Neurosyphilis

Aka: Neurosyphilis, Tabes Dorsalis, Argyll-Robertson Pupil, Prostitute Pupil, Syphilitic Paresis, Dementia Paralytica, Meningovascular Syphilis, Ocular Syphilis, Otosyphilis

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II. Epidemiology

  1. Occurs in 8-10% of untreated Syphilis (as part of Tertiary Syphilis)

III. Pathophysiology

  1. Complication of Tertiary Syphilis
  2. Onset as Meningitis or meningovasculitis
  3. Degenerative parenchymal changes in any part of the CNS

IV. Symptoms

  1. Slow mental deterioration
  2. Headaches
  3. Dizziness
  4. Personality change

V. Signs

  1. Altered Mental Status
  2. Cranial Nerve palsy
    1. Any ophthalmic or auditory symptoms in Syphilis should be treated as Neurosyphilis regardless of Lumbar Puncture results
  3. Tremor of lips, Tongue or hands
  4. Seizures
  5. Ataxia or impaired gait
  6. Aphasia
  7. Hyperreflexia
  8. Cognitive changes to progressive Dementia
  9. Argyll Robertson Pupil
    1. See Miosis
    2. Mnemonic (Prostitute's Pupil): Pupil accomodates but does not react
      1. Pupil constricts during accommodation, but does not constrict to light
    3. Due to Midbrain lesion (superior colliculus, pretectal region)
      1. Typically bilateral lesions resulting in small, irregular pupils

VI. Types

  1. Asymptomatic (at onset of Neurosyphilis)
    1. Cerebrospinal Fluid positive VDRL (Meningitis)
    2. Argyll-Robertson Pupil (Prostitute Pupil) may be present
    3. Asymptomatic Syphilis may persist
  2. Meningeal Neurosyphilis (subacute Meningitis)
    1. Subacute Meningitis presenting with fever, stiff neck and Headache
    2. CSF with high Lymphocyte Count, high Protein, low Glucose and Syphilis Serology positive
    3. Intracranial Pressure increased
  3. Ocular Syphilis
    1. Iritis, Uveitis or chorioretinitis
    2. Painless Vision Loss
    3. Evaluate with a complete Cranial Nerve exam and a Slit Lamp exam by ophthalmology
  4. Otosyphilis
    1. Sudden onset Sensorineural Hearing Loss (endocochlear Syphilis)
    2. Vertigo (Vestibular Neuronitis due to Syphilis)
    3. Tinnitus
  5. Meningovascular Syphilis
    1. Begins 5 to 10 years after initial infection in up to 10% of patients with Neurosyphilis (3% of overall Syphilis)
    2. Acute or subacute Aseptic Meningitis presenting with a meningo-encephalopathic syndrome
    3. Inflammatory arteriopathy affecting adventitia of large and medium-sized vessels
    4. Risk of Cerebrovascular Accident (multiple small infarctions) with variable distribution
  6. Tabes Dorsalis (Tabetic Neurosyphilis)
    1. Demyelination of Posterior Column dorsal roots and dorsal root ganglia
    2. Onset 20 to 30 years after initial infection
    3. Results in sensory Ataxia of the legs
    4. Lancinating pain and Paresthesias
    5. Urinary Overflow Incontinence
    6. Absent knee and ankle Deep Tendon Reflexes
    7. Proprioception loss
    8. Abnormal Romberg Test and Ataxia
    9. Progressive degeneration of spinal cord (posterior roots, Posterior Columns)
    10. Charcot's Joints
    11. Argyll-Robertson Pupil (Prostitute Pupil)
  7. Syphilitic Paresis (Dementia Paralytica, Paretic Neurosyphilis)
    1. Chronic meningoencephalitis
    2. Emotional lability and altered personality as well as affect
    3. Memory deficits
    4. Progressive Dementia
    5. Dysarthria and other speech changes
    6. Myoclonic Jerks
    7. Action Tremor
    8. Seizures
    9. Hyperreflexia
    10. Positive Babinski Reflex
    11. Evolves into Psychosis
    12. Argyll-Robertson Pupil (Prostitute Pupil) may be present

VII. Labs: CSF Evaluation

  1. See Syphylis Testing
  2. Screening
    1. CSF VDRL (high Specificity)
    2. CSF white cell count >10/mm3
    3. CSF Protein >50 mg/dl
  3. Retesting if CSF VDRL negative
    1. Treponemal specific CSF tests (e.g. TPHA)
      1. High False Positive Rate
      2. Consider TPHA index (compares CSF to serum titer)
    2. Spirochete DNA PCR from CSF sample
      1. Higher Specificity than TPHA
      2. Not yet widely available
  4. Experimental markers
    1. B-Cell chemoattractant chemokinge (CXCL13 or CXC motif)
      1. Increased CSF concentrations may reliably predict Neurosyphilis
      2. Marra (2010) Sex Transm Dis 37(5):283-7 [PubMed]

VIII. Management

  1. See Syphilis
  2. Penicillin (with probenacid 500 mg orally four times daily for 10-14 days)
    1. Aqueous crystalline Penicillin G
      1. Dose: 3-4 MU IV every 4 hours for 10-14 days (18-24 MU daily)
      2. Alternative: 0.75 to 1 MU/hour continuous IV
    2. Procaine Penicillin G (only in compliant patients)
      1. Dose 2.4 MU IM once daily for 14 days
      2. Use with Probenecid 500 mg qid for 14 days
  3. Penicillin Allergy
    1. Desensitize and treat with Penicillin
    2. Ceftriaxone 2 g IM/IV qd for 14 days
  4. Repeat testing for Syphilis
    1. Repeat Syphilis Testing at 6 and 12 months
    2. Expect four fold reduction in titer after treatment
      1. Reevaluate and HIV Test if fails to have four fold drop in titer

IX. References

  1. (2019) Sanford Guide, accessed on IOS 11/6/2019
  2. Green, Cohen, Billington (2016) Crit Dec Emerg Med 30(11): 4-10
  3. Kirk, McHugh and Parnell (2023) Crit Dec Emerg Med 37(8): 23-9
  4. Ricco (2020) Am Fam Physician 102(2): 91-8 [PubMed]
  5. Workowski (2010) MMWR Recomm Rep 59(RR-12): 1-110 [PubMed]

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