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|Year : 2021 | Volume
| Issue : 5 | Page : 1427-1429
Neuro-Behçet's Disease Mimicking Low-Grade Glioma Presenting with Drop Foot
Mustafa Emre Sarac1, Kadir Oktay2, Ebru Guzel3, Mansur Mammadov2, Ibrahim Sari4, Aslan Guzel5
1 Department of Neurosurgery, Adana City Training and Research Hospital, Adana, Turkey
2 Department of Neurosurgery, Cukurova University School of Medicine, Adana, Turkey
3 Department of Radiology, Gaziantep Medical Park Hospital, Gaziantep, Turkey
4 Department of Pathology, Patomer Pathology Laboratory, Gaziantep, Turkey
5 Department of Neurosurgery, Bahcesehir University School of Medicine, İstanbul, Turkey
|Date of Submission||28-Feb-2021|
|Date of Decision||11-Apr-2021|
|Date of Acceptance||25-Jul-2021|
|Date of Web Publication||30-Oct-2021|
Cukurova University School of Medicine, Department of Neurosurgery, Saricam District, Balcali Campus, Adana 01000
Source of Support: None, Conflict of Interest: None
Behçet's disease is a multisystem inflammatory disease that manifests with oral–genital ulcers, skin lesions, arthritis, and ophthalmologic and neurovascular findings. Neurological involvement of Behçet's disease is called neuro-Behçet's disease, and it is a difficult entity to diagnose because of insufficient symptoms. We present a 26-year-old male patient with complaints of headache, dizziness, and drop foot in the right lower extremity. He underwent gross total tumor resection with the preliminary diagnosis of low-grade glioma, according to the preoperative magnetic resonance imaging. Histopathological examination and further investigation revealed an interesting neuro-Behçet's disease case because of the localization and clinical occurrence of the lesion.
Keywords: Behçet's disease, low-grade glioma, neuro-Behçet's disease
Key Messages: This case report showed that parenchymal neuro-Behçet's disease should be kept in mind in the differential diagnosis of low-grade gliomas. Further imaging techniques such as magnetic resonance spectroscopy and cerebrospinal fluid examinations should be performed in the suspected cases.
|How to cite this article:|
Sarac ME, Oktay K, Guzel E, Mammadov M, Sari I, Guzel A. Neuro-Behçet's Disease Mimicking Low-Grade Glioma Presenting with Drop Foot. Neurol India 2021;69:1427-9
Behçet's disease (BD) is classified as a multisystem inflammatory disease that manifests with oral–genital ulcers, skin lesions, arthritis, and ophthalmologic and neurovascular findings. Although it is known as vasculitis, its etiology is unknown., Neurological involvement of BD is referred to as neuro-Behçet disease (NBD) and occurs in 13% of male and 6% of female patients. Because it demonstrates insufficient symptoms and findings, it is difficult to diagnose NBD in this patient group.
NBD is classified as two entities – parenchymal and nonparenchymal. The brain stem is affected 80% in the parenchymal form. Basal ganglia, cerebral hemispheres, and spinal cord are affected less., Parenchymal NBD generally manifests with subacute brain stem syndrome comprising ophthalmoparesis, cranial nerve disorders, dysarthria, and corticospinal disorders such as weakness and ataxia. Hemiparesis and behavior and sphincter disorders are the other findings related to parenchymal involvement., The nonparenchymal form typically presents with intracranial hypertension, venous infarct, and acute meningeal syndrome pertinent to dural sinus thrombosis.
In this report, we define an extraordinary case of NBD presenting with a drop foot due to a tumor-like lesion on the motor cortex of the left brain hemisphere. Because of the uncommon location and atypical presentation of the tumor-like neuro-Behçet lesion, we think it is considerable to notify this case.
| » Case Report|| |
A 26-year-old male patient presented to the neurosurgery department with headache, dizziness, numbness, and weakness in the right-sided upper and lower extremities. His complaints were continuing almost for a year, and he noted an increase in his complaints for the last one month. He had no history of chronic disease and medication. His neurological examination revealed a drop foot and weakness at the distal part of the right lower extremity. Contrast-enhanced brain magnetic resonance imaging (MRI) revealed a 19 × 12 × 19 mm lesion with heterogeneous contrast enhancement, including diffusion limitation in some areas. In the non-contrast series, the lesion was hypointense on T1 and hyperintense on the T2 series. The lesion was demonstrating an extension from the cortex to the white matter [Figure 1]. According to these findings, surgical resection was planned for the lesion, which is considered low-grade glioma. After the gross total resection of the lesion, drop foot finding at the right lower extremity ameliorated. Histopathological examination of the samples revealed perivascular lymphocytic infiltration and parenchymal macrophage infiltration [Figure 2]. After consultation with the pathology department, the lesion was suspected as NBD, and the patient was referred to the neurology department. On further questioning, the patient divulged a short-standing history of his self-limited rare oral aphthous ulcers for less than a year. He negated ever having genital ulcers or eye inflammation. He did not have a family history of BD or prior history of smoking. The neurology department indicated erythema nodosum–like lesions and a positive pathergy test in the patient. He was diagnosed as NBD and underwent follow-up in the neurology department.
|Figure 1: (a) Preoperative axial and sagittal brain magnetic resonance imaging revealing lesion with the size of 19 × 12 × 19 mm in the left motor cortex area. The lesion was hypointense on T1-weighted images and hyperintense on T2-weighted images and demonstrated heterogeneous contrast enhancement. (b) Postoperative axial and sagittal contrast-enhanced brain magnetic resonance imaging revealing total resection of the lesion|
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|Figure 2: (a) Perivascular lymphocytic infiltration and parenchymal macrophage infiltration (hematoxylin and eosin, 400× magnification). (b) Perivascular lymphocytic infiltration and parenchymal macrophage infiltration detected by CD68 immunohistochemical stain (DAB, 400× magnification)|
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| » Discussion|| |
Any of two situations such as genital ulcers, ophthalmologic findings, skin lesions, and positive patergy test in addition to oral aphthous ulcers are necessary to identify BD. Turkey has the highest rate of BD occurrence compared with other Middle Eastern countries. The typical age of onset is between 20 and 40 years, and it is more frequent in men than in women.
NBD is a seldom manifestation of BD. Although it occurs 3 to 6 years after the onset of the disease, it may be seen either simultaneously or prior to full-blown BD.,, Brain stem involvement is the most common appearance of the parenchymal form of NBD. Cerebral hemispheres are affected less, and clinical findings are usually due to the brain stem involvement in this form. Hemispheric lesions with NBD may mimic low-grade gliomas in appearance. These tumor-like lesions usually have heterogeneous contrast enhancement especially at the peripheral zone and are properly limited in contrast series of MRI. The images are hypointense on T1, hyperintense on T2 series, and perilesional edema may be seen in the acute period of parenchymal NBD similar to low-grade gliomas. The increased amount of cells and protein levels may emerge in the cerebrospinal fluid (CSF) with parenchymal NBD in contrast with the nonparenchymal form.,,
This case manifests that although clinical features of the patient are persuasive for low-grade glioma, an aggressive workup is necessary for the differential diagnosis according to the radiologic features; however, the radiological findings, localization, and presentation of the lesion are not characteristic of NBD. Given the radiologically low-grade glioma-like lesion and the age of the patient compatible with low-grade glioma occurrence period, any of the other differential diagnoses such as NBD is surmised. Furthermore, the seldom manifestation of NBD particularly on cerebral hemispheres and absence of presentation with drop foot in the literature directs us to low-grade glioma on diagnosis and treatment. However, there is not any clinical and historical finding attributing to BD, in case NBD may strongly be considered in differential diagnosis and magnetic resonance spectroscopy may be planned to distinguish the lesion from the low-grade glioma., In addition, a small amount of CSF may be obtained for examination to find out if the results are coherent with NBD. Moreover, further questioning maintains convenience to assess the patient properly. In this case, because the patient did not report his rare self-limited oral aphthous ulcers, NBD is not considered in the differential diagnosis. Therefore MR-spectroscopy was not planned to distinguish the low-grade glioma from a nontumoral lesion.
In conclusion; parenchymal NBD is a potentially treatable entity mimicking a wide variety of pathologies, particularly brain stem gliomas due to its common localization. Hemispheric NBD may be seen rarely and may mimic low-grade glioma. NBD is not only a radiologically defined situation but also a clinically diagnosed entity. Apart from being a report of a rare case, this report highlights the importance of being meticulous while evaluating a patient. A carefully taken history and a meticulously performed physical examination would have picked up important findings like aphthous ulcers and skin lesions (erythema nodosum) that could have potentially helped make correct diagnosis preoperatively. Also, the presence of diffusion restriction was an important point against the lesion being low-grade glioma. As a result, questioning the patient is still significant in spite of technological advances in radiologic imaging.
Declaration of patient consent
A written informed consent was obtained from the patient who participated in this study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]