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Neurocritical Care Copyright © 2005 Humana Press Inc. All rights of any nature whatsoever are reserved. ISSN 1541-6933/05/2:300–302 DOI: 10.1385/Neurocrit. Care 2005;2:300–302

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Cerebral Gumma Mimicking Glioblastoma Multiforme Beau M. Ances,1 Shabbar F. Danish,2 Dennis L. Kolson,1 Kevin D. Judy,2 and David S. Liebeskind1,3,* Departments of 1Neurology, 2Neurosurgery and 3Radiology, University of Pennsylvania, Philadelphia, PA

Abstract This article reports an unusual case of a syphilitic gumma with a clinical and radiographical presentation initially suggestive of glioblastoma multiforme. Pathological evaluation was essential in establishing the diagnosis of neurosyphilis and in excluding neoplastic involvement. Cerebral gumma should be considered as part of the differential diagnosis of a midline intracranial lesion observed on magnetic resonance imaging. Key Words: Syphilis; magnetic resonance imaging; gumma; glioblastoma.

(Neurocrit. Care 2005;2:300–302)

Introduction

*Correspondence and reprint requests to: David S. Liebeskind UCLA Stroke Center, 710 Westwood Plaza, Los Angeles, CA 90095 E-mail: [email protected]

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Increasing use of magnetic resonance imaging (MRI) has augmented the characterization of intracranial mass lesions. Determination of specific signal intensities on various MRI has facilitated the differentiation of these lesions, but pathological evaluation via brain biopsy is often needed for confirmation and treatment. Radiographical and pathological correlation is essential in excluding differential diagnoses. Glioblastoma multiforme is commonly entertained as the diagnosis of a midline mass; however, other entities should be considered in the differential diagnosis (1).

Case Report A 42-year-old right-handed man was brought to the hospital by a close friend because of frequent falling. The patient had lived only in the United States and had not traveled elsewhere. He was without a medical history. The patient offered minimal historical details, but his friend noted that he had been experiencing visual hallucinations, headaches, diminished appetite, and prominent weight loss over a period of several months. At presentation, he was noted to be

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cachectic and minimally interactive. General physical examination was unremarkable, with an absence of fever, heart murmur, adenopathy, skin lesions, jaundice, or hepatosplenomegaly. Neurological examination revealed prominent abulia, psychomotor retardation, and tremulousness of bilateral upper extremities. The patient exhibited diminished proprioception with a shuffling gait. Initial laboratory evaluation demonstrated hyponatremia but was otherwise normal. Noncontrast computed tomography (CT) scan revealed a heterogeneous perifalcine mass extending from the corpus callosum bilaterally into subcortical regions of the frontal lobes with considerable mass effect. Emergent MRI demonstrated heterogeneous enhancement of a partially cystic midline butterfly-shaped intra- and extra-axial mass as well as extensive subcortical vasogenic edema (Figure 1A). Coronal postcontrast T1-weighted images showed a butterfly midline lesion with mild surrounding edema (Figure 1B). Interspersed necrotic regions within the lesion and surrounding inflammation and edema were identified on

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Fig. 1. (A) Axial T1-weighted postgadolinium images showed a heterogeneous ring-enhancing midline mass with adjacent vasogenic edema. (B) Coronal T1-weighted postgadolinium images revealed the butterfly-shaped enhancing lesion. (C) FLAIR images demonstrated regions of interspersed necrosis within the lesion and surrounding edema. (D) DWI showed hypointensity of the lesion.

fluid attenuated inversion recovery (FLAIR) images (Figure 1C). Diffusion-weighted imaging (DWI; Figure 1D) failed to reveal hyperintensity in the lesion. The patient was started on steroids to reduce the edema and was subsequently taken to the operating room for a neurosurgical procedure. In the early postoperative period, laboratory studies revealed no evidence of immunosuppression and human immunodeficiency virus seronegativity. Serum angiotensin-

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converting enzyme levels and toxoplasma antibody titers were normal, and subsequent mycobacterial cultures were negative. Previous pre-operative laboratory values demonstrated a positive rapid plasma reagin (titer, 1:16), and treponemal pallidum antibodies were also positive. The patient became hypothermic approximately 24 hours following surgery and was noted to be unresponsive. His hyponatremia worsened, and he rapidly developed diabetes insipidus and became

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302 _________________________________________________________________________________________________Ances et al. comatose. Repeat CT demonstrated increased cerebral edema of bilateral frontal lobes, with effacement of the basal cistern and bilateral uncal herniation. Emergent hemicraniectomy was performed, but the patient died. Neurosurgical biopsy results demonstrated necrotic areas with extensive mixed inflammation, consisting of lymphocytes, plasma cells, neutrophils, and focal collagen deposits. Inflammation was also present in several midsized arteries, with extensive infiltration by macrophages and severe narrowing of the lumens. Stains of the biopsy were negative for micro-organisms. Neoplastic features were not identified on pathology.

Conclusion Syphilis is caused by the spirochete treponema pallidum, a small flagellated spirochete that is an obligate human parasite. Infection often occurs through sexual contact, but lesions can result from direct needle sharing, inoculation, and transplants (2). Over recent years, a significant increase in syphilis, with a concomitant increase incidence of neurosyphilis, has occurred because of increasing numbers of individuals diagnosed with acquired immune deficiency syndrome (AIDS; ref. 3). Cerebral syphilitic gummas are rare and can result from an exuberant cell-mediated response consisting of plasma cells and lymphocytes. Spirochetes are rarely found within these lesions (2). CT findings of cerebral gumma may include hypodensity with a relative paucity of mass effect; however, more dramatic changes may also include focal enhancement with adjacent edema and resultant mass effect. Sporadic case reports of MRI findings in cerebral gumma describe T1-weighted hypo- or isointensity with T2-weighted hyperintensity (4). Enhancement patterns may also be variable (3). The neuro-imaging findings observed in this case are consistent with prior case descriptions but are also consistent with the appearance of a glioblastoma multiforme. Similar findings were noted in a cerebral gumma case mimicking a glioblastoma (5). In the previously reported case, the mass lesion was located at the skull base, with extension into basal aspects of the right fronto-temporal lobes. The ring-enhancing lesion surrounded the right middle cerebral artery and was suspected to be a glioblastoma based on neuroradiological findings, despite the fact that the patient had known active syphilis. At least 15 other imaging reports of cerebral gumma include solitary lesions (5). The neuro-imaging findings of cerebral gumma, including enhancement patterns, may be nonspecific or may mimic intracranial neoplasms.

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The presence of a solitary, enhancing, midline, frontal butterfly-shaped mass is highly suggestive of a glioblastoma multiforme. However, the differential diagnosis of such a midline mass may include several etiological categories of disease, including infection (tuberculosis, fungal, abscess), inflammatory processes (sarcoid, lymphoma), other neoplastic disorders, and atypical infarction (1). Pathological evaluation of this lesion excluded these alternative diagnoses. The unusual MRI findings observed in our case suggest the necessity of adding neurosyphilis to the list of midline mass mimicries. This is particularly important because the clinical presentation of neurosyphilis is often unremarkable, and treatment may be beneficial. Our case and previous reports of cerebral gumma suggest a predilection for these lesions in close proximity to the basal cerebral vessels. Although cerebral gummas are considered a distinct entity regarding meningovascular syphilis, the vascular relationship of cerebral gumma has not been wellcharacterized. Meningovascular syphilis typically affects the anterior or middle cerebral artery lenticulostriate perforators (2). Interestingly, our case and the prior report of cerebral gumma mimicking glioblastoma demonstrated involvement in the region of these perforators (5). This localization may be a helpful diagnostic clue in the recognition of a syphilitic mass. The vascular relationship of cerebral gumma also suggests similarities with the meningovascular manifestations of neurosyphilis.

References 1. Hartmann M, Heilman S, Harting I, et al. Distinguishing of primary cerebral lymphoma from high-grade glioma with perfusion weighted magnetic resonance imaging. Neurosci Lett 2003;338(2):119–122. 2. Knox JM, Musher D, Guzick ND. The pathogenesis of syphilis and related treponematoses. In: Johnson RC, ed., The Biology of Parasitic Spirochetes, San Diego: Academic Press, 1976, pp. 249–259. 3. Brightbill TC, Ihmeidan IH, Post MJ, et al. Neurosyphilis in HIV-positive and HIV-negative patients: neuroimaging findings. Am J Neuroradiol 1995;16:703–711. 4. Bash S, Hathout GM, Cohen S. Mesiotemporal T2-weighted hyperintensity: neurosyphilis mimicking herpes encephalitis. Am J Neuroradiol 2001;22:314–316. 5. Uemura K, Yamada T, Tsukada A, et al. Cerebral gumma mimicking glioblastoma on magnetic resonance images—case report. Neurol Med Chir (Tokyo) 1995;35(7):462–466.

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