Neuro-Behcet's disease: An update on diagnosis, differential diagnoses, and treatment

Borhani‐Haghighi, Afshin; Kardeh, Bahareh; Banerjee, Shubhasree; Yadollahikhales, Golnaz; Safari, Anahid; Sahraian, Mohammad Ali; Shapiro, Lee

doi:10.1016/j.msard.2019.101906

Cited by 92 publications

(89 citation statements)

References 178 publications

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“…First, CSF examination for tumors in the basal ganglia or thalamus is not a new or additional method. In fact, CSF examination is required for differentiating among degenerative diseases such as MS, NMO, ADEM, and Behcet's disease, by measuring factors that are speci c to each [23,[3][4][5]. Moreover, the measurement of tumor markers such as HCG, HCGbeta, and AFP is well established and routine [6,10,20].…”

Section: Discussionmentioning

confidence: 99%

Precise detection of the germinomatous component of intracranial germ cell tumors of the basal ganglia and thalamus using placental alkaline phosphatase in cerebrospinal fluid

Chiba

Aihara

Kawamata

2021

Preprint

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Purpose: The disadvantages of biopsy for lesions in the basal ganglia and thalamus include a risk of various complications, difficulty in selecting the target tissue in some cases due to indistinct neuroimaging findings and limited availability of sample tissue. Placental alkaline phosphatase (PLAP) plays a decisive role in the diagnosis and management of intracranial germ cell tumors (IGCTs) in the basal ganglia and thalamus. The present study aimed to demonstrate the ability, specificity, and optimal use of PLAP values obtained from cerebrospinal fluid (CSF).Methods: Twenty patients with lesions in the basal ganglia and thalamus were enrolled in this study: 11 had IGCTs and 9 had non-IGCTs. The values of PLAP and other established tumor markers in the CSF were measured in all patients before treatment.Results: The mean follow-up period was 76.0 months (range, 3–168) for all lesions. PLAP was elevated in all 11 patients with IGCTs in the basal ganglia or thalamus, whereas none of the patients with non-IGCT exhibited elevated PLAP. Thus, the sensitivity and specificity of PLAP were both 100%.Conclusion: Our data demonstrated that the PLAP value can specifically identify the germinomatous component even in cases of IGCTs in the basal ganglia or thalamus with high sensitivity and specificity. PLAP is undoubtedly beneficial for the safe and timely detection of the germinomatous component of IGCTs in the basal ganglia and thalamus, because reliance on PLAP measurement enables us to avoid invasive surgical procedures and facilitates the prompt initiation of chemoradiation therapy.

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Section: Discussionmentioning

confidence: 99%

Precise detection of the germinomatous component of intracranial germ cell tumors of the basal ganglia and thalamus using placental alkaline phosphatase in cerebrospinal fluid

Chiba

Aihara

Kawamata

2021

Preprint

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“…The treatment of intravenous methylprednisolone 1,000 mg/day for 5 days was administrated with rapid improvement after 1 week, and this was followed by high-dose prednisone 60 mg/d Abbreviations: BD, Behçet's disease; NBD, neuro Behçet's disease; RTX, rituximab; CSF, Cerebrospinal fluid; MRI, Magnetic resonance imaging; ICR, International Consensus Recommendation; ESR, erythrocyte sedimentation rate; CRP, Creactive protein; NMOSD, neuromyelitis optica spectrum disorders; MS, multiple sclerosis; TNF-α, tumor necrosis factor alpha; IL, interleukin; γδ T, gammadelta T lymphocytes; Tregs, regulatory T lymphocytes; AQP4, aquaporin-4; MOG, myelin oligodendrocyte glycoprotein. and 100 mg azathioprine, gradually adding to 200 mg per day for maintenance (about 3 mg/kg/day), which is adequate for recommended dosage of azathioprine 2-3 mg/kg/day in NBD (6). A total of 9 months later, the patient barely tolerated the side effects of prednisone and quickly withdrew after 1 month.…”

Section: Case Presentationmentioning

confidence: 99%

“…Therefore, additional therapies for a subset of relapsing patients with NBD are inevitably needed. Indeed, accompanied by progression in the pathogenetic mechanisms of NBD, the treatments have been revolutionized by biological agents such as anti-cytokines and anti-B-lymphocytes (6).…”

mentioning

confidence: 99%

Case Report: Repeated Low-Dose Rituximab Treatment Is Effective in Relapsing Neuro Behçet's Disease

Zhao

Duan

et al. 2021

Front. Neurol.

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Neuro Behçet's disease (NBD) is a rare but most aggressive manifestation of Behçet's disease (BD) with a poor prognosis, and some patients even present a relapsing and treatment-resistant progressive course. In some relapsing NBD cases, traditional corticosteroids and immunosuppressive drugs show limited efficacy, while benefits of biological agents, such as anti-B-lymphocyte CD20 biological agent rituximab (RTX), gradually represent potential therapeutic advantages with clinical rapid remission and long-time maintenance. However, up to now, the optimal dosage of RTX in NBD is still elucidated. Here, we report two patients with relapsing NBD, despite continuous high dose steroids and sufficient azathioprine treatment, still presenting severe and relapsing meningoencephalitis or brainstem involvement. Repeated low-dose RTX (100 mg × 3/1 week apart, 100 mg repeated every 6 months) is then attempted with rapid recovery and sustained remission. The approach in our cases may expand therapeutic options and provide helpful references for relapsing NBD treatment.

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“…For non-parenchymal disease with CVST, first-line therapy is also high-dose steroids with an oral taper but further immunosuppression is not usually necessary as relapses are uncommon [24]. Use of anticoagulation is debated given increased risk of bleeding, but a short course is advised (Grade C) [22,25,26]. Recurrence of CVST is rare if systemic inflammation is well controlled, and accordingly, long-term anticoagulation is not indicated [24,25].…”

Section: Behcet's Diseasementioning

confidence: 99%

“…For severe parenchymal NBD, TNF-alpha antagonists, such as infliximab should be considered for first line and breakthrough disease (level III, grade C) [ 24 , 25 ]. Given the finding of elevated CSF IL-6, tocilizumab has also been trialed and shown promising efficacy [ 26 – 28 ]. Cyclosporine should be avoided due to neurotoxicity [ 22 , 24 ].…”

Section: Inflammation Of the Central Nervous System From Rheumatologimentioning

confidence: 99%

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

Lapides

McDonald

2020

Curr Treat Options Neurol

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Purpose of Review This review presents the current recommended therapeutic interventions for inflammatory disease in the central nervous system (CNS) secondary to systemic diseases of immune dysregulation. Treatment recommendations for CNS inflammation associated with rheumatologic conditions, immune-related adverse effects from immune checkpoint inhibitors (ICIs), and demyelinating disease from tumor necrosis factor-α (anti-TNFs) are explored. Additional therapeutic options for inflammation related to postviral syndromes and genetic immunodeficiencies are also discussed. Recent Findings In addition to treatment of mild, moderate, and severe CNS rheumatologic disease as guided by the European League Against Rheumatism (EULAR), early consideration of rituximab for severe IgG4-related disease and induction with anti-TNF therapy for severe neurosarcoidosis should be considered. Although often not first line, treatment options for CNS inflammatory diseases based on disease mechanism are emerging, including tocilizumab for Behcet's disease, natalizumab for ICI associated autoimmune encephalitis, and abatacept for treatment of infiltrative disease secondary to CTLA-4 deficiency. Hematopoietic stem cell treatments represent highly efficacious but risky options for autoimmunity related to genetic immunodeficiency. Summary While early high dose steroids remains first line therapy for most CNS inflammatory conditions, a rapidly expanding arsenal of immune targeted therapies offers clinicians tailored disease specific options for treatment.

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Neuro-Behcet's disease: An update on diagnosis, differential diagnoses, and treatment

Cited by 92 publications

References 178 publications

Precise detection of the germinomatous component of intracranial germ cell tumors of the basal ganglia and thalamus using placental alkaline phosphatase in cerebrospinal fluid

Precise detection of the germinomatous component of intracranial germ cell tumors of the basal ganglia and thalamus using placental alkaline phosphatase in cerebrospinal fluid

Case Report: Repeated Low-Dose Rituximab Treatment Is Effective in Relapsing Neuro Behçet's Disease

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

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