Do people recover from LHON?
A small number of LHON affected people recover some of their eyesight without treatment.
You should be very careful when looking for information about recovery.
When medical papers talk about a recovery, they may mean a very small change in eyesight, or a change in a measurement like OCT with no noticeable improvement in eyesight.
The best researchers say what they mean by a recovery, such as “3 lines on a Snellen chart” or “15 letters on a ETDRS chart”.
Doctors call this “spontaneous” recovery.
Any recovery is usually in the first year or two after the Acute phase, but there are very rare cases of recovery after many years.
Judging recovery from LHON can be complicated by the way vision changes during and just after the Acute phase.
An affected person might also feel that they have more useful sight once they have had some emotionhal recovery and rehabilitation / orientation training.
Dr Patrick Yu-Waui-Man gives the following figures for recovery in the GeneReviews article on LHON.
G11778A – between 4 and 25 percent (different studies have found the chance to be 1 in 25 and 1 in 4)
T14484C – between 37 and 58 percent (different studies have found the chance of some sight recovery to be around 4 in 10 and around 6 in 10)
G3460A – between 22 and 25 percent (different studies have found the chance of some recovery to be around 1 in 5 and around 1 in 4)
An early study of 8 families found that patients with the G11778A mutation did not show significant recovery. Other patients without the G11778A mutation did show some recovery (2575667)
Onue study in the Netherlands found that the chance of any sight recovery was dependent on the LHON mutation (16564802)
G11778A: around 22% – just over 1 in 5.
G3460A: around 15% – about 1 in 6
T14484C: over 50% – a little more than half.
This study, looking at 25 different families, found that sight recovery took place between 1 and 4 years after the acute phase.
Another study found that the figures were different in their patient group. Only 7% of those with the G11778A mutation showed any recovery, and 38% of those with the G3460A mutation.
The page on Medical Tests for LHON describes the tests used to diagnose LHON and measure the severity of the symptoms.
Studies and Case Reports
The Bascom-Palmer Eye Institute carried out a study of untreated LHON affected patients March 2008 to September 2012.(24525545)
They tested 44 untreated LHON patients every six months.
8 out of 44 or 18 percent of paitients showed some recovery, 3 of them in only one eye. The best recovery achieved was 20/100.
Visual Acuity using Early Treatment Diabetic Retinopathy Recovery Chart. Recovery was defined as 15 or more letters.
PERG Pattern electroretinogram
OCT optical coherence tomography
A 50 year old woman lost sight in one eye due to LHON but visual acuity recovered to 0.3 after 3 years. (18173420)
A 10 year old boy in Switzerland lost central vision in both eyes due to LHON G3460A mutation. After 18 months the central visual field loss was smaller. After 2 years his optic nerve electrical signal PERG test results had improved. (22684678)
A 28 year old man with the T14484C mutation who showed some recovery between 13 and 21 months after the Ac ute phase. (23905692)
Recovery with treatment
A double-blind randomized placebo-controlled study of 85 LHON affected patients tested daily treatment with 900mg Idebenone. It covered the three primary mutations G11778A, G3460A ande T14484C. After 24 weeks patients treated with Idebenone had some improvement in visual acuity. About 1/3 of patients who could not read an eye chart at all could see 1 line afer 24 weeks. (21788663)(http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3170530/)
A 31 year old woman was affected by LHON with the G11778A mutation. the Acute Phase lasted two weeks and her Visual Acuity was reduced to 20/200. She was treated with 900 mg of Idebenone daily and after 9 months her visual acuity improved to 20/25. (22269948)
A trial using EPI-743 to treat 5 patients in the USA found recovery of visual acuity in 4 patients, with “total recovery” in 2. (22410442)