Pub Date : 2018-03-01DOI: 10.17925/USN.2018.14.1.29
D. Hanley, W. Ziai, I. Awad
Intracerebral hemorrhage (ICH) with intraventricular extension is a devastating disease occurring in 40% of patients with spontaneous ICH. Although the CLEAR III trial (NCT00784134) demonstrated mortality reduction with intraventricular alteplase (versus saline), lessons learned warrant a therapeutic trial focusing on disease severity and treatment endpoints that support a high likelihood of improvement. We must answer questions of maximizing therapy intensity in large intraventricular hemorrhage (IVH) to promote good neurologic outcomes if we are committed to treating ICH and the full spectrum of stroke severity.
{"title":"Not “doing the same thing over and over again”","authors":"D. Hanley, W. Ziai, I. Awad","doi":"10.17925/USN.2018.14.1.29","DOIUrl":"https://doi.org/10.17925/USN.2018.14.1.29","url":null,"abstract":"Intracerebral hemorrhage (ICH) with intraventricular extension is a devastating disease occurring in 40% of patients with spontaneous ICH. Although the CLEAR III trial (NCT00784134) demonstrated mortality reduction with intraventricular alteplase (versus saline), lessons learned warrant a therapeutic trial focusing on disease severity and treatment endpoints that support a high likelihood of improvement. We must answer questions of maximizing therapy intensity in large intraventricular hemorrhage (IVH) to promote good neurologic outcomes if we are committed to treating ICH and the full spectrum of stroke severity.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"14 1","pages":"29-30"},"PeriodicalIF":0.0,"publicationDate":"2018-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46635690","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-01-01DOI: 10.17925/ENR.2018.13.2.72
P. Ryvlin, T. Tomson, O. Devinsky
Sudden unexpected death in epilepsy (SUDEP) is the most common cause of death related to epilepsy and is associated with treatment resistance and the presence of generalised tonic-clonic seizures (GTCS, of either focal or generalised onset). While the causative mechanisms of SUDEP are yet to be fully elucidated, it is thought that seizure-induced brainstem suppression, and respiratory and cardiac dysfunction may be involved. Research into SUDEP has identified several risk factors (including frequency of GTCS and male gender) but has also indicated proven or potential preventive strategies, including more effective seizure control. Despite increasing awareness and research into SUDEP, its underlying mechanisms and preventive strategies remain poorly defined. More research is needed into the pathophysiology of SUDEP and to identify predictive biomarkers. Furthermore, clinical trials are warranted to assess outcomes with preventive interventions. We review SUDEP epidemiology and risk factors, and discuss potential measures to reduce SUDEP risk.
{"title":"Prevention of Sudden Unexpected Death in Epilepsy","authors":"P. Ryvlin, T. Tomson, O. Devinsky","doi":"10.17925/ENR.2018.13.2.72","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.72","url":null,"abstract":"Sudden unexpected death in epilepsy (SUDEP) is the most common cause of death related to epilepsy and is associated with treatment resistance and the presence of generalised tonic-clonic seizures (GTCS, of either focal or generalised onset). While the causative mechanisms of SUDEP are yet to be fully elucidated, it is thought that seizure-induced brainstem suppression, and respiratory and cardiac dysfunction may be involved. Research into SUDEP has identified several risk factors (including frequency of GTCS and male gender) but has also indicated proven or potential preventive strategies, including more effective seizure control. Despite increasing awareness and research into SUDEP, its underlying mechanisms and preventive strategies remain poorly defined. More research is needed into the pathophysiology of SUDEP and to identify predictive biomarkers. Furthermore, clinical trials are warranted to assess outcomes with preventive interventions. We review SUDEP epidemiology and risk factors, and discuss potential measures to reduce SUDEP risk.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"72"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592319","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-01-01DOI: 10.17925/ENR.2018.13.2.86
S. Jacob
Myasthenia gravis (MG) is an autoimmune disorder that leads to skeletal muscle weakness and fatigue. The autoimmune attack is caused by autoantibodies against the acetylcholine postsynaptic receptors at the neuromuscular junction of skeletal muscles. However, other antigenic targets that are components of the neuromuscular junction have also been implicated in the pathogenesis of MG. The current standard of care is immunosuppressive therapy; however, many existing therapeutic options have not been validated for use in MG in large randomised controlled trials. Furthermore, around 10% of patients with generalised MG are refractory to treatment. The complement system is involved in numerous inflammatory, neurodegenerative and autoimmune diseases, and is a key factor in the pathogenesis of acetylcholine receptor antibody-related MG. Targeting complement and other components involved in the underlying pathogenesis of the disease may provide useful treatment options, particularly for refractory patients.
{"title":"Myasthenia Gravis – A Review of Current Therapeutic Options","authors":"S. Jacob","doi":"10.17925/ENR.2018.13.2.86","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.86","url":null,"abstract":"Myasthenia gravis (MG) is an autoimmune disorder that leads to skeletal muscle weakness and fatigue. The autoimmune attack is caused by autoantibodies against the acetylcholine postsynaptic receptors at the neuromuscular junction of skeletal muscles. However, other antigenic targets that are components of the neuromuscular junction have also been implicated in the pathogenesis of MG. The current standard of care is immunosuppressive therapy; however, many existing therapeutic options have not been validated for use in MG in large randomised controlled trials. Furthermore, around 10% of patients with generalised MG are refractory to treatment. The complement system is involved in numerous inflammatory, neurodegenerative and autoimmune diseases, and is a key factor in the pathogenesis of acetylcholine receptor antibody-related MG. Targeting complement and other components involved in the underlying pathogenesis of the disease may provide useful treatment options, particularly for refractory patients.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"86"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592401","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-01-01DOI: 10.17925/ENR.2018.13.1.21
J. Manson
Multiple sclerosis (MS) is a disabling disease affecting the central nervous system. Despite the high frequency of this disease in women of childbearing age, it has previously been found that female patients with MS are often uninformed regarding the effects of pregnancy on MS and there is little available research on family planning decisions in females with MS. In this commentary we examine the results of a recent multi-country study, carried out by Wakefield Research for Teva Pharmaceuticals, of 1,000 women, aged 25–35 years, who were diagnosed with relapsing forms of MS (RMS) in the last 5 years. The survey sampled 200 women from each of the following five countries: Germany, Italy, the Netherlands, Spain and the United Kingdom. Results from this survey highlight a lack of open communication regarding family planning between women in Europe with MS and healthcare professionals (HCPs), and show that insufficient information on family planning is being provided to the majority of those surveyed. We discuss the importance of family planning for European women with MS, and their top concerns regarding this issue. In addition, we outline how family planning concerns are addressed with HCPs, and discuss how family planning education for patients with MS can be improved.
{"title":"European Women With Multiple Sclerosis Feel Unprepared and Uneducated About Family Planning and Their Ability to Have Children – How Do We Improve Patient Education?","authors":"J. Manson","doi":"10.17925/ENR.2018.13.1.21","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.1.21","url":null,"abstract":"Multiple sclerosis (MS) is a disabling disease affecting the central nervous system. Despite the high frequency of this disease in women of childbearing age, it has previously been found that female patients with MS are often uninformed regarding the effects of pregnancy on MS and there is little available research on family planning decisions in females with MS. In this commentary we examine the results of a recent multi-country study, carried out by Wakefield Research for Teva Pharmaceuticals, of 1,000 women, aged 25–35 years, who were diagnosed with relapsing forms of MS (RMS) in the last 5 years. The survey sampled 200 women from each of the following five countries: Germany, Italy, the Netherlands, Spain and the United Kingdom. Results from this survey highlight a lack of open communication regarding family planning between women in Europe with MS and healthcare professionals (HCPs), and show that insufficient information on family planning is being provided to the majority of those surveyed. We discuss the importance of family planning for European women with MS, and their top concerns regarding this issue. In addition, we outline how family planning concerns are addressed with HCPs, and discuss how family planning education for patients with MS can be improved.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"21"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-01-01DOI: 10.17925/ENR.2018.13.2.116
B. Purbasari, S. Kurniawan
Background: Motor neuropathy is an extremely rare herpes complication, with a mere prevalence of 0.5–5%. The case of segmental zoster paresis of limbs, resulting from motor radiculopathy, is especially limited, with cervical and thoracic segments being the least frequent. Setting: Neurology outpatient clinic. Case Description: We report a case of a 16-year-old female who presented sudden-onset right upper extremity weakness, a week after her herpes zoster lesions first appeared. As she was diagnosed with systemic lupus erythematosus (SLE) 4 months prior, she routinely consumed steroids and azathioprine. Initial examinations revealed multiple vesicles along right C5-C6 roots dermatome accompanied by upper right extremity weakness (manual muscle test [MMT] 3) corresponding to the myotome of C5-C6 roots. An electromyography assessment uncovered results relevant to motor root neuritis in C5-C6. Magnetic resonance imaging of the cervical radix with contrast showed no abnormality. Thus, she received acyclovir, gabapentin and physiotherapy. Results: A follow-up visit after 2 weeks revealed an improvement of the weakness along C5-C6 myotome (MMT 4). A month later, all motor functions were restored with hypoesthesia and hypoalgesia sensory sequelae along C5-C6 dermatome. Conclusion: Herpes zoster radiculopathy, though rare, can occur after the onset of characteristic rash. Since cellular-mediated immunity holds crucial roles in varicella zoster virus activation, SLE and immunosuppression therapy is pertinent to this rare motoric complication of herpes. The prognosis is good. Acyclovir, gabapentin and physiotherapy treatments resulted in satisfactory recovery.
{"title":"Herpes Zoster Radiculopathy in a Systemic Lupus Erythematosus Patient – A Case Report","authors":"B. Purbasari, S. Kurniawan","doi":"10.17925/ENR.2018.13.2.116","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.116","url":null,"abstract":"Background: Motor neuropathy is an extremely rare herpes complication, with a mere prevalence of 0.5–5%. The case of segmental zoster paresis of limbs, resulting from motor radiculopathy, is especially limited, with cervical and thoracic segments being the least frequent. Setting: Neurology outpatient clinic. Case Description: We report a case of a 16-year-old female who presented sudden-onset right upper extremity weakness, a week after her herpes zoster lesions first appeared. As she was diagnosed with systemic lupus erythematosus (SLE) 4 months prior, she routinely consumed steroids and azathioprine. Initial examinations revealed multiple vesicles along right C5-C6 roots dermatome accompanied by upper right extremity weakness (manual muscle test [MMT] 3) corresponding to the myotome of C5-C6 roots. An electromyography assessment uncovered results relevant to motor root neuritis in C5-C6. Magnetic resonance imaging of the cervical radix with contrast showed no abnormality. Thus, she received acyclovir, gabapentin and physiotherapy. Results: A follow-up visit after 2 weeks revealed an improvement of the weakness along C5-C6 myotome (MMT 4). A month later, all motor functions were restored with hypoesthesia and hypoalgesia sensory sequelae along C5-C6 dermatome. Conclusion: Herpes zoster radiculopathy, though rare, can occur after the onset of characteristic rash. Since cellular-mediated immunity holds crucial roles in varicella zoster virus activation, SLE and immunosuppression therapy is pertinent to this rare motoric complication of herpes. The prognosis is good. Acyclovir, gabapentin and physiotherapy treatments resulted in satisfactory recovery.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"116"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-01-01DOI: 10.17925/ENR.2018.13.1.31
E. Mercuri, R. Quinlivan, S. Tuffery-Giraud
The understanding of the natural history of Duchenne muscular dystrophy (DMD) is increasing rapidly and new treatments are emerging that have the potential to substantially improve the prognosis for patients with this disabling and life-shortening disease. For many, however, there is a long delay between the appearance of symptoms and DMD diagnosis, which reduces the possibility of successful treatment. DMD results from mutations in the large dystrophin gene of which one-third are de novo mutations and two-thirds are inherited from a female carrier. Roughly 75% of mutations are large rearrangements and 25% are point mutations. Certain deletions and nonsense mutations can be treated whereas many other mutations cannot currently be treated. This emphasises the need for early genetic testing to identify the mutation, guide treatment and inform genetic counselling. Treatments for DMD include corticosteroids and more recently, ataluren has been approved in Europe, the first disease-modifying therapy for treating DMD caused by nonsense mutations. The use of ataluren in DMD is supported by positive results from phase IIb and phase III studies in which the treatment produced marked improvements in the 6-minute walk test, timed function tests such as the 10 m walk/run test and the 4-stair ascent/descent test compared with placebo. In these trials, ataluren was well tolerated and adverse event profiles were similar to placebo. As such disease-modifying treatments become more widely available, the outlook for children with DMD will improve but physicians must be aware of the disease, rapidly initiate testing where it is suspected and promptly begin appropriate treatment.
{"title":"Early Diagnosis and Treatment – The Use of Ataluren in the Effective Management of Duchenne Muscular Dystrophy","authors":"E. Mercuri, R. Quinlivan, S. Tuffery-Giraud","doi":"10.17925/ENR.2018.13.1.31","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.1.31","url":null,"abstract":"The understanding of the natural history of Duchenne muscular dystrophy (DMD) is increasing rapidly and new treatments are emerging that have the potential to substantially improve the prognosis for patients with this disabling and life-shortening disease. For many, however, there is a long delay between the appearance of symptoms and DMD diagnosis, which reduces the possibility of successful treatment. DMD results from mutations in the large dystrophin gene of which one-third are de novo mutations and two-thirds are inherited from a female carrier. Roughly 75% of mutations are large rearrangements and 25% are point mutations. Certain deletions and nonsense mutations can be treated whereas many other mutations cannot currently be treated. This emphasises the need for early genetic testing to identify the mutation, guide treatment and inform genetic counselling. Treatments for DMD include corticosteroids and more recently, ataluren has been approved in Europe, the first disease-modifying therapy for treating DMD caused by nonsense mutations. The use of ataluren in DMD is supported by positive results from phase IIb and phase III studies in which the treatment produced marked improvements in the 6-minute walk test, timed function tests such as the 10 m walk/run test and the 4-stair ascent/descent test compared with placebo. In these trials, ataluren was well tolerated and adverse event profiles were similar to placebo. As such disease-modifying treatments become more widely available, the outlook for children with DMD will improve but physicians must be aware of the disease, rapidly initiate testing where it is suspected and promptly begin appropriate treatment.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"93 1","pages":"31"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-01-01DOI: 10.17925/ENR.2018.13.1.38
Mia Birkholm Lausten, S. Rasmussen, P. Gazerani
number of genetic factors such as gender and hair colour have been associated with pain. ABO blood types have been linked to a diverse range of diseases such as various types of cancer, but only two studies have investigated a possible link between ABO blood types and pain. Thus, the aim of this study was to investigate if an association exists between a certain blood type and post-operative pain. Patients (18–40 years) who had an anterior cruciate ligament (ACL) reconstruction at Aalborg University Hospital, Aalborg, Denmark between January 2012 and August 2017 were included in this retrospective study. Blood type and post-operative analgesic use were extracted from the patients’ medical journals. The post-operative analgesics were converted to milligrams of morphine using equivalent doses for comparison between blood types and consumption of the analgesics. Sixty-six patients undergoing ACL reconstruction were divided into blood types A, B or O. None of the enrolled patients had AB blood type. No significant difference was found between a certain blood type and the amount of post-operative analgesics consumed (p=0.517). Findings from this study demonstrated that patients undergoing ACL reconstruction with blood types A, B and O were not significantly different concerning consumption of post-operative analgesics.
{"title":"Association Between the ABO Blood Types and Post-operative Pain","authors":"Mia Birkholm Lausten, S. Rasmussen, P. Gazerani","doi":"10.17925/ENR.2018.13.1.38","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.1.38","url":null,"abstract":"number of genetic factors such as gender and hair colour have been associated with pain. ABO blood types have been linked to a diverse range of diseases such as various types of cancer, but only two studies have investigated a possible link between ABO blood types and pain. Thus, the aim of this study was to investigate if an association exists between a certain blood type and post-operative pain. Patients (18–40 years) who had an anterior cruciate ligament (ACL) reconstruction at Aalborg University Hospital, Aalborg, Denmark between January 2012 and August 2017 were included in this retrospective study. Blood type and post-operative analgesic use were extracted from the patients’ medical journals. The post-operative analgesics were converted to milligrams of morphine using equivalent doses for comparison between blood types and consumption of the analgesics. Sixty-six patients undergoing ACL reconstruction were divided into blood types A, B or O. None of the enrolled patients had AB blood type. No significant difference was found between a certain blood type and the amount of post-operative analgesics consumed (p=0.517). Findings from this study demonstrated that patients undergoing ACL reconstruction with blood types A, B and O were not significantly different concerning consumption of post-operative analgesics.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"6 1","pages":"38-43"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592669","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-01-01DOI: 10.17925/ENR.2018.13.2.78
A. Scalfari, P. Muraro
{"title":"Monoclonal Antibody Therapy and Long-term Outcomes in Multiple Sclerosis – The Challenge of Treatment Optimisation","authors":"A. Scalfari, P. Muraro","doi":"10.17925/ENR.2018.13.2.78","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.78","url":null,"abstract":"","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"78"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592385","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-01-01DOI: 10.17925/ENR.2018.13.2.93
S. Ozyurt, P. Sfikakis, A. Siva, C. Constantinescu
Background: Behçet’s disease is a relatively uncommon, inflammatory disorder with characteristic mucocutaneous lesions and multisystem involvement, of unknown aetiology; presumably a vascular autoinflammatory syndrome that develops under combined environmental and genetic influences. As neuro-Behçet’s disease affects the central nervous system in about 10% of cases and in ways that can mimic other neuroinflammatory conditions, awareness of its manifestations, significance, and management is important for neurologists. In March 2017, a mini-symposium at the 11th Congress of Controversies in Neurology in Athens, Greece, was dedicated to specific aspects of Behçet’s and neuro-Behçet’s disease. These included an introduction to Behçet’s disease, pathogenesis and treatment, an overview of its neurological manifestations (neuro-Behçet’s disease) and the differential diagnosis from other neuroinflammatory conditions. Illustrative case reports were used. Objectives: To provide a brief overview of neuro-Behçet’s disease that is informative for clinical neurological practice and that follows the structure of the 2017 mini-symposium. Data sources: Relevant recent comprehensive reviews of the subject and relevant original articles and case reports were provided by each speaker at the mini-symposium. This article contains some of these sources and some additions where necessary to emphasise specific points. References are also provided for more comprehensive recent reviews. Limitations: The mini-symposium was an opportunity for providing a brief update and overview of neuro-Behçet’s disease and to exchange ideas and experience among neurologists. As such, it was found to be helpful, but also limited in scope. This resultant article refers to comprehensive reviews on the topic but is not in itself a comprehensive systematic review. Conclusions: Neuro-Bechet’s disease comprises largely two forms, parenchymal and a non-parenchymal. These manifestations seldom overlap in the same individual and may reflect different pathogenetic mechanisms. The principles of treatment largely follow the principles of treating Bechet’s disease in general, with the mainstay being corticosteroids for exacerbations and immunosuppressive treatments for prevention of exacerbations. One notable exception is cyclosporine, which is typically avoided in neuro-Bechet’s disease. Anti-tumour necrosis factor biologicals play an increasing role in treatment. Distinguishing neuro-Behçet’s disease from other neuroinflammatory conditions, such as multiple sclerosis, is essential for both management and prognostic reasons.
{"title":"Neuro-Behçet’s Disease – Clinical Features, Diagnosis and Differential Diagnosis","authors":"S. Ozyurt, P. Sfikakis, A. Siva, C. Constantinescu","doi":"10.17925/ENR.2018.13.2.93","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.93","url":null,"abstract":"Background: Behçet’s disease is a relatively uncommon, inflammatory disorder with characteristic mucocutaneous lesions and multisystem involvement, of unknown aetiology; presumably a vascular autoinflammatory syndrome that develops under combined environmental and genetic influences. As neuro-Behçet’s disease affects the central nervous system in about 10% of cases and in ways that can mimic other neuroinflammatory conditions, awareness of its manifestations, significance, and management is important for neurologists. In March 2017, a mini-symposium at the 11th Congress of Controversies in Neurology in Athens, Greece, was dedicated to specific aspects of Behçet’s and neuro-Behçet’s disease. These included an introduction to Behçet’s disease, pathogenesis and treatment, an overview of its neurological manifestations (neuro-Behçet’s disease) and the differential diagnosis from other neuroinflammatory conditions. Illustrative case reports were used. Objectives: To provide a brief overview of neuro-Behçet’s disease that is informative for clinical neurological practice and that follows the structure of the 2017 mini-symposium. Data sources: Relevant recent comprehensive reviews of the subject and relevant original articles and case reports were provided by each speaker at the mini-symposium. This article contains some of these sources and some additions where necessary to emphasise specific points. References are also provided for more comprehensive recent reviews. Limitations: The mini-symposium was an opportunity for providing a brief update and overview of neuro-Behçet’s disease and to exchange ideas and experience among neurologists. As such, it was found to be helpful, but also limited in scope. This resultant article refers to comprehensive reviews on the topic but is not in itself a comprehensive systematic review. Conclusions: Neuro-Bechet’s disease comprises largely two forms, parenchymal and a non-parenchymal. These manifestations seldom overlap in the same individual and may reflect different pathogenetic mechanisms. The principles of treatment largely follow the principles of treating Bechet’s disease in general, with the mainstay being corticosteroids for exacerbations and immunosuppressive treatments for prevention of exacerbations. One notable exception is cyclosporine, which is typically avoided in neuro-Bechet’s disease. Anti-tumour necrosis factor biologicals play an increasing role in treatment. Distinguishing neuro-Behçet’s disease from other neuroinflammatory conditions, such as multiple sclerosis, is essential for both management and prognostic reasons.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"93"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592455","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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