Neuro-Behçet's Syndrome in a Patient not Fulfilling Criteria for Behçet's Disease: Clinical Features and Value of Brain Imaging

2000 ◽  
Vol 19 (3) ◽  
pp. 231-234 ◽  
Author(s):  
S. M. Weiner ◽  
A. Otte ◽  
M. Schumacher ◽  
F. D. Juengling ◽  
I. Brink ◽  
...  
Keyword(s):  
Brain Imaging ◽  
Clinical Features ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behçet’S Syndrome ◽  
Behcet's Disease ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

scholarly journals Neuro-Behçet’s Syndrome: case report

2021 ◽  
Author(s):  
Izabel Feitosa da Mata Leite ◽  
Adelina Mouta Moreira Neto ◽  
Guilherme de Aguiar Moraes ◽  
Lucas Cardoso Siqueira Albernaz ◽  
Matheus de Campos Medeiros
Keyword(s):  
Case Report ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behçet’S Syndrome ◽  
Behcet's Disease ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Genital Ulcers ◽  
Behcet's Syndrome

Context: Behçet’s Disease (BD) is a multisystem chronic disorder characterized by perivasculitis within several tissues, including the Central Nervous System – Neuro-Behçet’s Disease, which accounts for 3 – 9 % of the BD patients. Neuro- Behçet’s Syndrome may present as brainstem or pyramidal syndromes, myelopathies, meningoencephalitis, intracranial hypertension and movement or psychiatric disorders. The objective of the present work is to report a case of Neuro-Behçet’s Disease, a rare and disabling disorder. Case report: 24-year-old male presenting focal neurological deficits – hemiparesis on the right side and motor aphasia, associated with unstoppable hiccups and visual blurring. His previous pathological history featured several self-limiting episodes of the same neurological presentation, as well as acute exacerbations of oral and genital ulcers. Erythema Nodosum and Folliculitis were his main skin lesions. The ophthalmological evaluation showed bilateral cicatricial chorioretinitis. Laboratorial diagnostic procedures revealed left shift leukocytosis, high ESR, high protein level in the CSF and negative serology for B and C hepatitis, HTLV, HIV and toxoplasmosis. P-ANCA, C-ANCA, ANA and Rheumatoid factor were all found negative. Magnetic Resonance Imaging of the brain showed multiple oval- shaped T2- hyperintensity foci, with adjacent vasogenic edema, in the brainstem and basal ganglia areas – suggesting vasculitis. Treatment involved pulse therapy with Methylprednisolone, followed by Azathioprine and Prednisone. The patient has had full recovery and no other relapses. Conclusions: This case illustrates the importance of investigating Neuro-Behçet’s Disease in patients with neurological symptoms and oral/genital ulcers. The goal is to establish adequate and early treatment to improve the quality of life.

Efficacy and safety of apremilast for Behçet’s syndrome: a real-life single-centre Italian experience

Rheumatology ◽  
2019 ◽  
Vol 59 (1) ◽  
pp. 171-175 ◽  
Author(s):  
Giacomo De Luca ◽  
Adriana Cariddi ◽  
Corrado Campochiaro ◽  
Daniele Vanni ◽  
Nicola Boffini ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Real Life ◽  
Behçet’S Syndrome ◽  
Efficacy And Safety ◽  
Behcet's Disease ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Objectives To evaluate the efficacy and safety of apremilast in treating oral ulcers (OUs), the cardinal and high-disabling feature of Behçet’s disease (BD). Methods Twelve consecutive patients affected by BD with recurrent/relapsing OUs resistant and/or intolerant to conventional therapy were enrolled and prospectively followed. The primary endpoint was the number of OUs at week 12. Secondary endpoints were modification from baseline to week 12 in Behçet’s Syndrome Activity Score (BSAS), Behçet’s Disease Current Activity Form (BDCAF) score, Behçet’s Disease Quality of Life (BDQOL) scale and pain of OUs, as measured by a visual analogue scale (VAS). All adverse events (AEs) were recorded during follow-up. Non-parametric tests (Wilcoxon rank test) were used and a P-value <0.05 was considered statistically significant. Results After 12 weeks of apremilast, there was a significant reduction in the number of OUs [0.58 (s.d. 0.67) vs 3.33 (s.d. 1.45) at baseline, P = 0.02] that was paralleled by improvement in disease activity: BSAS was 16.8 (s.d. 9.1) [from 45.9 (s.d. 19.6) at baseline] (P = 0.02), BDCAF score was 0.72 (s.d. 0.65) [vs 2.45 (s.d. 1.0) at baseline] (P = 0.04) and the VAS score for pain decreased to 23.3 (s.d. 13.7) [vs 67.9 (s.d. 17.2) at baseline] (P = 0.02). Consistently, an improvement of BDQOL was assessed (P = 0.02). Clinical improvement led to complete steroid discontinuation in six patients and a tapering of the prednisone dose in two patients (P = 0.016). Colchicine was discontinued in six of nine patients (P = 0.031). AEs related to apremilast occurred in four patients (mainly due to gastrointestinal AEs), leading to drug discontinuation in all of them. Conclusion Our preliminary real-world data support the use of apremilast as an effective therapeutic strategy against BD-related recurrent OUs resistant or intolerant to first-line therapy.

Rare health conditions 23: Behçet's disease, Mayer Rokitansky Küster Hauser (MRKH) syndrome and Wilson's disease

2019 ◽  
Vol 13 (5) ◽  
pp. 214-218
Author(s):  
Chris Barber
Keyword(s):  
Behcet’S Disease ◽  
Wilson’S Disease ◽  
Behçet's Disease ◽  
Wilson's Disease ◽  
Health Conditions ◽  
Behçet’S Syndrome ◽  
Mrkh Syndrome ◽  
Behcet's Disease ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

The purpose of this series is to briefly highlight a range of rare health conditions. Rare health conditions are those that affect no more and usually fewer than 1 person in every 2000 and many healthcare assistants and nurses will encounter some of these conditions, given the high number of them. This 23rd article will explore three of these conditions: Behçet's syndrome; Mayer Rokitansky Küster Hauser syndrome (MRKH); and Wilson's disease.

Coexistence of Behçet's Disease and Autoimmune Disease: Clinical Features of 11 Cases

2005 ◽  
Vol 32 (7) ◽  
pp. 614-616 ◽  
Author(s):  
Sung Bin Cho ◽  
Eun Young Chun ◽  
Dongsik Bang ◽  
Kwang Hoon Lee ◽  
Eun-So Lee ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Clinical Features ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease

Clinical features of Behcet’s disease in Mongolia: a multicenter study

2020 ◽  
Vol 39 (9) ◽  
pp. 2697-2706
Author(s):  
Javzandulam Balt ◽  
Baasankhuu Jamyanjav ◽  
Sarantuya Jav ◽  
Zulgerel Dandii ◽  
Chimedlkhamsuren Ganbold ◽  
...  
Keyword(s):  
Clinical Features ◽  
Behçet’S Disease ◽  
Multicenter Study ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease

Contribution of Hla-B51 in the susceptibility and specific clinical features of Behcet's disease in Tunisian patients

2012 ◽  
Vol 23 (4) ◽  
pp. 347-349 ◽  
Author(s):  
Amira Hamzaoui ◽  
Mohamed Habib Houman ◽  
Meriem Massouadia ◽  
Thouraya Ben Salem ◽  
Monia Smiti Khanfir ◽  
...  
Keyword(s):  
Clinical Features ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease ◽  
Tunisian Patients

Clinical features of early-stage possible Behçet’s disease patients with a variant-type major organ involvement in Japan

2018 ◽  
Vol 29 (4) ◽  
pp. 640-646 ◽  
Author(s):  
Takeharu Suzuki ◽  
Nobuyuki Horita ◽  
Masaki Takeuchi ◽  
Takehito Ishido ◽  
Yuki Mizuki ◽  
...  
Keyword(s):  
Clinical Features ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Early Stage ◽  
Organ Involvement ◽  
Behcet's Disease ◽  
Variant Type ◽  
Major Organ

Single center study on ethnic and clinical features of Behcet’s disease in Moscow, Russia

2013 ◽  
Vol 34 (2) ◽  
pp. 321-327 ◽  
Author(s):  
Anton Lennikov ◽  
Zemfira Alekberova ◽  
Regina Goloeva ◽  
Nobuyoshi Kitaichi ◽  
Lev Denisov ◽  
...  
Keyword(s):  
Clinical Features ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Single Center ◽  
Behcet's Disease ◽  
Single Center Study ◽  
Center Study
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