ID: 3 Disease Name: Huntington Disease Description: A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive chorea and dementia in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; depression; hallucinations; and delusions. Eventually intellectual impairment; loss of fine motor control; athetosis; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including seizures; ataxia; dementia; and chorea. Disease Type: Amyloidosis ICD-10 Classification: Diseases of the Nervous System Tissue: Central Nervous System (CNS) Cross References http://apps.who.int/classifications/icd10/browse/2016/en#/G10 https://meshb.nlm.nih.gov/record/ui?ui=D006816 https://www.omim.org/entry/143100 https://www.ncbi.nlm.nih.gov/pubmed/9267034 https://www.ncbi.nlm.nih.gov/pubmed/10410676 https://www.ncbi.nlm.nih.gov/pubmed/21163446 Alternative Names: Akinetic-Rigid Variant of Huntington Disease Chorea, Chronic Progressive Hereditary (Huntington) Chronic Progressive Hereditary Chorea (Huntington) Huntington Chorea Huntington Chronic Progressive Hereditary Chorea Huntington Disease, Akinetic-Rigid Variant Huntington Disease, Juvenile Huntington Disease, Juvenile-Onset Huntington Disease, Late Onset Huntington's Chorea Huntington's Disease Juvenile Huntington Disease Juvenile-Onset Huntington Disease Late-Onset Huntington Disease Progressive Chorea, Chronic Hereditary (Huntington) Progressive Chorea, Hereditary, Chronic (Huntington) Precursor Proteins: P42858 | Huntingtin Co-deposited Proteins: No information available Link to Amyloidosis: No information available //