Short and long term outcome of bilateral pallidal stimulation in chorea-acanthocytosis

BACKGROUND: Chorea-acanthocytosis (ChAc) is a neuroacanthocytosis syndrome presenting with severe movement disorders poorly responsive to drug therapy. Case reports suggest that bilateral deep brain stimulation (DBS) of the ventro-postero-lateral internal globus pallidus (GPi) may benefit these patients. To explore this issue, the present multicentre (n=12) retrospective study collected the short and long term outcome of 15 patients who underwent DBS. METHODS: Data were collected in a standardized way 2-6 months preoperatively, 1-5 months (early) and 6 months or more (late) after surgery at the last follow-up visit (mean follow-up: 29.5 months). RESULTS: Motor severity, assessed by the Unified Huntington's Disease Rating Scale-Motor Score, UHDRS-MS), was significantly reduced at both early and late post-surgery time points (mean improvement 54.3% and 44.1%, respectively). Functional capacity (UHDRS-Functional Capacity Score) was also significantly improved at both post-surgery time points (mean 75.5% and 73.3%, respectively), whereas incapacity (UHDRS-Independence Score) improvement reached significance at early post-surgery only (mean 37.3%). Long term significant improvement of motor symptom severity (≥ 20 % from baseline) was observed in 61.5 % of the patients. Chorea and dystonia improved, whereas effects on dysarthria and swallowing were variable. Parkinsonism did not improve. Linear regression analysis showed that preoperative motor severity predicted motor improvement at both post-surgery time points. The most serious adverse event was device infection and cerebral abscess, and one patient died suddenly of unclear cause, 4 years after surgery. CONCLUSION: This study shows that bilateral DBS of the GPi effectively reduces the severity of drug-resistant hyperkinetic movement disorders such as present in ChAc

Multicriterial characterisation of plant pathogens for agroterrorism prevention in Europe - An emerging field of research.

International audienc

Short and long term outcome of bilateral pallidal stimulation in chorea-acanthocytosis

BACKGROUND: Chorea-acanthocytosis (ChAc) is a neuroacanthocytosis syndrome presenting with severe movement disorders poorly responsive to drug therapy. Case reports suggest that bilateral deep brain stimulation (DBS) of the ventro-postero-lateral internal globus pallidus (GPi) may benefit these patients. To explore this issue, the present multicentre (n=12) retrospective study collected the short and long term outcome of 15 patients who underwent DBS. METHODS: Data were collected in a standardized way 2-6 months preoperatively, 1-5 months (early) and 6 months or more (late) after surgery at the last follow-up visit (mean follow-up: 29.5 months). RESULTS: Motor severity, assessed by the Unified Huntington's Disease Rating Scale-Motor Score, UHDRS-MS), was significantly reduced at both early and late post-surgery time points (mean improvement 54.3% and 44.1%, respectively). Functional capacity (UHDRS-Functional Capacity Score) was also significantly improved at both post-surgery time points (mean 75.5% and 73.3%, respectively), whereas incapacity (UHDRS-Independence Score) improvement reached significance at early post-surgery only (mean 37.3%). Long term significant improvement of motor symptom severity (≥ 20 % from baseline) was observed in 61.5 % of the patients. Chorea and dystonia improved, whereas effects on dysarthria and swallowing were variable. Parkinsonism did not improve. Linear regression analysis showed that preoperative motor severity predicted motor improvement at both post-surgery time points. The most serious adverse event was device infection and cerebral abscess, and one patient died suddenly of unclear cause, 4 years after surgery. CONCLUSION: This study shows that bilateral DBS of the GPi effectively reduces the severity of drug-resistant hyperkinetic movement disorders such as present in ChAc

The Weaponisation of Mycotoxins

Mycotoxins as weapons is a serious issue. The word weaponisation in the title of this chapter is poor grammatically, although it is useful as it is generally understood. Crucially, access to accurate information is required to enable appropriate responses for potential threats. The high interest created by a recent review of fungi and toxins as weapons indicated that further publications in the field are desirable: Paterson (2006a) remained at number 1 in Science Direct's Top 25 Hottest Articles (Mycological Research) for a year. Citation numbers put it fourth since 2006: Holstege et al. (2007) is instructive as it indicates just how seriously the threat is taken in the United States of America. The authors focus on trichothecene mycotoxins and particularly T-2 toxin. Of course, the reasons for the topicality were the mass attacks on citizens which have occurred this century, and the claim that aflatoxins had the potential to be used by Iraq. The recent attacks in the USA using anthrax spores via internal post also caused a great deal of concern: massive casualties have been predicted from anthrax released into very large cites. Dohnal et al. (2007) are also concerned with T-2 toxin. Latxague et al. (2007) focuses on anticrop bioterrorism and bioweapons against the agricultural sector. They appear to be more concerned with whole organisms, rather than purified mycotoxins per se. However, it is difficult to obtain a list of the fungi with which Latxague et al. are concerned, no doubt for security reasons. Mycotoxin-producing fungi need to be on the list, as do plant pathogens. Also, countries where crops are developed almost as a monoculture are at particular risk from natural pathogens [e.g. the fungus Ganoderma and the oil palm crop (Paterson et al. 2009)]. Pohanka et al. (2007) considered the issue of developing bioassays to detect mycotoxins. Finally, Casadevall and Pirofski (2006) provided a well-argued assessment concerning why human pathogenic fungi could usefully be given greater consideration as biowarfarFundação para a Ciência e a Tecnologia (FCT) - bolsa SFRH/BPD/34879/200