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In This Issue of JAMA Neurology
July 2015


JAMA Neurol. 2015;72(7):735. doi:10.1001/jamaneurol.2014.2856


Ringelstein and colleagues evaluate the long-term safety and efficacy of tocilizumab, a humanized antibody targeting the IL-6 receptor, in neuromyelitis optica (NMO) and NMO spectrum disorder using the annualized relapse rate, Expanded Disability Status Scale score, spinal cord and brain magnetic resonance imaging, aquaporin 4 antibody titers, pain levels (numerical rating scale), and adverse effects. Patients were followed up for a mean (SD) of 30.9 (15.9) months after switching to tocilizumab. The authors find that prolonged tocilizumab therapy may be safe and effective from early treatment phases onward for otherwise therapy-resistant highly active NMO and NMO spectrum disorder. Editorial perspective is provided by Sarosh R. Irani, MD, DPhil, and Angela Vincent, FRCPath.


Strain and coauthors assess the relationship of hippocampal volume, memory performance, and the influence of concussion history in retired National Football League (NFL) athletes with and without mild cognitive impairment (MCI). Hippocampal volume, age, California Verbal Learning Test scores, the number of grade 3 concussions, and the number of games played were examined as an objective variable pertaining to football history. They show that prior concussion that results in loss of consciousness is a risk factor for increased hippocampal atrophy and the development of MCI.

Continuing Medical Education

Ozaki et al identify the causative gene of spinocerebellar ataxia (SCA) in 2 Japanese families with distinct neurological symptoms and radiological presentations. A clinical genetic study at a referral center of 11 members from 2 Japanese families was conducted. Combined with the results of the family with SCA34 reported previously, this report confirms that mutations in ELOVL4 can cause dominantly inherited neurodegeneration severely affecting the cerebellum and brainstem.

Clinical Review & Education

Kremer and colleagues summarize the literature on advanced quantitative imaging measures reported for patients with neuromyelitis optica (NMO) spectrum disorder, including proton magnetic resonance (MR) spectroscopy, diffusion tensor imaging, magnetization transfer imaging, quantitative MR volumetry, and ultrahigh-field strength magnetic resonance imaging (MRI). They undertook an exhaustive literature review of the advanced MRI techniques used for patients with NMO by different specialists in the field. The use of nonconventional MRI techniques may further our understanding of the pathogenic processes in NMO spectrum disorders and may help us identify the distinct radiographic features corresponding to specific phenotypic manifestations of this disease.