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Correspondence

Cerebral Syphilitic Gumma

N Engl J Med 1997; 336:1027-1028April 3, 1997

Article

To the Editor:

The Image in Clinical Medicine involving syphilitic gummas in a patient with human immunodeficiency virus (HIV) infection who presented with focal seizures (Oct. 10 issue)1 caused us much concern. Our concern was heightened by the letter from Suarez et al. about a syphilitic gumma in an HIV-negative patient with focal seizures.2 Both patients had important focal neurologic findings coupled with severely abnormal magnetic resonance imaging (MRI) findings (in the case of the HIV-positive patient, there was a clear mass effect from the lesions and subfalcine herniation). Both patients underwent a lumbar puncture on the basis of which, according to the authors, the diagnosis of neurosyphilitic gummas was made.

However, the cerebrospinal fluid findings described in the HIV-positive patient do not establish the diagnosis of neurosyphilis. The patient had a negative serologic test for syphilis and the lymphocytic pleocytosis could have been consistent with HIV meningitis. In the case of the HIV-negative patient, the cerebrospinal fluid Venereal Disease Research Laboratory (VDRL) titer did not show a fourfold increase. In both patients, the diagnosis was established by empirical antibiotic therapy, with subsequent improvement in the imaging findings. The cerebrospinal fluid findings did not appear to be as important in establishing the diagnosis. With the potential risk of untoward tragic effects, the need for lumbar puncture can be seriously questioned in both cases.

Lumbar puncture is contraindicated in the presence of mass lesions, because the risk of transtentorial herniation and death is quite high. The risk appears to be even greater in HIV-positive patients than in HIV-negative patients. Fortunately, these two patients had no serious complications. When neurosyphilitic gummas are suspected, the patients should be presumptively treated and followed with serial radiologic studies.

Pamela Quinn, M.D.
Leon Weisberg, M.D.
Tulane University Medical Center, New Orleans, LA 70112

2 References
  1. 1

    Roeske LC, Kennedy PR. Syphilitic gummas in a patient with human immunodeficiency virus infection. N Engl J Med 1996;335:1123-1123
    Full Text | Web of Science | Medline

  2. 2

    Suarez JI, Mlakar D, Snodgrass SM. Cerebral syphilitic gumma in an HIV-negative patient presenting as prolonged focal motor status epilepticus. N Engl J Med 1996;335:1159-1160
    Full Text | Web of Science | Medline

Author/Editor Response

The authors reply:

To the Editor: We appreciate the concern of Drs. Quinn and Weisberg and agree that in the presence of a mass lesion, the risks associated with lumbar puncture must be carefully considered. At the time of the lumbar puncture, our patient had no signs of increased intracranial pressure and was awake and alert. The basilar cisterns were widely patent on both the initial computed tomographic (CT) scan and subsequent MRI.

We disagree with Drs. Quinn and Weisberg about the need for lumbar puncture. In our patient, who had a history of drug abuse, mouth sores, and recent incarceration and whose HIV status was unknown, it was necessary to exclude bacterial abscess, tuberculosis, toxoplasmosis, and cryptococcal meningitis as potential causes. Although the MRI findings of peripherally enhancing nodules with leptomeningeal and dural components are characteristic of syphilitic gummas,1,2 the diagnosis cannot be established on the basis of the radiographic appearance alone. Furthermore, although the latency period before the development of tertiary neurosyphilis may be shortened in an HIV-positive patient,3 syphilitic gummas are rare enough to warrant the consideration of other causes.

The diagnosis of syphilitic gummas was not based on the results of the lumbar puncture alone. It was based on the combination of several factors: the prior treatment of asymptomatic syphilis, the clinical presentation of focal seizures,1 the positive serologic status, the characteristic MRI findings, and the clinical response to penicillin. The cerebrospinal fluid findings were nonspecific but compatible with neurosyphilis, and they helped rule out other treatable infectious processes. The patient's positive syphilitic serologic status was stated in the legend (the rapid plasma reagin titer was 1:32, and the fluorescent treponemal-antibody-absorption test of serum was positive).

Lisa C. Roeske, M.D.
Philip R. Kennedy, M.D., Ph.D.
Emory University School of Medicine, Atlanta, GA 30322

3 References
  1. 1

    Berger JR, Waskin H, Pall L, Hensley G, Ihmedian I, Post MJ. Syphilitic cerebral gumma with HIV infection. Neurology 1992;42:1282-1287
    Web of Science | Medline

  2. 2

    Brightbill TC, Ihmeidan IH, Post MJ, Berger JR, Katz DA. Neurosyphilis in HIV-positive and HIV-negative patients: neuroimaging findings. AJNR Am J Neuroradiol 1995;16:703-711
    Web of Science | Medline

  3. 3

    Johns DR, Tierney M, Felsenstein D. Alteration in the natural history of neurosyphilis by concurrent infection with the human immunodeficiency virus. N Engl J Med 1987;316:1569-1572
    Full Text | Web of Science | Medline

Author/Editor Response

Regarding the concern expressed by Drs. Quinn and Weisberg about the diagnosis of neurosyphilis and the safety of lumbar puncture in our HIV-negative patient with syphilitic gumma,1 the recommended criteria for the diagnosis of active neurosyphilis in an HIV-negative patient include a reactive cerebrospinal fluid VDRL test, a reactive serum fluorescent treponemal-antibody-absorption test, and pleocytosis of more than 5 to 10 leukocytes per cubic millimeter.2 In the presence of a nontraumatic lumbar puncture and a truly reactive cerebrospinal fluid VDRL test, we believe that our patient most certainly had neurosyphilis and thus that the treatment was not instituted empirically.

The report of the quality-standards subcommittee of the American Academy of Neurology on practice guidelines for lumbar puncture contraindicates the use of that procedure in the presence of suspected elevated intracranial pressure and recommends obtaining a head CT scan to screen patients at risk for herniation.3 Although we strongly agree with those recommendations, there are several points to consider. First, in the era before CT scanning, nonrandomized studies showed that the incidence of actual complications of careful diagnostic lumbar puncture in the presence of papilledema was 1.2 percent.4 Second, with the advent of CT scanning, several anatomical criteria have been proposed as risk factors for herniation, including lateral shifting of midline structures, sulcal effacement, loss of the suprachiasmatic and basilar cisterns, obliteration of the fourth ventricle, and obliteration of the superior cerebellar and quadrigeminal-plate cisterns with sparing of the ambient cisterns.5 Since our patient did not meet those clinical or radiologic criteria, we believed that he was at low risk for cerebral herniation. The analysis of cerebrospinal fluid helped rule out other neurologic conditions, such as inflammatory or neoplastic vasculitis, and other infectious processes that require different treatment methods.

Jose I. Suarez, M.D.
Diane Mlakar, R.N.
Susan M. Snodgrass, M.D.
University Hospitals of Cleveland, Cleveland, OH 44106

5 References
  1. 1

    Suarez JI, Mlakar D, Snodgrass SM. Cerebral syphilitic gumma in an HIV-negative patient presenting as prolonged focal motor status epilepticus. N Engl J Med 1996;335:1159-1160
    Full Text | Web of Science | Medline

  2. 2

    Jordan KG. Modern neurosyphilis -- a critical analysis. West J Med 1988;145:47-57

  3. 3

    Practice parameters: lumbar puncture (summary statement): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 1993;43:625-627
    Web of Science | Medline

  4. 4

    Korein J, Cravioto H, Leicach M. Reevaluation of lumbar puncture: a study of 129 patients with papilledema or intracranial hypertension. Neurology 1959;9:290-297
    Web of Science | Medline

  5. 5

    Gower DJ, Baker AL, Bell WO, Ball MR. Contraindications to lumbar puncture as defined by computed cranial tomography. J Neurol Neurosurg Psychiatry 1987;50:1071-1074
    CrossRef | Web of Science | Medline