Pub Date : 2018-01-01DOI: 10.17925/ENR.2018.13.2.120
H. Santos, Pedro Agrela, C. Parente, Kelly Lopes, S. Santos, A. Cardoso, F. Campante
C harles Bonnet Syndrome (CBS) is a rare cause of complex visual hallucinations (VH) in individuals with preserved cognitive status. We report a case of an elderly patient with VH, diplopia and headaches for 1 week, with preserved visual acuity and without any strength or focal neurological findings. Computed tomography revealed a subacute ischaemic stroke with temporal and occipital involvement. The patient was admitted to the Department of Internal Medicine where neurological, ophthalmological and psychiatric primary disease were ruled out, as well as dementia. CBS was assumed as a result of a subacute ischaemic stroke. Valproic acid was initiated to treat the symptoms, which resolved the VH. After 3 months of follow up, the patient remained completely recovered without any dementia signs. CBS is a benign disease, usually controlled without pharmacological therapy and not associated with a psychiatric disease; nevertheless, its association with dementia is not clear.
C - harles Bonnet综合征(CBS)是一种罕见的导致复杂视幻觉(VH)的原因，发生在认知状态完好的个体中。我们报告一例老年VH患者，复视和头痛1周，视力保留，没有任何力量或局灶性神经学发现。计算机断层扫描显示亚急性缺血性脑卒中伴颞部和枕部受累。患者被送往内科，在那里排除了神经、眼科和精神原发疾病，以及痴呆症。CBS被认为是亚急性缺血性中风的结果。开始使用丙戊酸治疗症状，解决了VH。随访3个月后，患者完全康复，无痴呆症状。CBS是一种良性疾病，通常无需药物治疗即可控制，与精神疾病无关;然而，它与痴呆症的关系尚不清楚。
{"title":"Charles Bonnet Syndrome as a Rare Complication in an Ischaemic Stroke","authors":"H. Santos, Pedro Agrela, C. Parente, Kelly Lopes, S. Santos, A. Cardoso, F. Campante","doi":"10.17925/ENR.2018.13.2.120","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.120","url":null,"abstract":"C harles Bonnet Syndrome (CBS) is a rare cause of complex visual hallucinations (VH) in individuals with preserved cognitive status. We report a case of an elderly patient with VH, diplopia and headaches for 1 week, with preserved visual acuity and without any strength or focal neurological findings. Computed tomography revealed a subacute ischaemic stroke with temporal and occipital involvement. The patient was admitted to the Department of Internal Medicine where neurological, ophthalmological and psychiatric primary disease were ruled out, as well as dementia. CBS was assumed as a result of a subacute ischaemic stroke. Valproic acid was initiated to treat the symptoms, which resolved the VH. After 3 months of follow up, the patient remained completely recovered without any dementia signs. CBS is a benign disease, usually controlled without pharmacological therapy and not associated with a psychiatric disease; nevertheless, its association with dementia is not clear.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"120"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592276","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.2018.13.1.44
Gebbie A. R. Nielsen, H. H. Nielsen, Z. Illés, P. Gazerani
{"title":"Prevalence and pattern of craniofacial pain and headache in Danish patients with neuromyelitis optica spectrum disorder","authors":"Gebbie A. R. Nielsen, H. H. Nielsen, Z. Illés, P. Gazerani","doi":"10.17925/ENR.2018.13.1.44","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.1.44","url":null,"abstract":"","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"44-52"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592678","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.2018.13.1.18
S. Jacob
Myasthenia gravis (MG) is an autoimmune disorder characterised by muscle weakness and fatigue. Symptoms include slurred speech, weakness in the arms and legs and difficulty swallowing and breathing, which can lead to life-threatening myasthenic crisis. Despite receiving recommended therapies, many patients with MG report health-related quality of life is low, largely due to impaired mobility and depression. Furthermore, around 10% of patients with generalised MG fail to respond to treatment. Patients with refractory MG have a severely compromised quality of life and it is important that new treatments are evaluated for this group of patients.
{"title":"Refractory Myasthenia Gravis – Patient Burden and the Need for New Therapeutic Targets","authors":"S. Jacob","doi":"10.17925/ENR.2018.13.1.18","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.1.18","url":null,"abstract":"Myasthenia gravis (MG) is an autoimmune disorder characterised by muscle weakness and fatigue. Symptoms include slurred speech, weakness in the arms and legs and difficulty swallowing and breathing, which can lead to life-threatening myasthenic crisis. Despite receiving recommended therapies, many patients with MG report health-related quality of life is low, largely due to impaired mobility and depression. Furthermore, around 10% of patients with generalised MG fail to respond to treatment. Patients with refractory MG have a severely compromised quality of life and it is important that new treatments are evaluated for this group of patients.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"18"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592579","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.2018.13.1.25
P. Vermersch
The introduction of oral disease modifying therapies has transformed the treatment landscape for patients with multiple sclerosis (MS). Fingolimod (Gilenya®, Novartis, Basel, Switzerland), the first oral therapy to be approved, has demonstrated clinical efficacy as a result of modulation of subtype 1 sphingosine-1-phosphate (S1P1) receptors. This leads to retention of lymphocytes in the lymph nodes, preventing their entry into the central nervous system. However, fingolimod can cause adverse effects as a result of its interaction with other S1P receptor subtypes, which are expressed in numerous tissues, including cardiac myocytes. More selective S1P receptor agents are currently in phase II and III clinical development. Siponimod, ozanimod, ponesimod and amiselimod have demonstrated efficacy with improved safety profiles compared with fingolimod. While more long-term data are needed, these selective S1P receptor modulators appear to be promising options for the treatment of MS and other disorders associated with autoimmunity and inflammation.
{"title":"Sphingosine-1-phosphate Receptor Modulators in Multiple Sclerosis","authors":"P. Vermersch","doi":"10.17925/ENR.2018.13.1.25","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.1.25","url":null,"abstract":"The introduction of oral disease modifying therapies has transformed the treatment landscape for patients with multiple sclerosis (MS). Fingolimod (Gilenya®, Novartis, Basel, Switzerland), the first oral therapy to be approved, has demonstrated clinical efficacy as a result of modulation of subtype 1 sphingosine-1-phosphate (S1P1) receptors. This leads to retention of lymphocytes in the lymph nodes, preventing their entry into the central nervous system. However, fingolimod can cause adverse effects as a result of its interaction with other S1P receptor subtypes, which are expressed in numerous tissues, including cardiac myocytes. More selective S1P receptor agents are currently in phase II and III clinical development. Siponimod, ozanimod, ponesimod and amiselimod have demonstrated efficacy with improved safety profiles compared with fingolimod. While more long-term data are needed, these selective S1P receptor modulators appear to be promising options for the treatment of MS and other disorders associated with autoimmunity and inflammation.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"25"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592632","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.2018.13.2.103
Pinay Kainth, N. Murphy, S. Rossi, Jean-Noël Talabardon, M. Pedrazzoli, Leonardo Mascagni, Irene Menarini, Dublin Ireland Novartis Ireland Limited, Basel Switzerland Novartis Pharma Ag
Introduction: Migraine is one of the leading reasons for patient access to neurology services. Waiting lists can limit patients’ ability to access specialist care, even at specialised headache centres. Our study aims to investigate this issue, identify possible root causes and also document existing good practices. Methods: We conducted a study in a sample of 28 headache centres and their networks in six countries by performing in-depth interviews with 166 healthcare professionals. Results: The waiting list for new patients and follow-up visits exceeded 3 months in 61% and 36% of centres, respectively. Patients waited on average 6 months for their first consultation, with peaks beyond 12 months. Five areas were identified as common root causes: (1) inappropriate referral of patients with low-frequency episodic migraine or patients under acute treatment, (2) lack of triage/priority allocation, (3) limited resource availability or resources dedicated to migraine, (4) limited delegation of activities, and (5) suboptimal management of follow-up visits. Conclusion: Our work highlights a gap between best practices for migraine management proposed in the literature and current real-world practice. Guidelines recommend a “network” approach to bridge different levels of care. Based on our findings, consistency in practice amongst specialised headache clinics and integration with primary care represent an important area for further improvement.
{"title":"Management of Migraine and the Accessibility of Specialist Care – Findings from a Multi-national Assessment of 28 Healthcare Networks","authors":"Pinay Kainth, N. Murphy, S. Rossi, Jean-Noël Talabardon, M. Pedrazzoli, Leonardo Mascagni, Irene Menarini, Dublin Ireland Novartis Ireland Limited, Basel Switzerland Novartis Pharma Ag","doi":"10.17925/ENR.2018.13.2.103","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.103","url":null,"abstract":"Introduction: Migraine is one of the leading reasons for patient access to neurology services. Waiting lists can limit patients’ ability to access specialist care, even at specialised headache centres. Our study aims to investigate this issue, identify possible root causes and also document existing good practices. Methods: We conducted a study in a sample of 28 headache centres and their networks in six countries by performing in-depth interviews with 166 healthcare professionals. Results: The waiting list for new patients and follow-up visits exceeded 3 months in 61% and 36% of centres, respectively. Patients waited on average 6 months for their first consultation, with peaks beyond 12 months. Five areas were identified as common root causes: (1) inappropriate referral of patients with low-frequency episodic migraine or patients under acute treatment, (2) lack of triage/priority allocation, (3) limited resource availability or resources dedicated to migraine, (4) limited delegation of activities, and (5) suboptimal management of follow-up visits. Conclusion: Our work highlights a gap between best practices for migraine management proposed in the literature and current real-world practice. Guidelines recommend a “network” approach to bridge different levels of care. Based on our findings, consistency in practice amongst specialised headache clinics and integration with primary care represent an important area for further improvement.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"103"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592220","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.2018.13.1.14
A. Diaz-Ponce, J. Georges
{"title":"Residential Care for People with Dementia in Europe – Not the Same Policy Standards Everywhere","authors":"A. Diaz-Ponce, J. Georges","doi":"10.17925/ENR.2018.13.1.14","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.1.14","url":null,"abstract":"","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"14"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592513","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.2018.13.2.100
J. Reuck, Degenerative
Mixed dementias are clinically under-recognised and need neuropathological confirmation. The most frequent types are those composed of Alzheimer’s dementia (AD) associated with cerebral amyloid angiopathy (CAA), cerebral arteriosclerotic micro-angiopathy (CAMA) and Lewy body disease (LBD). The present neuropathological study with 7.0-tesla magnetic resonance imaging (MRI) compares the impact and the distribution of cerebrovascular lesions between unmixed AD brains to those with different types of associated disorders. Twenty-six unmixed AD brains were compared to 12 associated with LBD, eight with CAMA and 24 with CAA. On neuropathological examination brains with AD-CAA had the most severe cerebrovascular lesions, those with AD-CAMA had a higher number of lacunes and in the AD-LBD brains only an increase of cortical micro-infarcts was observed. On MRI examination, white matter changes were only increased in the AD-CAA group. Cortical micro-infarcts were significantly more frequent in all the sections of the AD-CAA and AD-CAMA groups. In the AD-LBD brains, they were only moderately more common in the occipital section. Cortical micro-bleeds were observed in all mixed dementia cases. A moderate amount of superficial siderosis was only seen in the AD-CAA brains. The present study confirms our previous findings that CAA and CAMA are the main causes of occurrence of different cerebrovascular lesions in the mixed AD brains.
{"title":"Cerebrovascular Lesions in Alzheimer-associated Diseases – A Neuropathological Study with 7.0-tesla Magnetic Resonance Imaging","authors":"J. Reuck, Degenerative","doi":"10.17925/ENR.2018.13.2.100","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.100","url":null,"abstract":"Mixed dementias are clinically under-recognised and need neuropathological confirmation. The most frequent types are those composed of Alzheimer’s dementia (AD) associated with cerebral amyloid angiopathy (CAA), cerebral arteriosclerotic micro-angiopathy (CAMA) and Lewy body disease (LBD). The present neuropathological study with 7.0-tesla magnetic resonance imaging (MRI) compares the impact and the distribution of cerebrovascular lesions between unmixed AD brains to those with different types of associated disorders. Twenty-six unmixed AD brains were compared to 12 associated with LBD, eight with CAMA and 24 with CAA. On neuropathological examination brains with AD-CAA had the most severe cerebrovascular lesions, those with AD-CAMA had a higher number of lacunes and in the AD-LBD brains only an increase of cortical micro-infarcts was observed. On MRI examination, white matter changes were only increased in the AD-CAA group. Cortical micro-infarcts were significantly more frequent in all the sections of the AD-CAA and AD-CAMA groups. In the AD-LBD brains, they were only moderately more common in the occipital section. Cortical micro-bleeds were observed in all mixed dementia cases. A moderate amount of superficial siderosis was only seen in the AD-CAA brains. The present study confirms our previous findings that CAA and CAMA are the main causes of occurrence of different cerebrovascular lesions in the mixed AD brains.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"100"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592215","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.2018.13.2.113
F. Mena-Martín, A. Gutiérrez-García, J. Martín‐Escudero, O. Fernández-Arconada
L evetiracetam (LEV) is one of the most common anti-epileptic drugs available. In general, it is tolerated relatively well; the majority of adverse effects are moderate and normally occur during the initial titration. We present a patient who developed two moderately serious adverse effects after an initial LEV dose: a 28-year-old male was admitted to intensive care unit after suffering two generalised seizures, and was given 1000 mg of LEV. Twenty-four hours after admittance, the laboratory tests showed a serum creatinine of 2.84 mg/dL and creatine kinase (CK) of 421 U/L (normal, 0–171 U/L). At all times the diuresis was normal, with a maximum value of creatinine of 4.67 mg/dL 48 hours following admittance, and the CK values ranged between 421–681 U/L with proteinuria of 840 mg/day. On the seventh day, blood tests showed a CK of 1,559 U/L and a creatinine of 1.55 mg/dL. LEV was progressively substituted for lacosamide, after which creatinine, CK and albumin excretion rate were normalised. Thus, CK and renal function during treatment with LEV should be monitored, and acute kidney injury due to LEV should be considered in the differential diagnosis for any unexplained acute renal failure.
{"title":"Acute Kidney Injury and Creatine Kinase Elevation After Beginning Treatment with Levetiracetam","authors":"F. Mena-Martín, A. Gutiérrez-García, J. Martín‐Escudero, O. Fernández-Arconada","doi":"10.17925/ENR.2018.13.2.113","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.113","url":null,"abstract":"L evetiracetam (LEV) is one of the most common anti-epileptic drugs available. In general, it is tolerated relatively well; the majority of adverse effects are moderate and normally occur during the initial titration. We present a patient who developed two moderately serious adverse effects after an initial LEV dose: a 28-year-old male was admitted to intensive care unit after suffering two generalised seizures, and was given 1000 mg of LEV. Twenty-four hours after admittance, the laboratory tests showed a serum creatinine of 2.84 mg/dL and creatine kinase (CK) of 421 U/L (normal, 0–171 U/L). At all times the diuresis was normal, with a maximum value of creatinine of 4.67 mg/dL 48 hours following admittance, and the CK values ranged between 421–681 U/L with proteinuria of 840 mg/day. On the seventh day, blood tests showed a CK of 1,559 U/L and a creatinine of 1.55 mg/dL. LEV was progressively substituted for lacosamide, after which creatinine, CK and albumin excretion rate were normalised. Thus, CK and renal function during treatment with LEV should be monitored, and acute kidney injury due to LEV should be considered in the differential diagnosis for any unexplained acute renal failure.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"113"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592229","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.2018.13.2.68
N. Lund
Support: No funding was received for the publication of this article. C luster headache (CH) is a primary headache disorder affecting up to 0.1% of the population. 1 It is characterised by the extreme nature of the attacks, the unilateral distribution of pain and the accompanying ipsilateral autonomic symptoms and restlessness. Attacks may arise between once every other day and eight times a day during the attack periods, the bouts, and patients are highly burdened and disabled. Although the clinical features are distinct, CH may be misdiagnosed, and is suboptimally managed. In an expert interview, Nunu Lund discusses the unmet needs in the management of CH, as well as the findings of a recent study that may improve the diagnosis and management of this debilitating condition.
{"title":"New Insights into Cluster Headache","authors":"N. Lund","doi":"10.17925/ENR.2018.13.2.68","DOIUrl":"https://doi.org/10.17925/ENR.2018.13.2.68","url":null,"abstract":"Support: No funding was received for the publication of this article. C luster headache (CH) is a primary headache disorder affecting up to 0.1% of the population. 1 It is characterised by the extreme nature of the attacks, the unilateral distribution of pain and the accompanying ipsilateral autonomic symptoms and restlessness. Attacks may arise between once every other day and eight times a day during the attack periods, the bouts, and patients are highly burdened and disabled. Although the clinical features are distinct, CH may be misdiagnosed, and is suboptimally managed. In an expert interview, Nunu Lund discusses the unmet needs in the management of CH, as well as the findings of a recent study that may improve the diagnosis and management of this debilitating condition.","PeriodicalId":12047,"journal":{"name":"European neurological review","volume":"13 1","pages":"68"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67592311","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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