Background: Behcet's disease is a chronic inflammatory disorder usually characterized by the triad of oral ulcers, genital ulcers, and uveitis. Central to the pathogenesis of Behcet's disease is an autoimmune vasculitis. Neurological involvement, so called "Neuro-Behcet's disease", occurs in 10-20% of patients, usually from a meningoencephalitis or venous thrombosis.
Case Report: We report the case of a 46-year-old patient with Neuro-Behcet's disease who presented with central neurogenic hyperventilation as a result of brainstem involvement from venulitis.
Conclusions: To the best of our knowledge, central neurogenic hyperventilation has not previously been described in a patient with Neuro-Behcet's disease.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4791089 | PMC |
http://dx.doi.org/10.12659/ajcr.895382 | DOI Listing |
Zhonghua Er Ke Za Zhi
January 2025
Department of Rheumatology and Immunology, Children's Hospital Affiliated to Capital Institute of Pediatrics, Beijing100020, China.
To investigate the clinical features and prognosis of neuro-Behçet's syndrome (NBS) in children. The clinical, brain magnetic resonance imaging and laboratory data of 5 children with NBS diagnosed in the Department of Pediatrics, General Hospital of Ningxia Medical University and Department of Rheumatology and Immunology, Children's Hospital Affiliated to Capital Institute of Pediatrics from April 2014 to April 2024 were analyzed retrospectively. The follow-up method was retrospective outpatient or inpatient visit to evaluate the treatment effect of NBS.
View Article and Find Full Text PDFClin Case Rep
December 2024
Multiple Sclerosis Research Center, Neuroscience Institute Tehran University of Medical Sciences Tehran Iran.
This case highlights the importance of considering Neuro-Behçet's disease (NBD) in the differential diagnosis of multiple sclerosis (MS), particularly in patients presenting with neurological manifestations, abnormal magnetic resonance imaging (MRI) findings, and systemic symptoms consistent with Behçet's disease (BD).
View Article and Find Full Text PDFRadiol Case Rep
January 2025
Central Radiology Department, Ibn Sina University Hospital Center, Mohamed V University of Rabat, Rabat, Morocco.
Neuro-Behçet disease is often difficult to diagnose due to its complex and severe clinical presentation. This article reports the case of a 35-year-old female patient with a history of Behçet's disease, admitted for a deep coma. Brain MRI performed upon admission revealed extended lesions in the basal ganglia, thalami, and midbrain, along with leptomeningeal contrast enhancement in the temporal region, suggesting meningoencephalitis compatible with parenchymal involvement of neuro-Behçet's disease.
View Article and Find Full Text PDFNEJM Evid
November 2024
Department of Internal Medicine and Clinical Immunology, Sorbonne Universités, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière University Hospital, Centre national de références Maladies Autoimmunes et systémiques rares, National Reference Centre for Rare Autoinflammatory Diseases and Inflammatory Amyloidosis, INSERM, UMR S959, Immunology-Immunopathology-Immunotherapy, Paris.
J Clin Med
September 2024
Rheumatology Division, Hospital Universitario Marqués de Valdecilla, Immunopathology Group-IDIVAL, Avda. Valdecilla s/n., 39008 Santander, Cantabria, Spain.
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