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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 915-917

Neurosyphilis masquerading as papillodema unmasked by tell-tale vitreous cells and peripapillary hemorrhages


1 Department of Ophthalmology, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India
2 Department of Neurology, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India
3 Department of Radiology, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India

Date of Submission26-Jun-2022
Date of Acceptance09-Aug-2022
Date of Web Publication11-Oct-2022

Correspondence Address:
Dr. Mayur R Moreker
4th Floor, New Wing, Bombay Hospital. 12, New Marine Lines, Mumbai - 400 020, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1537_22

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  Abstract 


A 35-year-old lady presented with headache and diminution of vision in both eyes for 30–35 days. She was being treated for idiopathic intracranial hypertension with documented high intracranial pressures. In view of the past history of positive blood RPR and the current picture of peripapillary hemorrhage along with papillodema and telltale vitreous cells, we suspected neurosyphilis and repeated Blood-CSF TPHA, both of which were positive. She was treated with a single dose of benzathine penicillin G 1.2 MIU I.M and tablet doxycycline 100 mg twice a day for 14 days, with oral acetazolamaide, to achieve resolution of symptoms and improvement of vision.

Keywords: Neurosyphilis, papillodema, peripapillary hemorrhages


How to cite this article:
Moreker MR, Pandya D, Singh R, Pardasani V, Shah S. Neurosyphilis masquerading as papillodema unmasked by tell-tale vitreous cells and peripapillary hemorrhages. Indian J Ophthalmol Case Rep 2022;2:915-7

How to cite this URL:
Moreker MR, Pandya D, Singh R, Pardasani V, Shah S. Neurosyphilis masquerading as papillodema unmasked by tell-tale vitreous cells and peripapillary hemorrhages. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Nov 27];2:915-7. Available from: https://www.ijoreports.in/text.asp?2022/2/4/915/358155



Syphilis is a sexually transmitted disease caused by the spirochete Treponema pallidum.[1] Ocular involvement in syphilis may be silent or present as anterior uveitis, choroiditis, interstitial keratitis, retinal vasculitis, retinitis, optic neuritis, dacryoadenitis, or scleritis.[2] It is known that evidence of optic nerve involvement and vitreous cells can be suggestive of central nervous system (CNS) involvement in syphilis and should be considered synonymous with neurosyphilis.[3]


  Case Report Top


A 35-year-old lady presented to us with headache for 35 days and diminution of vision in both eyes, 5 days after the onset of headache. Headache was severe, holo-cranial, throbbing in nature, and without any diurnal or postural variation. It was associated with multiple episodes of vomiting and subsided after analgesics. Diminution of vision was acute onset progressive in both eyes for both distant and near objects and for the entire field of vision, 5 days after the onset of headache. She had undergone treatment elsewhere for idiopathic intracranial hypertension and had multiple lumbar punctures with documented high opening pressures in this period. She had been referred to us for consideration of an optic nerve sheath fenestration for a benign intracranial hypertension.

Her best corrected visual acuity was perception of light with accurate projection of rays in both eyes. She had sluggishly reacting pupils with a relative afferent pupillary defect in the left eye. Slit lamp bio-microscopy of the anterior segment of both eyes was unremarkable, and the intra-ocular pressure was 14 mmHg in the right eye and 16 mmHg in the left eye. Fundoscopy showed a swollen mildly hyperemic disc with raised rims with dilated vessels and a peripapillary hemorrhage in the right eye [Figure 1]a and also a swollen disc with early gliosis around the disc and a small peripapillary hemorrhage in the left eye [Figure 1]b.
Figure 1: Fundus picture of a 35-year-old lady showing (a) swollen mildly hyperemic disc with raised rims with dilated vessels and a peripapillary hemorrhage in the right eye, (b) swollen disc with early gliosis around the disc and a small peripapillary hemorrhage in the left eye, and (c and d) resolution of disc edema in both eyes

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Magnetic resonance imaging of brain and orbit showed prominence of the bilateral peri-optic CSF spaces with partially empty sella, that is, signs s/o idiopathic intracranial hypertension [Figure 2]a and [Figure 2]b.
Figure 2: (a and b) Magnetic resonance imaging of brain and orbit of a 35-year-old lady showing prominence of the bilateral peri-optic CSF spaces with partially empty sella, that is, signs s/o idiopathic intracranial hypertension

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With a clinical impression of papillodema; a lumbar puncture for cerebrospinal fluid (CSF) examination was performed, which showed an opening pressure of 180 mm of H2O. This opening pressure was thought to be out of sync and lesser than expected for her complaints and duration of symptoms and was lesser than her opening pressure measured at previous CSF analyses elsewhere. CSF proteins and glucose were in the normal range with mild pleocytosis. A repeat ophthalmic examination was performed, which on closer examination showed occasional vitreous cells with no vitritis in both eyes, which prompted a suspicion of inflammation/infection being the cause of her condition. A repeat detailed look at her history drew our attention to the fact that she was positive for blood RPR and negative for VDRL and had received Injection Penicillin prior to the onset of her current headache and ocular symptoms. In view of this history and because of the presence of vitreous cells suggesting an inflammatory/infective etiology to her condition, suspecting neurosyphilis, we repeated a Blood TPHA and repeated CSF analysis with TPHA, both of which were positive with 1:1280 and 1:2560 titers, respectively. Her husband was tested and was also found positive for syphilis (VDRL and TPHA).

A diagnosis of neurosyphilis masquerading as papillodema was made, and the patient was treated with a single dose of benzathine penicillin G 1.2 MIU I.M and tablet doxycycline 100 mg twice a day for 14 days, with oral acetazolamaide, to achieve resolution of symptoms. Two months later, at follow-up, she had achieved a best corrected visual acuity of 20/60, N6 in the right eye and finger counting at 3 meters, N24 in the left eye with complete resolution of symptoms and disc odema in both eyes [Figure 1]c and [Figure 1]d, resolution of color vision lost in the right eye, and partial resolution of field of vision loss in both eyes in central 10 degrees to give her a good ambulatory vision [Figure 3]a and [Figure 3]b, which she maintains at 15 months follow-up.
Figure 3: (a and b) Grayscale map of the visual field analysis of a 35-year-old lady showing resolution of field of vision loss in both eyes in central 10 degrees. [Figure 3]a shows lighter regions indicating higher sensitivity in the supero-temporal area in the left eye; whereas [Figure 3]b shows lighter regions indicating higher sensitivity in the temporal area in the right eye

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  Discussion Top


Ocular involvement in syphilis is quite rare and typically occurs with secondary or tertiary syphilis. Uveitis is reported to be the most common manifestation of ocular syphilis.[4] CNS involvement can occur at any stage of syphilis. Ocular syphilis has been reported in both immuno-compromised and immuno-competent individuals; therefore, patients with syphilis should be tested for human immunodeficiency virus. In order to diagnose neuro-syphilis in a patient with ocular features, CSF study is required in the form of CSF VDRL and CSF TPHA. However, negative CSF study does not rule out neurosyphilis.[5] Syphilis masquerading as papillodema has been previously reported in the literature in immuno-competent people.[6],[7],[8],[9] In neurosyphilis, disc findings more commonly reported previously are perineuritis and papillitis.[8] Of these reports notably, the fundus finding of peripapillary hemorrhage with papillodema has been noted in only a single case report in which the hemorrhage was present unilaterally.[7] In our patient, peripapillary hemorrhage was noticed in both eyes along with papillodema and telltale vitreous cells. With the recent resurgence of syphilis and also knowing that syphilis is a great masquerader, it may thus be pertinent that ophthalmologists closely look for inflammatory vitreous cells in patients of papillodema and request for appropriate tests to look for syphilis when felt necessary as emphasized by our case.

Although we remain mindful that the presence of neurosyphilis does not rule out other causes of raised intra-cranial pressure, we believe that in our patient, the inflammation in the central nervous system because of syphilis would have resulted in raised intra-cranial pressure which manifested as papillodema. In fact, the optic nerve involvement and vitreous cells with the past history of syphilis prompted us to investigate for central nervous system involvement in syphilis, that is, neurosyphilis. This patient had been referred to us for consideration of an optic nerve sheath fenestration for a benign intracranial hypertension as mentioned earlier. Herein, we would like to emphasize that benign intracranial hypertension and papillodema are not inter-changeable terms. Benign intracranial hypertension should be diagnosed only after excluding all causes, including inflammation/infection in any patient with papillodema as highlighted by our case.


  Conclusion Top


This report describes an extremely infrequent case of bilateral secondary intracranial hypertension with papillodema and peripapillary hemorrhage as a presenting feature of neurosyphilis in an immuno-competent young lady.

Acknowledgements

The Authors wish to acknowledge Dr. Varsha Patil and Dr. Asmita Sakle for their technical help in the management of this patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Lutchman C, Weisbrod DJ, Schwartz CE. Diagnosis and management of syphilis after unique ocular presentation. Can Fam Physician 2011;57:896-9.  Back to cited text no. 1
    
2.
Foti C, Carnimeo L, Delvecchio S, Guerriero S, Cassano N, Vena GA. Secondary syphilis with progressive ocular involvement in an immunocompetent patient. Eur J Dermatol 2009;19:288.  Back to cited text no. 2
    
3.
Puech C, Gennai S, Pavese P, Pelloux I, Maurin M, Romanet JP, et al. Ocular manifestations of syphilis: Recent cases over a 2.5-year period. Graefes Arch Clin Exp Ophthalmol 2010;248:1623-9.  Back to cited text no. 3
    
4.
Tamesis RR, Foster CS. Ocular syphilis. Ophthalmology 1990;97:1281-7.  Back to cited text no. 4
    
5.
Bandettini di Poggio M, Primavera A, Capello E, Bandini F, Mazzarello G, Viscoli C, et al. A case of secondary syphilis presenting as optic neuritis. Neurol Sci 2010;31:365-7.  Back to cited text no. 5
    
6.
Cooper S, Razvi S, Alani A, Winter A, Browne B, Metcalfe R. Syphilis presenting with headache and papilloedema. BMJ Case Rep 2009;2009:bcr 09.2008.1008.  Back to cited text no. 6
    
7.
Quesada D, Stapleton M, Heer J, Aguìñiga-Navarrete P, Kim L. An unusual case of unilateral papilledema. Clin Pract Cases Emerg Med 2019;3:444-5.  Back to cited text no. 7
    
8.
Gonzalez-Martinez A, Quintas S, Celdrán Vivancos D, Cebrián J, Vivancos J. Diagnosis of syphilitic bilateral papillitis mimicking papilloedema. Emerg Infect Dis 2020;26:171-3.  Back to cited text no. 8
    
9.
Mcnally MA, Murira A, Dillard CM, Aisenberg G. The great masquerader: Syphilis mimicking papilledema and traction alopecia. Cureus 2020;12:e7391. doi: 10.7759/cureus 7391.  Back to cited text no. 9
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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