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Parental transmission in Huntington's disease
Lancet (London, England)
|May 19, 1984
Summary
Juvenile onset Huntington's chorea is nearly always inherited paternally. Late-onset Huntington's chorea shows no parental bias, with inheritance equally likely from mothers or fathers.
Area of Science:
- Genetics
- Neurology
- Inheritance patterns
Background:
- Huntington's chorea is a progressive neurodegenerative disorder.
- Previous studies suggested differing parental transmission for juvenile versus late-onset forms.
Purpose of the Study:
- To investigate the parental origin of Huntington's chorea based on age of onset.
- To clarify inheritance patterns in a larger cohort.
Main Methods:
- Survey of parental transmission data in Huntington's chorea patients.
- Categorization of patients by age of symptom onset (pre-pubertal vs. late-onset).
Main Results:
- 12 of 13 patients with juvenile onset (before age 10) inherited Huntington's chorea from their father.
- 205 late-onset patients (onset after age 65) showed equal likelihood of paternal or maternal inheritance.
Conclusions:
- Juvenile onset Huntington's chorea demonstrates a strong paternal inheritance pattern.
- Late-onset Huntington's chorea inheritance is not biased towards either parent.