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A 59-year-old woman presented with subacute onset of hand clumsiness followed by memory loss. She reported difficulty reaching for objects, although she could see them. Examination (video) showed optic ataxia, visual acuity of 20/30 OU, intact visual field, and near-normal finger-to-nose test. Hyperintense signals in the left temporo-parieto-occipital cortex in diffusion-weighted MRI, periodic triphasic complexes in EEG (figure), and elevated t-tau in CSF suggested the Heidenhain variant of Creutzfeldt-Jakob disease.1 The patient received supportive care and progressed to akinetic mutism. Optic ataxia—inaccuracy of visually guided arm movements—is a visuomotor disorder associated with lesions involving the posterior parietal cortex.2

Aicardi-Goutières syndrome (AGS) is a rare, single-gene disorder, characterized by neurologic and skin involvement with an increased level of interferon-α (IFN-α) in the CSF. We describe the case of a young patient presenting with recurrent ischemic stroke. Evaluation revealed the presence of chilblains, white matter abnormalities, cerebral atrophy, and raised IFN-α in the CSF. Compound heterozygous variants of TREX1 were detected, confirming a diagnosis of AGS. After excluding other causes, we attributed the stroke to AGS. Tofacitinib, a Janus kinase inhibitor, was administered to our patient in addition to antiplatelet drugs. There was no recurrence of stroke during 3-month follow-up. This is a rare case of recurrent stroke in TREX1-mutated AGS. Small vessel involvement has been previously demonstrated to play a significant role in the pathogenesis of AGS. This microvascular mechanism might explain the occurrence of ischemic stroke in our patient. For young patients with stroke and multiple system involvement, genetic disorders including AGS should be considered.

A 22-year-old man complained of progressive bilateral blurred vision that had developed 6 years ago. He also noted dispersed scotomas and graying of vison in both eyes, without photopsia, orbital pain, or double vision. He had not seen an ophthalmologist until he could hardly recognize people by appearance 1 week previously. Bilateral optic atrophy (figure, A) was recorded with best-corrected visual acuities of counting fingers OD and 20/400 OS. Perimetry revealed global visual field loss OD and tunnel vision OS (figure, B). He was referred to our neurology clinic, where he reported recurrent mild dull headache from age 10.

Objective: To describe the characteristics of post-stroke Chinese pure alexia and associated neuropsychological profiles. Methods: All patients with post-stroke pure alexia from the neurology department of our hospital were enrolled from June 2018 to July 2019. Brain MRI was performed to evaluate the stroke radiologically. The Aphasia Battery of Chinese (ABC) was used to evaluate the general language functions. A Chinese individual character reading test including 68 commonly used characters was used to evaluate the ability to read individual Chinese characters. The numbers of correct reading and misreading were recorded, and the error types of misreading were analyzed. MMSE, auditory verbal learning test, cube copying task, clock drawing test, and picture copying tests were performed to evaluate other cognitive impairments. Results: A total of 5 male patients were included, with a median age of 58 years (50-64 years). They all had Chinese pure alexia with intact verbal comprehension and speech and relatively preserved writing skills. The etiologies were all acute infarction involving the left temporo-parietal lobe and splenium of corpus callosum. In the Chinese individual character reading test, the median number of correct reading was 21 (18-44) out of 68 characters, with many shape-similar errors (one character was read as another one with a similar shape but different meaning and pronunciation). The percentage of shape-similar errors among all type errors was 81.5%, 60.0%, 23.7%, 18.5%, and 57.9% respectively, and 43.1% overall, much higher than other type errors. All the patients had obvious impairment in memory and visuospatial function with an exception that one patient had relatively preserved visuospatial ability. Conclusion: Shape-similar error is a remarkable feature of Chinese pure alexia. Chinese pure alexia is associated with impaired visuospatial function, but visuospatial dysfunction is not necessary for the development of Chinese pure alexia.

Restenosis caused by neointimal hyperplasia significantly decreases long-term efficacy of percutaneous transluminal angioplasty (PTA), stenting, and by-pass surgery for managing coronary and peripheral arterial diseases. A major cause of pathological neointima formation is abnormal vascular smooth muscle cell (VSMC) proliferation and migration. Notoginsenoside R1 (NGR1) is a novel saponin that is derived from Panax notoginseng and has reported cardioprotective, neuroprotective and anti-inflammatory effects. However, its role in modulating VSMC neointima formation remains unexplored. Herein, we report that NGR1 inhibits serum-induced VSMC proliferation and migration by regulating VSMC actin cytoskeleton dynamics. Using a mouse femoral artery endothelium denudation model, we further demonstrate that systemic administration of NGR1 had a potent therapeutic effect in mice, significantly reducing neointimal hyperplasia following acute vessel injury. Mechanistically, we show that NGR1’s mode of action is through inhibiting the activation of phosphatidylinositol 3-kinase (PI3K)/Akt signaling. Taken together, this study identified NGR1 as a potential therapeutic agent for combating restenosis after PTA in cardiovascular diseases.

Wall-eyed monocular internuclear ophthalmoplegia (WEMINO) is a rare variant of internuclear ophthalmoplegia (INO), consisting of unilateral INO and ipsilateral exotropia. This distinctive syndrome is probably associated with damage to the medial longitudinal fasciculus. However, WEMINO caused by a midbrain lesion has not been previously reported. We herein report a 50-year-old man presenting with WEMINO and vertical gaze dysfunction resulting from infarction of the midbrain tegmentum.

A novel palladium-catalyzed oxidative three-component coupling of easily accessible N-substituted anthranilamides with isocyanides and arylboronic acids is achieved. This protocol offers an alternative approach toward 2,3-disubstituted quinazolinones with a wide substrate scope and good functional group tolerance.

A 64-year-old literate Chinese man presented with sudden onset of difficulty reading. Examination revealed right homonymous hemianopsia and pure alexia. The oral reading test showed that he often read one character as another one with a similar shape but different meaning and pronunciation (figure 1). Brain MRI showed acute infarction involving the left occipital lobe, medial temporal lobe, and splenium of corpus callosum (figure 2). Pure alexia is a clinical disconnection syndrome that was first described in a patient with a similar infarction by Dejerine in 1892.1 Shape-similar errors may be a phenomenon characteristic of Chinese pure alexia.

A 27-year-old man presented with fever, somnolence, seizure, and nuchal rigidity. CSF culture indicated Streptococcus pneumoniae , confirming the diagnosis of bacterial meningitis. Ping-pong gaze was observed initially, and then replaced by what we called ocular nodding after IV administration of diazepam (video at [Neurology.org][1]). The eyes went downward and returned to primary position slowly, smoothly, and periodically (with a cycle lasting 12–17 seconds and a pause between 2 cycles), resembling a nodding head. He became alert with free eye movement within 3 days of antibiotic treatment. Ping-pong gaze has been reported,1 but ocular nodding is a novel finding. [1]: http://neurology.org/lookup/doi/10.1212/WNL.0000000000004633

A new ferulic acid ester named 4-methyl-3-trans-hexenylferulate (1), together with eight known phenolic acid esters (2-9), was isolated from the methanolic extract of the roots and rhizomes of Notopterygium incisium. Their structures were elucidated by extensive spectroscopic techniques, including 2D NMR spectroscopy and mass spectrometry. 4-Methoxyphenethyl ferulate (8) NMR data is reported here for the first time. The uptake and transepithelial transport of the isolated compounds 1-9 were investigated in the human intestinal Caco-2 cell monolayer model. Compounds 2 and 6 were assigned for the well-absorbed compounds, compound 8 was assigned for the moderately absorbed compound, and compounds 1, 3, 4, 5, 7, and 9 were assigned for the poorly absorbed compounds. Moreover, all of the isolated compounds were assayed for the inhibitory effects against nitric oxide (NO) production in the lipopolysaccharide-activated RAW264.7 macrophages model and L-N⁶-(1-iminoethyl)-lysine (L-NIL) was used as a positive control. Compounds 1, 5, 8, and 9 exhibited potent inhibitory activity on NO production with the half maximal inhibitory concentration (IC50) values of 1.01, 4.63, 2.47, and 2.73 μM, respectively, which were more effective than L-NIL with IC50 values of 9.37 μM. These findings not only enriched the types of anti-inflammatory compounds in N. incisum but also provided some useful information for predicting their oral bioavailability and their suitability as drug leads or promising anti-inflammatory agents.

Cellulosome is a kind of multienzyme complex that displays high activity, selectivity and stability. Here, we report a novel, non-cellulolytic, cellulosome-like multienzyme complex that produced by the Cellulosimicrobium cellulans wild-type strain F16 isolated from soil microflora. This multienzyme complex, with excellent catalytic efficiency of kcat 13.2 s−1 to remove the C-7 xylosyl group from 7-xylosyl-10-deacetylpaclitaxel (10-DAXP), has an outstanding tolerance against organic solvents and an excellent general stability, with the long half-life of 214 hours. This cellulosome-like multienzyme complex has a novel structure distinct from the well-documented ones. The key catalytic subunit responsible for the β-xylosidase activity against 10-DAXP is identified to be a novel protein, indicating a new glycoside hydrolase (GH) family. The pioneering work described here offers a novel nanoscale biocatalyst for the production of biofuels and chemicals from renewable plant-based natural resources.

To investigate the saponins from whole plants of Lysimachia capillipes Hemsl., two new saponins, named capilliposide E (1) and capilliposide F (2), were isolated. The structures of the new saponins were elucidated as 3β,22α-dihydroxy-16α-acetat-2813-lactone-oleanane-3-O-[β-D-glucopyranosyl-(12)-α-Z-arabinpyranoyi]-22-O-β-D-glucopyranoside (1) and 3β, 22α-dihydroxy-16α-acetat-28-l3-lactone-oleanane-3-O-{[β-D-glucopyranosy-(l2)-β-D-glucopyranosyl-(l4)]-α-L-arabinpyranoyl}-22-O-β-D-glucopyranoside (2). The structures of these compounds were determined by 1D- and 2D-NMR, MS techniques, and chemical methods. (Managing editor: Wei WANG)

To observe the toxicity of Pulsatilla chinensis (Bunge) Regel saponins (PRS) against Oncomelania hupensis (O. hupensis).O. hupensis snails were exposed to 40% and 80% of 24 h LC50 of PRS for 24 h, and then choline esterase (CHE), alanine aminotransferase (ALT), alkaline phosphatase (ALP), lactate dehydrogenase (LDH) activities in cephalopodium and liver of snails were determined. Niclosamide (NIC) was used as the reference molluscicide. Zebra fish lethality test was evaluated to non-target aquatic species of PRS.The molluscicidal activity of PRS (LC50 at 24 h: 0.48 mg/L) was similar to that of NIC (LC50 at 24 h: 0.16 mg/L). Significant alterations about CHE, ALP, and ALT activities both in the cephalopodium and the liver of snails were observed when O. hupensis was exposed to 40% and 80% LC50 of PRS or NIC for 24 h. PRS and NIC could not affect LDH activity in the cephalopodium and the liver. Lower toxicity to fish of PRS was observed up to the highest concentration tested than NIC.PRS, as compared with the reference molluscicide NIC, is thought to be used for the control of harmful vector snails safely.

A new method using gradient elution of reversed-phase high performance liquid chromatography was set up for the separation and determination of 12 natural phenolic acids simultaneously. The separation conditions were studied with a Zorbax ODS (5 microns) column, and optimized conditions were suggested with the mobile phase gradient elution of CH3CN-H2O (with 0.5% HAc) (5:95--35:65, V/V, in 40 min) at room temperature, flow rate at 1.0 mL/min and detection wavelength at 280 nm. And the effects of acidity on separations and the relationships between the phenolic acids structures and their retention behaviors were discussed. Qualitative and quantitative analysis of the 12 compounds were performed. The regression equation, correlation coefficient, linear range, detection limit and relative standard deviation (RSD) of each compound were obtained under the optimized conditions. The correlation coefficients of the 12 compounds were between 0.9980 and 0.9999. The detection limits ranged from 0.96 ng to 4.10 ng and RSDs wereor = 2.64%. These results demonstrate that the optimized determination method obtained is successful for the separation and determination of the 12 phenolic acids analysed.