Publications by authors named "T A Zesiewicz"

Introduction: The Friedreich Ataxia Rating Scale-Activities of Daily Living (FARS-ADL) is a validated and highly utilized measure for evaluating patients with Friedreich Ataxia. While construct validity of FARS-ADL has been shown for spinocerebellar ataxia (SCA), content validity has not been established.

Methods: Individuals with SCA1 or SCA3 (n = 7) and healthcare professionals (HCPs) with SCA expertise (n = 8) participated in qualitative interviews evaluating the relevance, clarity, and clinical meaningfulness of FARS-ADL for assessment of individuals with SCA.

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Background: Currently no curative treatment exists for spinocerebellar ataxias (SCAs). Riluzole repurposing was proposed as a symptomatic treatment in different types of cerebellar ataxia. We report a long-term-follow up under riluzole treatment in SCA type 7.

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Article Synopsis
  • The Scale for Assessment and Rating of Ataxia (SARA) is a key clinical tool for evaluating cerebellar ataxia but has received criticism regarding the relevance of its assessment items.
  • A study involving 850 patients across different types of spinocerebellar ataxias (SCA1, SCA2, SCA3, SCA6) revealed significant differences in progression rates among cohorts, particularly with the EUROSCA cohort showing the fastest rates.
  • The analysis found that not all items on the SARA scale contribute equally to measuring ataxia severity, and while some items are more sensitive to changes early or late in the disease, overall adjustments to the scale did not enhance responsiveness across different patient
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Background: Fatigue is a prevalent and debilitating symptom in neurological disorders, including spinocerebellar ataxias (SCAs). However, the risk factors of fatigue in the SCAs as well as its impact have not been well investigated.

Objectives: To study the prevalence of fatigue in SCAs, the factors contributing to fatigue, and the influence of fatigue on quality of life.

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