The Functional Rating Scale Taskforce for pre-Huntington Disease: Results So Far
2011

Assessing Day-to-Day Functioning in Early Huntington Disease

Sample size: 192 publication Evidence: moderate

Author Information

Author(s): Anthony L Vaccarino, Terrence Sills, Karen E. Anderson, Jean Endicott, Joseph Giuliano, Mark Guttman, Aileen K Ho, Peter Kupchak, Jane S. Paulsen, John H. Warner, Janet Evans, Ken Evans

Primary Institution: Ontario Cancer Biomarker Network

Hypothesis

The study aims to develop a comprehensive rating scale for assessing symptoms and functional ability in prodromal and early Huntington disease gene expansion carriers.

Conclusion

The study successfully developed and beta tested interview questions to assess day-to-day functioning in individuals with prodromal and early Huntington disease.

Supporting Evidence

  • Field testing of interview questions was conducted with 192 participants.
  • 11 out of 23 interview questions were found to produce scoring and discrimination across ranges of overall severity.
  • Mean total composite scores indicated higher functional impact in HD subjects compared to prHD subjects.

Takeaway

Researchers created questions to help understand how Huntington disease affects daily life for people who haven't been diagnosed yet.

Methodology

The study involved developing interview questions through a working group process, followed by field testing in individuals with prodromal and early Huntington disease.

Potential Biases

Potential under-reporting of symptoms due to lack of insight or awareness among participants.

Limitations

Some interview questions were poorly endorsed, limiting their usefulness for assessment.

Participant Demographics

{"total":192,"prHD":112,"HD":78,"male_percentage":44,"mean_age":47.3}

Statistical Information

P-Value

p=0.10

Statistical Significance

p<0.05

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