Results 21 to 30 of about 2,427 (143)
Juvenile Huntington disease in Argentina [PDF]
Arquivos de Neuro-Psiquiatria, 2015 ABSTRACT We analyzed demographic, clinical and genetic characteristics of juvenile Huntington disease (JHD) and it frequency in an Argentinean cohort. Age at onset was defined as the age at which behavioral, cognitive, psychiatric or motor abnormalities suggestive of JHD were first reported. Clinical and genetic data were similar to other international
Gatto, Emilia Mabel +7 more
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Juvenile Huntington's disease: a clinical case from a pediatric's practice
Клинический разбор в общей медицине, 2023 Background. Huntington's disease (Hd) is a hereditary neurodegenerative disease with an autosomal dominant type of inheritance and manifested by motor, behavioral and mental disorders.
Emilia E. Alieva +3 more
doaj +1 more source
Stem Cell Research, 2023 Huntington’s disease (HD) is an inherited neurodegenerative disorder caused by an abnormal length of CAG repeats in the gene HTT, leading to an elongated poly-glutamine (poly-Q) sequence in huntingtin (HTT).
Duncan C. Miller +6 more
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Oromandibular Dyskinesia as the Initial Manifestation of Late-Onset Huntington Disease [PDF]
Journal of Movement Disorders, 2011 Huntington’s disease (HD) is a neurodegenerative disorder characterized by a triad of choreoathetosis, dementia and dominant inheritance. The cause of HD is an expansion of CAG trinucleotide repeats in the HD gene.
Dong-Seok Oh +5 more
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Subcortical T1-Rho MRI Abnormalities in Juvenile-Onset Huntington’s Disease
Brain Sciences, 2020 Huntington’s disease (HD) is a fatal neurodegenerative disease caused by the expansion of cytosine-adenine-guanine (CAG) repeats in the huntingtin gene. An increased CAG repeat length is associated with an earlier disease onset.
Alexander V. Tereshchenko +6 more
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Pediatric Neurology Briefs, 1989 The positron-emission tomography (PET) findings in a seven year old girl with the juvenile form of Huntington's disease are described from the Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, Montreal, Canada.
J Gordon Millichap
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Neurobiology of Disease, 2012 The pathophysiology of Huntington's disease (HD) is primarily associated with striatal degeneration and a number of behavioral symptoms such as involuntary movements, cognitive decline, psychiatric disorders, and in the most juvenile-onset cases with ...
Michele Pignatelli +3 more
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Fluids and Barriers of the CNS, 2022 Huntington’s disease (HD) is an inherited neurodegenerative disease caused by expansion of cytosine–adenine–guanine (CAG) repeats in the huntingtin gene, which leads to neuronal loss and decline in cognitive and motor function.
Raleigh M. Linville +5 more
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Juvenile‐Onset Huntington Disease Pathophysiology and Neurodevelopment: A Review [PDF]
Movement Disorders, 2021 ABSTRACTHuntington disease is an autosomal dominant inherited brain disorder that typically becomes manifest in adulthood. 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.
Bakels, H.S. +3 more
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The Association between CAG Repeat Length and Age of Onset of Juvenile-Onset Huntington’s Disease
Brain Sciences, 2020 There is a known negative association between cytosine–adenine–guanine (CAG) repeat length and the age of motor onset (AMO) in adult-onset Huntington’s Disease (AOHD).
Jordan L. Schultz +2 more
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