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In This Issue of JAMA Neurology
September 2016


JAMA Neurol. 2016;73(9):1043. doi:10.1001/jamaneurol.2015.2479


Wang and coauthors evaluate the usefulness of [18F]-AV-1451 positron emission tomography imaging to stage Alzheimer disease (AD) and assess the associations among Aβ, tau, and volume loss. An imaging study was conducted, and a total of 59 participants who were cognitively normal or had AD dementia were included. They report that the use of [18F]-AV-1451 has a potential for staging of the preclinical and clinical phases of AD. Aβ interacts with hippocampal and cortical tauopathy to affect neurodegeneration. In the absence of Aβ, hippocampal tau deposition may be insufficient for the neurodegenerative process that leads to AD. Editorial perspective is provided by William Jagust, MD.



Ranasinghe et al identify subtypes of behavioral variant frontotemporal dementia (bvFTD) syndrome based on distinctive patterns of atrophy defined by selective vulnerability of specific functional networks targeted in bvFTD using statistical classification approaches. In their retrospective observational study, 90 patients meeting the Frontotemporal Dementia Consortium consensus criteria for bvFTD underwent evaluation. They conclude that divergent patterns of vulnerability in specific functional network components make an important contribution to the clinical heterogeneity of bvFTD. Editorial perspective is provided by David S. Knopman, MD.


Joubert and colleagues characterize the clinical and biological presentations of patients with anti–contactin-associated protein-like 2 (CASPR2) antibodies in the cerebrospinal fluid (CSF). They conducted a retrospective cohort analysis of 18 patients who had anti-CASPR2 antibodies in their CSF between March 2009 and November 2015 and conclude that anti-CASPR2 antibodies in the CSF are associated with a subtype of autoimmune encephalitis with prominent limbic involvement and seizures that is rarely associated with cancer. Editorial perspective is provided by Christian G. Bien, MD.


Groeschel and colleagues compare the long-term outcome of patients who underwent allogeneic hematopoietic stem cell transplantation (HSCT) with control patients who did not among a cohort with juvenile metachromatic leukodystrophy (MLD). Patients with juvenile MLD born between 1975 and 2009 and who received HSCT at a median age of 7 years (age range, 1.5-18.2 years) and nontransplanted patients with juvenile MLD born between 1967 and 2007 were included in this case-control study. The authors found that among patients with juvenile MLD, patients who underwent HSCT had a better gross motor and language outcome and lower magnetic resonance imaging severity scores compared with nontransplanted patients.