Neurology. Clinical practice最新文献

{"title":"Spinal Muscular Atrophy Update in Best Practices: Recommendations for Treatment Considerations.","authors":"Mary K Schroth, Jennifer Deans, Diana X Bharucha Goebel, W Bryan Burnette, Basil T Darras, Bakri H Elsheikh, Marcia V Felker, Andrea Klein, Jena Krueger, Crystal M Proud, Aravindhan Veerapandiyan, Robert J Graham","doi":"10.1212/CPJ.0000000000200374","DOIUrl":"https://doi.org/10.1212/CPJ.0000000000200374","url":null,"abstract":"<p><strong>Background and objectives: </strong>Spinal muscular atrophy (SMA) is an autosomal recessive disorder caused by biallelic variants of the <i>Survival Motor Neuron 1</i> gene (<i>SMN1</i>) that affects approximately 1 in 15,000 live births. Availability of 3 SMN-enhancing treatments for SMA has led to urgency to review how clinicians and patients use these treatments for SMA, while additional research and real-world data and experience are being collected. This work describes important factors to assist with decision-making for SMN-enhancing treatments.</p><p><strong>Methods: </strong>A systematic literature review was conducted on SMN-enhancing treatments for SMA and related studies. A working group of American and European health care providers with expertise in SMA care identified barriers and developed recommendations through a modified Delphi technique with serial surveys and feedback through virtual meetings to fill gaps for information where evidence is limited. A community working group of an individual living with SMA and caregivers provided insight and perspective on SMA treatments and support through a virtual meeting to guide recommendations.</p><p><strong>Results: </strong>The health care provider working group and the community working group agreed that when determining whether to start, change, add, or discontinue a treatment, essential considerations include patient and family/caregiver perspective, and treatment safety and side effects. When initiating treatment for patients newly diagnosed with SMA, important patient characteristics are age and <i>Survival Motor Neuron 2</i> gene <i>(SMN2)</i> copy number. Furthermore, when initiating, changing, or adding treatment, current clinical status and comorbidities drive decision-making. When considering a medication or treatment plan change, unless there is an urgent indication, a treatment and associated patient outcomes should be monitored for a minimum of 6-12 months. When determining a treatment plan with an adolescent or adult with SMA, consider factors such as quality of life, burden vs benefit of treatment, and reproductive issues. Access to care coordination and interdisciplinary/multidisciplinary care are essential to treatment success.</p><p><strong>Discussion: </strong>Sharing information about current knowledge of treatments and shared decision-making between health care providers and patients living with SMA and caregivers are essential to overcoming barriers to providing SMN-enhancing treatments.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11464225/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142470882","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Uncertainties Regarding Cerebral Palsy Diagnosis: Opportunities to Clarify the Consensus Definition.","authors":"Bhooma R Aravamuthan, Darcy L Fehlings, Iona Novak, Paul Gross, Noor Alyasiry, Ann H Tilton, Michael I Shevell, Michael C Fahey, Michael C Kruer","doi":"10.1212/CPJ.0000000000200353","DOIUrl":"10.1212/CPJ.0000000000200353","url":null,"abstract":"<p><strong>Background and objectives: </strong>We have established that physicians, including neurologists, variably diagnose cerebral palsy (CP) when using the most recent CP definition from 2006. We also know that child neurologists and neurodevelopmentalists view themselves to be optimally suited to diagnose CP based on their training backgrounds. Therefore, to reduce variability in CP diagnosis, our objective was to elucidate uncertainties child neurologists and neurodevelopmentalists may have regarding practical application of the 2006 definition.</p><p><strong>Methods: </strong>We conducted a cross-sectional survey of child neurologists and neurodevelopmentalists built into a discussion seminar at the 2022 Child Neurology Society (CNS) Annual Meeting, the largest professional meeting of these specialists in North America. Seminar attendees were provided the 2006 definition and asked whether they had any uncertainties about the practical application of the definition across 4 hypothetical clinical vignettes. A group of national and international CP leaders then processed these data through iterative discussions to develop recommendations for clarifying the 2006 definition.</p><p><strong>Results: </strong>The seminar was attended by 50% of all conference attendees claiming CME (202/401). Of the 164 closing survey respondents, 145 (88%) expressed uncertainty regarding the clinical application of the 2006 definition. These uncertainties focused on 1) age, both regarding the minimum and maximum ages of brain disturbance or motor symptom onset (67/164, 41%), and 2) interpretation of the term \"nonprogressive\" (48/164, 29%). Almost all respondents (157/164, 96%) felt that we should revise the 2006 consensus definition of CP.</p><p><strong>Discussion: </strong>To address the most common CP diagnostic uncertainties we identified, we collectively propose 4 points of clarification to the 2006 definition: 1) motor symptoms/signs should be present by 2 years old; 2) CP can and should be diagnosed as early as possible; 3) the clinical motor disability phenotype should be nonprogressive through 5 years old; and 4) a CP diagnosis should be re-evaluated if motor disability is progressive or absent by 5 years old. We anticipate that clarifying the 2006 definition of CP in this manner could address the uncertainties we identified among child neurologists and neurodevelopmentalists and reduce the diagnostic variability that currently exists.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11347036/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142081050","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"An Interprofessional Team for Disease-Modifying Therapy in Alzheimer Disease Implementation.","authors":"Katherine W Turk, Mark D Knobel, Alexandra Nothern, Garrett Friedman, Hannah Noah, Brendan Campbell, Diana C Anderson, Andreas Charidimou, Andrew Mills, Vanessa Coronel, Nacha Pierre, Beverly V Reynolds, Caroline Wagner, Leanne M Varga, John Roefaro, Laura Triantafylidis, Andrew E Budson","doi":"10.1212/CPJ.0000000000200346","DOIUrl":"10.1212/CPJ.0000000000200346","url":null,"abstract":"<p><strong>Background: </strong>Lecanemab and other new amyloid-targeting immunotherapies for Alzheimer disease show notable promise but may also pose significant risk for patients.</p><p><strong>Recent findings: </strong>To facilitate the implementation and monitoring of lecanemab infusions at our tertiary medical center, we convened an interprofessional team. The team created a number of resources including patient handouts and medical documentation templates as well as systems and processes that are likely to be useful to other clinical care settings and centers.</p><p><strong>Implications for practice: </strong>It is our intent to widely share the resources and processes developed.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11340999/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142054135","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Imaging-Guided Subthalamic Nucleus Deep Brain Stimulation Programming for Parkinson Disease: A Real-Life Pilot Study.","authors":"Mickael Aubignat, Alexis Berro, Mélissa Tir, Michel Lefranc","doi":"10.1212/CPJ.0000000000200326","DOIUrl":"https://doi.org/10.1212/CPJ.0000000000200326","url":null,"abstract":"<p><strong>Background and objectives: </strong>Deep brain stimulation (DBS) is a well-established treatment for Parkinson disease (PD), with programming methods continually evolving. This study aimed to compare the efficacy and patient burden between conventional ring-mode programming (CP-RM) and image-guided volume of tissue activated (IG-VTA) programming for subthalamic nucleus (STN) DBS in PD.</p><p><strong>Methods: </strong>In this retrospective study, patients with PD who underwent STN-DBS between 2011 and 2014 (CP-RM group) and 2019 and 2021 (IG-VTA group) were evaluated. The primary outcome was the improvement in the UPDRS III score from preoperative OFF to postoperative ON state without medication at one-year follow-up. Secondary outcomes included hospital stay duration and programming sessions.</p><p><strong>Results: </strong>A total of 26 patients were analyzed (IG-VTA: n = 12, CP-RM: n = 14). Both groups showed similar improvements in UPDRS III scores (IG-VTA: 43.62, CP-RM: 41.29). However, the IG-VTA group experienced shorter immediate postoperative hospital stays and fewer hospitalizations after discharge.</p><p><strong>Discussion: </strong>IG-VTA programming preserved the clinical efficacy of STN-DBS over 1 year and reduced the patient and clinician burden of hospital stay and programming sessions. However, conclusions drawn must consider the limitations of retrospective design, differing time epochs, and evolving clinical practices. Further multicentric and prospective studies are warranted to validate these findings in the evolving field of neurostimulation.</p><p><strong>Trial registration information: </strong>The trial is registered on clinicaltrials.gov (NCT05103072).</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11396028/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142292112","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Utility of CSF Correction Factors for Traumatic Lumbar Puncture in Adults.","authors":"Ryan W Zhou, Kamala Sangam, Adrian Budhram","doi":"10.1212/CPJ.0000000000200350","DOIUrl":"10.1212/CPJ.0000000000200350","url":null,"abstract":"<p><strong>Objectives: </strong>To identify indicators of false pleocytosis in adults with traumatic lumbar puncture (LP), and determine specificities and sensitivities of commonly used CSF correction factors.</p><p><strong>Methods: </strong>Adults who underwent 4-tube CSF collection were reviewed. Study inclusion required elevated tube 1 red blood cell (RBC) count, tube 1 pleocytosis, and normalized tube 4 RBC count. Tube 4 white blood cell (WBC) count served as the reference standard. Specificities and sensitivities of 3 correction factors (1 WBC:500 RBC, 1 WBC:1000 RBC, and 1 WBC:1500 RBC) were calculated.</p><p><strong>Results: </strong>One hundred ninety-five adults were included. Among them, 106 (54%) had false tube 1 pleocytosis; these patients had a significantly higher median CSF RBC count and lower median CSF WBC count than those with true tube 1 pleocytosis. Specificities and sensitivities of correction factors ranged from 71.7% to 29.2% and 84.3% to 97.8%, respectively; 1 WBC:500 RBC had highest specificity for pleocytosis, while 1 WBC:1500 RBC had highest sensitivity. Irrespective of correction factor used, false-positive and false-negative determinations of pleocytosis were usually mild (≤20 WBCs/μL).</p><p><strong>Discussion: </strong>Indicators of false pleocytosis in adults with traumatic LP include bloodier CSF and milder pleocytosis, suggesting that correction factors are most useful in such cases. Across correction factors, an expected specificity/sensitivity tradeoff is observed. Corrected CSF WBC counts suggesting only mild pleocytosis should be interpreted cautiously.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11341082/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142056158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Geographic Access to High-Volume Mechanical Thrombectomy Centers in Florida, 2019.","authors":"Liza Solovey, Renee Y Hsia, Yu-Chu Shen, Elan L Guterman, Jay Chol Choi, Anthony S Kim","doi":"10.1212/CPJ.0000000000200337","DOIUrl":"https://doi.org/10.1212/CPJ.0000000000200337","url":null,"abstract":"<p><strong>Background and objectives: </strong>Mechanical thrombectomy (MT) improves outcomes for acute ischemic stroke (AIS) due to large vessel occlusion, but is time sensitive and requires specialized infrastructure. Professional organizations and certification bodies have promulgated minimum procedural volume standards for centers and for individual proceduralists but it is unclear whether enforcing these requirements would decrease geographic access to MT. Therefore, we sought to evaluate the potential impact of applying a minimum procedural volume threshold on geographic access to MT.</p><p><strong>Methods: </strong>We identified all hospital discharges for stroke where an MT procedure was performed at any nonfederal hospital in Florida in 2019 using statewide hospital discharge data. We then generated geographic service area maps based on prespecified ground transport distances for the subset of hospitals that performed at least 1 MT and for those that performed at least 15 MTs that year, the minimum volume threshold required for thrombectomy capable and comprehensive stroke centers by the Joint Commission. Then, using zip code centroids and patient-level discharge hospital data, we computed the proportion of patients with AIS who lived within each of the generated service areas.</p><p><strong>Results: </strong>A total of 105 of 297 hospitals performed MT; of those, 51 (17%) were low-volume centers (1-14 MTs/year) and 54 (18%) were high-volume centers (≥15 MTs/year). High-volume centers accounted for nearly 95% of all MTs performed in the state. Most patients hospitalized with AIS (87%) lived within 20 miles (or an estimated as a 1-hour driving time) of a hospital that performed at least 1 MT, and all (100%) lived within 115 miles (or estimated as 3-hour driving time). Setting a minimum MT volume threshold of 15 would decrease the proportion of stroke patients living within 1-hour driving time of an MT center from 87% to 77%.</p><p><strong>Discussion: </strong>In 2019, most Florida stroke patients lived within a 1-hour ground transport time to a center that performed at least 1 MT and all lived within 3-hour driving time of an MT center, irrespective of whether a minimum procedural volume threshold of 15 cases per year was applied or not.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11396029/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142292111","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Virtual Compared With In-Person Neurologic Examination Study.","authors":"Lauren Hophing, Tiffany Tse, Nicole Naimer, Mario Masellis, Saira S Mirza, Aaron Izenberg, Houman Khosravani, Charles D Kassardjian, Sara B Mitchell","doi":"10.1212/CPJ.0000000000200339","DOIUrl":"10.1212/CPJ.0000000000200339","url":null,"abstract":"<p><strong>Background and objectives: </strong>The COVID-19 pandemic forced a shift to virtual care in several neurologic care settings. Little is known about the validity of the virtual neurologic examination (VNE) for clinical decision making when compared with the in-person neurologic examination (IPNE). The objective of this study was to investigate the utility of the VNE in arriving at an accurate localization and diagnosis in comparison with the traditional IPNE in an ambulatory outpatient setting.</p><p><strong>Methods: </strong>A retrospective chart review of patients examined virtually and in-person within 4 months at outpatient general neurology and neuromuscular clinics from 2 tertiary academic care centers during the COVID-19 pandemic was conducted. The Cohen kappa coefficient was calculated to test agreement between virtual and in-person assessment results, and descriptive statistical methods were used to compare accuracy, localization, and diagnosis.</p><p><strong>Results: </strong>A total of 81 patients met the inclusion criteria. Overall, there was fair agreement between VNE and IPNE (64% agreement, <i>p</i> = 0.003). Substantial agreement between VNE and IPNE was observed for gait abnormalities; moderate agreement for extraocular movements, facial weakness, dysarthria, fasciculation, and lower limb weakness; and fair agreement for bulk, upper limb weakness, and sensation. No agreement between VNE and IPNE was seen for hypokinetic or hyperkinetic movements and cerebellar signs. Compared with the IPNE, specificity of the VNE was 86% and sensitivity was 56%. Some cases demonstrated a consistent localization (44%) and diagnosis (57%) after virtual and in-person assessments. The localization was changed in 15% and refined in 41% of cases between visits. The diagnosis was changed in 14% and refined in 30% of cases.</p><p><strong>Discussion: </strong>The high rates of agreement in detecting an abnormality on the VNE and IPNE for some maneuvers and resultant clinical impressions may support the validity of the VNE for initial consultation depending on the clinical scenario. The VNE seems to be a good surrogate evaluation compared with the IPNE for certain chief complaints. The low sensitivity suggests that a normal VNE should warrant further in-person clinical correlation, especially in the context of a highly concerning history. The IPNE is more sensitive in detecting subtle abnormalities on examination, and a low threshold should be used to bring a patient in for an IPNE if the VNE is normal in certain clinical contexts.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11341002/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142056129","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Psychological and Cognitive Landscape of Adult Idiopathic Intracranial Hypertension: A Scoping Review.","authors":"Menaka P Paranathala, Katie T Yoganathan, Patrick Mitchell, Isobel A Williams, Lois Gourley, Ian C Coulter","doi":"10.1212/CPJ.0000000000200348","DOIUrl":"10.1212/CPJ.0000000000200348","url":null,"abstract":"<p><strong>Purpose of review: </strong>Idiopathic intracranial hypertension (IIH) is a chronic headache disorder with increasing prevalence. Although characterized by raised intracranial pressure causing papilledema with a risk of visual impairment, psychological symptoms such as depression, anxiety, and cognitive impairment are frequently seen but are understudied and under-reported. Together, these can negatively affect quality of life. These were explored through a systematic PRISMA scoping review. OVID, EMBASE, Cochrane, and PubMed databases were searched in April 2023, limited to those in English and published during January 2000-March 2023. PROSPERO ID CRD42023399410.</p><p><strong>Recent findings: </strong>From 454 identified studies, 24 relevant articles were identified from a range of countries including the United Kingdom, the United States, Israel, Egypt, Canada, Germany, and Brazil. Data on study design, participants, tests performed, and outcomes were collated. There is a considerable burden of psychiatric, affective, and cognitive conditions, as well as impaired quality of life, in adult patients with IIH. Notably, these were independently correlated with the presence of headache in a number of studies and with obesity in others.</p><p><strong>Summary: </strong>Aside from optimizing symptom control, including reducing the severity of headaches and protecting visual function, clinicians should be mindful of the biopsychosocial difficulties and potential cognitive impairments patients with IIH face. Assistance may be required with managing concurrent depression and anxiety, as well as optimizing cognitive function. Incorporating neuropsychological assessment and individualized management strategies may be beneficial at the point of diagnosis and throughout the illness.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11341086/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142056162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Using Design Thinking to Understand the Reason for Headache Referrals and Reduce Referral Rates.","authors":"Ashish D Patel, Mallory Sponenberg, Leeann Webster, Sara Cole, Edward Stefanowicz, Jason A Dinko, Brian Seeley, Scott Friedenberg","doi":"10.1212/CPJ.0000000000200336","DOIUrl":"10.1212/CPJ.0000000000200336","url":null,"abstract":"<p><strong>Background: </strong>The demand for neurology services exceeds the current supply. We convened multiple stakeholders to learn what drives our primary care providers (PCPs) to refer patients with headache to our neurology practice. This information guided a collaborative effort to evaluate the impact of an electronic health record (EHR) headache tool on care delivery in our PCP clinics.</p><p><strong>Recent findings: </strong>Neurology referrals and MRI ordering declined by 77% and 35%, respectively, after the release of the EHR tool for an estimated savings of $207,600 over 3 months. PCPs prescribing habits minimally changed.</p><p><strong>Implications for practice: </strong>Electronically embedding a neurologist's knowledge in our PCP office was an effective way to shape the demand for headache consultation. By further leveraging stakeholder collaboration, we plan to improve the tool and disseminate it across our health system to reduce headache burden and health care costs.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11341001/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142056164","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A General Neurologist's Practical Diagnostic Algorithm for Atypical Parkinsonian Disorders: A Consensus Statement.","authors":"Michiko K Bruno, Rohit Dhall, Antoine Duquette, Ihtsham U Haq, Lawrence S Honig, Guillaume Lamotte, Zoltan Mari, Nikolaus R McFarland, Leila Montaser-Kouhsari, Federico Rodriguez-Porcel, Jessica Shurer, Junaid Siddiqui, Christopher C Spears, Anne-Marie A Wills, Kristophe Diaz, Lawrence I Golbe","doi":"10.1212/CPJ.0000000000200345","DOIUrl":"10.1212/CPJ.0000000000200345","url":null,"abstract":"<p><strong>Purpose of review: </strong>The most common four neurodegenerative atypical parkinsonian disorders (APDs) are progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal syndrome (CBS), and dementia with Lewy bodies (DLB). Their formal diagnostic criteria often require subspecialty experience to implement as designed and all require excluding competing diagnoses without clearly specifying how to do that. Validated diagnostic criteria are not available at all for many of the other common APDs, including normal pressure hydrocephalus (NPH), vascular parkinsonism (VP), or drug-induced parkinsonism (DIP). APDs also include conditions of structural, genetic, vascular, toxic/metabolic, infectious, and autoimmune origin. Their differential diagnosis can be challenging early in the course, if the presentation is atypical, or if a rare or non-neurodegenerative condition is present. This review equips community general neurologists to make an early provisional diagnosis before, or in place of, referral to a tertiary center. Early diagnosis would allay diagnostic uncertainty, allow prompt symptomatic management, provide disease-specific information and support resources, avoid further pointless testing and treatments, and create the possibility of trial referral.</p><p><strong>Recent findings: </strong>We address 64 APDs using one over-arching flow diagram and a series of detailed tables. Most instances of APDs can be diagnosed with a careful history and neurological exam, along with a non-contrast brain MRI. Additional diagnostic tests are rarely needed but are delineated where applicable. Our diagnostic algorithm encourages referral to a tertiary center whenever the general neurologist feels it would be in the patient's best interest. Our algorithm emphasizes that the diagnosis of APDs is an iterative process, refined with the appearance of new diagnostic features, availability of new technology, and advances in scientific understanding of the disorders. Clinicians' proposals for all diagnostic tests for the APDs, including repeat visits, should be discussed with patients and their families to ensure that the potential information to be gained aligns with their larger clinical goals.</p><p><strong>Summary: </strong>We designed this differential diagnostic algorithm for the APDs to enhance general neurologists' diagnostic skills and confidence and to help them address the less common or more ambiguous cases.</p>","PeriodicalId":19136,"journal":{"name":"Neurology. Clinical practice","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11341009/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142056155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}