LATTICE CORNEAL-DYSTROPHY TYPE-II WITH FAMILIAL AMYLOID POLYNEUROPATHY TYPE-IV

Lattice corneal dystrophy type II with familial amyloid polyneuropathy type IV (Finnish type, Meretoja's syndrome, FAP-IV) has not been repo rted in Japan to date. In this study we report on 7 cases in a Japanes e family which we recently examined. The proband, a 64-year-old man, s uffering from itching in his limbs, impaired lip movement and dysarthr ia, consulted the Department of Neurology, University of Tokyo. Neurol ogical examinations revealed bilateral facial, glossopharyngeal, vagal and hypoglossal nerve palsies, and also impaired distal vibratory per ception. Immunohistological and biochemical studies confirmed the diag nosis of FAP-IV. Ophthalmological examinations showed his vision was 1 .2 with fine lattice corneal dystrophy in both eyes. The lattice dystr ophy was randomly scattered with short glassy lines. Corneal sensation was normal and there was no evidence of recurrent corneal erosion. Si x family members with similar lattice corneal dystrophies also were su spected to be affected neurologically by FAP-IV. The family pedigree s uggested an autosomal dominant trait of inheritance.