Clinical Characteristics of m.11778G>A Leber Hereditary Optic Neuropathy with Favorable Outcomes.

The prognosis of 11,778 mitochondrial mutations in Leber hereditary optic neuropathy (LHON) is poor. Patients with favorable outcomes (visual acuity better than 20/100) who could be observed for more than 6 months were analyzed. Among 74 patients (57 male, 17 female), 6 (8.1%) showed improvement in visual acuity of 20/100 or higher. The patients with favorable outcomes have better visual acuity at nadir (logMAR 0.98 ± 0.69 in the favorable patients and logMAR 2.32 ± 0.93 in the unfavorable patients, p = .003). Among the favorable group, four patients (36, 32, 19, and 7 years of age at onset) took idebenone within 6 months of onset. However, fifty-one percent of the patients with unfavorable outcomes took idebenone (p = .008). Although the age at onset in the favorable patients is relatively younger than that of the unfavorable patients (20.3 ± 10.8 versus 28.8 ± 12.8 years), a significant difference was not found (p = .138). In conclusion, better visual acuity in nadir and administration of idebenone may affect vision recovery.

The Effect of Horizontal Extraocular Muscle Surgery on the Heimann-Bielschowsky Phenomenon.

We report the effect of horizontal extraocular muscle surgery of Heimann-Bielshowsky phenomenon. A 52-year-old woman complained of binocular diplopia for a few months. In 2000, she underwent extracapsular cataract extraction with intraocular lens implantation in the right eye. After surgery, the diplopia persisted and developed further. She showed an esodeviation of 60 prism dioptres at near fixation and distance fixation. Cortical opacity and nucleus sclerosis were observed in the left lens, and phacoemulsification with intraocular lens implantation was performed in the left eye in October 2011. After cataract surgery, the patient suffered from oscillopsia and diplopia. Examination revealed unilateral coarse, slow, pendular vertical oscillations in the right eye and an esodeviation of 45 prism dioptres and a right hypodeviation of 8 prism dioptres, which were observed before the surgery. We performed a medial rectus muscle 6 mm recession and lateral rectus muscle 7 mm resection surgery on the right eye. After the operation, the oscillations disappeared and diplopia greatly improved. Moreover, the patient no longer complained of oscillopsia. We conclude that surgery performed on horizontal extraocular muscles could be an effective treatment for Heimann-Bielschowsky phenomenon also.

Fresnel prism on hess screen test.

A 65-year-old male patient complained of diplopia after a cataract surgery. He had esotropia of 18 prism diopters (PDs) at distant and near deviation, and therefore, we performed the Hess screen test to identify any abnormal eye movement. However, the indicator was found to be out of bounds, and therefore, the test could not be completed. Therefore, the test was subsequently performed with a 20 PD base-out Fresnel prism, and an abduction deficit was observed in the right eye, but not in the left eye. Therefore, we speculated that the patient had abducens nerve palsy in the right eye. The results obtained in the present study imply that performing the Hess screen test with the Fresnel prism may be very useful in diagnosing ambiguous abnormalities in patients with extraocular movement. The Hess screen test can be performed for patients with a strabismus of greater than 15 PD by using a Fresnel prism. Thus, a Fresnel prism may be useful for performing both the Hess screen test and Lancaster screen test.