Huntington’s Part Three!!!

Huntington’s is a progressive neurodegenerative disease that causes specific nerve cells to waste away. Currently there is no cure for Huntington’s and no way to stop the disease from getting worse. Treatments focus on slowing the course of the disease and treating the individual symptoms. Conventional antipsychotic drugs like haloperidol can be used to try and control extra movements but the use of such drugs can lead to the development of tardive dyskinesia so the are generally only recommended in the chorea is completely debilitating. Dopamine agonists can also be used to treat irregular movements. All medications should be prescribed at the lowest dose possible and patients should be periodically taken off their medications (drug holidays).
There are 5 main ways that the research on Huntington’s is being conducted. Basic neurobiology is when the scientists study the specific gene to see how it is affecting the body. They can also use imaging to identify the structures in the brain that are most affected by the mutated Huntington’s gene. Imaging is generally carried out through the use of PET scans. Animal models are bred to show the clinical symptoms of Huntington’s in order to observe the progression of the disease. Animal models have also been used to study the effectiveness of fetal tissue in restoring or replacing functions lost by nerve degradation. The final pathway fro studying Huntington’s is through genetic studies that look at markers and inheritance factors within families.
Recent studies suggest that co-enzyme Q10 may help slow down the course of the disease. There is currently a study being conducted by Mass Gen and the National Institute of Neurological Disorders and Stroke to determine if coenzyme Q10 is effective in slowing the effects of Huntington’s disease. He study is also looking at the practicality of the drug as a treatment and whether it is safe or not. Looking at an actual ongoing research study was interesting because I got to see the research precautions that had been discussed in my seminar actually used in an actual research setting. For example the allocation of the co enzyme and of the placebo was randomized and the masking used was double blind meaning that neither the subject, caregiver nor investigator actually knew for certain which subject was given which drug.
Other areas that are currently being studied in relation to Huntington’s are its excitotoxicity (the over-stimulation of cells by natural chemicals found in the body), defective energy metabolism (a defect in the mitochondria), oxidative stress (normal metabolic activity in the brain that produces toxic compounds called free radicals) and tropic factors (natural chemical substances found in the human body that may protect against cell death). Hoprefully advancements in any one of these areas will lead to a break through in the study of Huntington’s.