Juvenile Huntington’s disease (JHD)
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Juvenile Huntington’s disease (JHD). The onset age is under 21 years. It accounts for 3-10% of cases of HD. Fully referenced neurology checklist.
Genetics
- The onset age is under 21 years
- It accounts for 3-10% of cases of HD
- It is usually with paternal transmission
- It is associated with larger CAG repeats
Phenotypes
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Early features
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Later features
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Magnetic resonance imaging (MRI) brain: atrophy sites
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Prognosis
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References
- Gonzalez-Alegre P, Afifi AK. Clinical characteristics of childhood-onset (juvenile) Huntington's disease: report of 12 patients and review of the literature. J Child Neurol 2006; 21:223-229.
- Ruocco HH, Lopes-Cendes I, Laurito TL, Li LM, Cendes F. Clinical presentation of juvenile Huntington disease. Arq Neuropsiquiatr 2006; 64:5-9.
- Gómez-Tortosa E, del Barrio A, García Ruiz PJ, et al. Severity of cognitive impairment in juvenile and late-onset Huntington disease. Arch Neurol 1998; 55:835-843.
- Lammert DB, Bang J, Stafstrom CE. Pearls & Oy-sters: Epilepsy is a key feature of pediatric-onset Huntington disease. Neurology 2023; 101:e2051-e2055.
- Cloud LJ, Rosenblatt A, Margolis RL, et al. Seizures in juvenile Huntington's disease: frequency and characterization in a multicenter cohort. Mov Disord 2012; 27:1797-1800.
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