Fig. 1.

Timeline of STM dosage and visual acuity in two unrelated juvenile patients with LHON. a Patient #1 already had visual impairment with visual acuity of 0.5 before start of STM treatment. Shortly after initiation of antiepileptic medication with STM 3.3 mg/kg BW per day the patient complained of further visual impairment with visual acuity of 0.2 as assessed 3 weeks later. Due to persistently recurring seizures STM dosage was increased to 6.6 mg/kg BW per day. A few days later (9 weeks after start of STM) the girl complained marked further visual decline with visual acuity of 0.02. Hereupon STM was stopped. Under treatment with idebenone a slight recovery of visual acuity was observed in the following months. b Patient #2 had no history of previous visual impairment. Antiepileptic treatment with STM was started with 4.2 mg/kg BW per day. Due to persistently recurring seizures the dosage was increased in two steps to 8.7 mg/kg BW per day after 4 months. The next day the boy complained marked visual loss on his right eye, visual acuity was 0.1 in the subsequent ophthalmologic examination. Thus STM was stopped and a treatment with idebenone was initiated. In the further course a complete recovery of visual acuity could be observed