Pseudotumoural presentation of neuro-Behcet's disease: case series and review of literature

Rheumatology
Nicolas NoelDavid Saadoun

Abstract

To describe the pseudotumoural presentation of neuro-Behçet's disease (NBD). We report here the main characteristics, treatment and outcome of 23 patients (5 personal cases and 18 patients from the literature) with a pseudotumoural presentation of NBD. Pseudotumoural NBD patients were compared with 69 consecutive patients, with a classical form of NBD. The median age was 39 (range 27-48 years) years, with a male predominance (65.2%). Clinical features of the pseudotumoural NBD included hemi- or tetra-pyramidal symptoms (n = 20), headache (n = 17), cerebellar syndrome (n = 3), sphincter impotence (n = 3) and pseudobulbar signs (n = 2). CNS imaging showed pseudotumoural lesions mainly in the capsulo-thalamic area (69.6 vs 11.6% for classical NBD; P < 0.01). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoural or infectious lesions. Patients with pseudotumoural NBD had more severe initial disability status (Rankin's score ≥3 in 65.2 vs 24.7%; P < 0.01) and had a 3 years' longer duration between neurological signs and BD diagnosis (P = 0.01) compared with patients with classical NBD. Treatment consisted of CSs (n = 21, 95.5%) and immunosuppressive agents (n = 10, 35.7%)...Continue Reading

References

Feb 1, 1990·The New England Journal of Medicine·H YaziciG E Lovatt
Jan 1, 1987·Neuroradiology·I LitvanJ Ruscalleda
Jan 1, 1993·Neuroradiology·M NeudorferI Reider
May 1, 1993·Surgical Neurology·C GenyJ D Degos
Apr 1, 1997·Arthritis and Rheumatism·V HamuryudanH Yazici
Sep 19, 1998·Journal of the Neurological Sciences·S HirohataT Hashimoto
Oct 17, 1998·La Revue de médecine interne·S VignesB Wechsler
Oct 10, 2002·Neurologia Medico-chirurgica·Hirochika ImotoMichiyasu Suzuki
Oct 16, 2002·Journal of Korean Medical Science·Jeong-Ho ParkJe G Chi
Aug 16, 2003·Advances in Experimental Medicine and Biology·Hirotoshi KikuchiShunsei Hirohata
Aug 25, 2004·Neurology·D L H BennettG Giovannoni
Aug 10, 2005·Neurology·Heike Schmolck
Dec 6, 2005·Rheumatology International·Simone AppenzellerSandra Regina M Fernandes
Apr 6, 2006·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Emrah CanIşik Yalçin
May 20, 2006·Revue neurologique·M DarmoulM Hedi Dougui
Jan 6, 2007·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Yoshifumi AraiYoshihiro Tsutusi
Jan 26, 2007·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·F G Joseph, N J Scolding
Sep 14, 2007·Journal of the Neurological Sciences·Jae-Hyeok HeoManho Kim
Nov 3, 2007·Journal of the Neurological Sciences·Shunsei Hirohata
Jan 24, 2009·Lancet Neurology·Adnan Al-Araji, Desmond P Kidd
Apr 1, 2009·Arthritis and Rheumatism·D SaadounJ C Piette
Aug 18, 2009·Annals of the Rheumatic Diseases·Akira MeguroNobuhisa Mizuki
Oct 3, 2009·AJNR. American Journal of Neuroradiology·A O Varoglu
Feb 4, 2010·Journal of Neurology·Haruko IdeguchiYoshiaki Ishigatsubo
Nov 30, 2010·European Journal of Internal Medicine·A Riera-MestreA Vidaller
Dec 18, 2010·Clinical Rheumatology·Afshin Borhani HaghighiSiamak Farahangiz

❮ Previous
Next ❯

Citations

Dec 3, 2013·La Revue de médecine interne·N NoelD Saadoun
Jul 6, 2014·Tidsskrift for den Norske lægeforening : tidsskrift for praktisk medicin, ny række·Monika MocholEmilia Kerty
Feb 19, 2016·Pediatric Rheumatology Online Journal·Isabelle Koné-Paut
Mar 24, 2016·Current Treatment Options in Neurology·Murat KürtüncüGulsen Akman-Demir
Sep 28, 2014·Diagnostic and Interventional Imaging·D LeclercqA Drier
Dec 18, 2016·The Journal of Obstetrics and Gynaecology Research·Zehra Vural YılmazDilek Uygur
May 2, 2014·Arthritis & Rheumatology·Nicolas NoelDavid Saadoun
Apr 28, 2018·The British Journal of Ophthalmology·Ammr AlghamdiDavid Saadoun
Jun 20, 2013·Arthritis and Rheumatism·Nicolas NoelDavid Saadoun
Apr 3, 2019·JRSM Open·Theodoros PaschalisConstantinos G Missouris
Feb 25, 2021·Orphanet Journal of Rare Diseases·Isabelle Kone-PautUNKNOWN Collaborators
Dec 15, 2020·Seminars in Ultrasound, CT, and MR·Benjamin R GrayNicholas A Koontz

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.