[Lattice corneal dystrophy type II with familial amyloid polyneuropathy type IV]

Lattice corneal dystrophy type II associated with familial amyloid polyneuropathy type IV (Finish type, FAP-IV) has not yet been reported in Japan. We report 7 cases in a Japanese family. 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 examination revealed bilateral facial, glossopharyngeal, vagal, and hypoglossal nerve palsy, and impaired distal vibratory perception. His vision was 1.2 and he had fine lattice corneal dystrophy in both eyes. Short glassy lines were randomly scattered in the lattice dystrophy. Corneal sensation was normal and there was no evidence of recurrent corneal erosion. Immunohistological and biochemical studies confirmed the diagnosis of FAP-IV. Six siblings were neurologically suspected to be FAP-IV patients with similar lattice corneal dystrophy. The family pedigree suggested an autosomal dominant trait of inheritance.