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 reported in Japan to date. In this study we report on 7 cases in a Japanese family which we recently examined. The proband, a 64-year-old man, suffering from itching in his limbs, impaired lip movement and dysarthria, consulted the Department of Neurology, University of Tokyo. Neurological examinations revealed bilateral facial, glossopharyngeal, vagal and hypoglossal nerve palsies, and also impaired distal vibratory perception. Immunohistological and biochemical studies confirmed the diagnosis of FAP-IV. Ophthalmological examinations showed his vision was 1.2 with fine lattice corneal dystrophy in both eyes. The lattice dystrophy was randomly scattered with short glassy lines. Corneal sensation was normal and there was no evidence of recurrent corneal erosion. Six family members with similar lattice corneal dystrophies also were suspected to be affected neurologically by FAP-IV. The family pedigree suggested an autosomal dominant trait of inheritance.