Neuro-behçet's disease in South Korea: clinical characteristics and treatment response

Autor:	Bon San Koo, Chang-Keun Lee, You Jae Kim, Yong-Gil Kim, Da-Lim Yoon, Bin Yoo
Rok vydání:	2014
Předmět:	Adult Male medicine.medical_specialty Time Factors Internal capsule Disease Behcet's disease Pharmacotherapy Rheumatology Recurrence Internal medicine Republic of Korea medicine Humans Medical history Cyclophosphamide Retrospective Studies Neurologic Examination business.industry Behcet Syndrome Remission Induction Retrospective cohort study Middle Aged medicine.disease Magnetic Resonance Imaging Surgery Treatment Outcome Drug Therapy Combination Female Steroids Nervous System Diseases Neuro-Behçet's disease business Complication Immunosuppressive Agents
Zdroj:	International Journal of Rheumatic Diseases. 17:453-458
ISSN:	1756-1841
DOI:	10.1111/1756-185x.12265
Popis:	Aim Neuro-behcet's disease (NBD) is a rare complication of Behcet's disease (BD) but is still important due to its morbidity and mortality. In this study, we sought to identify the characteristics of NBD by examining the clinical characteristics, and whether there were differences in the clinical characteristics or the treatment between relapsed and non-relapsed groups. Methods We retrospectively reviewed the medical history of all patients diagnosed with BD at the Asan Medical Center in South Korea from January 1996 to May 2012. NBD was defined as neurological symptoms and compatible abnormalities identified by magnetic resononance imaging. Results Among 624 BD patients, 22 were diagnosed with NBD. The common initial neurological symptoms were headache, gait disturbance and dysarthria. The common involved sites were the diencephalon, including internal capsule, the thalamus and basal ganglia, followed by the brainstem. An increased signal intensity on T2-weighted images was the most common finding. All patients had an improved disease state after steroid treatment, regardless of combined treatment with cyclophosphamide. However, eight patients experienced a disease recurrence at 7.3 ± 5.1 months after treatment. There were no significant differences in treatment patterns between the relapsed group and the non-relapsed group. Conclusions NBD may present with various neurologic manifestations with multifocal diencephalon and brainstem involvement. Most NBD patients had a good clinical outcome after steroid treatment, with or without a cytotoxic agent, but showed a pattern of relapse–remission. Therefore, careful long-term follow-up is needed in most cases of NBD.
Databáze:	OpenAIRE
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