Neurosyphilis
1990 Case Definition

Background

Syphilis is a complex, sexually transmitted disease with a highly variable clinical course. Classification by a clinician with expertise in syphilis may take precedence over the following case definitions developed for surveillance purposes.

Clinical Description

The characteristic lesion of primary syphilis is the chancre, but atypical primary lesions may occur.

Laboratory Criteria For Diagnosis

Demonstration of Treponema pallidum in clinical specimens by darkfield, fluorescent antibody, or equivalent microscopic methods

Probable

A clinically compatible case with one or more ulcers (chancres) consistent with primary syphilis and a reactive serologic test

Confirmed

A clinically compatible case that is laboratory confirmed

Clinical Description

A stage of infection due to Treponema pallidum, characterized by localized or diffuse mucocutaneous lesions and generalized lymphadenopathy. Constitutional symptoms are common, and clinical manifestations are protean. The primary chancre may still be present.

Laboratory Criteria For Diagnosis

Demonstration of T. pallidum in clinical specimens by darkfield, fluorescent antibody, or equivalent microscopic methods

Probable

A clinically compatible case with a reactive nontreponemal (VDRL, RPR) test titer of greater than or equal to 4

Confirmed

A clinically compatible case that is laboratory confirmed

Clinical Description

A stage of infection due to Treponema pallidum in which organisms persist in the body of the infected person without causing symptoms or signs. Latent syphilis is subdivided into early, late, and unknown syphilis categories based upon the length of elapsed time from initial infection.

Presumptive

No clinical signs or symptoms of syphilis and the presence of one of the following:

• No past diagnosis of syphilis and a reactive nontreponemal test, and a reactive treponemal (fluorescent treponemal antibody-absorbed [FTA-ABS], microhemagglutination assay for antibody to Treponema pallidum [MHA-TP]) test
• A past history of syphilis therapy and a current nontreponemal test titer demonstrating fourfold or greater increase from the last nontreponemal test titer

Clinical Description

A subcategory of latent syphilis. When initial infection has occurred within the previous 12 months, latent syphilis is classified as early.

Presumptive

Latent syphilis (see above) of a person who has evidence of having acquired the infection within the previous 12 months based on one or more of the following criteria:

• A nonreactive serologic test for syphilis or a nontreponemal titer that has dropped fourfold within the past 12 months
• A history of symptoms consistent with primary or secondary syphilis without a history of subsequent treatment in the past 12 months
• A history of sexual exposure to a partner with confirmed or presumptive primary or secondary syphilis, or presumptive early latent syphilis, and no history of treatment in the past 12 months
• Reactive nontreponemal and treponemal tests from an individual whose only possible exposure occurred within the preceding 12 months

Clinical Description

A subcategory of latent syphilis. When initial infection has occurred greater than 1 year previously, latent syphilis is classified as late.

Presumptive

Latent syphilis (see above) of a patient who shows no evidence of having acquired the disease within the past 12 months (see Early Latent Syphilis) and whose age and titer do not meet the criteria specified for unknown latent syphilis

Clinical Description

A subcategory of latent syphilis. When the date of initial infection cannot be established as occurring within the previous year, and the patient's age and titer meet criteria described below, latent syphilis is classified as unknown latent.

Presumptive

Latent syphilis (see above) that does not meet the criteria for early latent syphilis, and the patient is 13-35 years of age with a nontreponemal test serologic titer of greater than or equal to 32

Clinical Description

A fetal death that occurs after a 20-week gestation or in which the fetus weighs greater than 500g, and the mother had untreated or inadequately treated* syphilis at delivery

Comments

For reporting purposes, syphilitic stillbirths should be reported as cases of congenital syphilis.

*Inadequate treatment consists of any non-penicillin therapy or penicillin given less than 30 days before delivery.

Clinical Description

A condition caused by infection in utero with Treponema pallidum. A wide spectrum of severity exists, and only severe cases are clinically apparent at birth. An infant (less than 2 years) may have signs such as hepatosplenomegaly, characteristic skin rash, condyloma lata, snuffles, jaundice (non-viral hepatitis), pseudoparalysis, anemia, or edema (nephrotic syndrome and/or malnutrition). An older child may have stigmata such as interstitial keratitis, nerve deafness, anterior bowing of shins, frontal bossing, mulberry molars, Hutchinson teeth, saddle nose, rhagades, or Clutton joints.

Laboratory Criteria For Diagnosis

Demonstration of T. pallidum by darkfield microscopy, fluorescent antibody, or other specific stains in specimens from lesions, placenta, umbilical cord, or autopsy material.

Presumptive

The infection of an infant whose mother had untreated or inadequately treated* syphilis at delivery, regardless of signs in the infant; or the infection of an infant or child who has a reactive treponemal test for syphilis and any one of the following:

• Any evidence of congenital syphilis on physical examination
• Any evidence of congenital syphilis on long bone x-ray
• A reactive cerebrospinal fluid (CSF) VDRL
• An elevated CSF cell count or protein (without other cause)
• A reactive test for fluorescent treponemal antibody absorbed-19S-IgM antibody

Confirmed

A case (among infants) that is laboratory confirmed.

Comments

Congenital and acquired syphilis may be difficult to distinguish when a child is seropositive after infancy. Signs of congenital syphilis may not be obvious, and stigmata may not yet have developed.
Abnormal values for CSF VDRL, cell count, and protein, as well as IgM antibodies, may be found in either congenital or acquired syphilis. Findings on long bone x-rays may help, since x-ray changes in the metaphysis and epiphysis are considered classic for congenitally acquired disease. The decision may ultimately be based on maternal history and clinical judgment. The possibility of sexual abuse should be considered.
For reporting purposes, congenital syphilis includes cases of congenitally acquired syphilis among infants and children, as well as syphilitic stillbirths.

*Inadequate treatment consists of any non-penicillin therapy or penicillin given less than 30 days before delivery.