1. Pediatric autoimmune encephalitis: Recognition and diagnosis
Marienke A A M de Bruijn, et al. Neurol Neuroimmunol Neuroinflamm. 2020 Feb 11;7(3):e682. doi: 10.1212/NXI.0000000000000682. Print 2020 May.
Objective: The aims of this study were (1) to describe the incidence of autoimmune encephalitis (AIE) and acute disseminated encephalomyelitis (ADEM) in children, (2) to validate the currently used clinical criteria to diagnose AIE, and (3) to describe pitfalls in the diagnosis of pediatric autoimmune (AI) and inflammatory neurologic disorders. Methods: This study cohort consists of 3 patient categories: (1) children with antibody-mediated AIE (n = 21), (2) children with ADEM (n = 32), and (3) children with suspicion of an AI etiology of their neurologic symptoms (n = 60). Baseline and follow-up clinical data were used to validate the current guideline to diagnose AIE. In addition, patient files and final diagnoses were reviewed. Results: One-hundred three of the 113 included patients fulfilled the criteria of possible AIE. Twenty-one children had antibody-mediated AIE, of whom 19 had anti-N-methyl-D-aspartate receptor (NMDAR), 1 had anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, and 1 had anti-leucine-rich glioma-inactivated protein 1 encephalitis. Finally, 34 children had ADEM, and 2 children had Hashimoto encephalopathy. Mean incidence rates were 1.54 children/million (95% CI 0.95-2.35) for antibody-mediated AIE and 2.49 children/million (95% CI 1.73-3.48) for ADEM. Of the other 48 children, treating physicians' diagnoses were reviewed. In 22% (n = 6) of children initially diagnosed as having an AI/inflammatory etiology (n = 27), no support for AI/inflammation was found. Conclusion: Besides anti-NMDAR encephalitis and ADEM, other AIEs are rare in children. The current guideline to diagnose AIE is also useful in children. However, in children with nonspecific symptoms, it is important to review data critically, to perform complete workup, and to consult specialized neuroinflammatory centers.
2. N-Methyl-l-leucyl-l-leucine hydro-chloride monohydrate
Tao Lu, Mu-Wu Xu, Xiao-Jian Liao, Shi-Hai Xu Acta Crystallogr Sect E Struct Rep Online. 2011 Sep 1;67(Pt 9):o2394. doi: 10.1107/S1600536811031126. Epub 2011 Aug 27.
In the title compound C(13)H(27)N(2)O(3) (+)·Cl(-)·H(2)O, obtained by deprotecting the amino and carboxyl groups of an inter-mediate in the synthesis of the cyclic penta-peptide Galaxamide, a number of hydrogen-bonding inter-actions occur including aminium N-H⋯Cl, amide-carboxyl N-H⋯O, water O-H⋯Cl and carbox-yl-water O-H⋯O associations. The aminium N-H⋯Cl⋯H-N bridging extensions give rise to zigzag chains extending along the a axis, the overall two-dimensional structure lying in the (110) plane.
3. N-{N-[N-(1,1-Dimethyl-ethoxy-carbon-yl)-l-leuc-yl]-N-methyl-l-leuc-yl}-N-methyl-l-leucine benzyl ester
Wen Jie Xu, Xiao Jian Liao, Jian Zhong Diao, Lei Zhou, Shi Hai Xu Acta Crystallogr Sect E Struct Rep Online. 2008 Oct 25;64(Pt 11):o2178. doi: 10.1107/S1600536808034247.
The tripeptide title compound, C(32)H(53)N(3)O(6), synthesized in 80% yield by coupling of N-methyl-l-leucine benzyl ester with tert-butoxy-carbonyl-l-leucyl-N-methyl-l-leucine at 273 K, conjugates through two amide linkages and includes two protecting groups: a tert-butyl-oxycarbonyl group at the C-tip and a benzyl group at the N-tip. A classical inter-molecular N-H⋯O hydrogen bond and a weak non-conventional inter-molecular C-H⋯O contact connect the mol-ecules, forming layers parallel to (001).
