A population-specific mutational spectrum of CADASIL was found in the Chinese patients on Taiwan. The Chinese patients carrying NOTCH3 R544C may descend from a common ancestor. Anterior temporal hyperintensity on T2-weighted MRI may not be a sensitive marker for CADASIL. ICH is a relatively common manifestation of CADASIL in East Asians, especially in the presence of hypertension.

Stable stationary solutions correspond to memory capacity in the application of associative memory for neural networks. In this presentation, existence of multiple stable stationary solutions for Hopfield-type neural networks with delay and without delay is investigated. Basins of attraction for these stationary solutions are also estimated. Such a scenario of dynamics is established through formulating parameter conditions based on a geometrical setting. The present theory is demonstrated by two numerical simulations on the Hopfield neural networks with delays. Introduction.The studies of neural networks have attracted considerable multidisciplinary research interest in recent years. The developments for neural network models and the theory for the models are, on the one hand, driven by application motif or inspired by biological neuronal behaviors. On the other hand, the neural network theory has motivated and elicited further progress in dynamical system theory. For example, theory for existence of many stable patterns or chaotic dynamics for systems in phase space of large dimension is in strong demand for neural network applications. The progress in this direction of research has also enriched dynamical system theory [6,17,27].The applications of neural networks range from classifications, associative memory, image processing, and pattern recognition to parallel computation and its ability to solve optimization problems. The theory on the dynamics of the networks has been developed according to the purposes of the applications. In the application to parallel computation and signal processing involving finding the solution of an optimization problem, the existence of a computable solution for all possible initial states is the best situation. Mathematically, this means that the network needs to have a unique equilibrium which is globally attractive. Such a convergent behavior is referred to as "monostability" of a network. On the other hand, when a neural network is employed as an associative memory storage or for pattern recognition, the existence of many equilibria is a necessary feature [7,11,16,21]. The notion of "multistability" of a neural network is used to describe coexistence of multiple stable patterns such as equilibria or periodic orbits. In general, if the dynamics for a system are bounded, the existence of multiple stable patterns is accompanied with coexistence of stable and unstable equilibria or periodic orbits. The existence of unstable equilibria is essential in certain applications of neural network. For example, unstable equilibria are related to digital constraints on selection in winner-take-all problems [32,33].

Bajiaolian ( Dysosma pleianthum), one species in the Mayapple family, has been widely used as a general remedy and for the treatment of snake bite, weakness, condyloma accuminata, lymphadenopathy and tumours in China for thousands of years. However, the textbooks of traditional Chinese medicine mention little about the toxicity of Bajiaolian. Within 1 year, the authors saw five people who manifested nausea, vomiting, diarrhoea, abdominal pain, thrombocytopenia, leucopenia, abnormal liver function tests, sensory ataxia, altered consciousness and persistant peripheral tingling or numbness after drinking infusions made with Bajiaolian. The herb was recommended by either traditional Chinese medical doctors or herbal pharmacies for postpartum recovery and treatment of a neck mass, hepatoma, lumbago and dysmenorrhoea. Podophyllotoxin is one of the main ingredients of the Bajiaolian root. The clinical manifestations observed in our patients were consistent with podophyllum intoxication. Podophyllotoxin intoxication usually results from the accidental ingestion or topical application of podophyllum resin. However, these cases of Bajiaolian intoxication were iatrogenic and results from 'therapeutic doses' of Bajiaolian cited in the textbooks of traditional Chinese medicine.

Objective The clinical and genetic profiles of hereditary transthyretin amyloidosis ( ATTR ) in Chinese populations remain elusive. We aim to characterize the features of ATTR in a Taiwanese cohort of Han Chinese descent. Methods Seventy‐nine patients with molecularly confirmed ATTR from 57 Taiwanese families were identified by sequencing the transthyretin gene ( TTR ). The clinical and electrophysiological data were scrutinized. Cardiac involvement of ATTR was evaluated by echocardiography and cardiac scintigraphy. Four microsatellite and seven single‐nucleotide polymorphism markers flanking TTR were genotyped to investigate the founder effect of the TTR Ala97Ser mutation. Results Most of the patients had a peripheral neuropathy with variable autonomic symptoms. The average age at disease onset ( AO ) was 58.2 ± 7.2 years, and the male patients had an earlier AO than female patients (56.6 ± 5.7 years vs. 61.8 ± 8.9 years, P = 0.013). Electrophysiological studies revealed a generalized axonal sensorimotor polyneuropathy and isolated median neuropathy in 84.5% and 15.5% of the patients, respectively. Up to 80% of the patients with ATTR had symptomatic or subclinical cardiac involvement. Six TTR mutations were identified in the participants including one novel mutation Glu89Asp. Among them, Ala97Ser was the most common mutation, accounting for 91.2% of the ATTR pedigrees. Detailed haplotype analyses demonstrated a shared haplotype in the 47 patients with the Ala97Ser mutation, suggesting a founder effect. Interpretation The present study delineates the distinct features of ATTR in Taiwan and provides useful information for the diagnosis and management of ATTR , especially in patients of Chinese descent.

BackgroundMutations in the PRRT2 gene have recently been identified in patients with familial paroxysmal kinesigenic dyskinesia with infantile convulsions (PKD/IC) and patients with sporadic PKD/IC from several ethnic groups. To extend these recent genetic reports, we investigated the frequency and identities of PRRT2 mutations in a cohort of Taiwanese patients with PKD/IC.Methodology and Principal FindingsWe screened all 3 coding exons of PRRT2 for mutations in 28 Taiwanese patients with PKD/IC. Among them, 13 had familial PKD/IC and 15 were apparently sporadic cases. In total, 7 disparate mutations were identified in 13 patients, including 8 familial cases and 5 apparently sporadic cases. The mutations were not present in 500 healthy controls. Four mutations were novel. One patient had a missense mutation and all other patients carried PRRT2 mutations putatively resulting in a protein truncation. Haplotype analysis revealed that 5 of the 7 patients with the PRRT2 p.R217Pfs*8 mutation shared the same haplotype linked to the mutation.Conclusions and Significance PRRT2 mutations account for 61.5% (8 out of 13) of familial PKD/IC and 33.3% (5 out of 15) of apparently sporadic PKD/IC in the Taiwanese cohort. Most patients with the PRRT2 p.R217Pfs*8 mutation in Taiwan likely descend from a single common ancestor. This study expands the spectrum of PKD/IC-associated PRRT2 mutations, highlights the pathogenic role of PRRT2 mutations in PKD/IC, and suggests genetic heterogeneity within idiopathic PKD.