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Parkinsonism in Huntington's disease
1George-Huntington-Institute, Muenster, Germany; Department of Radiology, Universitaetsklinikum Muenster (UKM), Westfaelische Wilhelms-University, Muenster, Germany; Department of Neurodegenerative Diseases and Hertie-Institute for Clinical Brain Research, University of Tuebingen, Tuebingen, Germany.
Insights
Huntington's disease (HD) motor symptoms evolve over time. Early Parkinsonian signs like bradykinesia appear years before chorea, challenging diagnosis in some patient groups.
Area of Science:
- Neurology
- Neurodegenerative Diseases
- Movement Disorders
Background:
- Huntington's disease (HD) is primarily known for chorea, involuntary hyperkinetic movements.
- Chorea's intensity peaks mid-stage and declines as HD progresses.
- Parkinsonian signs (bradykinesia, rigidity, postural instability) are often underestimated in HD.
Purpose of the Study:
- To highlight the progression and diagnostic challenges of Parkinsonian motor signs in Huntington's disease.
- To emphasize the early detection of bradykinesia using quantitative motor assessments.
Main Methods:
- Utilized quantitative motor (Q-Motor) assessments, including finger tapping (digitomotography).
- Analyzed motor symptom progression, comparing chorea and Parkinsonian signs over disease stages.
- Examined specific patient groups with atypical motor phenotypes.
Main Results:
- Bradykinesia is detectable in premanifest gene carriers up to two decades before clinical HD onset.
- Parkinsonian signs progress linearly and contribute to falls in advanced HD stages.
- A predominantly Parkinsonian motor phenotype, with minimal chorea, occurs in late-manifesting and pediatric HD patients, complicating early diagnosis.
Conclusions:
- The motor phenotype of Huntington's disease is more complex than just chorea.
- Early quantitative motor assessments can reveal Parkinsonian signs preceding overt HD symptoms.
- Diagnostic challenges exist for HD patients presenting with primarily Parkinsonian features, necessitating refined diagnostic criteria.
Abstract:
Huntington's disease (HD) is usually characterized by involuntary hyperkinetic movements, called chorea. The intensity of chorea exhibits a peak in middle stages of HD and then decreases as HD progresses. In contrast, Pakinsonian signs of HD are often less appreciated. They typically progress in a fairly linear pattern over time. In fact, bradykinesia is detectable early on in premanifest gene carriers up to two decades prior to the clinical manifestation of HD symptoms using quantitative motor (Q-Motor) assessments such as finger tapping (digitomotography). Other Parkinsonian symptoms besides bradykinesia are rigidity and postural instability. They typically results in falls and injuries in advanced stages of HD. A primarily Parkinsonian motor phenotype, often seen with little to no chorea, is characteristically observed in older, late manifesting patients and in pediatric HD subjects. Establishing a diagnosis of HD is difficult in these groups and patients are often misdiagnosed in early stages.
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