The Electrophysiological Features in X-Linked Charcot-Marie-Tooth Disease With Transient Central Nervous System Deficits

Background: Electrophysiological examination plays an important role in the diagnosis of X-linked Charcot-Marie-Tooth disease (CMTX1) with transient central nervous system deficits. However, the electrophysiological features are seldom reported.Methods: We reviewed and analyzed published reports to determine the electrophysiological features of CMTX1 patients with transient central nervous system deficits.Results: A total of 21 CMTX1 patients with transient central nervous system deficits were found in 17 published case reports/series. The age of onset ranged from 0.5 to 18 years (mean 12.02 ± 0.78 years). All patients were male. Recurrent episodes of central nervous system deficits were reported in all 21 cases and resolved in periods ranging from several minutes to 3 days. All 20 patients who had MRIs at presentation had bilaterally symmetrical abnormal T2/Diffusion signals in the white matter without enhancement of gadolinium. All subsequent MRIs showed improvement or were within normal limits. The median motor nerve conduction velocity (MNCV), motor latencies, and compound muscle action potential (CMAP) amplitude were the most commonly measurable electrophysiological parameters (85.7%). All cases that had MNCV at presentation had slower and significantly decreased MNCV compared with the normal value (34.1 ± 1.10 m/s vs. 46.8±2.05 m/s, P < 0.0001; 95% CI, −17.4 to −7.92). The average variations of MNCV in median nerve, ulnar nerve, peroneal nerve, and tibial nerve were 22.0 ± 5.96%, 27.6 ± 11.9%, 25.9 ± 4.36%, and 27.3 ± 4.30%, respectively. All cases with measured sensory nerve conduction velocity (SNCV) at presentation had slower and significantly decreased SNCV compared with the normal value (35.3 ± 1.33 m/s vs. 47.7 ± 2.40 m/s, P < 0.001; 95% CI −18.2 to −6.46). The average variations of SNCV in median nerve, ulnar nerve, and sural nerve were 19.9 ± 8.24%, 25.2 ± 7.75%, and 23.2 ± 3.95%, respectively.Conclusion: This case series serves as a reminder that electrophysiological examination should be included in the diagnosis of recurrent and episodic neurological deficit with white matter lesions. Median MNCV is a sensitive and valuable parameter to support the diagnosis of CMTX1 with transient central nervous system deficits

Effects of habitat usage on hypoxia avoidance behavior and exposure in reef-dependent marine coastal species

Reef habitat in coastal ecosystems is increasingly being augmented with artificial reefs (ARs) and is simultaneously experiencing increasing hypoxia due to eutrophication and climate change. Relatively little is known about the effects of hypoxia on organisms that use complex habitat arrangements and how the presence of highly preferred AR habitat can affect the exposure of organisms to low dissolved oxygen (DO). We performed two laboratory experiments that used video recording of behavioral movement to explore 1) habitat usage and staying duration of individuals continuously exposed to 3, 5, and 7 mg/L dissolved oxygen (DO) in a complex of multiple preferred and avoided habitat types, and 2) the impact of ARs on exposure to different DO concentrations under a series of two-way replicated choice experiments with or without AR placement on the low-oxygen side. Six common reef-dependent species found in the northeastern sea areas of China were used (i.e., rockfish Sebastes schlegelii and Hexagrammos otakii, filefish Thamnaconus modestus, flatfish Pseudopleuronectes yokohamae, sea cucumber Stichopus japonicus, and crab Charybdis japonica). Results showed that lower DO levels decreased the usage of preferred habitats of the sea cucumber and the habitat-generalist filefish but increased the habitat affinity to preferred habitat types for the two habitat-specific rockfishes. Low DO had no effect on the crab’s habitat usage. In the choice experiment, all three fish species avoided 1 mg/L, and the rockfish S. schlegelii continued to avoid the lower DO when given choices involving pairs of 3, 5, and 7 mg/L, while H. otakii and the flatfish showed less avoidance. The availability of ARs affected exposure to low DO for the habitat-preferring rockfishes but was not significant for the flatfish. This study provides information for assessing the ecological effects and potential for adaptation through behavioral movement for key reef-dependent species under the increasing overlap of ARs and hypoxia anticipated in the future

Data_Sheet_1_The Electrophysiological Features in X-Linked Charcot-Marie-Tooth Disease With Transient Central Nervous System Deficits.docx

<p>Background: Electrophysiological examination plays an important role in the diagnosis of X-linked Charcot-Marie-Tooth disease (CMTX1) with transient central nervous system deficits. However, the electrophysiological features are seldom reported.</p><p>Methods: We reviewed and analyzed published reports to determine the electrophysiological features of CMTX1 patients with transient central nervous system deficits.</p><p>Results: A total of 21 CMTX1 patients with transient central nervous system deficits were found in 17 published case reports/series. The age of onset ranged from 0.5 to 18 years (mean 12.02 ± 0.78 years). All patients were male. Recurrent episodes of central nervous system deficits were reported in all 21 cases and resolved in periods ranging from several minutes to 3 days. All 20 patients who had MRIs at presentation had bilaterally symmetrical abnormal T2/Diffusion signals in the white matter without enhancement of gadolinium. All subsequent MRIs showed improvement or were within normal limits. The median motor nerve conduction velocity (MNCV), motor latencies, and compound muscle action potential (CMAP) amplitude were the most commonly measurable electrophysiological parameters (85.7%). All cases that had MNCV at presentation had slower and significantly decreased MNCV compared with the normal value (34.1 ± 1.10 m/s vs. 46.8±2.05 m/s, P < 0.0001; 95% CI, −17.4 to −7.92). The average variations of MNCV in median nerve, ulnar nerve, peroneal nerve, and tibial nerve were 22.0 ± 5.96%, 27.6 ± 11.9%, 25.9 ± 4.36%, and 27.3 ± 4.30%, respectively. All cases with measured sensory nerve conduction velocity (SNCV) at presentation had slower and significantly decreased SNCV compared with the normal value (35.3 ± 1.33 m/s vs. 47.7 ± 2.40 m/s, P < 0.001; 95% CI −18.2 to −6.46). The average variations of SNCV in median nerve, ulnar nerve, and sural nerve were 19.9 ± 8.24%, 25.2 ± 7.75%, and 23.2 ± 3.95%, respectively.</p><p>Conclusion: This case series serves as a reminder that electrophysiological examination should be included in the diagnosis of recurrent and episodic neurological deficit with white matter lesions. Median MNCV is a sensitive and valuable parameter to support the diagnosis of CMTX1 with transient central nervous system deficits.</p

Carbon stocks and potential carbon storage in the mangrove forests of China

Chinese Academy of Sciences [XDA05050206]Mangrove forests provide important ecosystem services, and play important roles in terrestrial and oceanic carbon (C) cycling. Although the C stocks or storage in terrestrial ecosystems in China have been frequently assessed, the C stocks in mangrove forests have often been overlooked. In this study, we estimated the C stocks and the potential C stocks in China's mangrove forests by combining our own field data with data from the National Mangrove Resource Inventory Report and from other published literature. The results indicate that mangrove forests in China store about 6.91 +/- 0.57 Tg C, of which 81.74% is in the top 1 m soil, 18.12% in the biomass of mangrove trees, and 0.08% in the ground layer (i.e. mangrove litter and seedlings). The potential C stocks are as high as 28.81 +/- 4.16 Tg C. On average, mangrove forests in China contain 355.25 +/- 82.19 Mg C ha(-1), which is consistent with the global average of mangrove C density at similar latitudes, but higher than the average C density in terrestrial forests in China. Our results suggest that C storage in mangroves can be increased by selecting high C-density species for afforestation and stand improvement, and even more by increasing the mangrove area. The information gained in this study will facilitate policy decisions concerning the restoration of mangrove forests in China. (C) 2013 Elsevier Ltd. All rights reserved