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Lattice corneal dystrophy type II with familial amyloid polyneuropathy type IV. 1993

T Asaoka, and S Amano, and Y Sunada, and M Sawa
Department of Ophthalmology, University of Tokyo School of Medicine, Japan.

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.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009422 Nervous System Diseases Diseases of the central and peripheral nervous system. This includes disorders of the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscle. Neurologic Disorders,Nervous System Disorders,Neurological Disorders,Disease, Nervous System,Diseases, Nervous System,Disorder, Nervous System,Disorder, Neurologic,Disorder, Neurological,Disorders, Nervous System,Disorders, Neurologic,Disorders, Neurological,Nervous System Disease,Nervous System Disorder,Neurologic Disorder,Neurological Disorder
D010243 Paralysis A general term most often used to describe severe or complete loss of muscle strength due to motor system disease from the level of the cerebral cortex to the muscle fiber. This term may also occasionally refer to a loss of sensory function. (From Adams et al., Principles of Neurology, 6th ed, p45) Palsy,Plegia,Todd Paralysis,Todd's Paralysis,Palsies,Paralyses,Paralysis, Todd,Paralysis, Todd's,Plegias,Todds Paralysis
D010375 Pedigree The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition. Family Tree,Genealogical Tree,Genealogic Tree,Genetic Identity,Identity, Genetic,Family Trees,Genealogic Trees,Genealogical Trees,Genetic Identities,Identities, Genetic,Tree, Family,Tree, Genealogic,Tree, Genealogical,Trees, Family,Trees, Genealogic,Trees, Genealogical
D003317 Corneal Dystrophies, Hereditary Bilateral hereditary disorders of the cornea, usually autosomal dominant, which may be present at birth but more frequently develop during adolescence and progress slowly throughout life. Central macular dystrophy is transmitted as an autosomal recessive defect. Corneal Dystrophies,Granular Dystrophy, Corneal,Groenouw's Dystrophies,Macular Dystrophy, Corneal,Stromal Dystrophies, Corneal,Corneal Dystrophy,Corneal Dystrophy, Hereditary,Corneal Granular Dystrophies,Corneal Granular Dystrophy,Corneal Macular Dystrophies,Corneal Macular Dystrophy,Corneal Stromal Dystrophies,Corneal Stromal Dystrophy,Dystrophy, Corneal,Dystrophy, Corneal Granular,Dystrophy, Corneal Macular,Dystrophy, Corneal Stromal,Dystrophy, Hereditary Corneal,Groenouw Dystrophies,Groenouws Dystrophies,Hereditary Corneal Dystrophies,Hereditary Corneal Dystrophy,Stromal Dystrophy, Corneal
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly

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