US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.1.14
A. Wimo, L. Jönsson, G. Johansson, B. Winblad
{"title":"Lecanemab: The Price of a Breakthrough","authors":"A. Wimo, L. Jönsson, G. Johansson, B. Winblad","doi":"10.17925/usn.2023.19.1.14","DOIUrl":"https://doi.org/10.17925/usn.2023.19.1.14","url":null,"abstract":"On 6 January 2023, the amyloid beta-targeting antibody lecanemab was approved by the US Food and Drug Administration for the treatment of mild cognitive impairment due to Alzheimer’s disease (AD) and mild AD dementia via its Accelerated Approval pathway. However, two important questions need to be highlighted: What is the appropriate patient population to receive treatment? How much should healthcare systems be prepared to pay for the drug? The healthcare infrastructure is not prepared for the expected huge demands for diagnostics and eventual treatment, especially given the suggested list price and a potentially large population of people with mild cognitive impairment due to AD and mild AD dementia. A lower price point would allow wider access to the treatment and reduce disparities in health equity.","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67613781","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}
US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.1.41
Alex M. T. Russell, Bridget Marcinkowski, M. Chin
{"title":"Patients’ Beliefs in Optimal Pain Management: A Pilot Study","authors":"Alex M. T. Russell, Bridget Marcinkowski, M. Chin","doi":"10.17925/usn.2023.19.1.41","DOIUrl":"https://doi.org/10.17925/usn.2023.19.1.41","url":null,"abstract":"Opioids are commonly prescribed for chronic pain despite potential adverse side effects, risks of misuse and abuse, and limited evidence for improved outcomes. Patients’ perspectives and understanding of pain management may impact treatment approaches and the success of therapies. Yet, few studies have investigated how patients’ beliefs in optimal pain management may influence their care. We prospectively surveyed 200 patients with chronic pain with the aim of assessing patients’ beliefs on the optimal management of their chronic pain. The following significant findings were made: 1) most responders agreed that opioid medication was the best way to control pain (p<0.05), especially those currently taking opioids (p<0.0001); 2) those who received injections for pain disagreed that opioids were the best way to control pain (p<0.0001); 3) most participants were aware of other options to control pain (p<0.05), but patients on opioids were significantly less aware of other options for pain control (p<0.05). This study demonstrated that most patients with chronic pain surveyed in our clinic believed that opioids were the best option for treating their chronic pain, but this finding did not hold true for those who were exposed to other interventions for their pain, such as injections. Pain physicians may encounter resistance when attempting to incorporate non-opioid and or non-pharmacological therapies for chronic pain management. Further research may help determine whether patient education and exposure to such therapies can help combat these challenges.","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67614235","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}
US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.2.1
R. Karda
{"title":"The Future of SCN1A Gene-targeting Research for the Treatment of Dravet Syndrome","authors":"R. Karda","doi":"10.17925/usn.2023.19.2.1","DOIUrl":"https://doi.org/10.17925/usn.2023.19.2.1","url":null,"abstract":"<p />","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67614348","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}
US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.2.4
Prashant Bhatele, M. Khanal
{"title":"A Rare Presentation of Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis with Neuropsychiatric Symptoms: A Case Report","authors":"Prashant Bhatele, M. Khanal","doi":"10.17925/usn.2023.19.2.4","DOIUrl":"https://doi.org/10.17925/usn.2023.19.2.4","url":null,"abstract":"Background: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is an autoimmune disease that appears as a fulminant, subacute or chronic course of altered mental status, often accompanied by seizures and myoclonus. This case report demonstrates that SREAT can be present with solitary neuropsychiatric signs long before seizures and myoclonus. Methods: A 42-year-old female presented with abrupt-onset whole-body myoclonic jerks. She had been suffering from depression for 15 years before being diagnosed with hypothyroidism. She was conscious, oriented and alert. At rest, all four limbs had multifocal mild-to-moderate myoclonus, which was significantly aggravated by muscle activation; her Hamilton Depression Rating Scale score was 21. Results: Antithyroglobulin and antithyroid peroxidase autoantibodies were both above 2,000 IU/mL. The thyroid-stimulating hormone level was 5.65 mIU/mL, free triiodothyronine level was 3.36 pg/mL and free thyroxine level was 1.02 ng/dL. Vasculitis profile and the serum test for autoimmune encephalitis panel were negative. Brain neuroimaging was normal. Pulse dose of methylprednisolone followed by oral steroids resulted in significant clinical improvement. Conclusion: SREAT can present with chronic neuropsychiatric symptoms with abrupt exacerbation of seizures and myoclonus. This case study emphasizes the importance of screening individuals with depression and thyroid problems for serum antithyroid antibody levels. In most cases, steroid treatment yields positive results.","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"49 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67614421","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}
US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.1.8
I. Subramanian, K. Perepezko, B. Stone, R. Chaudhuri, G. Pontone
{"title":"Wellness in Parkinson’s Disease: A Framework for Management Using a Holistic Culturally Sensitive Approach","authors":"I. Subramanian, K. Perepezko, B. Stone, R. Chaudhuri, G. Pontone","doi":"10.17925/usn.2023.19.1.8","DOIUrl":"https://doi.org/10.17925/usn.2023.19.1.8","url":null,"abstract":"The concept of wellness has yet to be formally adopted in Parkinson’s disease (PD) management despite its potential to improve quality of life. Currently, PD treatment is usually focused on symptomatic therapies for motor symptom relief using medications or devices. While the focus on non-motor symptoms has increased in the last decade, it remains suboptimal. Future clinical practice should strive to incorporate the more holistic 'dashboard vitals of PD', which include comorbidities/polypharmacy and dental, vision, bone, and gut health and go beyond just motor and non-motor issues. Furthermore, recognizing and addressing the cultural context of the patient and practising healthcare from a place of cultural humility can help decrease barriers to care and build trust. Promoting self-agency through personalized lifestyle interventions, such as healthy diet, exercise, sleep routines, mind–body approaches and social support, can make them more accessible and easier to maintain. Building upon the concept of personalized medicine in PD, wellness programmes can proactively target non-motor issues and mental and psychosocial health by enhancing resilience and supporting social connections. The desired outcome of a wellness approach extends beyond the traditional medical model to improve quality of life in a more enduring manner.","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67614299","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}
US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.2.2
Riley Kessler, Sonal Sharma, D. Lynch
{"title":"Omaveloxolone for the Treatment of Friedreich’s Ataxia","authors":"Riley Kessler, Sonal Sharma, D. Lynch","doi":"10.17925/usn.2023.19.2.2","DOIUrl":"https://doi.org/10.17925/usn.2023.19.2.2","url":null,"abstract":"Friedrich’s ataxia (FRDA), a neurodevelopmental and progressive neurodegenerative disease, is the most common inherited form of ataxia. Omaveloxolone was approved by the US Food and Drugs Administration in early 2023, making it the first treatment available to patients with FRDA. This approval was made possible by combining a compelling cellular mechanism and strong clinical evidence provided through the MOXIe study, the multipart clinical trial evaluating the efficacy of omaveloxolone in patients with FRDA. This review discusses the underlying cellular pathology and proposed mechanism of omaveloxolone in FRDA. The MOXIe study is presented in detail, including a discussion of the challenges faced in clinical trials in FRDA, and rare diseases more broadly. Finally, other therapies under investigation are reviewed briefly.","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67614357","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}
US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.2.25
C. Ahern
{"title":"Paradoxical Tumefactive Worsening of Multiple Sclerosis After Natalizumab Initiation: A Case Report","authors":"C. Ahern","doi":"10.17925/usn.2023.19.2.25","DOIUrl":"https://doi.org/10.17925/usn.2023.19.2.25","url":null,"abstract":"Background: Natalizumab is a widely used anti-α4 integrin inhibitor for treating highly active multiple sclerosis. Although clinical and radiological relapses were observed in the pivotal natalizumab trials, severe disease activity after initiation of the drug is a rare phenomenon and has been reported only in isolated cases. Objective: To present a case of a patient who experienced a paradoxical increase in disease activity after the second dose of natalizumab. Methods: We describe the case, review the literature concerning similar cases and suggest possible mechanisms for this phenomenon. Results: Our case involves a patient who developed extensive tumefactive demyelinating lesions and multiple gadolinium-enhancing lesions detected on magnetic resonance imaging after receiving the second dose of natalizumab. A brain biopsy confirmed the presence of demyelination, and the patient’s condition improved after treatment with intravenous methylprednisolone, intravenous immunoglobulin and plasma exchange. Tests for anti-natalizumab antibodies were negative. Conclusions: Paradoxical worsening can occur in the setting of natalizumab treatment, which warrants careful attention and should prompt anti-natalizumab antibody testing. We discuss potential mechanisms. Further research is needed to better understand the mechanisms and risk factors for paradoxical worsening and to develop strategies for mitigating this adverse effect with significant patient impact.","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67614363","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}
US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.1.2
Owen Miller
{"title":"Dementia in Europe Yearbook 2022: Employment and Related Social Protection for People with Dementia and Their Carers","authors":"Owen Miller","doi":"10.17925/usn.2023.19.1.2","DOIUrl":"https://doi.org/10.17925/usn.2023.19.1.2","url":null,"abstract":"<p />","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67614153","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}
US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.1.4
D. Gove, J. Georges
{"title":"Sex, Gender and Sexuality in the Context of Dementia: A Guide to Raise Awareness Amongst Health and Social Care Workers","authors":"D. Gove, J. Georges","doi":"10.17925/usn.2023.19.1.4","DOIUrl":"https://doi.org/10.17925/usn.2023.19.1.4","url":null,"abstract":"<p />","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67614226","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}
US neurologyPub Date : 2023-01-01DOI: 10.17925/usn.2023.19.1.6
D. Gove, Ana Diaz
{"title":"Guidelines for the Ethical and Inclusive Communication About/Portrayal of Dementia and People with Dementia","authors":"D. Gove, Ana Diaz","doi":"10.17925/usn.2023.19.1.6","DOIUrl":"https://doi.org/10.17925/usn.2023.19.1.6","url":null,"abstract":"<p />","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67614289","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}