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In This Issue of JAMA Neurology |

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JAMA Neurol. 2015;72(8):847. doi:10.1001/jamaneurol.2014.2861.
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Ariño and colleagues report the clinical and immunological features of patients with paraneoplastic neurological syndromes and glutamic acid decarboxylase antibodies (GAD-abs). They find that 8 of the 15 patients with cancer presented with classic paraneoplastic syndromes (5 limbic encephalitis, 1 paraneoplastic encephalomyelitis, 1 paraneoplastic cerebellar degeneration, and 1 opsoclonus-myoclonus syndrome). Patients with GAD-abs must be screened for an underlying cancer if they have clinical presentations different from those typically associated with this autoimmunity or develop classic paraneoplastic neurological syndromes.

Gershen and colleagues determine whether patients with temporal lobe epilepsy (TLE) have widespread translocator protein 18 kDa (TSPO) overexpression using carbon 11 ([11C])–labeled PBR28 positron emission tomographic (PET) imaging and replicate relative ipsilateral TSPO increases in patients with TLE using [11C]PBR28 and another TSPO radioligand, [11C]DPA-713. They find that the [11C]PBR28 distribution volume to free fraction ratio was higher in patients with TLE than in controls for all ipsilateral temporal regions (27%-42%; P < .05) and in the contralateral hippocampus, amygdala, and temporal pole (approximately 30%-32%; P < .05). In conclusion, binding of TSPO is increased both ipsilateral and contralateral to seizure foci in patients with TLE, suggesting ongoing inflammation.

Selcen and coauthors characterize clinical, structural, electrophysiologic, and genetic features of a congenital myasthenic syndrome (CMS) and search for optimal therapy. They find that most end plates had poorly differentiated or degenerate junctional folds, and some appeared denuded of nerve terminals. They identified a second CMS kinship harboring mutations in LRP4, identified the mechanisms that impair neuromuscular transmission, and mitigated the disease by appropriate therapy.

Quiroz and colleagues characterize and compare structural magnetic resonance imaging (MRI), resting-state and task-dependent functional MRI, and plasma amyloid-β (Aβ) measurements in presenilin 1 (PSEN1) E280A mutation–carrying and noncarrying children with autosomal dominant Alzheimer disease (ADAD). They report that similar to findings in adult mutation carriers, in the later preclinical and clinical stages of ADAD, mutation-carrying children were distinguished from control individuals by significantly higher plasma Aβ1-42 levels (mean [SD]: carriers, 18.8 [5.1] pg/mL and noncarriers, 13.1 [3.2] pg/mL; P < .001) and Aβ1-42:Aβ1-40 ratios (mean [SD]: carriers, 0.32 [0.06] and noncarriers, 0.21 [0.03]; P < .001), as well as less memory encoding task–related deactivation in parietal regions (eg, mean [SD] parameter estimates for the right precuneus were −0.590 [0.50] for noncarriers and −0.087 [0.38] for carriers; P < .005 uncorrected). They conclude that children at genetic risk for ADAD have functional and structural brain changes and abnormal levels of plasma Aβ1-42, and that the extent to which the underlying brain changes are either neurodegenerative or developmental remains to be determined; furthermore, this study provides additional information about the earliest known biomarker changes associated with ADAD.





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