Case Presentation: A 37-year-old man who has sex with men (MSM) with extensive cerebrovascular history, including non-traumatic internal carotid artery dissection, cerebrovascular accident (CVA) with left-sided hemiparesis, Circle of Willis aneurysm, presented with acute painless central scotoma in his left eye for one day. He was seen by an outpatient ophthalmologist, who visualized a placoid yellowing of the retina and macula. A fluorescein retinal angiography was also done, which showed no evidence of occlusion in retinal vessels. MRA showed stable findings. In setting of his high-risk sexual history, ocular findings were concerning for neurosyphilis, and patient was sent in for further workup. Rapid Plasma Reagin (RPR) Assay was reactive with titer of 1:32 and Treponema pallidum antibody was positive. However, lumbar puncture was unremarkable with negative cerebral spinal fluid Venereal Disease Research Laboratory test (CSF-VDRL) and no pleocytosis. Other etiologies of acute vision loss, such as multiple sclerosis, vasculitides, systemic autoimmune disease, and other infectious causes were ruled out. After penicillin treatment for 2 weeks, his visual acuity improved from 20/400 on admission to 20/20 by discharge.
Discussion: Our patient was a MSM presenting with nonspecific visual symptoms, who was found to have a characteristic finding for acute syphilitic posterior placoid chorioretinitis (ASPPC) on ophthalmoscopic and fluorescein exam. These ocular findings in setting of positive RPR titers and T. pallidum antibodies, as well as an excellent response to penicillin, made his presentation consistent with ocular syphilis. While LP, the gold standard for diagnosis, was unremarkable without any pleocytosis and negative CSF-VDRL, this does not rule out diagnosis, as this marker is specific but has low sensitivity. No clear cardiovascular or hypercoagulable risk factors to explain his history of cerebrovascular disease were identified. While APLS antibodies were positive, there is cross-reactivity between syphilis reagin antibodies and anticardiolipin; APLS antibodies were likely falsely positive. The patient’s cerebrovascular history is likely a manifestation of undetected neurosyphilis. Months to years after infection, patients can develop arteritis of the cerebral vasculature known as syphilitic meningovascular disease, which often manifests as a focal neurological deficit in territory of cerebral vessels. Hemiparesis/plegia from MCA territory stroke is the most common manifestation and was interestingly also a part of our patient’s clinical history.
Conclusions: We have presented a case of a MSM who presented to us with ASPPC. This patient’s syphilis infection remained undetected and untreated until he was found to have the more specific ocular findings. Had this patient been diagnosed earlier on in the course of his disease, other cerebrovascular pathologies may not have developed. This case highlights the importance of syphilis screening in high risk patients with a history of nonspecific neurological signs and symptoms.
To cite this abstract:Satoh, S; Kim, E. Blind to the diagnosis: a case of undetected neurosyphilis presenting as unilateral central scotoma.. Abstract published at Hospital Medicine 2018; April 8-11; Orlando, Fla. Abstract 833. https://www.shmabstracts.com/abstract/blind-to-the-diagnosis-a-case-of-undetected-neurosyphilis-presenting-as-unilateral-central-scotoma/. Accessed February 21, 2020.