Behçet's disease classification

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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]


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.


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 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.


  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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