Neuro-Behçet's disease: epidemiology, clinical characteristics, and management

Al-Araji, Adnan; Kidd, Desmond P.

doi:10.1016/s1474-4422(09)70015-8

Cited by 446 publications

(478 citation statements)

References 124 publications

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“…In a study with a limited number of patients, CSF constituents were shown to be altered in only around 70–80% of patients with parenchymal NBD [17]. In fact, in the present study, elevation of the CSF cell count was not observed in 9 of 35 patients with CP NBD.…”

Section: Discussioncontrasting

confidence: 70%

Clinical characteristics of neuro-Behcet’s disease in Japan: a multicenter retrospective analysis

et al. 2011

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To delineate the clinical characteristics of neuro-Behçet’s disease (NBD), a multicenter retrospective survey was performed in BD patients who had presented any neurological manifestations between 1988 and 2008. The diagnosis of acute NBD, chronic progressive (CP) NBD, and non-NBD was confirmed by retrospective review of clinical records. Data on a total of 144 patients were collected; 76 with acute NBD, 35 with CP NBD, and 33 with non-NBD. High-intensity lesions on T2-weighted magnetic resonance imaging (MRI) were found in 60.5% of the patients with acute NBD, 54.2% with CP NBD, and 42.4% with non-NBD, whereas brainstem atrophy was observed in 7.5% with acute NBD, 71.4% with CP NBD, and 9.0% with non-NBD. The cerebrospinal fluid (CSF) cell count was prominently elevated in patients with acute NBD, but was normal in about 15% of those with CP NBD. The sensitivity and specificity of the CSF cell count for the diagnosis of acute NBD versus non-NBD were 97.4 and 97.0%, respectively (cut-off 6.2/mm3). The sensitivity and specificity of CSF interleukin (IL)-6 for the diagnosis of CP NBD versus the recovery phase of acute NBD were 86.7 and 94.7%, respectively (cut-off 16.55 pg/ml). The results indicate that elevation of the CSF cell count and CSF IL-6 and the presence of brainstem atrophy on MRI are useful for the diagnosis of NBD.

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Section: Discussioncontrasting

confidence: 70%

Clinical characteristics of neuro-Behcet’s disease in Japan: a multicenter retrospective analysis

et al. 2011

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“…Although no consensus definition for NBD exists, the diagnosis is generally made in a BD patient with headache or neurological symptoms in the presence of compatible abnormalities on neuroimaging. 2,6,7 Approximately 5-15 % of those with BD subsequently develop NBD, 6,8 but 45 % of those with NBD will have moderate to severe neurological disability at 10-year follow-up. 2 It remains unclear if the prompt recognition and specific treatment of NBD would improve long-term prognosis, 6,8 but the diagnostic approach to headache and use of neuroimaging in BD can be categorized by the types of headaches experienced by BD patients and associated clinical features (Fig.…”

Section: Discussionmentioning

confidence: 99%

“…Of those, 70 % are migraine and tension headaches, similar to the distribution seen in the general population. 6 A study of BD patients presenting with migraine or tension headache and a normal neurological exam demonstrated that these patients had normal MRIs. 9 Neuroimaging for migraine and tension headaches in BD is not recommended unless there are neurological signs, other red-flag symptoms, or worsening presentation.…”

Section: Discussionmentioning

confidence: 99%

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Neuro-Behçet’s Disease: An Unusual Cause of Headache

Fountain

Dhurandhar

2014

J GEN INTERN MED

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“…Behçet's disease (BD) is a multisystemic, recurrent, inflammatory disorder of unknown etiology [107]. Neurological involvement is characterized either by primary parenchymal lesions or secondary to major vascular involvement.…”

Section: Roles Of Immune and Inflammation In Drementioning

confidence: 99%

Modulation of Immunity and the Inflammatory Response: A New Target for Treating Drug-resistant Epilepsy

Líu²,

Qin³

2013

Current Neuropharmacology

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Until recently, epilepsy medical therapy is usually limited to anti-epileptic drugs (AEDs). However, approximately 1/3 of epilepsy patients, described as drug-resistant epilepsy (DRE) patients, still suffer from continuous frequent seizures despite receiving adequate AEDs treatment of sufficient duration. More recently, with the remarkable progress of immunology, immunity and inflammation are considered to be key elements of the pathobiology of epilepsy. Activation of inflammatory processes in brain tissue has been observed in both experimental seizure animal models and epilepsy patients. Anti-inflammatory and immunotherapies also showed significant anticonvulsant properties both in clinical and in experimental settings. The above emerging evidence indicates that modulation of immunity and inflammatory processes could serve as novel specific targets to achieve potential anticonvulsant effects for the patients with epilepsy, especially DRE. Herein we review the recent evidence supporting the role of inflammation in the development and perpetuation of seizures, and also discuss the recent achievements in modulation of inflammation and immunotherapy applied to the treatment of epilepsy. Apart from medical therapy, we also discuss the influences of surgery, ketogenic diet, and electroconvulsive therapy on immunity and inflammation in DRE patients. Taken together, a promising perspective is suggested for future immunomodulatory therapies in the treatment of patients with DRE.

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Neuro-Behçet's disease: epidemiology, clinical characteristics, and management

Cited by 446 publications

References 124 publications

Clinical characteristics of neuro-Behcet’s disease in Japan: a multicenter retrospective analysis

Clinical characteristics of neuro-Behcet’s disease in Japan: a multicenter retrospective analysis

Neuro-Behçet’s Disease: An Unusual Cause of Headache

Modulation of Immunity and the Inflammatory Response: A New Target for Treating Drug-resistant Epilepsy

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