The retinopathy of dystrophia myotonia Steinert.
Ophthalmologic examination with ERG investigation in "Ganzfeld" of 11 patients with dystrophia myotonia Steinert, showed decreased "b" wave amplitude in photopic and scotopic conditions, and delayed scotopic "implicit time" in all subjects. That therefore concerns the so called "Rod dysfunction syndrome," a type of ERG abnormality found to appear in retinitis pigmentosa dominantly inherited. A possible relation between ERG abnormality and inheritance in dystrophia myotonia Steinert is suggested. ERG appears to be an useful investigation for ill-defined, congenital and unapparent form of disease, in affected families.
Segal
B S
BS
Département d'Ophtalmologie, Chiniques Universitaires St Luc. Université Catholique de Louvain, Bruxelles.
D000328:Adult; D004596:Electroretinography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000175:diagnosis*; D012174:Retinitis Pigmentosa / Q000175:diagnosis*