JUVENILE ONSET HUNTINGTON DISEASE
\d͡ʒˈuːvənˌa͡ɪl ˈɒnsɛt hˈʌntɪŋtən dɪzˈiːz], \dʒˈuːvənˌaɪl ˈɒnsɛt hˈʌntɪŋtən dɪzˈiːz], \dʒ_ˈuː_v_ə_n_ˌaɪ_l ˈɒ_n_s_ɛ_t h_ˈʌ_n_t_ɪ_ŋ_t_ə_n d_ɪ_z_ˈiː_z]\
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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. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)
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Word of the day
Dopamine Acetyltransferase
- An enzyme that catalyzes the of groups from acetyl-CoA to arylamines. They have wide specificity for aromatic amines, particularly serotonin, and can also catalyze acetyl transfer between arylamines without CoA. EC 2.3.1.5.
Nearby Words
- juvenile myoclonic epilepsy of janz
- juvenile neuroaxonal dystrophies
- juvenile neuroaxonal dystrophy
- juvenile neurosyphilis
- juvenile onset diabetes mellitus
- Juvenile Onset Huntington Disease
- juvenile pareses
- juvenile paresis
- juvenile parkinson disease
- juvenile parkinsonism
- juvenile parkinsonism, autosomal recessive