Background:Benign essential blepharospasm(BEB),aberrant facial nerve degeneration and hemifacial spasm(HFS)are all examples dystonia which,though not life-threatening,can have a significant impact on patient quality o...Background:Benign essential blepharospasm(BEB),aberrant facial nerve degeneration and hemifacial spasm(HFS)are all examples dystonia which,though not life-threatening,can have a significant impact on patient quality of life.The need for reliable self-rating surveys to monitor functional disability is fundamental.The Blepharospasm Disability Index(BSDI)is already a widely utilised and validated selfrating score for blepharospasm whilst the functional disability score(FDS)requires further validation.The principle aim of this study is to repeat validation of the FDS against the BSDI,which has been validated by several groups since its original description but only in patients with BEB.Methods:A randomised blinded prospective cohort study was conducted at a single unit on 38 patients with BEB,aberrant facial nerve degeneration and HFS.Patients were blinded to complete the FDS followed by the BSDI or the BSDI followed by the FDS with a 30-minute interval.Results:Both the FDS and BSDI were found to be reliable with high internal consistency and test-retest reliability.Both scales were also found to be moderately correlated with the Jankovic disease severity score.Conclusions:This study is the first to use the FDS as a rating scale in patients with HFS and aberrant facial nerve degeneration.It is also the first study to formally validate the FDS as an acceptable rating scale for patients with dystonia and in particular it provides validation for its use in patients with HFS and aberrant facial nerve degeneration.展开更多
Background and objective:Botulinum toxin type A(BoNT/A)is a metalloprotease that blocks synaptic transmission via the cleavage of a synaptosomal-associated protein of 25 kDa(SNAP-25).It has gained widespread use as a ...Background and objective:Botulinum toxin type A(BoNT/A)is a metalloprotease that blocks synaptic transmission via the cleavage of a synaptosomal-associated protein of 25 kDa(SNAP-25).It has gained widespread use as a treatment for cerebral palsy and skeletal muscle hypertrophy.In China,Chinese botulinum toxin type A(CBTX-A),a type of BoNT/A,is in widespread clinical use.However,the changes in the morphological and biochemical properties of treated muscles and in remote muscles from the CBTX-A injection site are relatively unknown.Therefore,we investigated the changes in histomorphology and myosin heavy chain(MyHC)isoform composition and distribution in rat gastrocnemius muscles after intramuscular injection of CBTX-A.Methods:The weakness of the injected muscles was assessed periodically to identify their functional deficiency.Muscle slices were stained with hematoxylin-eosin(HE)and adenosine triphosphatase(ATPase).MyHC isoform composition was analyzed by sodium dodecyl sulfatepolyacrylamide gel electrophoresis(SDS-PAGE)to uncover changes in morphological and biochemical properties.Results:Our findings demonstrate that following injection of CBTX-A 5 U into rat gastrocnemius muscles,shifts in MyHC isoform composition emerged on the third day after injection and peaked in the fourth week.The composition remained distinctly different from that of the control group after the twelfth week.More specifically,there was a decrease in the proportion of the type IIb isoform and an increase in the proportions of type IIx,type IIa,and type I isoforms.Conclusions:Data revealed that CBTX-A led to a shift in MyHC composition towards slower isoforms and that the MyHC composition remained far from normal six months after a single injection.However,no noticeable remote muscle weakness was induced.展开更多
文摘Background:Benign essential blepharospasm(BEB),aberrant facial nerve degeneration and hemifacial spasm(HFS)are all examples dystonia which,though not life-threatening,can have a significant impact on patient quality of life.The need for reliable self-rating surveys to monitor functional disability is fundamental.The Blepharospasm Disability Index(BSDI)is already a widely utilised and validated selfrating score for blepharospasm whilst the functional disability score(FDS)requires further validation.The principle aim of this study is to repeat validation of the FDS against the BSDI,which has been validated by several groups since its original description but only in patients with BEB.Methods:A randomised blinded prospective cohort study was conducted at a single unit on 38 patients with BEB,aberrant facial nerve degeneration and HFS.Patients were blinded to complete the FDS followed by the BSDI or the BSDI followed by the FDS with a 30-minute interval.Results:Both the FDS and BSDI were found to be reliable with high internal consistency and test-retest reliability.Both scales were also found to be moderately correlated with the Jankovic disease severity score.Conclusions:This study is the first to use the FDS as a rating scale in patients with HFS and aberrant facial nerve degeneration.It is also the first study to formally validate the FDS as an acceptable rating scale for patients with dystonia and in particular it provides validation for its use in patients with HFS and aberrant facial nerve degeneration.
基金Project (No.491030-w10011) supported by the Zhejiang Provincial Natural Science Foundation of China
文摘Background and objective:Botulinum toxin type A(BoNT/A)is a metalloprotease that blocks synaptic transmission via the cleavage of a synaptosomal-associated protein of 25 kDa(SNAP-25).It has gained widespread use as a treatment for cerebral palsy and skeletal muscle hypertrophy.In China,Chinese botulinum toxin type A(CBTX-A),a type of BoNT/A,is in widespread clinical use.However,the changes in the morphological and biochemical properties of treated muscles and in remote muscles from the CBTX-A injection site are relatively unknown.Therefore,we investigated the changes in histomorphology and myosin heavy chain(MyHC)isoform composition and distribution in rat gastrocnemius muscles after intramuscular injection of CBTX-A.Methods:The weakness of the injected muscles was assessed periodically to identify their functional deficiency.Muscle slices were stained with hematoxylin-eosin(HE)and adenosine triphosphatase(ATPase).MyHC isoform composition was analyzed by sodium dodecyl sulfatepolyacrylamide gel electrophoresis(SDS-PAGE)to uncover changes in morphological and biochemical properties.Results:Our findings demonstrate that following injection of CBTX-A 5 U into rat gastrocnemius muscles,shifts in MyHC isoform composition emerged on the third day after injection and peaked in the fourth week.The composition remained distinctly different from that of the control group after the twelfth week.More specifically,there was a decrease in the proportion of the type IIb isoform and an increase in the proportions of type IIx,type IIa,and type I isoforms.Conclusions:Data revealed that CBTX-A led to a shift in MyHC composition towards slower isoforms and that the MyHC composition remained far from normal six months after a single injection.However,no noticeable remote muscle weakness was induced.
