Seminars in Ophthalmology, 2014; 29(4): 189–191 ! Informa Healthcare USA, Inc. ISSN: 0882-0538 print / 1744-5205 online DOI: 10.3109/08820538.2013.810278


A Rare Presentation of Neurosyphilis Mimicking a Unilateral Adie’s Tonic Pupil* Imran Jivraj and Michael Johnson

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University of Alberta, Department of Ophthalmology, Edmonton, Alberta, Canada

ABSTRACT We describe a case of a female patient who presented with anisocoria and was initially diagnosed with a right acute Adie’s pupil on the basis of a right tonic pupil with absent oculomotor or neurological findings and hyper-responsiveness to dilute Pilocarpine. Two months later, the patient returned with bilateral tonic pupils and limitation of extraocular movement in the right eye. Subsequent laboratory testing revealed neurosyphilis. Our case, only the second ever reported, emphasizes the importance of considering the diagnosis of neurosyphilis in patients presenting with an isolated acute tonic pupil. Keywords: Adie’s pupil, neurosyphilis, tonic pupil


constricted to 3.5 mm 45 minutes after instilling 0.125% Pilocarpine. Confrontational visual fields were full to counting fingers bilaterally, extraocular movements were full, and no manifest ocular misalignment or eyelid ptosis was found. As there were no further concerning symptoms or signs, she was felt to have an acute unilateral Adie’s tonic pupil. The patient presented two months later after noticing her left pupil had also become dilated. Best corrected visual acuities measured 20/30 OD and 20/ 25 OS. Her pupils measured 8.5 mm OD and 8 mm OS in darkness, ambient light, and bright light (Figure 1). There was poor pupil constriction with accommodation, but both pupils constricted to 4 mm 45 minutes after instilling 0.1% Pilocarpine. Mild eyelid ptosis and limitation of adduction of the right eye were noted. Orthoptic measurements demonstrated a right exotropia of 45 prism diopters and a right hypotropia of 10 prism diopters in primary position with alternate cover testing. Anterior segment examination demonstrated vermiform movements of the pupil margins bilaterally.

Argyll Robertson pupils are the most common pupillary manifestation of neurosyphilis and are described as bilateral small pupils with diminished constriction to light but brisk response to near stimuli. Bilateral dilated tonic pupils are a less common but well recognized pupillary manifestation of neurosyphilis. Here, we describe a rare case of an isolated dilated tonic pupil, initially attributed to an Adie’s tonic pupil, which was later found to be the sole presenting feature of neurosyphilis.

MATERIALS AND METHODS A 38-year-old woman presented with a three-week history of blurred vision out of the right eye and an enlarged right pupil. Best corrected visual acuities measured 20/25 OD and 20/20 OS. Her right pupil measured 8.5 mm in dark, ambient, and bright light conditions. The left pupil measured 6 mm in the dark, 4.5 mm in ambient light, and 4 mm in bright light. Pupils constricted to 7 mm OD and 4 mm OS with accommodation, and her right pupil demonstrated a more remote near point of accommodation. The right pupil constricted to 5 mm and her left pupil

RESULTS Laboratory Treponema

results showed a positive serum pallidum enzyme immunoassay.

Received 18 October 2012; revised 21 February 2013; accepted 26 May 2013; published online 24 July 2013 *Presented via Poster Presentation at the North American Neuro-Ophthalmology Society Meeting in San Antonio, Texas, in February 2012. Correspondence: Imran Jivraj, M.D., University of Alberta, Department of Ophthalmology, 927 Burley Drive, Edmonton, Alberta T6R1X5, Canada. E-mail: [email protected]


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190 I. Jivraj and M. Johnson

FIGURE 2. Axial T1 post-contrast MRI demonstrates enhancement of the right third cranial nerve as it exits the brain stem (arrow). There was also enhancement of the left third cranial nerve (not well demonstrated in this image).

FIGURE 1. Patient returned with bilateral tonic pupils, mild right eyelid ptosis, and extraocular movement abnormalities and was found to have neurosyphilis.

Cerebrospinal fluid demonstrated a protein level of 0.6 g/L, glucose 2.0 mmol/L, white blood cell count 83  106/L (89% lymphocytes, 6% monocytes, 5% polymorphs), and no red blood cells. Cerebrospinal fluid was reactive to four dilutions to VDRL and this result was confirmed with reactive cerebrospinal FTAABS. MRI brain demonstrated bilateral enhancement of the oculomotor nerves (Figure 2). The patient was diagnosed with neurosyphilis and treated with intravenous Penicillin G. Improvement of her internal and external ophthalmoplegia was noted five months after treatment.

dissociation with tonic redilation after constriction. Acutely, pupil constriction to light is diminished more markedly than to near stimuli because of the 30:1 ratio of fibers subserving accommodation versus constriction. Slit lamp examination reveals an irregular pupil with sectoral paralysis. The pupillary abnormality results from damage to the ciliary ganglion or postganglionic short ciliary nerves, which innervate the pupillary sphincter and ciliary muscles. Vermiform movements result from partial preservation of the pupil’s parasympathetic innervation, which causes partial pupillary constriction in regions adjacent to areas of paralysis. Iris sphincter denervation leads to cholinergic hypersensitivity, which forms the basis for the use of dilute (0.1– 0.125%) Pilocarpine: a tonic pupil will constrict to its administration, but a normal pupil will remain indifferent.1 The causes of tonic pupils fall into three groups. A tonic pupil in association with absent deep tendon reflexes is part of a complex known as Holmes Adie Syndrome. Local ocular processes affecting the ciliary ganglion or short ciliary nerves, such as orbital trauma, sarcoidosis, viral illnesses, ischemia, or tumor involvement, may secondarily cause tonic pupils. Finally, tonic pupils may uncommonly occur in association with a multitude of systemic conditions related to autonomic dysfunction.1 Our patient’s initial presentation was consistent with an acute unilateral Adie’s tonic pupil. Only after the evolution of other symptoms and clinical signs did the diagnosis of neurosyphilis become known. There is great diversity in the ophthalmic presentations of neurosyphilis. Pupil abnormalities are prevalent in roughly 45–53% of patients.2 The most common pupil abnormalities include bilaterally small pupils which react briskly to near stimuli, called Argyll Robertson pupils, or bilaterally large tonic pupils.2–4 To the best of our knowledge, a single case report of neurosyphilis presenting as an isolated unilateral tonic pupil, similar to acute Adie’s pupil, has previously been reported.5,6 We propose that syphilis serology be considered in the work-up of patients presenting with a presumed unilateral Adie’s tonic pupil.

ACKNOWLEDGMENTS We would like to acknowledge Dr. Ameeta Singh, Infectious Diseases Specialist, University of Alberta, for her clinical contributions.

DISCUSSION DECLARATION OF INTEREST Patients who present with a tonic pupil describe a new onset of anisocoria or complaints of monocular photophobia and difficulty reading. The tonic pupil is classically dilated and demonstrates light-near

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. Seminars in Ophthalmology

Neurosyphilis Mimicking Adie’s Tonic Pupil 191


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1. Loewenfeld IE. Lesions in the ciliary ganglion and short ciliary nerves: The tonic pupil (Adie’s syndrome). In: The Pupil: Anatomy, Physiology, and Clinical Applications; pp 1080–1130; Detroit: Wayne State University Press, 1993. 2. Fletcher WA, Sharpe JA. Tonic pupils in neurosyphilis. Neurology 1986;36(2):188–192. 3. Luxon L, Lees AJ, Greenwood RJ. Neurosyphilis today. Lancet 1979;1(8107):90–93.


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4. Englestein ES, Ruderman MI, Troiano RA, et al. Dilated tonic pupils in neurosyphilis. J Neurol Neurosurg Psychiatry 1986;49(12):1455–1457. 5. Camoriano GD, Kassab JJ, Suchak AA, et al. Neurosyphilis masquerading as an acute Adie’s tonic pupil: Report of a case. Case Report Ophthalmol 2011;2(2):205–210. 6. Zeligs MA, Joseph GF. Unilateral internal ophthalmoplegia: Sole clinical sign in patient with syphilitic meningitis. Arch Neur Psych 1945;54(5):389–390.

A rare presentation of neurosyphilis mimicking a unilateral Adie's tonic pupil.

We describe a case of a female patient who presented with anisocoria and was initially diagnosed with a right acute Adie's pupil on the basis of a rig...
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