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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 11  |  Issue : 1  |  Page : 89-91

Neuro-Behcet's disease: A report of three clinically distinct cases from North-west India


Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India

Date of Web Publication17-Jan-2018

Correspondence Address:
Dr. Kaushik R Rana
Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kleuhsj.kleuhsj_150_17

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  Abstract 


Behcet's disease (BD) is a multisystem inflammatory disorder with various neurological manifestations common in the Middle-East countries but scarcely reported in the Indian literature. We report three patients of neuro-BD with different neurological manifestations. Two cases presented as cerebral venous sinus thrombosis and the third case as pachymeningitis. It should be included in the differential diagnosis of any neurological disorder with a diagnostic dilemma as it is potentially treatable condition.

Keywords: Behcet's disease, cerebral venous sinus thrombosis, pachymeningitis


How to cite this article:
Sharma C, Kumawat B, Rana KR, Saini T. Neuro-Behcet's disease: A report of three clinically distinct cases from North-west India. Indian J Health Sci Biomed Res 2018;11:89-91

How to cite this URL:
Sharma C, Kumawat B, Rana KR, Saini T. Neuro-Behcet's disease: A report of three clinically distinct cases from North-west India. Indian J Health Sci Biomed Res [serial online] 2018 [cited 2019 Jun 20];11:89-91. Available from: http://www.ijournalhs.org/text.asp?2018/11/1/89/223421




  Introduction Top


Behcet's disease (BD) is a multisystem inflammatory disorder of unknown etiology and characterized by the triad of recurrent oral ulcers, genital ulcers, and uveitis.[1] BD bears the name of Huluci Behcet, a Turkish dermatologist who described the disease in 1937. The prevalence is higher among populations with human leukocyte antigen (HLA) B5 and its split, HLA B51, in the Mediterranean countries and Japan basin.[2] Although the prevalence of HLA B5 in North Indian populations (30%) is comparable to the Mediterranean (27%) and the Japanese (36.9%) populations,[3],[4] exact prevalence of BD is not known in the Indian population. Dermatological, ophthalmological, and musculoskeletal manifestations of BD are thoroughly studied in the Indian literature, but neurological manifestations are scarcely reported.[5]


  Cases Report Top


  • First case: A 35-year-old male presented with a 6-month history of headache without any focal neurological deficit. His magnetic resonance (MR) venogram brain revealed superior sagittal sinus thrombosis [Figure 1]a
  • Second case: A 39-year-old male presented with a 3-month history of headache, left 9th and 10th cranial nerve palsy with left cerebellar ataxia. MR imaging (MRI) brain T2 and fluid attenuated inversion recovery revealed hyperintensities in the right pons, left midbrain, and middle cerebellar peduncle without contrast enhancement [Figure 2]
  • Third case: A 29-year-old female presented with a 4-month history of chronic headache and episodes of generalized tonic–clonic seizure. MRI brain revealed multiple T2 hyperintensity in the subcortical and pericallosal region with pachymeningitis [Figure 3].
Figure 1: (a) Magnetic resonance venogram brain showing thrombosis of posterior one-third of superior sagittal sinus, (b) positive pathergy test showing development of pustules after 48 h

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Figure 2: Magnetic resonance imaging brain T2-weighted axial and sagittal view showing hyperintensities in the right pons, left midbrain, and middle cerebellar peduncle

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Figure 3: MRI brain (a and b): T2 weighted axial and sagittal view showing multiple T2 hyper intensity in the subcortical and pericallosal region, (c) T1 weighted axial image with contrast showing pachymeningitis

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All patients had a history of recurrent oral ulcers and uveitis. The first and third cases also had a history of recurrent genital ulcers. In all patients, routine tests including hemogram, liver function test, renal function test, chest X-ray, and HIV test were normal. All patients had raised erythrocyte sedimentation rate and positive C-reactive protein. Immunological testing including anti-nuclear antibody and anti-double-stranded DNA was negative in all patients. Pathergy test was positive in all three patients [Figure 1]b.

All the three patients fulfilled the criteria for the diagnosis of neuro-BD (NBD) recommended by the International Consensus Recommendation (ICR).[6]


  Discussion Top


The neurological manifestations of Behcet's disease is known as neuro-Behçet's disease (NBD). The prevalence of neurological manifestation amongst BD is varies between 2-49%.[7],[8] The central nervous system (CNS) manifestations of BD can be parenchymal and nonparenchymal. The parenchymal involvement includes hemispheric, brainstem, spinal, and multifocal presentations. The nonparenchymal CNS involvement (also known as neurovascular BD) includes dural sinus thrombosis, arterial occlusion, and arterial aneurysms.[9] The ratio of parenchymal to nonparenchymal involvement is 4.3:1.[9]

The first and second cases presented with cerebral venous sinus thrombosis (CVST). The prevalence of CVST among patients with NBD was from 10% to 20%.[9],[10] Superior sagittal sinus is the most common site for the involvement in CVST. One of our patients (second case) also had brainstem manifestation in the form of 9th, 10th cranial nerve involvement and cerebellar ataxia. According to Akman Demir et al., brainstem manifestations are the most common presentation of NBD and include opthalmoparesis, cranial neuropathy, bulbar palsy with dysarthria, and dysphagia.[11]

Third case presented with recurrent seizures and chronic headache, and MRI brain was suggestive of multiple sclerosis-like picture and pachymeningitis. Generalized seizures are the predominant seizure type seen in NBD-related epileptic disorder.[12] The periventricular lesions found in the MRI study of the third case suggest a demyelinating process, similar to one found in multiple sclerosis, even though recurrent oral or genital ulcers are diagnostic for BD. After reviewing the literature, only a few case reports have demyelinating lesions similar to those of multiple sclerosis. Cerebrospinal fluid (CSF) analysis of this patient showed increased protein without pleocytosis. CSF oligoclonal band or immunoglobulin G index was not done because of financial constraint.

All three patients had a history of chronic headache. A headache is the most common neurological symptom in patients with BD and occurs in about 70% of patients.[13]

According to the ICR, for acute/subacute parenchymal NBD attack, a course of corticosteroids is recommended preferably intravenous methyl prednisolone for 3–10 days followed by a maintenance oral corticosteroid for a few months (up to 6 months). Azathioprine is recommended as a first-line disease-modifying therapy; alternatives include mycophenolate mofetil, methotrexate, and cyclophosphamide.[6] If anticoagulation is to be started for CVST, caution should be taken to rule out a systemic aneurysm.[6]

All the patients were treated with a short course of steroid followed by azathioprine. The first and second cases were treated with an anticoagulant. The first case had active uveitis at the time of presentation, and he was treated with topical glucocorticoid. All patients were responded to treatment, and there was no relapse in 3-month follow-up period.


  Conclusion Top


Clinician should be aware of various neurological manifestation of BD as it is potentially treatable condition. Diagnosis is based on the clinical ground mainly from history, examination, and pathergy test. Treatment strategies include immunosuppressive therapy and supportive management.

.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Behcet H. Uber residivierende, aphtose durch ein virus verursachtes geschwure am mund, am auge und an der genitalien. Derm Wschr 1937;105:1152-7.  Back to cited text no. 1
    
2.
Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M, et al. Close association of HLA-bw51 with Behçet's disease. Arch Ophthalmol 1982;100:1455-8.  Back to cited text no. 2
    
3.
Mehra NK, Taneja V, Kailash S, Raizada N, Vaidya MC. Distribution of HLA antigens in a sample of the North Indian Hindu population. Tissue Antigens 1986;27:64-74.  Back to cited text no. 3
[PUBMED]    
4.
Yazici H, Akokan G, Yalçin B, Müftüoǧlu A. The high prevalence of HLA-B5 in Behçet's disease. Clin Exp Immunol 1977;30:259-61.  Back to cited text no. 4
    
5.
Singal A, Chhabra N, Pandhi D, Rohatgi J. Behçet's disease in India: A dermatological perspective. Indian J Dermatol Venereol Leprol 2013;79:199-204.  Back to cited text no. 5
  [Full text]  
6.
Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS, et al. Diagnosis and management of Neuro-Behçet's disease: International consensus recommendations. J Neurol 2014;261:1662-76.  Back to cited text no. 6
    
7.
Treudler R, Orfanos CE, Zouboulis CC. Twenty-eight cases of juvenile-onset adamantiades-behçet disease in Germany. Dermatology 1999;199:15-9.  Back to cited text no. 7
    
8.
Zouboulis CC, Vaiopoulos G, Marcomichelakis N, Palimeris G, Markidou I, Thouas B, et al. Onset signs, clinical course, prognosis, treatment and outcome of adult patients with Adamantiades-Behçet's disease in Greece. Clin Exp Rheumatol 2003;21:S19-26.  Back to cited text no. 8
    
9.
Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet's disease: Evaluation of 200 patients. The Neuro-Behçet study group. Brain 1999;122(Pt 11):2171-82.  Back to cited text no. 9
    
10.
Wechsler B, Vidailhet M, Piette JC, Bousser MG, Dell Isola B, Blétry O, et al. Cerebral venous thrombosis in Behçet's disease: Clinical study and long-term follow-up of 25 cases. Neurology 1992;42:614-8.  Back to cited text no. 10
    
11.
Joseph FG, Scolding NJ. Neuro-Behçet's disease in Caucasians: A study of 22 patients. Eur J Neurol 2007;14:174-80.  Back to cited text no. 11
    
12.
Aykutlu E, Baykan B, Serdaroglu P, Gökyigit A, Akman-Demir G. Epileptic seizures in Behçet disease. Epilepsia 2002;43:832-5.  Back to cited text no. 12
    
13.
Kidd D, Steuer A, Denman AM, Rudge P. Neurological complications in Behçet's syndrome. Brain 1999;122(Pt 11):2183-94.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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