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Author

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Search Results for all work with filters:

  • 2015
  • Health Sciences, General
  • Biology, Neuroscience
  • Movement Disorders
  • Neurology: Movement Disor

Work 1-3 of 3

Sorted by relevance

Article

Oscillatory head movements in cervical dystonia: Dystonia, tremor, or both?

by Aasef Shaikh; David S. Zee; Hyder Jinnah

2015

Subjects
  • Biology, Neuroscience
  • Health Sciences, General
  • Health Sciences, Medicine and Surgery
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Abstract:Close

Cervical dystonia is characterized by abnormal posturing of the head, often combined with tremor-like oscillatory head movements. The nature and source of these oscillatory head movements is controversial, so they were quantified to delineate their characteristics and develop a hypothetical model for their genesis. A magnetic search coil system was used to measure head movements in 14 subjects with cervical dystonia. Two distinct types of oscillatory head movements were detected for most subjects, even when they were not clinically evident. One type had a relatively large amplitude and jerky irregular pattern, and the other had smaller amplitude with a more regular and sinusoidal pattern. The kinematic properties of these two types of oscillatory head movements were distinct, although both were often combined in the same subject. Both had features suggestive of a defect in a central neural integrator. The combination of different types of oscillatory head movements in cervical dystonia helps to clarify some of the current debates regarding whether they should be considered as manifestations of dystonia or tremor and provides novel insights into their potential pathogenesis.

Article

Coenzyme Q10 and spinocerebellar ataxias

by Raymond Y. Lo; Karla P. Figueroa; Stefan M. Pulst; Chi-Ying Lin; Susan Perlman; George Wilmot; Christopher Gomez; Jeremy Schmahmann; Henry Paulson; Vikram G. Shakkottai; Sarah Ying; Theresa Zesiewicz; Khalaf Bushara; Michael Geschwind; Guangbin Xia; S.H. Subramony; Tetsuo Ashizawa; Sheng-Han Sheng-Han Kuo

2015

Subjects
  • Health Sciences, General
  • Health Sciences, Pharmacology
  • Biology, Neuroscience
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Abstract:Close

The aim of this study was to investigate the association between drug exposure and disease severity in SCA types 1, 2, 3 and 6. The Clinical Research Consortium for Spinocerebellar Ataxias (CRC-SCA) enrolled 319 participants with SCA1, 2, 3, and 6 from 12 medical centers in the United States and repeatedly measured clinical severity by the Scale for Assessment and Rating of Ataxia (SARA), the Unified Huntington's Disease Rating Scale part IV (UHDRS-IV), and the 9-item Patient Health Questionnaire during July 2009 to May 2012. We employed generalized estimating equations in regression models to study the longitudinal effects of coenzyme Q10 (CoQ10), statin, and vitamin E on clinical severity of ataxia after adjusting for age, sex, and pathological CAG repeat number. Cross-sectionally, exposure to CoQ10 was associated with lower SARA and higher UHDRS-IV scores in SCA1 and 3. No association was found between statins, vitamin E, and clinical outcome. Longitudinally, CoQ10, statins, and vitamin E did not change the rates of clinical deterioration indexed by SARA and UHDRS-IV scores within 2 years. CoQ10 is associated with better clinical outcome in SCA1 and 3. These drug exposures did not appear to influence clinical progression within 2 years. Further studies are warranted to confirm the association.

Article

Development and Validation of a Clinical Scale for Rating the Severity of Blepharospasm

by Giovanni Defazio; Mark Hallett; Hyder Jinnah; Glenn T. Stebbins; Angelo F. Gigante; Gina Ferrazzano; Antonella Conte; Giovanni Fabbrini; Alfredo Berardelli

2015

Subjects
  • Biology, Neuroscience
  • Health Sciences, General
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Abstract:Close

Existing scales for rating the severity of blepharospasm (BSP) are limited by a number of potential drawbacks. We therefore developed and validated a novel scale for rating the severity of BSP. The development of the scale started with careful examination of the clinical spectrum of the condition by a panel of experts who selected phenomenological aspects thought to be relevant to disease severity. Thereafter, selected items were first checked for reliability, then reliable items were combined to generate the scale, and clinimetric properties of the scale were evaluated. Finally, the confidence with which the scale could be used by people without high levels of movement disorders skill was assessed. The new scale, based on objective criteria, yielded moderate to almost perfect reliability, acceptable internal consistency, satisfactory scaling assumptions, lack of floor and ceiling effects, partial correlations with a prior severity scale and with a quality of life scale, and good sensitivity to change. Despite a few limitations, the foregoing features make the novel scale more suitable than existing scales to assess the severity of BSP in natural history and pathophysiologic studies as well as in clinical trials.
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