Neurosyphilis with Syphilitic Anterior and Intermediate Uveitis OU (Grand Rounds)

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Original article contributed by: Michael I. Seider, MD
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Neurosyphilis with syphilitic anterior and intermediate uveitis OU.

Uveitis Grand Rounds Case Presentation

Michael I. Seider, MD

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Financial Disclosure

Dr. Seider states that he has no financial interest, affiliation, or other relationship with the manufacture of any commercial project discussed or with the manufacture of any competing commercial project.


History

  • Chief Complaint:
    • "My right eye is red and painful since I poked it 5 days ago. Also, the vision in both eyes is blurry"
  • History of Present Illness:
    • 31 year old man with 3 months of progressive blurred vision, OD>OS. He complained of pain, redness, severe photophobia OD x 5 days (believed by the patient to be secondary to mild trauma)
  • Review of Systems: negative, no rashes/ulcers
  • Past Medical History: HIV diagnosed 10 years ago, but did not follow up
  • Allergies: None
  • Medications: None
  • Social History: Caucasian, born near Krakow, Poland. No known history of TB/recent travel/tick bites. Sexually active with one man. 1pk/day tobacco, significant EtOH, no IV drugs

Examination

  • Vision (sc): HM, 20/200 (no improvement c PH)
  • Pupils: symmetrically reactive s RAPD
  • Tonometry: 12, 14
  • Extraocular Motility: full OU
  • Visual Fields: unable OD, full OS
  • Lids/lashes - normal OU
  • Conjunctiva/sclera -
    • OD: 2-3+ injected, no scleritis
    • OS: white/quiet
  • Cornea -
    • OD: 2-3+ diffuse edema, no KP
    • OS: moderate non-pigmented granulomatous KP in Arlt's triangle, no edema
  • Anterior chamber - No cell seen through hazy cornea OD, 0.5+ cell OS, deep OU
  • Iris - normal without nodules OU
  • Lens - clear OU
  • Anterior vitreous - significant WBC OD>OS
  • Diffuse, dense vitreous haze OD>OS, no retinal whitening seen, retina attached OU


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Laboratory Testing

  • CBC: WNL
  • CMP: WNL


  • HIV: Positive
  • CD4: 319 (19%)
  • VL: 64912


  • ACE: 117 (ref 14-67)
  • Lysozyme: >32


  • RPR : Negative


  • TPPA: Reactive (no history of syphilis treatment)


  • PPD: Negative


  • Hi-res chest CT: mild emphysema

Imaging Testing

  • Hi-res chest CT: mild emphysema only
  • B-Scan: moderate vitritis OD>OS, no masses or retinal detachment

Differential Diagnosis

  • Sarcoidosis
  • Tuberculosis
  • Syphilis
  • Endogenous endophthalmitis
  • Viral retinitis/uveitis (CMV, ARN)
  • Lyme disease
  • Behçet disease

Treatment Course

  • Admitted
  • Repeat serum RPR titer - 1:1024
  • CSF analysis:
    • WBC: 80 (93% lymphocytes)
    • Glucose: 39 (low-normal)
    • Protein: 100 (elevated)
    • VDRL: 1:16
  • 2 weeks of IV aqueous crystalline penicillin G for ocular and disseminated neurosyphilis

Final Diagnosis

  • Neurosyphilis with syphilitic anterior and intermediate uveitis OU


Syphilitic Uveitis

  • May occur during any classic "stage" of syphilis infection by the spirochete Treponema pallidum
  • Protean presentation: can present as interstitial keratitis, iris nodule, a myriad of forms of uveitis (may be granulomatous or non), optic neuritis, etc.
  • Symptoms depend on manifestation of disease, but may present with indolent course
  • Differential includes other causes of uveitis, including tuberculosis, sarcoidosis Lyme disease, etc.


Diagnosis

  • Treponemal tests (specific for treponemal disease)
    • FTA-Abs
    • TPPA
  • Non-treponemal tests (non-specific)
    • VDRL
    • RPR
    • Track with disease severity
    • Prozone phenomenon may occur resulting in false negatives
  • CSF analysis (usually reveals elevated WBC unless very immunosupressed, mostly lymphocytes, elevated protein, variable glucose, +VDRL)


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Treatment

  • IV aqueous crystalline penicillin G

Follow Up

  • Our patient completed 2 weeks of IV antibiotic therapy
  • Most recent follow-up (1 month after presentation):
    • VA sc: 20/25, HM
    • Significantly improved disease OD
    • Congealed, inactive appearing vitreous cell OS

References

  • Jurado RL, Campbell J, Martin PD. Prozone phenomenon in secondary syphilis: Has its time arrived? Archives of Internal Medicine. 1993;153: 2496-8.
  • Maves RC, Cachay ER, Young MA, et al. Secondary syphilis with ocular manifestations in older adults. Clinical Infectious Diseases. 2008;46: 142- 5.
  • Tucker JD, Li JZ, Robbine GK, et al. Ocular syphilis among HIV-infected patients: a systemic analysis of the literature. Sexually Transmitted Infections. 2011;87: 4-8.
  • Marra CM, Maxwell CL, Smith SL, et al. Cerebrospinal fluid abnormalities in patients with syphilis: association and clinical and laboratory features. Journal of Infectious Diseases. 2004;189: 369-76.

Discussion

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