- Neurosurgery Department, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil
- Neurology Department, Hospital de Clinicas de Porto Alegre, Porto Alegre, RS, Brazil
- Radiology Department, Hospital de Clinicas de Porto Alegre, Porto Alegre, RS, Brazil
- Department of Pharmacology, Institute for Basic Health Sciences, Federal University of Rio Grande do Sul, Porto Alegre, RS, Brazil
- Cancer and Neurobiology Laboratory, Experimental Research Center, Porto Alegre Clinical Hospital, Federal University of Rio Grande do Sul, Brazil
- Advanced Center of Neurology and Neurosurgery (CEANNE)-Brazil, Porto Alegre, RS, Brazil
Correspondence Address:
Pedro L. Tramontini
Neurosurgery Department, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil
Advanced Center of Neurology and Neurosurgery (CEANNE)-Brazil, Porto Alegre, RS, Brazil
DOI:10.4103/sni.sni_3_17
Copyright: © 2017 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.How to cite this article: Pedro L. Tramontini, Alessandro Finkelsztejn, Juliana Á. Duarte, Guilherme T. Santos, Rafael Roesler, Gustavo R. Isolan. Neuro-Behçet disease mimicking brain tumor: A case report. 05-Jun-2017;8:97
How to cite this URL: Pedro L. Tramontini, Alessandro Finkelsztejn, Juliana Á. Duarte, Guilherme T. Santos, Rafael Roesler, Gustavo R. Isolan. Neuro-Behçet disease mimicking brain tumor: A case report. 05-Jun-2017;8:97. Available from: http://surgicalneurologyint.com/surgicalint-articles/neuro%e2%80%91behcet-disease-mimicking-brain-tumor-a-case-report/
Abstract
Background:Behçet's disease (BD) is an inflammatory multisystem disease with unknown etiology, and consists of a TRIAD comprising recurrent oral ulcers, genital ulcers, and uveitis. In some cases, the disease affects the central nervous system, called Neuro-Behçet Disease (NBD). Few cases of NBD simulating a brain tumor have been previously reported.
Case Description:Here, we describe the case of a 46-year-old male patient with a previous diagnosis of brain tumor who was later diagnosed for BD.
Conclusion:This case highlights the importance of differential diagnosis of lesions with tumoral features. Checking for the possibility of NBD may help avoiding biopsy in these types of cases.
Keywords: Behçet syndrome, brain tumor-like lesions, brain disease diagnosis, neuro-Behçet Disease
INTRODUCTION
Behçet's disease (BD) is an inflammatory multisystem disease of unknown etiology with periods of relapses and remissions. The disease was named in the memory of “Hulusi Behçet,” a Turkish dermatologist who has described a trisymptom complex, characterized by recurrent oral ulcers, genital ulcers, and uveitis.[
Few cases of NBD mimicking a brain tumor have been reported. We report a case of a patient with a previous diagnostic of brain tumor who was diagnosed as BD. We discuss the case and review the literature regarding this rare presentation of BD.
CASE REPORT
MR, male, 46-year-old, came to the hospital with a complaint of subacute hemiparesis in the left lower limb, accompanied by mental confusion.
In the past, he had the same clinical presentation associated to oral ulcers. He was submitted to brain MRI and to lumbar puncture. The brain MRI showed a T2-hyperintense lesion in the regions of mesencephalon and right parahippocampus [
DISCUSSION
Epidemiology
Generally, BD starts in the third or fourth decade of life,[
Pathophysiology
In spite of the several immunological components demonstrated, the exact mechanism which leads to the inflammatory changes remains obscure. A plausible hypothesis is that an infectious agent or an autoantigen, such as heat shock proteins (HSP), triggers an inflammatory reaction that leads to the disease, in genetically predisposed individuals.[
Genetic factors play an important role in the development of BD. Ohno et al. has first described the association between the disease and HLA-B5(51) in 1973.[
It has been proposed that the genetic susceptibility induces the overexpression of pro-inflammatory cytokines, such as Th1 and Th17, thus enhancing the inflammatory reaction in BD.[
Clinical features
The typical features of BD include mucocutaneous lesions. Oral and genital ulcers, together with cutaneous, ocular, and articular lesions are the most frequent features of the disease in all countries.[
The genital ulcers are similar in appearance and course to the oral ones. They are most commonly found on the scrotum in men and the vulva in women. They are usually deeper and have a tendency to scar and are also debilitating.[
Other symptoms of BD include ocular, vascular, gastrointestinal, and neurological involvement. Recent studies demonstrated that these other lesions can have their onsets later in the course of the disease (5–10 years).[
Diagnosis
The diagnosis of BD is primarily based on clinical criteria following the exclusion of other conditions. The International Study Group for Behçet's Disease Criteria consists of presence of recurrent oral ulcers, plus any two of recurrent genital ulcers, typical ocular lesions, typical cutaneous lesions, or a positive skin pathergy test (SPT).
An international team developed new criteria under the name International Criteria for Behçet's Disease, briefly “ICBD” criteria, which were published in 2006. These new criteria were revised in 2013, becoming the 17th set of classification/diagnosis criteria for BD. In this criteria, oral ulcers (OU), genital ulcers (GU) and ocular manifestations get each 2 points, and others (skin lesions, neurological manifestations, vascular manifestations, and positive SPT) get 1 point. If a patient gets 4 points or more, the patient is diagnosed as having BD.[
Neurological features
Neurological involvement is one of the most serious causes of morbidity and mortality in BD.[
There are two categories of CNS involvement in BD: parenchymal or non-parenchymal. In the first, the principal finding is meningoencephalitis. Lesions can occur in brainstem, spinal cord, in the brain, or be diffuse.[
It is important to note that NBD is an inflammatory perivasculitis.[
Making a NBD diagnosis is easier when the patient has systemic findings of the disease. Also, it is uncommon for BD to arise in the absence of systemic features. No validated criteria exist.[
The characteristic MRI lesion in parenchymal involvement is an upper brainstem lesion that extends into the thalamus and basal ganglia on one side. Bilateral lesions are less common.[
In the acute phase, most patients have single lesions, but in the chronic phase, more diffuse involvement is noted, making it difficult to differentiate between NBD and multiple sclerosis.[
CSF constituent are altered in around 70–80% of patients with parenchymal complications.[
Subacute meningoencephalitis accounts for 75% of cases in parenchymal NBD.[
Brain tumor-like NBD
Few cases of NBD mimicking a brain tumor have been reported. The earliest case was reported in 1987.[
Differential diagnosis is extensive. It includes infectious causes (viral, bacterial, fungal), uveomeningitic syndromes (sarcoid, SLE, Sjögren), Neoplastic causes, complications of other systemic diseases or complications of treatment for BD (drug induced-meningitis, infections with immunosuppression).
Patients who have an acute parenchymal inflammatory episode usually recover well with corticosteroid treatment.[
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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