Since the accommodation reflex pathway is separate from the light reflex, it is unaffected. This causes a loss of both the direct and consensual pupillary light reflexes. As a result, signals carried from the retina are not relayed via the pretectal nucleus on the affected side to the Edinger-Westphal nuclei. The abnormal pupil is probably caused by damage to cells in the pretectal region of the midbrain. 8 Crucial to this were the discoveries by Schaudinn and Hoffmann of the treponema pallidum in 1905 and the serologic test in 1906 of August Paul von Wassermann (1866–1925). However, the role of syphilis in causing these disorders was at first debated, and not until the 20th century was it generally accepted. The Argyll Robertson pupil soon became a “pathognomonic sign” in tabes dorsalis, dementia paralytica, and meningovascular syphilis. Argyll Robertson also cited Romberg’s Lehrbuch der Nervenkrankheiten des Menschen (1840): “In tabes dorsalis I have found the pupil contracted to the size of a pin’s head…one or both eyes, consisting in a contraction with loss of motion.” Atropine drops produced only “medium dilatation of the pupils.”Įrnst Julius Remak had earlier shown the pupillary signs of tabes to Argyll Robertson, 7 whose reports of 1869 acknowledged Remak’s observation of the miotic pupil, its defective reaction to light, and preserved contraction on accommodation.
6 Three of his cases had slight optic atrophy one showed ptosis. In the second paper (1869), he reported four more cases and concluded the “myosis was due to paralysis of the cilio-spinal nerves,” 5 although miosis is not a constant sign. In the first, he reported the eye signs in a fifty-nine-year-old patient admitted on account of dimness of vision and an unsteady gait: 4 In 1869, the ophthalmologist Douglas Moray Cooper Lamb Argyll Robertson (1837–1908) described his famous pupillary sign in two lengthy papers. Unequal, irregular pupils without reaction to light had been known in cases of tabes dorsalis and dementia paralytica (GPI) since the end of the eighteenth century. Thanks to modern antibiotic treatment, the Argyll Robertson pupil (Fig 1) has become relatively rare. Incidental neuroimaging abnormalities may also alert the neurologist to suspect neurosyphilis: cerebral gummata arising from the dura can mimic meningiomas, and medial temporal lobe abnormalities can mimic herpes encephalitis. About half the patients with neurosyphilis are infected with HIV. 3 Meningovascular involvement is caused by an inflammatory, obliterating endarteritis of the leptomeninges, brain, and spinal cord with resulting ischemic attacks or infarcts. General Paralysis of the Insane (GPI or dementia paralytica) presents with insidious and variable psychological symptoms, dementia, epilepsy, focal neurological deficits, and often AR pupils.
Argyll Robertson pupils are a valuable sign of tabes and may precede its typical sensory ataxia, absent ankle jerks, lighting pains, and visceral crises. Symptomatic neurosyphilis most often presents with posterior column lesions, the hallmark of tabes dorsalis, typically fifteen to twenty-five years after primary infection. Neurosyphilis may be asymptomatic with abnormal cerebrospinal fluid (CSF) but no neurological symptoms or signs. 3 The Argyll Robertson (AR) pupil (Fig 1) is an important, classical sign of neurosyphilis. Tertiary syphilis includes not only neurosyphilis but also gummata, cardiovascular, and meningovascular varieties. Historically, neurosyphilis was so common that Kinnier Wilson in his unique textbook Neurology in 1940 devoted over one hundred pages to it. Because we now have effective antibiotic treatment, both clinical clues and screening tests are of vital importance, especially in early and in asymptomatic latent cases. 2 A clinical diagnosis of syphilis is no longer the occasional curiosity it was at the end of the twentieth century, yet routine screening tests have fallen into disuse. 1 In 2020, 133,945 cases of all stages of syphilis were reported in the US, and in the UK the rate of newly diagnosed cases in 2020 was twice that in 2012. Since reaching a historic low in 2000 and 2001, the incidence of syphilis has increased almost every year. Several direct and serological tests of varying sensitivity and specificity have now replaced the WR. In my student days, the Wasserman reaction (WR), though not specific, was performed almost routinely in patients on medical wards to detect syphilis.
Douglas Argyll Robertson and his pupils November 8, 2022įigure 1.
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