Neurology




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Spotlight on the June 1 Issue

José G. Merino

doi : 10.1212/WNL.0000000000012020

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Cognitive Heterogeneity in Alzheimer Clinical Trials

Rachel F. Buckley, David S. Knopman

doi : 10.1212/WNL.0000000000012027

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Modifiable Risk Factors in Critically Ill Children

Rod C. Scott,  View ORCID ProfileCourtney J. Wusthoff

doi : 10.1212/WNL.0000000000012026

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Quelling Public Fears About Guillain-Barré Syndrome and COVID-19 Vaccination

Dennis Bourdette, Joep Killestein

doi : 10.1212/WNL.0000000000011882

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Finding Treatment Effects in Alzheimer Trials in the Face of Disease Progression Heterogeneity

Roos J. Jutten, Sietske A.M. Sikkes,  View ORCID ProfileWiesje M. Van der Flier,  View ORCID ProfilePhilip Scheltens,  View ORCID ProfilePieter Jelle Visser,  View ORCID ProfileBetty M. Tijms, for the Alzheimer's Disease Neuroimaging Initiative

doi : 10.1212/WNL.0000000000012022

Objective To investigate the influence of heterogeneity in disease progression for detecting treatment effects in Alzheimer disease (AD) trials, using a simulation study.

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Progression to Dementia in Mild Cognitive Impairment With Lewy Bodies or Alzheimer Disease

Calum A. Hamilton,  View ORCID ProfileFiona E. Matthews,  View ORCID ProfilePaul C. Donaghy,  View ORCID ProfileJohn-Paul Taylor,  View ORCID ProfileJohn T. O'Brien,  View ORCID ProfileNicola Barnett, Kirsty Olsen, Rory Durcan,  View ORCID ProfileGemma Roberts,  View ORCID ProfileJoanna Ciafone, Sally A.H. Barker,  View ORCID ProfileMichael Firbank,  View ORCID ProfileIan G. McKeith,  View ORCID ProfileAlan J. Thomas

doi : 10.1212/WNL.0000000000012024

Objective To determine whether mild cognitive impairment with Lewy bodies or mild cognitive impairment with Alzheimer disease differ in their rates of clinical progression to dementia, we undertook longitudinal observation of mild cognitive impairment cases with detailed clinical assessment of Lewy body diagnostic characteristics.

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Association of Hemoglobin A1C With TDP-43 Pathology in Community-Based Elders

Shahram Oveisgharan, Ana W. Capuano, Sukriti Nag, Sonal Agrawal, Lisa L. Barnes, David A. Bennett, Zoe Arvanitakis, Julie A. Schneider

doi : 10.1212/WNL.0000000000012025

Objective To test the hypothesis that an inverse association exists between diabetes mellitus (DM) and hemoglobin A1C (A1C) with transactive response DNA binding protein 43 (TDP-43) levels in older adults.

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Association of Infarct Volume Before Hemicraniectomy and Outcome After Malignant Infarction

Dominik Lehrieder, Katharina Layer, Hans-Peter Müller, Viktoria Rücker, Jan Kassubek, Eric Juettler, Hermann Neugebauer, on behalf of the DESTINY-R (Decompressive Surgery for the Treatment of Malignant Infarction of the Middle Cerebral Artery Registry) Study Group and IGNITE (Initiative of German Neurointensive Trial Engagement) Study Group

doi : 10.1212/WNL.0000000000011987

Objective To determine the impact of infarct volume before hemicraniectomy in malignant middle cerebral artery infarction (MMI) as an independent predictor for patient selection and outcome prediction, we retrospectively analyzed data of 140 patients from a prospective multicenter study.

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Brain MRI and Ophthalmic Biomarkers of Intracranial Pressure

 View ORCID ProfileLinda D'Antona, Hasan Asif, Claudia Louise Craven, James Alexander McHugh, Anna Vassiliou,  View ORCID ProfileLewis Thorne, Manjit Singh Matharu, Laurence Dale Watkins, Fion Bremner, Ahmed Kassem Toma

doi : 10.1212/WNL.0000000000012023

Objective To evaluate the utility of brain MRI and ophthalmic biomarkers for the prediction of intracranial hypertension, we have studied the association between 6 biomarkers and 24-hour intracranial pressure (ICP) monitoring results in 45 patients.

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Long-term Efficacy and Safety of Erenumab

Peter J. Goadsby, Uwe Reuter, Michel Lanteri-Minet, Gabriel Paiva da Silva Lima, Peggy Hours-Zesiger, Chrystel Fernandes, Shihua Wen, Nadia Tenenbaum, Aditi Kataria, Michel D. Ferrari, Jan Klatt

doi : 10.1212/WNL.0000000000012029

Objective To report the efficacy and safety of erenumab among patients with episodic migraine (EM) who were unsuccessful on 2 to 4 preventive treatments observed at week 64 of the open-label extension phase (OLEP) of A Study Evaluating the Effectiveness of AMG 334 Injection in Preventing Migraines in Adults Having Failed Other Therapies (LIBERTY) study (ClinicalTrials.gov NCT03096834).

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Trajectory-Based Classification of Recovery in Sensorimotor Complete Traumatic Cervical Spinal Cord Injury

Blessing N.R. Jaja, Jetan Badhiwala, James Guest, James Harrop, Chris Shaffrey, Max Boakye, Shekar Kurpad, Robert Grossman, Elizabeth Toups, Fred Geisler, Brian Kwon, Bizhan Aarabi,  View ORCID ProfileMark Kotter, Michael G. Fehlings, Jefferson R. Wilson

doi : 10.1212/WNL.0000000000012028

Objective To test the hypothesis that sensorimotor complete traumatic cervical spinal cord injury (SCI) is a heterogenous clinical entity comprising several subpopulations that follow fundamentally different trajectories of neurologic recovery.

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Electrographic Seizures and Outcome in Critically Ill Children

France W. Fung, Zi Wang, Darshana S. Parikh, Marin Jacobwitz, Lisa Vala, Maureen Donnelly, Alexis A. Topjian, Rui Xiao, Nicholas S. Abend

doi : 10.1212/WNL.0000000000012032

Objective To determine the association between electroencephalographic seizure (ES) and electroencephalographic status epilepticus (ESE) exposure and unfavorable neurobehavioral outcomes in critically ill children with acute encephalopathy.

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[11C]PK11195-PET Brain Imaging of the Mitochondrial Translocator Protein in Mitochondrial Disease

 View ORCID ProfileJelle van den Ameele, Young T. Hong,  View ORCID ProfileRoido Manavaki,  View ORCID ProfileAntonina Kouli, Heather Biggs, Zoe MacIntyre, Rita Horvath,  View ORCID ProfilePatrick Yu-Wai-Man, Evan Reid,  View ORCID ProfileCaroline H. Williams-Gray,  View ORCID ProfileEd T. Bullmore,  View ORCID ProfileFranklin I. Aigbirhio, Tim D. Fryer, Patrick F. Chinnery

doi : 10.1212/WNL.0000000000012033

Objective To explore the possibilities of radioligands against the mitochondrial outer membrane translocator protein (TSPO) as biomarkers for mitochondrial disease, we performed brain PET-MRI with [11C]PK11195 in 14 patients with genetically confirmed mitochondrial disease and 33 matched controls.

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Futures Planning at the AAN

Carlayne E. Jackson, Lyell K. Jones, Brad C. Klein, Natalia R. Rost, Sarah M. Benish, Bruce T. Levi, Robert A. Gross, on behalf of the 2019–2021 AAN and AANI Boards of Directors and the AAN Executive Team

doi : 10.1212/WNL.0000000000012031

We describe a process of organizational strategic future forecasting, with a horizon of 2035, as implemented by the American Academy of Neurology (AAN) on behalf of its members, and as a model approach for other organizations. The participants were members of the 2018–2020 AAN Boards of Directors and Executive Team, moderated by a consultant with expertise in future forecasting. Four predetermined model scenarios of import to our field (1 “expectable,” 1 “challenging,” and 2 “visionary”) were discussed in small groups, with alternative scenarios developed in specific domains. Common themes emerged among all scenarios: the importance of thoughtful integration of biomedical and information technology tools into neurologic practice; continued demonstration of the value of neurologic care to society; and emphasis on population management and prevention of neurologic disease. Allowing for the inherent uncertainties of predicting the future, the AAN's integration of structured forecasting into its strategic planning process has allowed the organization to prepare more effectively for change, such as the disruptions stemming from the coronavirus disease 2019 (COVID-19) pandemic. The approaches outlined here will be integrated into future AAN operations and may be implemented to a similar effect by other organizations.

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Vagus Nerve Stimulation and Seizure Outcomes in Pediatric Refractory Epilepsy

 View ORCID ProfilePuneet Jain,  View ORCID ProfileRavindra Arya

doi : 10.1212/WNL.0000000000012030

Objective We synthesized evidence for effectiveness of vagus nerve stimulation (VNS) as adjuvant therapy in pediatric drug-resistant epilepsy (DRE) by obtaining pooled estimates for seizure outcomes and analyzing their determinants.

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Guillain-Barré Syndrome in the Placebo and Active Arms of a COVID-19 Vaccine Clinical Trial

Alejandra M. M?rquez Loza, Kathryn B. Holroyd, Stephen A. Johnson, David M. Pilgrim, Anthony A. Amato

doi : 10.1212/WNL.0000000000011881

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Pearls & Oy-sters: Rosai-Dorfman Disease of the CNS

Kia Gilani, Stephanie Kuntz, David G. Munoz,  View ORCID ProfileRaphael Schneider

doi : 10.1212/WNL.0000000000012035

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Clinical Reasoning: A 7-Year-Old Boy With Acute-Onset Altered Mental Status

Gregory J. Wong, Cristina M. Gaudioso, Eleanor Castro,  View ORCID ProfileNima Sharifai,  View ORCID ProfileSonika Dahiya, Louis P. Dehner, Jennifer L. Griffith

doi : 10.1212/WNL.0000000000012034

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Teaching NeuroImages: Transependymal Oozing of Intrathecal Contrast Mimicking Intracerebral Hemorrhage

Bryan J. Neth, Rafid Mustafa, Sherri A. Braksick,  View ORCID ProfileEelco F.M. Wijdicks

doi : 10.1212/WNL.0000000000011546

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Teaching Video NeuroImages: Wilson Disease Presenting With Catatonia

Roshni Abee Patel, Meagan Bailey

doi : 10.1212/WNL.0000000000012038

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Editors' Note: Novel ALDH5A1 Variants and Genotype: Phenotype Correlation in SSADH Deficiency

Ariane Lewis, Steven Galetta

doi : 10.1212/WNL.0000000000012045

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Reader Response: Novel ALDH5A1 Variants and Genotype: Phenotype Correlation in SSADH Deficiency

Antonio Siniscalchi

doi : 10.1212/WNL.0000000000012039

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Author Response: Novel ALDH5A1 Variants and Genotype: Phenotype Correlation in SSADH Deficiency

Phillip L. Pearl, K. Michael Gibson, Jean-Baptiste Roullet, Melissa DiBacco

doi : 10.1212/WNL.0000000000012040

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Editors' Note: Nusinersen in Adult Patients With Spinal Muscular Atrophy: Observations From a Single Center

Ariane Lewis, Steven Galetta

doi : 10.1212/WNL.0000000000012046

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Reader Response: Nusinersen in Adult Patients With Spinal Muscular Atrophy: Observations From a Single Center

Tim Hagenacker, Maggie Christine Walter, Valeria Sansone, Eugenio Mercuri

doi : 10.1212/WNL.0000000000012042

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Author Response: Nusinersen in Adult Patients With Spinal Muscular Atrophy: Observations From a Single Center

Chafic Karam,  View ORCID ProfileOrly Moshe-Lilie, Thomas Ragole, Nizar Chahin, Diana Dimitrova

doi : 10.1212/WNL.0000000000012043

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