[Progressive supranuclear palsy with macular degeneration--report of three cases]

Three cases of progressive supranuclear palsy (PSP) with decreased visual acuity were reported. Case 1 was a 50-year-old man, case 2 a 60-year old woman, and case 3 a 66-year-old woman. They all had vertical gaze palsy and unexplained falls, and were diagnosed as PSP by NINDS-SPSP clinical criteria. All cases had symptoms of difficulty in vision; therefore, detailed ophthalmological tests were performed. Ophthalmoscopic findings of macula revealed atrophy in case 1 and 2, and brownish discoloration in case 3. Fluorescein fundus angiography done in case 1 showed abnormal staining at the macula. Multifocal electroretinography performed in case 3 showed absence of big response in the macula. These results indicated macular degeneration. None of three cases had cataract. And visual evoked potentials of case 2 and 3 showed prolonged P100 latency. From these results, we suspected that decreased visual acuity of our cases were due to macular degeneration and difficulty to catch the object at the fovea because of gaze palsy. There had been no reports of macular degeneration with PSP, but we might have overlooked these findings, because of dementia, gaze palsy, and apraxia of lid opening. The possibility of macular degeneration is raised as a part of neurological signs is PSP.