Neural Repair Institute: Advancing the Field of Neural Repair

Huntington's disease (HD) is a neurodegenerative disease characterized by degeneration of some neurons in certain areas of the brain such as the frontal lobe, basal ganglia, and caudate nucleus. This degeneration causes motor, cognitive, and behavioral disturbances.  The motor manifestations include sudden, jerky, and uncontrollable movements (called chorea) affecting mainly the face and upper extremities.   The cognitive decline include poor judgement and having trouble driving, learning new things, remembering a fact, making a decision, initiating appropriate actions, or inhibiting inappropriate actions.  The behavioral disturbances of Huntington's disease may include anxiety, depression, mood swings, aggressive behavior, and compulsivity that may lead to addictions and hypersexuality.
At present, there is no way to stop or reverse the course of Huntington's disease as the pathophysiology of the disorder is poorly understood.  The Huntington's disease gene has been located and investigators are continuing to study this gene with more focus toward understanding the mechanism of the disease in humans.
Scientific investigations are currently underway to study how the defective gene affects various structures in the brain and how it affects the chemistry and metabolism of the body.  Animal models are created in the laboratory in the hope of duplicating the clinical features of Huntington's disease so that researchers can learn more about the symptoms and progression of Huntington's disease. Investigators are currently experimenting fetal tissue implantation in rodents and nonhuman primates with the hope of understanding, restoring, or replacing functions typically lost by neuronal damage in patients with Huntington's disease.

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