title: Managing juvenile Huntington's disease.
abstract: Huntington's disease (HD) is a well-recognized progressive neurodegenerative disorder that follows an autosomal dominant pattern of inheritance. Onset is insidious and can occur at almost any age, but most commonly the diagnosis is made between the ages of 35 and 55 years. Onset ≤20 years of age is classified as juvenile HD (JHD). This age-based definition is arbitrary but remains convenient. There is overlap between the clinical pathological and genetic features seen in JHD and more traditional adult-onset HD. Nonetheless, the frequent predominance of bradykinesia and dystonia early in the course of the illness, more frequent occurrence of epilepsy and myoclonus, more widespread pathology, and larger genetic lesion means that the distinction is still relevant. In addition, the relative rarity of JHD means that the clinician managing the patient is often doing so for the first time. Management is, at best, symptomatic and supportive with few or no evidence-based guidelines. In this article, the authors will review what is known of the condition and present some suggestions based on their experience.
authors.affiliation: Department of Clinical Genetics, Sheffield Children's Hospital, Sheffield, UK.
authors.forename: Oliver W J
authors.initials: OW
authors.lastname: Quarrell
authors.affiliation: Struthers Parkinson's Center, 6701 Country Club Drive, Golden Valley, MN 55427, USA.
authors.forename: Martha A
authors.initials: MA
authors.lastname: Nance
authors.affiliation: University of Iowa Carver College of Medicine W278 GH 200, Hawkins Drive, Iowa City, IA 52242, USA.
authors.forename: Peggy
authors.initials: P
authors.lastname: Nopoulos
authors.affiliation: Department of Psychiatry, University of Iowa, Iowa City, IA 52242-1000, USA.
authors.forename: Jane S
authors.initials: JS
authors.lastname: Paulsen
authors.affiliation: Department of Psychological Sciences, Birkbeck University of London, London, UK.
authors.forename: Jonathan A
authors.initials: JA
authors.lastname: Smith
authors.affiliation: Centre for Neurogenetics & Rare Diseases Neurological Research Institute Neuromed Via Atinense, 18-8607, Pozzilli (IS), Italy.
authors.forename: Ferdinando
authors.initials: F
authors.lastname: Squitieri