Psychiatric Issues in Patients with Huntington's Disease. Disclosures. Huntington s Disease. Disease Course. Huntington s Disease

Transcription

1 Psychiatric Issues in Patients with Huntington's Disease SCPA Sunday, January 27, 2019 Robert Breen, M.D. South Carolina Department of Mental Health University of South Carolina School of Medicine 1 2 Disclosures Dr. Breen discloses the following relevant relationships/affiliations: BRISTOL MYERS SQUIBB CO Global Blood Therapeutics GLAXOSMITHKLINE PLC SPON MCKESSON CORP MERCK & COMPANY INC NEW SANOFI SPON ADR HDSDA Center of Excellence USC SoM Department of Neurology Palmetto Health Richland HD clinic provides multidisciplinary services that include: on site neurological evaluations and tx, genetic counseling, neuropsychological consultations and psychological consultations. Our clinic provides genetic counseling and predictive HD testing for individuals at risk, as well as genetic counseling and family planning for families at risk. We also provide monthly in person support group and online support group for patients and families affected by HD Huntington s Disease Fatal genetic disorder Movement component chorea Neuropsychiatric component including behavioral, cognitive and emotional changes We estimate there are 30,000 symptomatic Americans and more than 200,000 at risk of inheriting the disease In SC we estimate diagnosed patients and 2,500 individuals at risk 3 4 Huntington s Disease Autosomal dominant disorder meaning the child of a parent with HD has a 50/50 chance of inheriting the faulty gene that causes Huntington s disease. There is also a juvenile form of HD An excessive number of CAG (cytosine, adenine, guanine) repeats vs. > 60 produces abnormal Huntingtin Disease Course Preclinical Early Stage (functioning affected) Intermediate Stage (functioning impaired) Late Stage (functionally disabled) Predictive testing is available End of Life Stage 5 6

2 Co morbid Psychiatric Conditions Mania OCD Neuropsychiatric conditions Suicide risk Suicide Risk is Considerable DEPRESSION and SUICIDAL IDEATION are NOT NORMAL in HD 7 8 There is a 40% lifetime prevalence of depression in person s with HD DEPRESSION and SUICIDAL IDEATION are NOT NORMAL in HD The response rate is close to if not equal to that of depressed persons without HD Most psychiatrists employ SSRI s and SNRI s 9 10 Potential complications include: Low threshold for delirium is seen often in HD but is less well researched than depression Estimated prevalence uncertain: 13 71% Occurs with and without depression 11 12

3 Higher levels of anxiety are associated with: Greater illness identity and a less strong belief in treatment control Coping strategies of venting, self blame, and behavioral disengagement A low acceptance of illness May or may not be a risk factor for suicide in Huntington s disease But it is definitely a risk factor for suicide in the general population Mania Mania is less common than anxiety or depression and Must be distinguished from disinhibition Mania To make a Mania diagnosis focus on: Increased activity Decreased need for sleep Grandiosity Delusions and Hallucinations Mania Treatment Mood Stabilizing Anticonvulsants Antipsychotics Lithium can be effective ( narrow therapeutic index makes it risky) Paranoia is more frequent Hallucinations less frequent Irritability, perseveration and apathy may be neuropsychiatric symptoms 17 18

4 Antipsychotics work well in Huntington s Disease and may also treat the movement disorder Delusional disorder may occur and is likely to be as difficult to treat as in persons without HD OCD OCD in HD looks similar to OCD in the general population OCD SSRI s remain the treatment of choice TCA s may be difficult to tolerate Dys executive syndrome Apathy Perseveration and fixation Irritability Delirium Toxicity Dehydration Infection Subdural Hematoma 23 24

5 Sleep Problems Chorea Lack of exercise and anxiety but also apathy Sexual Behavior Changes Loss of interest Disinhibition Spousal incompatibility Some patients may sleep too much Suicide Risk Demoralization Loss of Employment Station in life Independence Office set up Speech issues Supportive Therapy 29 30

6 Caretakers Summary Challenging but rewarding Many usual treatments will work The power of a narrative DEPRESSION and SUICIDAL IDEATION are NOT NORMAL in HD Additional Sources Understanding Behavior In Huntington s Disease: Additional Sources HD 101 CME Series 10 units of video based free cme A Guide For Professionals content/uploads/2015/03/understanding Behavior.pdf cme series/ 33 34