Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 51, Num. 3, 2003, pp. 376-378

Neurology India, Vol. 51, No. 3, July, 2003, pp. 376-378

Two cases of neuro-Behçet's disease mimicking cerebral tumor

Tuzgen S, Kaya AH, Erdincler D, Oguzoglu SA, Ulu O, Saip S
Departments of Neurosurgery, Neurology and Internal Medicine of Cerrahpasa Medical Faculty, University of Istanbul, Istanbul

Correspondence Address:
Cerrahpasa Tip Fakultesi, Beyin Cerrahisi ABD, 34303 Cerrahpasa, Istanbul,
ahmethilmikaya@yahoo.com

Code Number: ni03122

ABSTRACT

Two cases of neuro-Behçet's disease with isolated, solitary fronto-temporal and mesencephalic lesions respectively are reported. The cases were misdiagnosed as cerebral tumor. The postoperative outcome of the first patient was not satisfactory as he developed hemispheric edema. The second patient, with mesencephalic lesion, was treated only with corticosteroids, and the patient improved significantly. The lesion in this case resolved completely at six-month follow-up. We conclude that distinguishing the isolated solitary cerebral lesion of the Behçet's disease from a tumor may prevent surgical intervention.

INTRODUCTION

Behçet's disease is a chronic relapsing multisystem disorder, originally described in 1937 by the Turkish dermatologist Hulusi Behçet. It is characterized by the clinical triad of oral apthous ulceration, genital ulceration and ocular inflammation. The disease is now recognized as a systemic vasculitis.[1]

Neurological involvement is reported to occur in 4 to 49% of the cases and mainly, it is in the form of cerebral venous thrombosis.[2],[3] The most common sites of the neural parenchymal involvement are the diencephalic, pontobulbar, thalamic, hypothalamic, basal ganglia and cerebellar regions. It is rarely seen as an isolated, solitary lesion.[3],[4] In such a case, the differential diagnosis from a primary cerebral tumor is difficult and this may result in an unnecessary surgical intervention.

We present 2 cases of neuro-Behçet's disease, which presented with solitary intracerebral lesion mimicking primary cerebral tumor. The results of the surgical and conservative treatment modalities are discussed.

CASE REPORTS

Case 1
A 59-year-old woman having headache for one month was admitted with complaints of progressive left hemiparesis, lethargy and temporal fits as characterized by sensations of bad smell for the last 24 hours. Physical examination revealed left hemiparesis and bilateral papilloedema. Computerized tomography (CT) scans showed right fronto-temporal mass causing shift of the midline structures suggestive of high-grade glioma [Figure-1]. A right fronto-temporal craniotomy and subtotal surgical excision of the soft, infiltrative and avascular mass was performed. The patient did not regain full consciousness after surgery and CT scans demonstrated hemispheric edema, probably secondary to the perisylvian vascular injury. The patient progressively improved in her consciousness over a period of one month, but her left hemiparesis persisted. Histopathological examination of the lesion revealed reactive gliosis associated with panvasculitis, thrombosis, extensive infarction and vascular proliferation. Retrospective inquiry disclosed history of recurrent oral and genital apthous ulcers. On the basis of the clinical and histological findings, a diagnosis of neuro-Behçet's disease was considered.

Case 2
A 45-year-old woman was admitted with complaints of headache for 3 weeks, and right hemiparesis and somnolence for 48 hours. On physical examination, she was not fully alert and had moderate right hemiparesis. Magnetic resonance imaging (MRI) showed that there was a mass in the left mesodiencephalic junction that enhanced homogenously after contrast injection [Figure-2]. The patient was placed on steroids and was observed clinically. Improvement of the neurological signs was observed during the first 72 hours of corticosteroid therapy. One week after the institution of steroid therapy the patient recovered and had no neurological deficit and was alert. A retrospective history analysis disclosed that the patient had genital apthous ulceration and monoarthritis three months previously. At six-month follow-up, control MRI showed that the lesion had resolved completely [Figure-3].

DISCUSSION

Behçet's disease is a multisystemic disease. Central nervous system involvement can be suspected with a detailed history, careful systemic examination, and proper laboratory and radiological evaluation. Rarely, neuro-Behçet's disease manifests as an isolated, solitary cerebral lesion.[2],[3],[4] In these cases, the correct diagnosis is difficult.

Both CT and MRI are sensitive for the cerebral lesions of neuro-Behçet's disease, the latter investigation being superior and more sensitive. Neuroradiological findings are not specific and the differential diagnosis includes other vasculitic pathologies, multiple sclerosis, ischemic lesions, sarcoidosis, tuberculosis and a glial neoplasm. The lesion is hypodense on CT scans, and has high signal intensity on T2-weighted images and low signal intensity on T1-weighted MRI. A nodular contrast enhancement is possible as was seen in our second case.[3],[5],[6] Unlike multiple sclerosis wherein the white matter is involved primarily, neuro-Behçet's disease involves both gray and white matter and the brainstem. The brainstem involvement is rare in other vasculitic processes. Solitary tuberculoma must also be considered in the differential diagnosis of neuro-Behçet's lesions. In contrast with the usual appearance of the neuro-Behçet's lesions, which are hyperintense on T2-weighted images, the lesion in our second case was hypointense on T2-weighted MRI. This appearance was previously mentioned as a finding only in the subacute or chronic stage of the lesion and was thought to result from the deposits of hemosiderin.[7] No satisfactory explanation is available for this observation.

Although generally the neurological manifestations of Behçet's disease appear two to four years after the first clinical presentation, there are a few reported cases of neuro-Behçet's disease in which the neurological findings preceded the other more typical systemic manifestations of the disease.[3],[5] The majority of them were misdiagnosed as intracranial tumor and some of them were operated as in our first case.[8],[9],[10] Distinguishing a solitary cerebral lesion of neuro-Behçet's disease from the tumoral pathologies is difficult in the absence of other cardinal findings such as recurrent oral and genital apthous ulcerations.

The histopathological findings of the lesion are vessel wall and perivascular mononuclear cell infiltration that is consistent with vasculitis involving both arterial and venous systems.[3] As the underlying pathology in neuro-Behçet's lesion is the result of an inflammatory vasculitic process, the surgical intervention, unlike in the tumoral lesions, means the resection of normal cerebral tissue. Steroid pulse therapy has been found to be effective in the treatment of these lesions.[9],[10]

The suspicion of neuro-Behçet's disease in such a lesion offers a less invasive and effective treatment choice of corticosteroid therapy. Stereotactic biopsy may help establish a diagnosis. Stereotactic biopsy followed by prompt treatment with corticosteroid regimen has been reported to be a successful modality of treatment.[8],[11],[12]

REFERENCES

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