Behçet's disease classification: Difference between revisions

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*[[Central nervous system]] [[Lesion|lesions]] are detectable with [[Magnetic resonance imaging|MRI]].<ref name="pmid10545401">{{cite journal| author=Akman-Demir G, Serdaroglu P, Tasçi B| title=Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. | journal=Brain | year= 1999 | volume= 122 ( Pt 11) | issue=  | pages= 2171-82 | pmid=10545401 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10545401  }} </ref><ref name="pmid11428799">{{cite journal| author=Lee SH, Yoon PH, Park SJ, Kim DI| title=MRI findings in neuro-behçet's disease. | journal=Clin Radiol | year= 2001 | volume= 56 | issue= 6 | pages= 485-94 | pmid=11428799 | doi=10.1053/crad.2000.0675 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11428799  }} </ref>
*[[Central nervous system]] [[Lesion|lesions]] are detectable with [[Magnetic resonance imaging|MRI]].<ref name="pmid10545401">{{cite journal| author=Akman-Demir G, Serdaroglu P, Tasçi B| title=Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. | journal=Brain | year= 1999 | volume= 122 ( Pt 11) | issue=  | pages= 2171-82 | pmid=10545401 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10545401  }} </ref><ref name="pmid11428799">{{cite journal| author=Lee SH, Yoon PH, Park SJ, Kim DI| title=MRI findings in neuro-behçet's disease. | journal=Clin Radiol | year= 2001 | volume= 56 | issue= 6 | pages= 485-94 | pmid=11428799 | doi=10.1053/crad.2000.0675 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11428799  }} </ref>
*[[Acute (medicine)|Acute]] and [[subacute]] lesions are hypointense or isointense on T1-weighted images; hyperintense on T2-weighted, FLAIR, and diffusion-weighted images; and commonly enhance with contrast.  
*[[Acute (medicine)|Acute]] and [[subacute]] lesions are hypointense or isointense on T1-weighted images; hyperintense on T2-weighted, FLAIR, and [[Diffusion MRI|diffusion-weighted]] images; and commonly enhance with [[Contrast medium|contrast]].  
*In the chronic phase, lesions may be smaller or resolve, atrophy may be present, nonspecific white matter lesions may be present, and lesions usually do not enhance.  
*In the [[Chronic (medical)|chronic]] phase, lesions may be smaller or resolve, [[atrophy]] may be present, nonspecific [[white matter]] lesions may be present, and [[Lesion|lesions]] usually do not enhance.  
*Cerebrospinal fluid (CSF) may show increased protein and increased cells, and neutrophils may predominate.<ref name="pmid24366648">{{cite journal| author=Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS et al.| title=Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations. | journal=J Neurol | year= 2014 | volume= 261 | issue= 9 | pages= 1662-76 | pmid=24366648 | doi=10.1007/s00415-013-7209-3 | pmc=4155170 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24366648  }} </ref>
*[[Cerebrospinal fluid|Cerebrospinal fluid (CSF)]] may show increased [[protein]] and increased [[Cell (biology)|cells]], and [[Neutrophil|neutrophils]] may predominate.<ref name="pmid24366648">{{cite journal| author=Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS et al.| title=Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations. | journal=J Neurol | year= 2014 | volume= 261 | issue= 9 | pages= 1662-76 | pmid=24366648 | doi=10.1007/s00415-013-7209-3 | pmc=4155170 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24366648  }} </ref>
*Pathology reveals local perivenular lymphocytic cuffing, inflammatory cell infiltration, gliosis, necrosis, and neuronal loss. Although frank vasculitis is not always observed in parenchymal lesions, it is sometimes noted in larger cerebral vessels, including arteries or veins. Arteritis may lead to ischemic strokes, dissection, aneurysmal dilatation, and subarachnoid hemorrhage.
*[[Pathology]] reveals local perivenular [[Lymphocyte|lymphocytic]] cuffing, [[Inflammation|inflammatory]] [[Cell (biology)|cell]] infiltration, [[gliosis]], [[necrosis]], and [[Neuron|neuronal]] loss. Although frank [[vasculitis]] is not always observed in [[Parenchyma|parenchymal]] lesions, it is sometimes noted in larger [[cerebral]] [[Blood vessel|vessels]], including arteries or veins. [[Arteritis]] may lead to [[Ischemia|ischemic]] [[Stroke|strokes]], [[Dissection (medical)|dissection]], [[Aneurysmal disease|aneurysmal]] dilatation, and [[subarachnoid hemorrhage]].


====Non-parenchymal disease====
====Non-parenchymal disease====
Non-parenchymal disease of Behcet disease include the following:
Non-[[Parenchyma|parenchymal]] disease of Behcet disease include the following:
*Cerebral venous thrombosis, intracranial hypertension syndrome (pseudotumor cerebri), acute meningeal syndrome, and uncommonly stroke due to arterial thrombosis, dissection, or aneurysm.<ref name="pmid24366648">{{cite journal| author=Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS et al.| title=Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations. | journal=J Neurol | year= 2014 | volume= 261 | issue= 9 | pages= 1662-76 | pmid=24366648 | doi=10.1007/s00415-013-7209-3 | pmc=4155170 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24366648  }} </ref>
*[[Cerebral venous sinus thrombosis|Cerebral venous thrombosis]], [[intracranial hypertension]] syndrome ([[Idiopathic intracranial hypertension|pseudotumor cerebri]]), [[Acute (medicine)|acute]] [[Meninges|meningeal]] [[syndrome]], and uncommonly [[stroke]] due to [[Artery|arterial]] [[thrombosis]], [[Dissection (medical)|dissection]], or [[aneurysm]].<ref name="pmid24366648">{{cite journal| author=Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS et al.| title=Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations. | journal=J Neurol | year= 2014 | volume= 261 | issue= 9 | pages= 1662-76 | pmid=24366648 | doi=10.1007/s00415-013-7209-3 | pmc=4155170 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24366648  }} </ref>


*Central nervous system manifestations may result from arterial or venous thrombosis, including dural sinus thrombosis.<ref name="pmid9527155">{{cite journal| author=Farah S, Al-Shubaili A, Montaser A, Hussein JM, Malaviya AN, Mukhtar M et al.| title=Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations. | journal=J Neurol Neurosurg Psychiatry | year= 1998 | volume= 64 | issue= 3 | pages= 382-4 | pmid=9527155 | doi= | pmc=2169980 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9527155  }} </ref>  
*[[Central nervous system]] manifestations may result from [[Artery|arterial]] or [[Vein|venous]] [[thrombosis]], including [[Cerebral venous sinus thrombosis|dural sinus thrombosis]].<ref name="pmid9527155">{{cite journal| author=Farah S, Al-Shubaili A, Montaser A, Hussein JM, Malaviya AN, Mukhtar M et al.| title=Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations. | journal=J Neurol Neurosurg Psychiatry | year= 1998 | volume= 64 | issue= 3 | pages= 382-4 | pmid=9527155 | doi= | pmc=2169980 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9527155  }} </ref>  
*Cerebral venous thrombosis may present with headache, papilledema, sixth nerve palsy, and an elevated CSF pressure.<ref name="pmid9527155" /><ref name="pmid8031678">{{cite journal| author=O'Duffy JD| title=Behçet's disease. | journal=Curr Opin Rheumatol | year= 1994 | volume= 6 | issue= 1 | pages= 39-43 | pmid=8031678 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8031678  }} </ref><ref name="pmid19333987">{{cite journal| author=Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A et al.| title=Cerebral venous thrombosis in Behçet's disease. | journal=Arthritis Rheum | year= 2009 | volume= 61 | issue= 4 | pages= 518-26 | pmid=19333987 | doi=10.1002/art.24393 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19333987  }} </ref>  
*[[Cerebral venous sinus thrombosis|Cerebral venous thrombosis]] may present with [[headache]], [[papilledema]], [[sixth nerve palsy]], and an elevated [[CSF]] pressure.<ref name="pmid9527155" /><ref name="pmid8031678">{{cite journal| author=O'Duffy JD| title=Behçet's disease. | journal=Curr Opin Rheumatol | year= 1994 | volume= 6 | issue= 1 | pages= 39-43 | pmid=8031678 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8031678  }} </ref><ref name="pmid19333987">{{cite journal| author=Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A et al.| title=Cerebral venous thrombosis in Behçet's disease. | journal=Arthritis Rheum | year= 2009 | volume= 61 | issue= 4 | pages= 518-26 | pmid=19333987 | doi=10.1002/art.24393 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19333987  }} </ref>  
*An association has been observed between dural sinus thrombosis and peripheral deep venous thrombosis.<ref name="pmid10545401">{{cite journal| author=Akman-Demir G, Serdaroglu P, Tasçi B| title=Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. | journal=Brain | year= 1999 | volume= 122 ( Pt 11) | issue=  | pages= 2171-82 | pmid=10545401 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10545401  }} </ref>  
*An association has been observed between [[Cerebral venous sinus thrombosis|dural sinus thrombosis]] and peripheral [[Deep vein thrombosis|deep venous thrombosis]].<ref name="pmid10545401">{{cite journal| author=Akman-Demir G, Serdaroglu P, Tasçi B| title=Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. | journal=Brain | year= 1999 | volume= 122 ( Pt 11) | issue=  | pages= 2171-82 | pmid=10545401 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10545401  }} </ref>  
*Thrombosis of the cerebral arteries may also be observed.<ref name="pmid10545401" /><ref name="pmid9527155">{{cite journal| author=Farah S, Al-Shubaili A, Montaser A, Hussein JM, Malaviya AN, Mukhtar M et al.| title=Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations. | journal=J Neurol Neurosurg Psychiatry | year= 1998 | volume= 64 | issue= 3 | pages= 382-4 | pmid=9527155 | doi= | pmc=2169980 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9527155  }} </ref>  
*[[Thrombosis]] of the [[cerebral arteries]] may also be observed.<ref name="pmid10545401" /><ref name="pmid9527155">{{cite journal| author=Farah S, Al-Shubaili A, Montaser A, Hussein JM, Malaviya AN, Mukhtar M et al.| title=Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations. | journal=J Neurol Neurosurg Psychiatry | year= 1998 | volume= 64 | issue= 3 | pages= 382-4 | pmid=9527155 | doi= | pmc=2169980 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9527155  }} </ref>  
*One analysis of neurologic Behçet syndrome from Turkey, involving 26 children and 702 adults, found that dural venous sinus thrombosis was much more common in children than parenchymal neurologic involvement, although parenchymal disease was more frequent in adults.<ref name="pmid22076549">{{cite journal| author=Uluduz D, Kürtüncü M, Yapıcı Z, Seyahi E, Kasapçopur Ö, Özdoğan H et al.| title=Clinical characteristics of pediatric-onset neuro-Behçet disease. | journal=Neurology | year= 2011 | volume= 77 | issue= 21 | pages= 1900-5 | pmid=22076549 | doi=10.1212/WNL.0b013e318238edeb | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22076549  }} </ref>
*One analysis of neurologic Behçet syndrome from Turkey, involving 26 children and 702 adults, found that [[Cerebral venous sinus thrombosis|dural]] [[Cerebral venous sinus thrombosis|venous sinus thrombosis]] was much more common in children than [[Parenchyma|parenchymal]] [[Neurology|neurologic]] involvement, although [[Parenchyma|parenchymal]] [[disease]] was more frequent in adults.<ref name="pmid22076549">{{cite journal| author=Uluduz D, Kürtüncü M, Yapıcı Z, Seyahi E, Kasapçopur Ö, Özdoğan H et al.| title=Clinical characteristics of pediatric-onset neuro-Behçet disease. | journal=Neurology | year= 2011 | volume= 77 | issue= 21 | pages= 1900-5 | pmid=22076549 | doi=10.1212/WNL.0b013e318238edeb | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22076549  }} </ref>


*In one large series, the clinical features and outcomes of 200 patients with Behçet syndrome and neurologic involvement were reported.<ref name="pmid10545401">{{cite journal| author=Akman-Demir G, Serdaroglu P, Tasçi B| title=Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. | journal=Brain | year= 1999 | volume= 122 ( Pt 11) | issue=  | pages= 2171-82 | pmid=10545401 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10545401  }} </ref>  
*In one large series, the clinical features and outcomes of 200 patients with Behçet syndrome and [[Neurology|neurologic]] involvement were reported.<ref name="pmid10545401">{{cite journal| author=Akman-Demir G, Serdaroglu P, Tasçi B| title=Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. | journal=Brain | year= 1999 | volume= 122 ( Pt 11) | issue=  | pages= 2171-82 | pmid=10545401 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10545401  }} </ref>  
*On average, a period of approximately five to six years elapsed between the onset of the earliest non-neurologic symptoms of Behçet syndrome and the appearance of neurologic symptoms or findings.
*On average, a period of approximately five to six years elapsed between the onset of the earliest non-[[Neurology|neurologic]] symptoms of Behçet syndrome and the appearance of [[Neurology|neurologic]] symptoms or findings.
*Nevertheless, neurologic findings may also appear concurrently (7.5 percent) or precede non-neurologic features (3 percent). Twenty percent of those with neurologic findings were asymptomatic.
*Nevertheless, [[Neurology|neurologic]] findings may also appear concurrently (7.5 percent) or precede non-[[Neurology|neurologic]] features (3 percent). Twenty percent of those with [[Neurology|neurologic]] findings were asymptomatic.


==References==
==References==

Revision as of 20:38, 2 May 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

Neurologic disease of Behcet disease is classified into parenchymal or non-parenchymal. Parenchymal disease is due to lesions in the corticospinal tract, brainstem, periventricular white matter, spinal cord, and basal ganglia. Focal parenchymal lesions and complications of vascular thrombosis are the most common abnormalities. Progressive personality change, psychiatric disorders, and dementia may develop. Parenchymal disease may be divided into acute and chronic progressive neuro-Behçet syndrome. Central nervous system lesions are detectable with MRI. In the chronic phase, lesions may be smaller or resolve, atrophy may be present, nonspecific white matter lesions may be present, and lesions usually do not enhance. Cerebrospinal fluid (CSF) may show increased protein and increased cells, and neutrophils may predominate. Non-parenchymal disease of Behcet disease include cerebral venous thrombosis, intracranial hypertension syndrome (pseudotumor cerebri), acute meningeal syndrome, and uncommonly stroke due to arterial thrombosis, dissection, or aneurysm. On average, a period of approximately five to six years elapsed between the onset of the earliest non-neurologic symptoms of Behçet syndrome and the appearance of neurologic symptoms or findings.

Classification

Neurologic disease

Parenchymal disease

Non-parenchymal disease

Non-parenchymal disease of Behcet disease include the following:

  • In one large series, the clinical features and outcomes of 200 patients with Behçet syndrome and neurologic involvement were reported.[5]
  • On average, a period of approximately five to six years elapsed between the onset of the earliest non-neurologic symptoms of Behçet syndrome and the appearance of neurologic symptoms or findings.
  • Nevertheless, neurologic findings may also appear concurrently (7.5 percent) or precede non-neurologic features (3 percent). Twenty percent of those with neurologic findings were asymptomatic.

References

  1. Atasoy HT, Tunc TO, Unal AE, Emre U, Koca R, Esturk E; et al. (2007). "Peripheral nervous system involvement in patients with Behçet disease". Neurologist. 13 (4): 225–30. doi:10.1097/NRL.0b013e31805778d1. PMID 17622917.
  2. Akbulut L, Gur G, Bodur H, Alli N, Borman P (2007). "Peripheral neuropathy in Behçet disease: an electroneurophysiological study". Clin Rheumatol. 26 (8): 1240–4. doi:10.1007/s10067-006-0466-0. PMID 17149536.
  3. Benamour S, Naji T, Alaoui FZ, El-Kabli H, El-Aidouni S (2006). "[Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature]". Rev Neurol (Paris). 162 (11): 1084–90. PMID 17086145.
  4. Ishido M, Horita N, Takeuchi M, Shibuya E, Yamane T, Kawagoe T; et al. (2017). "Distinct clinical features between acute and chronic progressive parenchymal neuro-Behçet disease: meta-analysis". Sci Rep. 7 (1): 10196. doi:10.1038/s41598-017-09938-z. PMC 5579041. PMID 28860590.
  5. 5.0 5.1 5.2 5.3 Akman-Demir G, Serdaroglu P, Tasçi B (1999). "Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group". Brain. 122 ( Pt 11): 2171–82. PMID 10545401.
  6. Lee SH, Yoon PH, Park SJ, Kim DI (2001). "MRI findings in neuro-behçet's disease". Clin Radiol. 56 (6): 485–94. doi:10.1053/crad.2000.0675. PMID 11428799.
  7. 7.0 7.1 Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS; et al. (2014). "Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations". J Neurol. 261 (9): 1662–76. doi:10.1007/s00415-013-7209-3. PMC 4155170. PMID 24366648.
  8. 8.0 8.1 8.2 Farah S, Al-Shubaili A, Montaser A, Hussein JM, Malaviya AN, Mukhtar M; et al. (1998). "Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations". J Neurol Neurosurg Psychiatry. 64 (3): 382–4. PMC 2169980. PMID 9527155.
  9. O'Duffy JD (1994). "Behçet's disease". Curr Opin Rheumatol. 6 (1): 39–43. PMID 8031678.
  10. Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A; et al. (2009). "Cerebral venous thrombosis in Behçet's disease". Arthritis Rheum. 61 (4): 518–26. doi:10.1002/art.24393. PMID 19333987.
  11. Uluduz D, Kürtüncü M, Yapıcı Z, Seyahi E, Kasapçopur Ö, Özdoğan H; et al. (2011). "Clinical characteristics of pediatric-onset neuro-Behçet disease". Neurology. 77 (21): 1900–5. doi:10.1212/WNL.0b013e318238edeb. PMID 22076549.

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