37 Spontaneous recovery may be overrepresented in the NH cohort and could partly explain the apparent trend toward a negative treatment effect. It is feasible that this potentially “milder” disease course could also manifest as a reduced rate of CRW; however, this cannot be verified due to the lack of comparable data in the literature.…”

Grand Rounds: Could Occupational Exposure to n -Hexane and Other Solvents Precipitate Visual Failure in Leber Hereditary Optic Neuropathy?

“…Johns et al (1992) reported an isolated case of an LHON patient with occupational exposure to solvent fumes—a firefighter repeatedly exposed to xylene, toluene, and methylethylketone, as well as smoke and other fumes. It is reasonable to suppose that both in the case of this firefighter and in our patient, it may have been an acute exposure to solvents that triggered the onset of LHON.…”