Neurology

Shobi Sivathamboo, Daniel Friedman, Juliana Laze, Russell Nightscales, Zhibin Chen, Levin Kuhlmann, Sasha Devore, Vaughan Macefield, Patrick Kwan, Wendyl D'Souza, Samuel F. Berkovic, Piero Perucca, Terence J. O'Brien, Orrin Devinsky, on behalf of the MS-BioS Study Group

doi : 10.1212/WNL.0000000000012946

vol. 97 no. 24 e2357-e2367

We compared heart rate variability (HRV) in sudden unexpected death in epilepsy (SUDEP) cases and living epilepsy controls.

خرید پکیج و مشاهده آنلاین مقاله

Burden of Chronic and Acute Conditions and Symptoms in People With Epilepsy

Wyatt P. Bensken, Guadalupe Fernandez-Baca Vaca, Barbara C. Jobst, Scott M. Williams, Kurt C. Stange, Martha Sajatovic, Siran M. Koroukian

doi : 10.1212/WNL.0000000000012975

vol. 97 no. 24 e2368-e2380

People with epilepsy, one-third of whom in the United States are on Medicaid, experience a wide range of chronic and physical comorbidities that influence their care and outcomes. In this study, we examine the burden and racial/ethnic disparities of chronic and acute conditions, injuries, and symptoms in a large and diverse group of people with epilepsy on Medicaid.

خرید پکیج و مشاهده آنلاین مقاله

Diet Inflammatory Index and Dementia Incidence

Sokratis Charisis, Eva Ntanasi, Mary Yannakoulia, Costas A. Anastasiou, Mary H. Kosmidis, Efthimios Dardiotis, Antonios N. Gargalionis, Kostas Patas, Stylianos Chatzipanagiotou, Ioannis Mourtzinos, Katerina Tzima, Georgios Hadjigeorgiou, Paraskevi Sakka, Dimitrios Kapogiannis, Nikolaos Scarmeas

doi : 10.1212/WNL.0000000000012973

vol. 97 no. 24 e2381-e2391

Aging is characterized by a functional shift of the immune system toward a proinflammatory phenotype. This derangement has been associated with cognitive decline and has been implicated in the pathogenesis of dementia. Diet can modulate systemic inflammation; thus, it may be a valuable tool to counteract the associated risk for cognitive impairment and dementia. The present study aimed to explore the associations between the inflammatory potential of diet, assessed with an easily applicable, population-based, biomarker-validated diet inflammatory index (DII), and the risk for dementia in community-dwelling older adults.

خرید پکیج و مشاهده آنلاین مقاله

Neurofascin-155 Immunoglobulin Subtypes

Shahar Shelly, Christopher J. Klein, P. James B. Dyck, Pritikanta Paul, Michelle L. Mauermann, Sarah E. Berini, Benjamin Howe, James P. Fryer, Eati Basal, Hammami M. Bakri, Ruple S. Laughlin, Andrew McKeon, Sean J. Pittock, John Mills, Divyanshu Dubey

doi : 10.1212/WNL.0000000000012932

vol. 97 no. 24 e2392-e2403

Multiple studies highlighting the diagnostic utility of neurofascin-155 (NF155)–immunoglobulin G4 (IgG4) in chronic demyelinating inflammatory polyradiculoneuropathy (CIDP) have been published. However, few studies comprehensively address the long-term outcomes or clinical utility of NF155–immunoglobulin M (IgM) or NF155–immunoglobulin G (IgG) in the absence of NF155-IgG4. We evaluated phenotypic and histopathologic specificity and differences in outcomes between these NF155 antibody isotypes or IgG subclasses. We also compare NF155-IgG4-seropositive cases to other seropositive demyelinating neuropathies.

خرید پکیج و مشاهده آنلاین مقاله

Association of the Level of Neurofilament Light With Disease Severity in Patients With Spinocerebellar Ataxia Type 2

Lu Yang, Ya-Ru Shao, Xiao-Yan Li, Yin Ma, Yi Dong, Zhi-Ying Wu

doi : 10.1212/WNL.0000000000012945

vol. 97 no. 24 e2404-e2413

Few biochemical markers have been identified in spinocerebellar ataxia type 2 (SCA2). This study aimed to determine the levels of neurofilament light (NfL) in patients with SCA2 and to identify whether they are associated with disease severity.

خرید پکیج و مشاهده آنلاین مقاله

Simulation-Based Assessment of Graduate Neurology Trainees' Performance Managing Acute Ischemic Stroke

Melissa B. Pergakis, Wan-Tsu W. Chang, Ali Tabatabai, Michael S. Phipps, Benjamin Neustein, Jamie E. Podell, Gunjan Parikh, Neeraj Badjatia, Melissa Motta, David P. Lerner, Nicholas A. Morris

doi : 10.1212/WNL.0000000000012972

vol. 97 no. 24 e2414-e2422

Multidisciplinary acute stroke teams improve acute ischemic stroke management but may hinder trainees' education, which in turn may contribute to poorer outcomes in community hospitals on graduation. Our goal was to assess graduate neurology trainee performance independently of a multidisciplinary stroke team in the management of acute ischemic stroke, tissue plasminogen activator (tPA)–related hemorrhage, and cerebral herniation syndrome.

خرید پکیج و مشاهده آنلاین مقاله

CNS Involvement in Hereditary Transthyretin Amyloidosis

Luísa Sousa, Teresa Coelho, Ricardo Taipa

doi : 10.1212/WNL.0000000000012965

vol. 97 no. 24 1111-1119

Hereditary transthyretin amyloidosis (ATTRv amyloidosis) is predominantly a disease of the peripheral nerves, heart, kidney, and eye. CNS involvement has been a marginal issue in research and the clinical setting until recently. Growing evidence shows that leptomeningeal amyloid accumulation is frequent and present from early stages of ATTRv amyloidosis. Several recent studies show CNS symptoms arise as a common late complication in patients with the V30M mutation after at least 14 years of symptomatic peripheral nerve disease. Conversely, in non-V30M patients, there are several descriptions, mostly case reports, of patients presenting with severe phenotypes of ocular and CNS dysfunction (oculoleptomeningeal amyloidosis), with little systemic involvement. This phenotype is found in rare families worldwide, associated with at least 14 mutations. In both patients with late and early onset CNS dysfunction, symptoms include transient focal neurologic episodes, hemorrhagic and ischemic stroke, cognitive decline, and cranial nerve dysfunction. Pathologically, there is severe amyloid deposition in the leptomeninges and cerebral amyloid angiopathy of leptomeningeal and penetrating vessels. These amyloid aggregates are formed mostly by CSF-produced transthyretin (TTR) and seem resistant to the available ATTRv therapies that increase the stability or reduce the production of plasma TTR. This indicates that CNS involvement will become a meaningful issue in patient management in upcoming years.

خرید پکیج و مشاهده آنلاین مقاله

Comment: Introducing Neurologic Therapeutics in 2035

Lyell K. Jones, Robert A. Gross

doi : 10.1212/WNL.0000000000012977

vol. 97 no. 24 1120

خرید پکیج و مشاهده آنلاین مقاله

Neurologic Therapeutics in 2035

Nicholas E. Johnson, Ericka Greene

doi : 10.1212/WNL.0000000000012976

vol. 97 no. 24 1121-1127

Therapeutic development has accelerated rapidly in the past 5 years in many neurologic and neurodegenerative diseases. The therapeutic categories of development include small molecules, genetic therapies, and cell-based therapies. Current development has provided novel treatment approaches to disorders without available treatment. However, the regulatory procedures to allow for access to these therapies is challenging, as is the ability to provide wide access to increasingly expensive therapies. By 2035, these challenges are likely to have accelerated and have the potential to create bottlenecks in drug approval and reduced access to patients. Innovative regulatory and payer solutions are required. In addition, ethical considerations around genetic therapies should be considered in current and future development. These approaches will ensure that patients with neurologic disease have broad access to highly innovative therapies.

خرید پکیج و مشاهده آنلاین مقاله

From Writer to President

Orly Avitzur

doi : 10.1212/WNL.0000000000012978

vol. 97 no. 24 1128-1131

خرید پکیج و مشاهده آنلاین مقاله

Resident & Fellow Rounds: December 2021

Roy E. Strowd, Whitley Aamodt

doi : 10.1212/WNL.0000000000012980

vol. 97 no. 24 1132-1133

خرید پکیج و مشاهده آنلاین مقاله

Opinion & Special Article: Next Steps in Palliative Care Education for Neurology Residents

Tara Cook, Robert Arnold, Kwonho Jeong, Julie Childers

doi : 10.1212/WNL.0000000000012911

vol. 97 no. 24 1134-1137

Many neurologic diseases are life limiting and markedly impair patients' quality of life. Growing recommendations in the field recommend that neurologists have primary skills in palliative medicine that will allow them to manage symptoms and discuss end-of-life decisions with patients and families. Previous work has shown that formal palliative care training in neurology residencies is very limited. In this article, we briefly describe a national survey of neurology residents where we assess both the quantity and quality of the teaching they receive in end-of-life care as compared to a common and an uncommon neurologic condition. Based on the gaps we identified, as well as previous studies and recommendations in neuropalliative care, we provide 9 recommendations to help neurology residency programs improve their teaching of primary neuropalliative care skills.

خرید پکیج و مشاهده آنلاین مقاله

Clinical Reasoning: A Middle-aged Man With Progressive Gait Abnormalities

Junyu Lin, Yanbing Hou, Huifang Shang

doi : 10.1212/WNL.0000000000012756

vol. 97 no. 24 e2423-e2428

Progressive spastic paraplegia is the core symptom of hereditary spastic paraplegias (HSPs), a group of monogenic disorders characterized pathologically by degeneration of the corticospinal tract and dorsal column and leading to irreversible neurologic deficits. However, acquired causes, such as structural, vascular, inflammatory, infectious, metabolic, toxic, neurodegenerative, and iatrogenic causes, can also cause acquired spastic paraplegia. We describe the case of a middle-aged man presenting with progressive spastic paraplegia combined with ataxia and parkinsonism. No mutation of HSP genes was detected. After a comprehensive diagnostic workup, hyperintensities in the bilateral basal ganglia, mesencephalon, pons, and cerebellum on T1-weighted images were found, which demonstrated hypointensity on susceptibility-weighted imaging. Furthermore, an increased blood ammonia level and diffuse slow-wave activity in EEG were detected. The patient had a 7-year history of hypertension, alcoholic liver cirrhosis, and transjugular intrahepatic portosystemic shunt operation 2 years before the onset of spastic paraplegia symptoms. Current workup combined with patient history resulted in a diagnosis of acquired hepatocerebral degeneration and hepatic myelopathy.. This case provided a detailed diagnostic approach for progressive spastic paraplegias and exhaustive differential diagnoses of basal ganglia deposits. The take-home message from this case was that acquired causes, especially curable causes, should always be excluded first when dealing with patients with progressive spastic paraplegia.

خرید پکیج و مشاهده آنلاین مقاله

Teaching NeuroImage: A 41-Year-Old Woman Presented With Excessive Sweating of Contralateral Face and Extremity

Anjiao Peng, Yusha Tang, Tingting Song, Lei Chen

doi : 10.1212/WNL.0000000000012666

vol. 97 no. 24 e2429-e2430

خرید پکیج و مشاهده آنلاین مقاله

Teaching Video NeuroImage: Uncommon Neuro-ophthalmic Finding in a Patient With Miller Fisher Syndrome and Past SARS-CoV-2 Infection

Aldo F. Costa, Alba Rodríguez, Paula Martínez, Del Carmen M. Blanco

doi : 10.1212/WNL.0000000000012859

vol. 97 no. 24 e2431-e2432

خرید پکیج و مشاهده آنلاین مقاله

Editors' Note: Automated Quantitative Pupillometry in the Critically Ill: A Systematic Review of the Literature

Ariane Lewis, Steven Galetta

doi : 10.1212/WNL.0000000000012979

vol. 97 no. 24 1138

خرید پکیج و مشاهده آنلاین مقاله

Reader Response: Automated Quantitative Pupillometry in the Critically Ill: A Systematic Review of the Literature

Fabio S. Taccone, Giuseppe Citerio

doi : 10.1212/WNL.0000000000012981

vol. 97 no. 24 1138-1139

خرید پکیج و مشاهده آنلاین مقاله

Author Response: Automated Quantitative Pupillometry in the Critically Ill: A Systematic Review of the Literature

Petra Opic, Raoul Sutter

doi : 10.1212/WNL.0000000000012983

vol. 97 no. 24 1139

خرید پکیج و مشاهده آنلاین مقاله

Reader Response: Automated Quantitative Pupillometry in the Critically Ill: A Systematic Review of the Literature

Merlin D. Larson

doi : 10.1212/WNL.0000000000012982

vol. 97 no. 24 1140

خرید پکیج و مشاهده آنلاین مقاله

Author Response: Automated Quantitative Pupillometry in the Critically Ill: A Systematic Review of the Literature

Petra Opic, Raoul Sutter

doi : 10.1212/WNL.0000000000012984

vol. 97 no. 24 1140-1141

خرید پکیج و مشاهده آنلاین مقاله

Geriatric Syndromes and Treatment Toxicities in Older Patients With Malignant Gliomas (4327)

doi : 10.1212/WNL.0000000000013032

vol. 97 no. 24 1141

خرید پکیج و مشاهده آنلاین مقاله

Patient-Reported Symptom Severity in a Nationwide Myasthenia Gravis Cohort

doi : 10.1212/WNL.0000000000013021

خرید پکیج و مشاهده آنلاین مقاله