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

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.

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

Rheumatology ◽

10.1093/rheumatology/kez267 ◽

2019 ◽

Vol 59 (1) ◽

pp. 171-175 ◽

Cited By ~ 7

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.

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Behçet's Disease versus Behçet's Syndrome with Some Criteria for Systemic Lupus Erythematosus

Pediatric Asthma Allergy & Immunology ◽

10.1089/pai.1998.12.53 ◽

1998 ◽

Vol 12 (1) ◽

pp. 53-59 ◽

Cited By ~ 5

Author(s):

ABEERA MANSUR ◽

MARIANNE FRIERI

Keyword(s):

Systemic Lupus Erythematosus ◽

Behçet’S Disease ◽

Lupus Erythematosus ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Behçet’S Syndrome ◽

Systemic Lupus ◽

Behcet's Disease ◽

Behcet’S Syndrome ◽

Behcet's Syndrome

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

British Journal of Healthcare Assistants ◽

10.12968/bjha.2019.13.5.214 ◽

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.

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