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May 26, 2020; 94 (21) In Focus

Spotlight on the May 26 issue

José G. Merino
First published May 25, 2020, DOI: https://doi.org/10.1212/WNL.0000000000009503
José G. Merino
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Spotlight on the May 26 issue
José G. Merino
Neurology May 2020, 94 (21) 901-902; DOI: 10.1212/WNL.0000000000009503

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Notable in Neurology this week

This issue features an article that shows patients with cluster headaches have substantially more sickness absence and disability pension days compared to matched references; another investigates the association of aerobic exercise with improvements in cognition and cerebrovascular regulation. A featured Special Editorial discusses the effect of the global COVID-19 pandemic on the dissemination and implementation of telehealth services.

Articles

Liver transplantation as a rescue therapy for severe neurologic forms of Wilson disease

Liver transplantation (LT) for the management of patients with Wilson disease (WD) with severe neurologic worsening, but without liver failure, is controversial. Eighteen patients who underwent LT for pure neurologic indication were retrospectively studied. Most patients who were alive at last follow-up had moderate to major improvement in symptoms and less function impairment. LT may be a therapeutic option for patients with severe symptoms who are resistant to anticopper therapies.

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Page 911

From editorialists Bandmann, Weiss, and Hedera: “The study by Poujois and coworkers also provides circumstantial evidence that restoration of copper homeostasis may be superior to chelation for removal of excessive copper from the CNS. Thus, other, hopefully less risky approaches such as gene therapy for WD may 1 day result in marked improvement of neurologic WD.”

Page 907

The implications of different approaches to define AT(N) in Alzheimer disease

CSF and imaging Alzheimer disease change biomarkers for amyloid (A), tau (T), and neurodegeneration (N) can be combined in a classification system [AT(N)] to classify individuals as cognitively unimpaired and impaired and to predict cognitive decline. Different biomarker combinations are not interchangeable, however, and lead to differences in classification and prediction that change as the disease progresses.

Page 915

Intracranial hemodynamic relationships in patients with cerebral small vessel disease

In an MRI-based study, changes in hemodynamic measures (decreased cerebrovascular reactivity, higher venous pulsatility, and lower foramen magnum CSF stroke volume vascular dynamic dysfunction) were associated with enlarged perivascular spaces and white matter hyperintensities in patients with small vessel disease (SVD). These finding suggest that the study of microvascular dysfunction and CSF dynamics may help identify potential interventions for preventing clinical and cognitive decline in patients with SVD.

Page 917

From editorialists Shaaban and Molad: “Developing effective interventions for SVD requires identifying targets for intervention and measurable biomarkers of the disease. Blair and colleagues have added important additional candidates for both with these novel and important results.”

Page 909

Views & Reviews

Imaging of the vulnerable carotid plaque: Role of imaging techniques and a research agenda

Carotid atherothrombosis is a main cause of stroke and may depend on plaque vulnerability. Several imaging modalities, including ultrasound, MRI, CT, and nuclear medicine, can help identify vulnerable carotid plaque. Yet, few studies have evaluated the diagnostic yield of each modality and their relationship with clinical stroke outcomes.

Page 922

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  • NB: “Abnormal cerebellar foliation in EBF3 mutation,” p. 933. To check out other NeuroImages articles, point your browser to Neurology.org/N. At the end of the issue, check out the Resident & Fellow Section Teaching NeuroImages article illustrating an association of bilateral atrophic maculopathy with spinocerebellar ataxia type 3. This week also includes a Resident & Fellow Mystery Case titled “An infant with developmental delay, epileptic spasms, and acrocyanosis.”

  • COVID-19 Resources: NPub.org/COVID19

  • © 2020 American Academy of Neurology

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