Correspondence

Cerebral Syphilitic Gumma in an HIV-Negative Patient Presenting as Prolonged Focal Motor Status Epilepticus

N Engl J Med 1996; 335:1159-1160October 10, 1996

Article

To the Editor:

The incidence of seizures in neurosyphilis ranges from 14 to 60 percent,1 with no reports of focal status epilepticus as a presenting complaint. We describe a patient in whom cerebral syphilitic gumma was the underlying cause of focal motor seizures.

A 47-year-old previously healthy right-handed man experienced a generalized seizure followed by continuous clonic movement of his right foot 24 hours before admission. His general physical examination was unremarkable. Neurologic evaluation revealed a normal Mini–Mental State Examination, no cranial-nerve deficits, normal strength, and continuous clonic movements of the right foot. There was no reflex asymmetry, and sensory and coordination testing were unrevealing. The patient had no meningeal signs. Magnetic resonance imaging of the brain demonstrated an abnormal T₂-weighted signal of the left frontal area (Figure 1AFigure 1T₂-Weighted Magnetic Resonance Images of the Brain at Admission (Panel A) and after Treatment (Panel B).), which showed multifocal enhancement with the administration of gadolinium. An electroencephalogram indicated continuous slowing over the left frontal central region of the head. Analysis of cerebrospinal fluid showed a total protein concentration of 117 mg per deciliter, a glucose concentration of 69 mg per deciliter, a white-cell count of 11 per cubic millimeter, and no red cells. A differential count of the white cells revealed 82 percent lymphocytes and 18 percent polymorphonuclear cells. A Venereal Disease Research Laboratory (VDRL) test of serum was reactive at a titer of 1:16, and a test for fluorescent treponemal antibody, absorbed, was positive. A VDRL test of cerebrospinal fluid was reactive at a titer of 1:2. Cultures of cerebrospinal fluid were negative. Tests of serum for hepatitis and Lyme disease, as well as for antinuclear antibodies and rheumatoid factor, were negative, and levels of angiotensin-converting enzyme were negative. Tests for antibodies against the human immunodeficiency virus (HIV) and for HIV p24 antigen were negative, and the erythrocyte sedimentation rate was 53 mm per hour. The patient was treated intravenously with phenytoin and high-dose penicillin G for 21 days. Magnetic resonance imaging of the brain six days after the initiation of therapy showed no enhancement of the left frontal lesion, and one month later the abnormal signal had disappeared (Figure 1B). Studies of cerebrospinal fluid six months after the completion of therapy were normal, with a nonreactive VDRL test.

The findings of abnormalities on magnetic resonance imaging and a reactive VDRL test of cerebrospinal fluid, along with a therapeutic response to intravenous penicillin, made the diagnosis of neurosyphilis almost certain in our patient.2 The incidence of neurosyphilis has increased over the past two decades, particularly in immunocompromised hosts.3,4 In patients presenting with focal motor status epilepticus and corresponding abnormally enhancing lesions on magnetic resonance imaging of the brain, the diagnosis of neurosyphilis should be seriously considered regardless of their immune status. The response to treatment should be evaluated by follow-up radiologic and cerebrospinal fluid studies.

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

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Citing Articles (9)

Citing Articles

1. 1

   Jing-cheng Li, Ali Mahta, Ryan Y. Kim, Marlon Saria, Santosh Kesari. (2011) Cerebral syphilitic gumma: a case report and review of the literature. Neurological Sciences
   CrossRef

2. 2

   Kyle M. Fargen, Jorge E. Alvernia, Christine S. Lin, Miguel Melgar. (2009) CEREBRAL SYPHILITIC GUMMATA. Neurosurgery 64:3, 568-576
   CrossRef

3. 3

   Chan Woo Lee, Mi-Jin Lim, Dongwook Son, Jin-Soo Lee, Moon-Hyun Cheong, In Shu Park, Myoung-Kwan Lim, Eunsil Kim, Yoon Ha. (2009) A Case of Cerebral Gumma Presenting as Brain Tumor in a Human Immunodeficiency Virus (HIV)-Negative Patient. Yonsei Medical Journal 50:2, 284
   CrossRef

4. 4

   A. Sesar, M. Arias, I. Requena, I. Pereiro. (2008) Status epilepticus secondary to luetic encephalitis: evolution of neuroimaging findings. Journal of Neurology 255:3, 438-440
   CrossRef

5. 5

   Yang-Pei Chang, Ruey-Tay Lin, Ching-Kuan Liu, Hai-Lun Chao, A-Ching Chao. (2006) Neurosyphilis Presenting With Status Epilepticus. The Neurologist 12:6, 314-317
   CrossRef

6. 6

   Beau M. Ances, Renee Shellhaus, Mark J. Brown, O.Vanessa Rios, Susan T. Herman, Jacqueline A. French. (2004) Neurosyphilis and status epilepticus: case report and literature review. Epilepsy Research 59:1, 67-70
   CrossRef

7. 7

   Thanh G. Phan, Ernest R. Somerville, Sharon Chen. (1999) Intractable Epilepsy as the Initial Manifestation of Neurosyphilis. Epilepsia 40:9, 1309-1311
   CrossRef

8. 8

   Alberto Primavera, Claudio Solaro, Leonard Cocito. (1998) De Novo Status Epilepticus as the Presenting Sign of Neurosyphilis. Epilepsia 39:12, 1367-1369
   CrossRef

9. 9

   (1997) Cerebral Syphilitic Gumma. New England Journal of Medicine 336:14, 1027-1028
   Full Text

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