II. Epidemiology

  1. Occurs in 10% of untreated Syphilis

III. Pathophysiology

  1. Meningovasculitis
  2. Degenerative parenchymal changes in any part of the CNS

IV. Symptoms

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

V. Signs

  1. Tremor of lips, Tongue or hands
  2. Argyll Robertson Pupil
  3. Seizures
  4. Ataxia
  5. Aphasia
  6. Hyperreflexia
  7. Cognitive changes to progressive Dementia

VI. Types

  1. Asymptomatic (at onset of Neurosyphilis)
    1. Cerebrospinal Fluid positive VDRL (Meningitis)
    2. Argyll-Robertson Pupil (Prostitute Pupil) may be present
  2. Meningeal Neurosyphilis
    1. Intracranial Pressure increased
  3. Ocular Syphilis
    1. Iritis, Uveitis or chorioretinitis
    2. Painless Vision Loss
  4. Meningovascular Syphilis
    1. Begins 5 to 10 years after initial infection
    2. Acute or subacute Aseptic Meningitis
    3. Risk of Cerebrovascular Accident (multiple small infarctions) with variable distribution
  5. 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. Abnormal rhomberg sign
    8. Progressive degeneration of spinal cord (posterior roots, Posterior Columns)
    9. Charcot's Joints
    10. Argyll-Robertson Pupil (Prostitute Pupil)
      1. Accommodates but does not react
  6. 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

IX. References

  1. Green, Cohen, Billington (2016) Crit Dec Emerg Med 30(11): 4-10
  2. Workowski (2010) MMWR Recomm Rep 59(RR-12): 1-110 [PubMed]

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Ontology: Neurosyphilis (C0027927)

Definition (NCI) Infection of the brain or spinal cord by Treponema pallidum. It occurs many years following the original infection which remained untreated. Signs and symptoms include abnormal gait, blindness, depression, paralysis, seizures and dementia.
Definition (MSH) Infections of the central nervous system caused by TREPONEMA PALLIDUM which present with a variety of clinical syndromes. The initial phase of infection usually causes a mild or asymptomatic meningeal reaction. The meningovascular form may present acutely as BRAIN INFARCTION. The infection may also remain subclinical for several years. Late syndromes include general paresis; TABES DORSALIS; meningeal syphilis; syphilitic OPTIC ATROPHY; and spinal syphilis. General paresis is characterized by progressive DEMENTIA; DYSARTHRIA; TREMOR; MYOCLONUS; SEIZURES; and Argyll-Robertson pupils. (Adams et al., Principles of Neurology, 6th ed, pp722-8)
Concepts Disease or Syndrome (T047)
MSH D009494
ICD9 094.9, 094
ICD10 A52.3
SnomedCT 187356008, 186884004, 154384001, 186895005, 266210001, 26039008
LNC LA10492-9
French NEUROSYPHILIS, Neurosyphilis, non précisée, Neurosyphilis, Syphilis nerveuse, Neuro-syphilis, Syphilis du SNC, Syphilis du système nerveux central
English NEUROSYPHILIS, Neurosyphilis, unspecified, [X]Neurosyphilis, unspecified, CNS SYPHILIS, SYPHILIS CNS, neurosyphilis (diagnosis), neurosyphilis, Central Nervous System Syphilis, Syphilis, Central Nervous System, Syphilis, CNS, Neurosyphilis NOS, Neurosyphilis [Disease/Finding], cns syphilis, syphilis cns, syphilis cerebrospinal, cerebrospinal syphilis, cerebrospinal syphilis (diagnosis), [X]Neurosyphilis, unspecified (disorder), Neurosyphilis NOS (disorder), Neurosyphilis (disorder), Syphilis of central nervous system, central nervous system; syphilitic, cerebrospinal; syphilitic, nervous system; syphilitic, neurosyphilitic, syphilis; central nervous system, syphilis; cerebrospinal, syphilis; nervous system, Neurosyphilis, NOS, Syphilis of central nervous system, NOS, Neurosyphilis, Syphilis of central nervous system NOS
German NEUROSYPHILIS, Neurosyphilis, unspezifisch, Neurosyphilis, nicht naeher bezeichnet, Neurosyphilis, Lues, Zentralnervensystem, Neuroleus, Syphilis, Zentralnervensystem
Dutch neurosyfilis, niet-gespecificeerd, centraal zenuwstelsel; syfilis, cerebrospinaal; syfilis, syfilis; centraal zenuwstelsel, syfilis; cerebrospinaal, syfilis; zenuwstelsel, zenuwstelsel; syfilis, Neurosyfilis, niet gespecificeerd, neurosyfilis, Lues, neuro-, Neurolues, Neurosyfilis, Syfilis van het centrale zenuwstelsel
Italian Neurosifilide non specificata, Neurosifilide, Neurolue
Portuguese Neurossífilis NE, NEUROSIFILIS, Neurossífilis, Sífilis do Sistema Nervoso Central
Spanish Neurosífilis no especificada, neurosífilis, SAI (trastorno), [X]neurosífilis, no especificada (trastorno), [X]neurosífilis, no especificada, neurosífilis, SAI, Paresis Juvenil, neurosífilis (trastorno), neurosífilis, sífilis del SNC, sífilis del sistema nervioso central, Neurosífilis, Sífilis del Sistema Nervioso Central
Japanese 神経梅毒、詳細不明, シンケイバイドクショウサイフメイ, シンケイバイドク, 麻痺性神経梅毒, 痴呆-麻痺性, 進行麻痺, 麻痺-進行, 麻痺性痴呆, 進行性麻痺, 神経梅毒, 梅毒-神経
Swedish Neurosyfilis
Czech neurosyfilis, Neurosyfilis, blíže neurčená, Neurosyfilis
Finnish Neurosyfilis
Russian NEIROSIFILIS, PARALICH PROGRESSIVNYI, PROGRESSIVNYI PARALICH, НЕЙРОСИФИЛИС, ПАРАЛИЧ ПРОГРЕССИВНЫЙ, ПРОГРЕССИВНЫЙ ПАРАЛИЧ
Korean 상세불명의 신경매독
Croatian NEUROLUES
Polish Kiła układu nerwowego
Hungarian neurosyphilis, neurosyphilis, nem meghatározott
Norwegian Nevrosyfilis, Syfilis i sentralnervesystemet, Nevrolues

Ontology: Tabes Dorsalis (C0039223)

Definition (MSHCZE) Onemocnění CNS, zejm. míchy, které je součástí třetího stadia syfilis (neurolues). Vzniká za mnoho let (cca 5 až 20) po infekci. Postiženy bývají zadní kořeny a zadní provazce míšní a přilehlé mozkové pleny. Bývají změny i ve zrakové dráze, bývá endarteritida. Postižen může být zrak. Jsou četné neurologické příznaky – Argyll-Robertsonova zornice, hyporeflexie až areflexie, spinální ataxie, porucha hlubokého čití, porucha chůze, trofické poruchy kloubů (tabické artropatie). Příznaky z iritace zadních kořenů vedou k ostrým vystřelujícím bolestem a bolestem vycházejícím z různých vnitřních orgánů a imitujícím jejich onemocnění (tabická krize). Terapie antibiotiky (penicilin, ceftriaxon aj.). (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A form of neurosyphilis characterized by slowly progressive degeneration of the spinal cord. Signs and symptoms include pain, ataxia, loss of coordination, personality changes, blindness, urinary incontinence, dementia, and degeneration of the joints.
Definition (MSH) Parenchymatous NEUROSYPHILIS marked by slowly progressive degeneration of the posterior columns, posterior roots, and ganglia of the spinal cord. The condition tends to present 15 to 20 years after the initial infection and is characterized by lightening-like pains in the lower extremities, URINARY INCONTINENCE; ATAXIA; severely impaired position and vibratory sense, abnormal gait (see GAIT DISORDERS, NEUROLOGIC), OPTIC ATROPHY; Argyll-Robertson pupils, hypotonia, hyperreflexia, and trophic joint degeneration (Charcot's Joint; see ARTHROPATHY, NEUROGENIC). (From Adams et al., Principles of Neurology, 6th ed, p726)
Concepts Disease or Syndrome (T047)
MSH D013606
ICD9 094.0
ICD10 A52.11
SnomedCT 266134000, 266210001, 186885003, 154384001, 2844004, 316841006, 51928006, 315826004
English TABES DORSALIS, Posterior spinal scler.-syph, Neurosyphilis, Tabetic, Tabetic Neurosyphilis, Spinalis, Tabes, Tabes Spinalis, Tabes Dorsalis [Disease/Finding], dorsalis tabes, dorsalis neurosyphilis tabes, duchenne disease, tabes dorsalis, duchenne's disease, disease duchenne, Duchenne's disease, Tabes dorsalis, Syphilitic posterior spinal sclerosis, Tabetic neurosyphilis, Tabes dorsalis - neurosyphilis, Tabes dorsalis (disorder), Tabetic neurosyphilis (disorder), dorsalis; tabes, tabes; dorsalis, Tabes dorsalis (disorder) [Ambiguous], Tabes Dorsalis, Duchennes Disease, Posterior spinal sclerosis
Dutch tabes dorsalis, dorsalis; tabes, tabes; dorsalis, Ruggenmergsyfilis, Tabes dorsalis
French Ataxie locomotrice progressive, Tabès, Neurosyphilis tabétique
Portuguese Tabes dorsal, Sífilis da Medula Espinhal, Tabes Dorsal
Spanish Tabes dorsal, neurosífilis tábica, ataxia motora, Sífilis de la Columna Vertebral, enfermedad de Duchenne (trastorno), enfermedad de Duchenne, esclerosis espinal posterior sifilítica, neurosífilis tabética (trastorno), neurosífilis tabética, tabes dorsal (concepto no activo), tabes dorsal (trastorno), tabes dorsal, Tabes Dorsal
Swedish Syfilitisk ryggmärgsförtvining
Japanese セキズイロウ, 梅毒-脊髄, 脳梅毒, 脳脊髄梅毒, 脊髄梅毒, 梅毒-脳, 脊髄ろう, 脊髄癆, 脊髄後索硬化症
Czech tabes dorsalis, Tabes dorsalis, úbytě míchy
Finnish Selkäydinkato
Italian Atassia spinale, Tabe dorsale, Atassia locomotoria
Russian SPINNAIA SUKHOTKA, DINAMICHESKAIA ATAKSIIA, MOZGA SPINNOGO SIFILIS, SPINNOGO MOZGA SIFILIS, LOKOMOTORNAIA ATAKSIIA, TABES DORSALIS, ДИНАМИЧЕСКАЯ АТАКСИЯ, ЛОКОМОТОРНАЯ АТАКСИЯ, МОЗГА СПИННОГО СИФИЛИС, СПИННАЯ СУХОТКА, СПИННОГО МОЗГА СИФИЛИС
Polish Wiąd rdzenia, Kiła rdzenia kręgowego
Hungarian Tabes dorsalis
Norwegian Tabes dorsalis, Ryggmargssyfilis
German Tabes dorsalis

Ontology: General Paralysis (C0205858)

Concepts Disease or Syndrome (T047)
MSH D009494
ICD9 094.1
ICD10 A52.17
SnomedCT 186886002, 154384001, 266210001, 313231000009106, 51928006
English General Paralyses, Paralyses, General, Paralysis, General, General Paresis, Paretic Neurosyphilis, General Pareses, Pareses, General, Neurosyphilis, Paretic, Paresis, General, GPI - Gen paresis of insane, General paresis - neurosyph., paretic neurosyphilis (diagnosis), neurosyphilis paresis (GPI), general paresis, paretic neurosyphilis, General Paresis of the Insane, general paralysis, general paralysis of insane, progressive paralysis, dementia paralytica, paralytica dementia, GPI-general paralysis insane, Progressive paralysis (finding), Progressive paralysis, Dementia paralytica, General paresis, Paretic neurosyphilis, Progressive general paresis, GPI - General paresis of the insane, General paralysis of insane, General paresis - neurosyphilis, General paresis of the insane, General paresis - neurosyphilis (disorder), Paralytic dementia, general paresis; neurosyphilis, general; paralysis, general; paresis, neurosyphilis; general paresis, paralysis; general, paralysis; progressive, general, paralysis; progressive, paresis; general, paresis; syphilitic, progressive; paralysis, general, progressive; paralysis, syphilis; paresis, General paresis (disorder), Dementia Paralytica, Paresis (General), General Paralysis, General paralysis of the insane, General paralysis, Progressive general paralysis
Dutch gegeneraliseerde parese, gegeneraliseerd; paralyse, gegeneraliseerd; parese, gegeneraliseerde parese; neurosyfilis, neurosyfilis; gegeneraliseerde parese, paralyse; gegeneraliseerd, paralyse; progressief, gegeneraliseerd, paralyse; progressief, parese; gegeneraliseerd, parese; luetisch, progressief; paralyse, gegeneraliseerd, progressief; paralyse, syfilis; parese, Generale parese, Paretische neurolues
French Parésie générale, Démence paralytique, Paralysie générale, Maladie de Bayle
German allgemeine Parese, Progressive Paralyse, Paralysis progressiva
Italian Paralisi progressiva
Portuguese Paresia geral, Neurossífilis Parética, Paresia Geral
Spanish Paresia general, demencia paralítica, neurosífilis parética, paresia general (trastorno), paresia general progresiva, paresia general, Neurosífilis Parética, Paresia General
Czech Celková paréza
Hungarian Általános paresis
Norwegian Hjernesyfilis, Paretisk nevrosyfilis, Paralysis generalis

Ontology: Argyll-Robertson pupil (finding) (C0234668)

Concepts Finding (T033)
SnomedCT 267749005, 155204008, 4129001
Italian Pupille di Argyll-Robertson, Pupilla di Argyll Robertson
Dutch pupil van Argyll Robertson, fenomeen; Argyll Robertson, pupil; Argyll Robertson, Argyll-Robertson pupillen
French Pupille d'Argyll Robertson, Pupilles d'Argyll-Robertson
German Argyll-Robertson-Pupille, Argyll-Roberton Zeichen
Portuguese Pupila de Argyll Robertson, Pupilas de Argyll-Robertson
Spanish Pupila de Argyll Robertson, pupila de Argyll - Robertson (hallazgo), pupila de Argyll - Robertson, Pupilas de Argyll-Robertson
Japanese アーガイルロバートソン瞳孔, アーガイルロバートソンドウコウ
English Argyll-Robertson pupil (finding), Argyll Robertson pupil (small, react to convergence only), Argyll Robertson pupil (physical finding), Argyll Robertson pupil, the pupil was small and reacted to convergence only, Argyll-Robertson pupils, Pupil Argyll Robertson, argyll pupils robertson, argyll-robertson pupil, argyll robertson pupil, Argyll-Robertson pupil, Argyll Robertson (etiology), Argyll Robertson (manifestation), phenomenon; Argyll Robertson (etiology), phenomenon; Argyll Robertson (manifestation), pupil; Argyll Robertson (etiology), pupil; Argyll Robertson (manifestation)
Czech Argyll-Robertsonovy zornice, Argyll Robertsonova zornice
Hungarian Argyll Robertson pupilla, Argyll-Robertson-pupillák

Ontology: Meningovascular syphilis - quaternary stage (C0343695)

Concepts Disease or Syndrome (T047)
SnomedCT 240568004
English Meningovas syph - quat stage, Meningovascular syphilis - quaternary stage, Meningovascular syphilis - quaternary stage (disorder)
Spanish sífilis meningocardiovascular, sífilis meningovascular - etapa cuaternaria (trastorno), sífilis meningovascular - etapa cuaternaria