Westphal-strumpell disease (pseudosclerosis of basal ganglion) 1

General: Part of Wilson disease; mostly in men; onset at ages 11 to 25 yea 333d39d rs (see Wilson Disease).

Ocular: Brown-yellow-green ring in Descemet membrane, about 2 mm wide, which begins directly at the limbus and fades away toward the center; deposition of copper.

Clinical: Jaundice; difficulty in swallowing, speaking, and mastication; extensive muscular rigidity; coarse tremors.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Pau H. Differential diagnosis of eye diseases. New York: Thieme, 1987.