Treatment and management

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Currently, no treatment influences the course of illness of Huntington' disease, although advances in research on the function of the Huntington' disease gene may change that. Nevertheless, psychiatric treatments can relieve some of the troublesome symptoms. Clinical experience suggests that the depression, anxiety, and obsessive-compulsive disorder associated with Huntington' disease usually respond to the pharmacological treatments used for the similar idiopathic disorders. Because some patients seem unaware of their depressed mood (just as they can be unaware of their involuntary movements) an informant is needed to elicit the symptoms. It is also important to distinguish depression (from which the patient is miserable and sleepless) and apathy, which does not cause the patient distress. Occasionally, mood and anxiety disorders are chronic and unresponsive to treatment. Severe, unresponsive depression can be treated successfully with electroconvulsive therapy.(!8) Bipolar disorder in patients with Huntington' disease does not usually respond to lithium, but may improve with carbamazepine or valproic acid, which is also helpful for severely irritable patients. Lithium is difficult to administer because Huntington' disease patients require high volumes of liquid and easily become lithium toxic if fluid intake is insufficient. In one case report, high doses of sertraline were very effective for intractable aggression. (!9ยป Schizophrenic symptoms can be relatively unresponsive to treatment. Sometimes a combination of clozaril and antidepressants can provide some relief. Small doses of neuroleptics can be helpful in decreasing involuntary movements in the first stages of the illness. (29 Persons with advanced disease are often unresponsive to most medication, probably because few striatal receptors remain.

As with most dementias, psychopathology influences, and is influenced by, the patient' environment. Patients do best in a calm highly predictable environment. Patients whose jobs have become cognitively taxing can be irritable, especially towards their family. Often stopping work will alleviate this almost entirely. Huntington' disease seriously damages family relationships, which in turn affects the patient. The well spouse becomes responsible for supporting the family, caring for children and the patient, and making family and financial decisions. Spouses naturally find this very burdensome and may become irritable and resentful. Some patients are unwilling to give up financial and family decision making, even though they may make very poor decisions that damage family relationships and finances. Some patients neglect their children or treat them badly. If the other parent cannot prevent this, it is wisest to remove the patient from the home. There is no research on the treatment of sexual aggression, but the author has successfully treated a few males using progesterone.

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