Juvenile Huntington's disease (JHD) is rare. In the first decade of life speech difficulties, rigidity, and dystonia are common clinical motor symptoms, whereas onset in the second decade motor symptoms may sometimes resemble adult-onset Huntington's disease (AOHD). Cognitive decline is mostly detected by declining school performances.

Juvenile Huntington’s disease (JHD) is rare. In the first decade of life speech difficulties, rigidity, and dystonia are common clinical motor symptoms, whereas onset in the second decade motor symptoms may sometimes resemble adult-onset Huntington’s disease (AOHD). Cognitive decline is mostly detected by declining school performances.

Juvenile‐onset Huntington disease refers to approximately 5% of patients with symptom onset before the age of 21 years. The causal factor is a pathologically expanded CAG repeat in the Huntingtin gene. Age at onset is inversely correlated with CAG repeat length.

2017年2月7日 · Juvenile Huntington’s disease (JHD) is a neurodegenerative disease with onset prior to the age of 21. While it accounts for a relatively small proportion of Huntington’s disease (HD) diagnoses, its impact is significant on the quality of life for those affected.

Juvenile Huntington's disease (JHD) is a neurodegenerative disease with onset prior to the age of 21. While it accounts for a relatively small proportion of Huntington's disease (HD) diagnoses, its impact is significant on the quality of life for those affected.

2020年3月7日 · The juvenile form of Huntington’s disease (JHD) is rare. It is defined as HD with an onset of <21 years of age . The Juvenile HD Working Group of the European Huntington’s Disease Network (EHDN) recently redefined JHD as pediatric HD with an age of onset ≤18 years . However, as our study was conducted before this redefinition, we still ...

2006年2月1日 · Huntington's disease (HD) is caused by a CAG repeat mutation translating as a polyglutamine (poly (Q)) expansion in the huntingtin protein, whose main pathogenic mechanism is a gain of toxic function. In the case of large expansions beyond 60 repeats onset may result in juvenile HD (JHD, onset before 20 years of age).

Juvenile Huntington disease (JHD) is a form of Huntington disease (HD; see this term), characterized by onset of signs and symptoms before 20 years of age. Exact prevalence of the juvenile form is not known, but is estimated to be about 1/166,000. JHD is reported in 6% of the total cases of HD, which has a prevalence of 1/10,000.

Background: Juvenile-onset Huntington's disease (JHD) is defined by onset at the age of 20 or younger and represents approximately 5% of all HD cases. Patients with JHD present with a broad range of symptoms and signs that only overlap partially with adult-onset HD.

Juvenile Onset Huntington Disease (JHD) is a form of Huntington disease (HD) that affects children and teenagers. Huntington disease is a hereditary neurodegenerative disorder that is characterized by progressively worsening motor, cognitive, behavioral, and psychiatric symptoms.