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In This Issue of JAMA Neurology
November 2019


JAMA Neurol. 2019;76(11):1271. doi:10.1001/jamaneurol.2018.3016


Pathways to dementia in chronic traumatic encephalopathy (CTE) are unclear and likely multifaceted. In a cross-sectional study of 180 men who played football and had a diagnosis of CTE, Alosco and coauthors investigated the contribution of white matter (WM) rarefaction and cerebrovascular disease to dementia. More years of football play, a proxy for repetitive head impacts, corresponded to more severe WM rarefaction and frontal cortex neurofibrillary tangles; these pathologies were associated with dementia. Arteriolosclerosis was unrelated to years of play but contributed to dementia. In CTE, dementia is likely due to neuropathologic changes from repetitive head impacts, including WM rarefaction and phosphorylated tau, in addition to nonhead trauma–associated pathologic changes, like arteriolosclerosis. Editorial perspective is provided by Schneider.


The optimal blood pressure (BP) target of less than 120 mm Hg is superior for preventing vascular events in those without prior stroke, but an optimal BP target for secondary stroke prevention is unclear. In a randomized clinical trial that included 1263 patients with a history of stroke, Kitagawa and coauthors found that intensive BP control to less than 120/80 mm Hg tended to reduce stroke recurrence compared with standard blood pressure control (<140/90 mm Hg). When this finding was pooled with prior trials of intensive BP control for secondary stroke prevention, intensive BP treatment significantly reduced stroke recurrence by 22%. Intensive BP control should be recommended for secondary stroke prevention. Editorial perspective is provided by Anderson.


Continuing Medical Education

In Huntington disease, age at onset is associated with the length of the CAG trinucleotide sequence, but less is known about the association of repeat length with disease trajectory, especially early in the course of the disorder. In a prospective study, Langbehn and coauthors analyzed data from 2065 visits of 443 participants, including 290 gene carriers and 153 controls, and developed composite summary scores by combining motor-cognitive scores and brain volume measures. They found that initial progression age and acceleration rate were each dependent on CAG repeat length, predicting the rate of onset and decline in total functional capacity.

Over the last 10 years, multiple effective disease-modifying therapies have been approved for the treatment of multiple sclerosis (MS), but it is not known what more treatment options means for price, patient spending, and market share. In a cohort study using claims from Medicare Part D beneficiaries, San-Juan-Rodriguez and coauthors evaluated the association of rising prices with pharmaceutical spending on MS therapies and on the contribution of different stakeholders toward this spending. From 2006 to 2016, the out-of-pocket cost of self-administered MS drugs increased at rates substantially higher than specialty medications. From 2006 to 2016, annual costs of treatment with MS therapies increased in parallel by more than 4-fold, while Medicare Part D pharmaceutical spending increased by more than 10-fold and beneficiaries’ out-of-pocket spending increased by more than 7-fold. Editorial perspective is provided by Hartung and Bourdette.