NCS Exam Comprehensive Study Guide | 330+ Questions with 100% Correct Answers | Verified | Latest Update 2024 | 36 Pages
What is Huntington's Disease? - inherited disorder, malfunction of Huntington gene located on chromosome 4 - repeat of CAG triplets What is the pathology of HD? - severe loss of neurons in the caudate and putamen with subsequent astrocytosis; head of caudate becomes shrunken and there is dilatation of the anterior horns of lateral ventricles; also see neuronal degeneration within the temporal and frontal lobes of the cerebral cortex, globus pallidus, thalamus, subthalamic nucleus, substantial nigra, and cerebellum Why does HD produce hyperkinetic movements in early stage? - Because of loss of projection neurons in D2 pathway, causing disinhibition of the thalamus, which excites the cortex Why does HD produce hypokinetic movements in the late stage? - Loss of striatal projection neurons of the direct D1 pathway as well as cortical neurons When does juvenile onset of HD occur? - Before age 20 What characterizes the difference between juvenile onset HD and typical HD? - Early phase involves bradykinesia, rigidity, tremor, and myoclonus similar to PD; can have seizures and mental disability; faster disease progression (10 years vs. 19 years) What are the two main classes of medications involved in managing HD? - Antichoreic/antipsychotics and anti-depressants
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