Behçet's disease classification: Difference between revisions

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Latest revision as of 20:35, 29 July 2020

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

Neurologic disease of Behcet disease is classified into parenchymal or non-parenchymal.

Parenchymal disease

Parenchymal disease is due to lesions in the corticospinal tract, brainstem, periventricular white matter, spinal cord, and basal ganglia.
- Brainstem disease (which may extend to the midbrain, basal ganglia, and diencephalon) including focal lesions or atrophy with signs and symptoms including ophthalmoparesis, cranial neuropathy, and cerebellar or pyramidal dysfunction are more characteristic of Behçet syndrome than multiple sclerosis.
- Cerebral lesions are often multiple though may be single, are often subcortical, and are not particularly peri-ventricular, as in multiple sclerosis.
- Spinal cord lesions (myelitis) may occur in isolation, but are more common in patients with other central nervous system lesions.
- The clinical presentation of parenchymal disease is often subacute and manifestations may include headache, behavior changes, and deficits reflecting areas of parenchymal involvement.

Focal parenchymal lesions and complications of vascular thrombosis are the most common abnormalities.
Progressive personality change, psychiatric disorders, and dementia may develop.
Unlike many other systemic vasculitic disorders, peripheral neuropathy is uncommon in Behçet syndrome.^[1]^[2]^[3]
Parenchymal disease may be divided into acute and chronic progressive neuro-Behçet syndrome.
- A meta-analysis reviewed 184 acute and 114 chronic progressive cases, and fever and higher cerebrospinal fluid cell counts were found more commonly in acute disease, and sphincter disturbances, ataxia, confusion, brain stem atrophy on magnetic resonance imaging (MRI), and cerebral changes on MRI were more common in chronic progressive disease.^[4]

Central nervous system lesions are detectable with MRI.^[5]^[6]
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.
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.^[7]
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.

Non-parenchymal disease

Non-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.^[7]

Central nervous system manifestations may result from arterial or venous thrombosis, including dural sinus thrombosis.^[8]
Cerebral venous thrombosis may present with headache, papilledema, sixth nerve palsy, and an elevated CSF pressure.^[8]^[9]^[10]
An association has been observed between dural sinus thrombosis and peripheral deep venous thrombosis.^[5]
Thrombosis of the cerebral arteries may also be observed.^[5]^[8]
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.^[11]

In one large series, the clinical features and outcomes of 200 patients with Behçet syndrome and neurologic involvement were reported.^[5]
On an 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

↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ ^5.0 ^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.
↑ 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.
↑ ^7.0 ^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.
↑ ^8.0 ^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.
↑ O'Duffy JD (1994). "Behçet's disease". Curr Opin Rheumatol. 6 (1): 39–43. PMID 8031678.
↑ 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.
↑ 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.

Template:WH Template:WS

[pmid17622917-1] 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.

[pmid17149536-2] 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.

[pmid17086145-3] 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.

[pmid28860590-4] 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.

[pmid10545401-5] 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.

[pmid11428799-6] 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.

[pmid24366648-7] 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.

[pmid9527155-8] 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.

[pmid8031678-9] O'Duffy JD (1994). "Behçet's disease". Curr Opin Rheumatol. 6 (1): 39–43. PMID 8031678.

[pmid19333987-10] 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.

[pmid22076549-11] 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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 ==Overview==
+Neurologic disease of Behcet disease is classified into [[Parenchyma|parenchymal]] or non-[[Parenchyma|parenchymal]]. [[Parenchyma|Parenchymal]] disease is due to lesions in the [[corticospinal tract]], [[Brain stem|brainstem]], periventricular [[white matter]], [[spinal cord]], and [[basal ganglia]]. Focal [[Parenchyma|parenchymal]] [[Lesion|lesions]] and complications of [[vascular]] [[thrombosis]] are the most common abnormalities. Progressive [[Personality change due to another medical condition|personality change]], [[Mental disorder|psychiatric disorders]], and [[dementia]] may develop. [[Parenchyma|Parenchymal]] [[disease]] may be divided into [[Acute (medicine)|acute]] and [[Chronic (medical)|chronic]] progressive neuro-Behçet syndrome. [[Central nervous system]] lesions are detectable with [[Magnetic resonance imaging|MRI]]. In the [[Chronic (medical)|chronic]] phase, lesions may be smaller or resolve, [[atrophy]] may be present, nonspecific [[white matter]] [[Lesion|lesions]] may be present, and lesions usually do not enhance. [[Cerebrospinal fluid|Cerebrospinal fluid (CSF)]] may show increased [[protein]] and increased [[Cell (biology)|cells]], and [[Neutrophil|neutrophils]] may predominate. Non-[[Parenchyma|parenchymal]] [[disease]] of Behcet disease include [[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 [[arterial thrombosis]], [[Dissection (medical)|dissection]], or [[aneurysm]]. On average, a period of approximately five to six years elapsed between the onset of the earliest non-neurologic [[Symptom|symptoms]] of Behçet syndrome and the appearance of [[Neurology|neurologic]] [[Symptom|symptoms]] or findings.
 ==Classification==
 ===Neurologic disease===
-*Neurologic disease of Behcet disease is classified into parenchymal or non-parenchymal:
+*Neurologic disease of Behcet disease is classified into [[Parenchyma|parenchymal]] or non-[[Parenchyma|parenchymal]].
-===Parenchymal disease===
+====Parenchymal disease====
-*Parenchymal disease may be due to lesions in the corticospinal tract, brainstem, periventricular white matter, spinal cord, and basal ganglia.
+*[[Parenchyma|Parenchymal]] [[disease]] is due to [[Lesion|lesions]] in the [[corticospinal tract]], [[Brain stem|brainstem]], periventricular [[white matter]], [[spinal cord]], and [[basal ganglia]].
-**Brainstem disease (which may extend to the midbrain, basal ganglia, and diencephalon) including focal lesions or atrophy with signs and symptoms including ophthalmoparesis, cranial neuropathy, and cerebellar or pyramidal dysfunction are more characteristic of Behçet syndrome than multiple sclerosis.
+**[[Brain stem|Brainstem]] [[disease]] (which may extend to the [[Mesencephalon|midbrain]], [[basal ganglia]], and diencephalon) including focal [[Lesion|lesions]] or [[atrophy]] with [[Medical sign|signs]] and [[Symptom|symptoms]] including [[ophthalmoparesis]], [[cranial]] [[neuropathy]], and [[Cerebellum|cerebellar]] or pyramidal dysfunction are more characteristic of Behçet syndrome than [[multiple sclerosis]].
-**Cerebral lesions are often multiple though may be single, are often subcortical, and are not particularly peri-ventricular, as in multiple sclerosis.
+**[[Brain|Cerebral]] [[Lesion|lesions]] are often multiple though may be single, are often [[Cerebral cortex|subcortical]], and are not particularly peri-ventricular, as in [[multiple sclerosis]].
-**Spinal cord lesions (myelitis) may occur in isolation, but are more common in patients with other central nervous system lesions.
+**[[Spinal cord]] [[Lesion|lesions]] ([[myelitis]]) may occur in isolation, but are more common in patients with other [[central nervous system]] [[Lesion|lesions]].
-**The clinical presentation of parenchymal disease is often subacute and manifestations may include headache, behavior changes, and deficits reflecting areas of parenchymal involvement
+**The clinical presentation of [[Parenchyma|parenchymal]] disease is often [[subacute]] and manifestations may include [[headache]], [[behavior]] changes, and deficits reflecting areas of [[Parenchyma|parenchymal]] involvement.
-*Parenchymal disease may be divided into acute and chronic progressive neuro-Behçet syndrome.
+*Focal [[Parenchyma|parenchymal]] [[Lesion|lesions]] and complications of [[vascular]] [[thrombosis]] are the most common abnormalities.
-**Subdivided into brainstem disease, multifocal (diffuse) disease (including brainstem, cerebral, or spinal cord disease), myelopathy, cerebral disease (including encephalopathy, hemiparesis, hemisensory loss, seizures, dysphagia, and mental changes such as psychosis and cognitive dysfunction), and optic neuropathy.
+*Progressive [[personality]] change, [[psychiatric]] disorders, and [[dementia]] may develop.
-**A meta-analysis reviewed 184 acute and 114 chronic progressive cases, and fever and higher cerebrospinal fluid cell counts were found more commonly in acute disease, and sphincter disturbances, ataxia, confusion, brainstem atrophy on magnetic resonance imaging (MRI), and cerebral changes on MRI were more common in chronic progressive disease [50].
+*Unlike many other systemic [[Vasculitis|vasculitic]] disorders, [[peripheral neuropathy]] is uncommon in Behçet syndrome.<ref name="pmid17622917">{{cite journal| author=Atasoy HT, Tunc TO, Unal AE, Emre U, Koca R, Esturk E et al.| title=Peripheral nervous system involvement in patients with Behçet disease. | journal=Neurologist | year= 2007 | volume= 13 | issue= 4 | pages= 225-30 | pmid=17622917 | doi=10.1097/NRL.0b013e31805778d1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17622917  }} </ref><ref name="pmid17149536">{{cite journal| author=Akbulut L, Gur G, Bodur H, Alli N, Borman P| title=Peripheral neuropathy in Behçet disease: an electroneurophysiological study. | journal=Clin Rheumatol | year= 2007 | volume= 26 | issue= 8 | pages= 1240-4 | pmid=17149536 | doi=10.1007/s10067-006-0466-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17149536  }} </ref><ref name="pmid17086145">{{cite journal| author=Benamour S, Naji T, Alaoui FZ, El-Kabli H, El-Aidouni S| title=[Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature]. | journal=Rev Neurol (Paris) | year= 2006 | volume= 162 | issue= 11 | pages= 1084-90 | pmid=17086145 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17086145  }} </ref>
+*[[Parenchyma|Parenchymal]] [[disease]] may be divided into [[Acute (medicine)|acute]] and [[Chronic (medical)|chronic]] progressive neuro-Behçet syndrome.
+**A meta-analysis reviewed 184 acute and 114 chronic progressive cases, and [[fever]] and higher [[cerebrospinal fluid]] cell counts were found more commonly in acute disease, and sphincter disturbances, ataxia, confusion, [[brain stem]] [[atrophy]] on [[Magnetic resonance imaging|magnetic resonance imaging (MRI)]], and [[Brain|cerebral]] changes on [[Magnetic resonance imaging|MRI]] were more common in [[Chronic (medical)|chronic]] progressive disease.<ref name="pmid28860590">{{cite journal| author=Ishido M, Horita N, Takeuchi M, Shibuya E, Yamane T, Kawagoe T et al.| title=Distinct clinical features between acute and chronic progressive parenchymal neuro-Behçet disease: meta-analysis. | journal=Sci Rep | year= 2017 | volume= 7 | issue= 1 | pages= 10196 | pmid=28860590 | doi=10.1038/s41598-017-09938-z | pmc=5579041 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28860590  }} </ref>
-*Focal parenchymal lesions and complications of vascular thrombosis are the most common abnormalities.
+*[[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>
-*Progressive personality change, psychiatric disorders, and dementia may develop.
+*[[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]].
-*Unlike many other systemic vasculitic disorders, peripheral neuropathy is not a common feature of Behçet syndrome, though it may develop in a subset of patients [51-53].
+*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|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 [[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]].
-*These central nervous system lesions are detectable with MRI [46,54].
+====Non-parenchymal disease====
-*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.
+Non-[[Parenchyma|parenchymal]] disease of Behcet disease include the following:
-*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.
+*[[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>
-*Cerebrospinal fluid (CSF) may show increased protein and increased cells, and neutrophils may predominate [55].
-*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.
-===Non-parenchymal disease===
+*[[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, intracranial hypertension syndrome (pseudotumor cerebri), acute meningeal syndrome, and uncommonly stroke due to arterial thrombosis, dissection, or aneurysm [55].
+*[[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 [[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>
+*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>
-*Central nervous system manifestations may result from arterial or venous thrombosis, including dural sinus thrombosis [56].
+*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>
-*Cerebral venous thrombosis may present with headache, papilledema, sixth nerve palsy, and an elevated CSF pressure [12,23,46,56-59].
+*On an 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.
-*An association has been observed between dural sinus thrombosis and peripheral deep venous thrombosis [46].
+*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.
-*Thrombosis of the cerebral arteries may also be observed [46,56].
-*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 [22].
-*In one large series, the clinical features and outcomes of 200 patients with Behçet syndrome and neurologic involvement were reported [46].
-*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==
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-[[Category:Autoimmune diseases]]
+[[Category:Medicine]]
+[[Category:Up-To-date]]
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 [[Category:Rheumatology]]