Major or Mild Neurocognitive Disorder Due to Huntingtons Disease DSM-5 33.4(G10), 31.83(G31.84)
DSM-5 Category: Neurocognitive Disorders
Introduction
Huntington’s disease is a genetic disease with no cure. Symptoms include loss of control over muscle movement and severe mood and behavioral changes. According to the DSM-5, major or mild neurocogntivie disorder due to Huntington’s disease refers a decline in cognitive performance as a direct result of Huntington’s disease. Because no cure exists for Huntington’s disease, treatment is focused on reducing the impact of symptoms on the patient as well as prolonging normal functioning. Huntington’ disease is always inherited and therefore can be determined by blood test. Early detection can lead to improved quality of life.
Symptoms of Major or Mild Neurocognitive Disorder Due to Huntington's Disease
According to the DSM-5, symptoms of mild neurocognitive disorder due to Huntington’s disease include a modest decline in cognitive performance when compared to previous levels of cognitive performance. This is evidenced by self-report by the patient or the concern of a friend or family member. The impairment can be measured using standardized tests of cognitive performance. These impairments do not limit the patient’s ability to perform everyday activities and the deficits are not related to delirium. Behavioral disturbances maybe present but are not necessary for diagnosis (American Psychiatric Association, 2013).
The DSM-5 explains that symptoms of major neurocognitive disorder due to Huntington’s disease include a significant decline in cognitive performance when compared to previous levels of cognitive performance. This is evidenced by self-report of the patient or the concern of a friend or family member. The impairment can be measured using standardized tests of cognitive performance. These impairments significantly limit the patient’s ability to perform every-day activities such as shopping, driving, working, paying bills and self-care. These deficits are not related to delirium. Behavioral disturbances maybe present but are not necessary for diagnosis (American Psychiatric Association, 2013).
Both mild and major neurocognitive disorder due to Huntington’s disease are related to the feature impairment of Huntington’s disease. Early symptoms include changes to processing speed, organization and planning. Later symptoms involve motor abnormalities. Huntington’s disease not affect learning or memory (American Psychiatric Association, 2013).
Prevalence of Major or Mild Neurocognitive Disorder Due to Huntington’s Disease
The DSM-5 estimates that the prevalence of Neurocognitive disorder due to Huntington’s disease is about 5.6 per 100,000 people in the United States, Canada, Europe and Australia. Prevalence is Asia is less than half of that in other parts of the world (American Psychiatric Association, 2013).The genetic component of Huntington’s disease is responsible for these differences (Pringsheim, et al., 2012)
Onset and Course
Although most adults are diagnosed with Huntington’s disease in middle life, or around 40 years, the DSM-5 suggests that cognitive and psychological abnormalities begin in early adulthood. Early psychological symptoms include depression, anxiety and irritability as well as increased impulsivity and apathy. Early physical symptoms include fidgeting, difficulty with motor tasks and difficulty with intended movement. Difficulty with speech, walking and eventually swallowing also occurs (American Psychiatric Association, 2013). Typically, a patient with no family history of Huntington ’s disease will visit a doctor worried about being clumsy or more emotional than usual. When symptoms, such as jerky movements or difficulty with eye coordination worsen, the doctor will likely order magnetic resolution imaging (MRI) of the brain and a genetic test for Huntington’s disease (Lawrence, 2009).
Heritability and Genetic Testing for Huntington’s Disease
All cases of Huntington’s disease are directly caused by an abnormal gene passed from parent to child (Huntington Disease Society of America, 2014). A person who has one parent who has Huntington’s disease has a 50% change of having the disease himself. An accurate genetic test to diagnose Huntington’s disease before symptoms appear has existed since the 1990s. The testing process is simple. The patient’s blood is sampled and the IT15 gene is analyzed. The results can determine whether the individual will develop Huntington’s disease, are at risk for passing the gene even if they themselves are not at risk, or do not have the disease at all (Lawrence, 2009). Although the technical aspect of the test is straightforward, the emotional aspect is extremely difficult. It is strongly recommended that patient receive counseling before and after receiving test results. In many families, one sibling may be found to carry the gene while another sibling may not. This can create conflict as well as feelings of guilt or betrayal (Lawrence, 2009). Some people also feel that early diagnosis is of no use because no treatment or cure for Huntington’s disease is currently available (Huntington Disease Society of America, 2014).
Prognosis for Major of Mild Neurocognitive Disorder Due to Huntington’s Disease
Huntington’s disease is gradually progressive and cannot be cured. The DSM-5 explains that death typically occurs around 15 years after motor symptoms are diagnosed (American Psychiatric Association, 2013).
Social and Occupational Consequences
The physical and mental impairments related to Huntington’s disease are extremely debilitating. The emotional, cognitive and personality changes can create challenges in all relationships. The physical changes can affect work and day to day function, such as driving, showering and toileting. Because typical onset is 40 years old, most patients are in the heights of their careers and involved in raising families. This, of course can put great strain on the family members of the patient (American Psychiatric Association, 2013).
Treatment for Major or Mild Neurocognitive Disorder due to Huntington’s Disease
The major or mild Neurocognitive disorder associated with Huntington’s disease will be treated along the other symptoms of the disease. To date, no cure for Huntington’s disease exists. Treatment goals are to manage symptoms and when possible, delay or prevent the progression of the disease. For one, several medications can help with physical and psychological symptoms. Muscle relaxers and ant-convulsive drugs, for example, can help control involuntary movement. Antipsychotics are useful in managing hallucinations and aggressive outbursts. Antidepressants and anti-anxiety medications are also useful in controlling emotional issues related to Huntington’s disease (Lawrence, 2009).
Although drugs are helpful for controlling symptoms, patients also benefit from psychotherapy. Adjusting to a debilitating disease can be devastating for patients and families. Therapy can help patients and their loved ones adjust to new lifestyles and changing abilities. As the physical symptoms worsen, physical and occupational therapy are needed to help the patient adjust and re-learn skills necessary for daily living. Exercises and speech therapy can help the patient increase the time they are able to control their bodies and speak to others (Lawrence, 2009).
Towards the end of the disease, keeping the patient comfortable is top priority. Padding the bed and making the sure patient gets enough nutrition are common interventions. Because patients lose muscular control in their mouths, they are often fed through a tube towards the end of their lives (Lawrence, 2009).
References
American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (5th ed.). Arlington, VA: American Psychiatric Publishing. Huntington’s Disease Society of America (2014). Living with HD.
Lawrence, D. (2009). Huntington’s Disease. New York: Infobase Publishing
Spiney, L. (2010) Uncovering the true prevalence of Huntington's disease. Lancet Neurology, 9 (8): 760-761
Pringsheim, T., Wiltshire, K., Day, L., Dykeman, J. Steeves, T. & Jette, N. (2012). The incidence and prevalence of Huntington's disease: a systematic review and meta-analysis. Movement Disorders. 27(9): 1083-91
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