Whose sign is it anyway?

Take-home

Physicians’ namesake discoveries of various ocular signs provide a historical perspective.

Dr. Medow

Our Ophthalmic Heritage By Norman B. Medow, MD, FACS

In 1835, an Irish physician, Robert James Graves, described a case of exophthalmos associated with an enlargement of the thyroid gland (goiter).

In 1840, a German physician, Karl Adolph von Basedow, reported the same group of symptoms in three additional cases.

In Europe, the triad of hyperthyroidism, goiter, and exophthalmos is referred to as Basedow’s disease. In England and the United States, the preference is to call the condition Graves’ disease.

However, history shows us that neither physician was the first to describe this entity. Others-Parry in 1786, Flajani in 1802, Testa in 1810, and al-Jurjani, a Persian physician in the 1100s-all described portions of this disorder. Sadly, none of these received priority for their discoveries.

Eye signs over the centuries

Because of the effect of this disorder on the eyes and the orbit, many signs have been described during the past two centuries. Some authors indicate that 40 various eye signs have been described.

Here are a few common signs, as well as some less common ones.

Dalrymple’s sign. First described by English ophthalmologist John Dalrymple, this distinctive sign of a widened lid opening in exophthalmus with scleral show above the upper margin of the cornea leads to what is known as lid retraction. This sign is considered by many to be the most common lid signs in Graves’ disease and as close to a pathognomonic sign that we have.

Joffroy’s sign. Poor forehead wrinkling upon upgaze with forward bending of the head was first described by neurologist Alix Joffroy (1844 to 1908), who worked in Paris with the eminent neurologist, Jean-Martin Charcot.

Kocher’s sign. Theodor Kocher (1841 to 1917) was one of the most important surgeons of the late 1800s. Born and raised in Switzerland, “home of the goiter,” he had ample experience with this disorder. His sign-difficulty of the eye to look upward-is inconsistently found and rests most likely with the thickening and fibrosis of the inferior rectus muscle.

In 1909, Kocher was awarded the Nobel Prize for his work on the thyroid gland, reproduced in great part in his book, “Diseases of the Thyroid Gland.” Kocher was also one of the earliest surgeons to embrace aseptic technique.

Möbius’ sign. Weakness of convergences was first described by German neurologist Paul Julius Möbius in 1894. Möbius is also known for his description of this syndrome that bears his name, Möbius’ syndrome.

Stellwag’s sign. Austrian ophthalmologist Karl Stellwag von Carion (1823 to 1904) described this sign in patients who had exophthalmus and noted they most often had decreased or infrequent blinking. In 1856, Stellwag von Carion also coined the term “ectopia lentis” in Marfan’s syndrome.

Von Graefe’s sign. Friedrich Wilhelm Ernst Albrecht von Graefe (1828 to 1870), one of ophthalmology’s great physicians, first described the finding now given his name at the Berlin Medical Society on March 9, 1864.

Von Graefe noted that in exophthalmos, the upper eyelid fails to follow the downward movement of the eye. He believed it was present even in very slight exophthalmos, was most likely due to involvement of Müller’s muscle, and was pathognomonic of Basedow’s disease.

Von Graefe only lived until age 42, but also made other contributions to the profession. He described iridectomy in angle closure, moved the cataract incision from the lower cornea to the upper cornea, initiated visual field testing, and taught hundreds of ophthalmologists who visited his clinic in Berlin.

References

Graves RJ. Newly observed affection of the thyroid gland in females. London Medical and Surgical Journal. 1835;7:516-517.

Basedow KA. Exophthalmos durch Hypertrophie des Zellgewebes in der Augenhöhle: Wochenschrift für die gesamte Heilkunde, Berlin. March 28, 1840.

Norman B. Medow, MD, FACS, is editor of the Our Ophthalmic Heritage column. He is director, pediatric ophthalmology and strabismus, Montefiore Hospital Medical Center, and professor of ophthalmology and pediatrics, Albert Einstein College of Medicine, Bronx, NY. He did not indicate a financial interest in the subject matter.

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