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Neurosyphilis
Aka: Neurosyphilis, Tabes Dorsalis, Argyll-Robertson Pupil, Prostitute Pupil, Syphilitic Paresis, Dementia Paralytica, Meningovascular Syphilis
- See Also
- Syphilis
- Tertiary Syphilis
- Epidemiology
- Occurs in 10% of untreated Syphilis
- Pathophysiology
- Meningovasculitis
- Degenerative parenchymal changes in any part of the CNS
- Symptoms
- Slow mental deterioration
- Headaches
- Personality change
- Signs
- Cranial Nerve palsy
- Any ophthalmic or auditory symptoms in Syphilis should be treated as Neurosyphilis regardless of Lumbar Puncture results
- Tremor of lips, Tongue or hands
- Seizures
- Ataxia
- Aphasia
- Hyperreflexia
- Cognitive changes to progressive Dementia
- Argyll Robertson Pupil
- See Miosis
- Mnemonic (Prostitute's Pupil): Pupil accomodates but does not react
- Pupil constricts during accommodation, but does not constrict to light
- Due to Midbrain lesion (superior colliculus, pretectal region)
- Typically bilateral lesions resulting in small, irregular pupils
- Types
- Asymptomatic (at onset of Neurosyphilis)
- Cerebrospinal Fluid positive VDRL (Meningitis)
- Argyll-Robertson Pupil (Prostitute Pupil) may be present
- Meningeal Neurosyphilis
- Intracranial Pressure increased
- Ocular Syphilis
- Iritis, Uveitis or chorioretinitis
- Painless Vision Loss
- Meningovascular Syphilis
- Begins 5 to 10 years after initial infection
- Acute or subacute Aseptic Meningitis
- Risk of Cerebrovascular Accident (multiple small infarctions) with variable distribution
- Tabes Dorsalis (Tabetic Neurosyphilis)
- Demyelination of Posterior Column dorsal roots and dorsal root ganglia
- Onset 20 to 30 years after initial infection
- Results in sensory Ataxia of the legs
- Lancinating pain and Paresthesias
- Urinary Overflow Incontinence
- Absent knee and ankle Deep Tendon Reflexes
- Proprioception loss
- Abnormal Romberg Test and Ataxia
- Progressive degeneration of spinal cord (posterior roots, Posterior Columns)
- Charcot's Joints
- Argyll-Robertson Pupil (Prostitute Pupil)
- Syphilitic Paresis (Dementia Paralytica, Paretic Neurosyphilis)
- Chronic meningoencephalitis
- Emotional lability and altered personality as well as affect
- Memory deficits
- Progressive Dementia
- Dysarthria and other speech changes
- Myoclonic Jerks
- Action Tremor
- Seizures
- Hyperreflexia
- Positive Babinski Reflex
- Evolves into Psychosis
- Argyll-Robertson Pupil (Prostitute Pupil) may be present
- Labs: CSF Evaluation
- See Syphylis Testing
- Screening
- CSF VDRL (high Specificity)
- CSF white cell count >10/mm3
- CSF Protein >50 mg/dl
- Retesting if CSF VDRL negative
- Treponemal specific CSF tests (e.g. TPHA)
- High False Positive Rate
- Consider TPHA index (compares CSF to serum titer)
- Spirochete DNA PCR from CSF sample
- Higher Specificity than TPHA
- Not yet widely available
- Experimental markers
- B-Cell chemoattractant chemokinge (CXCL13 or CXC motif)
- Increased CSF concentrations may reliably predict Neurosyphilis
- Marra (2010) Sex Transm Dis 37(5):283-7 [PubMed]
- Management
- See Syphilis
- Penicillin (with probenacid 500 mg orally four times daily for 10-14 days)
- Aqueous crystalline Penicillin G
- Dose: 3-4 MU IV every 4 hours for 10-14 days (18-24 MU daily)
- Alternative: 0.75 to 1 MU/hour continuous IV
- Procaine Penicillin G (only in compliant patients)
- Dose 2.4 MU IM once daily for 14 days
- Use with Probenecid 500 mg qid for 14 days
- Penicillin Allergy
- Desensitize and treat with Penicillin
- Ceftriaxone 2 g IM/IV qd for 14 days
- Repeat testing for Syphilis
- Repeat Syphilis Testing at 6 and 12 months
- Expect four fold reduction in titer after treatment
- Reevaluate and HIV Test if fails to have four fold drop in titer
- References
- (2019) Sanford Guide, accessed on IOS 11/6/2019
- Green, Cohen, Billington (2016) Crit Dec Emerg Med 30(11): 4-10
- Ricco (2020) Am Fam Physician 102(2): 91-8 [PubMed]
- Workowski (2010) MMWR Recomm Rep 59(RR-12): 1-110 [PubMed]