Neuropsychology of Huntington's Disease
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Questions and Answers

Which cognitive domain is NOT listed as being affected in Huntington's Disease?

  • Memory
  • Executive function
  • Emotional recognition (correct)
  • Speed of processing
  • What is anosognosia in the context of Huntington's Disease?

  • Enhanced communication skills
  • Increased speed of cognitive processing
  • Loss of insight regarding one's condition (correct)
  • The ability to multitask effectively
  • How do individuals with Huntington's Disease typically view their symptoms compared to their caregivers?

  • They report more symptoms than their caregivers do.
  • They often underestimate their abilities.
  • They report fewer symptoms than their caregivers. (correct)
  • They have a clear understanding of their condition.
  • What is the significance of having an 'informant' present during mental wellbeing assessments for individuals with Huntington's Disease?

    <p>It improves the accuracy of the assessments.</p> Signup and view all the answers

    What aspect of cognitive flexibility is emphasized concerning Huntington's Disease?

    <p>Switching from one task to another</p> Signup and view all the answers

    What is one of the main focuses of neuropsychology?

    <p>Recognizing difficulties associated with neurological conditions</p> Signup and view all the answers

    Which of the following is NOT a learning objective stated for the session?

    <p>Evaluate the effectiveness of therapy dogs in clinical settings</p> Signup and view all the answers

    What type of neurological conditions will be discussed as causing distressing symptoms?

    <p>Neurological conditions that are life-limiting</p> Signup and view all the answers

    What is a potential associated issue discussed in relation to poor mental wellbeing?

    <p>Self-harm and suicidal thoughts</p> Signup and view all the answers

    Which population might neuropsychologists typically work with?

    <p>Individuals experiencing neurological conditions</p> Signup and view all the answers

    In which context will neuropsychologists apply their understanding during interventions?

    <p>Systemic contexts supporting neurological presentations</p> Signup and view all the answers

    What is a consequence of functioning with neurodegenerative conditions?

    <p>Gradual decline in cognitive and functional abilities</p> Signup and view all the answers

    Which of the following best describes functional neurological conditions?

    <p>Disorders where psychological factors significantly impact neurological function</p> Signup and view all the answers

    What was observed during the first six months following a significant loss according to the Kubler-Ross model?

    <p>High anxiety decreasing towards the end of the first year</p> Signup and view all the answers

    In the context of aging, what cognitive change typically begins after age 50?

    <p>Cognitive deterioration in various areas</p> Signup and view all the answers

    What peak in depressive symptoms was noted in relation to significant loss?

    <p>Around the 20th month</p> Signup and view all the answers

    What term describes the approach that addresses common difficulties across various conditions?

    <p>Transdiagnostic approaches</p> Signup and view all the answers

    Which cognitive ability is noted to show limited exceptions despite general decline in older adults?

    <p>Verbal ability</p> Signup and view all the answers

    How might therapy assist individuals coping with loss and changes in functioning?

    <p>By promoting adjustment and adaptation over time</p> Signup and view all the answers

    How many people aged 65 and older were reported in the UK as of the study, and what is the prediction for 2050?

    <p>12.3 million, rising to 25% by 2050</p> Signup and view all the answers

    What common memory issues do older adults face?

    <p>Worsened ability to memorize and retrieve information</p> Signup and view all the answers

    Which factor is associated with anxiety in the mental health difficulties observed?

    <p>Inability to relax</p> Signup and view all the answers

    What group showed consistent differences in symptoms of self-harm?

    <p>Manifest group</p> Signup and view all the answers

    Which symptom was reported as the most surprising finding across the groups?

    <p>Self-harm</p> Signup and view all the answers

    Which two factors were reported under depression difficulties?

    <p>Not enjoying life and Not looking forward to the future</p> Signup and view all the answers

    What was the observed difference in anxiety symptoms across the groups?

    <p>No difference between groups</p> Signup and view all the answers

    Which group experienced more significant difficulties with outward irritability?

    <p>Manifest group compared to genotype negative</p> Signup and view all the answers

    How many distinct factors were identified across the groups regarding mental health difficulties?

    <p>Four</p> Signup and view all the answers

    What was the overall conclusion regarding the differences in mental health difficulties between the groups?

    <p>Far less difference than expected was observed</p> Signup and view all the answers

    What is the primary purpose of defusion in ACT?

    <p>To detach from thoughts, emotions, and sensations</p> Signup and view all the answers

    How does ACT define acceptance?

    <p>Making space for distressing thoughts and emotions</p> Signup and view all the answers

    What metaphor is commonly associated with defusion in ACT?

    <p>Passengers on the Bus</p> Signup and view all the answers

    What is meant by the concept of 'dirty' pain in ACT?

    <p>Pain that is exacerbated by thoughts and beliefs</p> Signup and view all the answers

    Which metaphor is used to engage clients in ACT without being technical?

    <p>Dropping the Rope</p> Signup and view all the answers

    Which of the following does ACT assert about pain?

    <p>It is a necessary part of life</p> Signup and view all the answers

    What is the significance of metaphors in ACT?

    <p>They help simplify complex concepts</p> Signup and view all the answers

    Which of the following statements about ACT is TRUE?

    <p>It encourages acceptance of difficult experiences</p> Signup and view all the answers

    What is a primary focus of Acceptance and Commitment Therapy (ACT)?

    <p>Promoting valued living and enjoyment of life.</p> Signup and view all the answers

    Which of the following is NOT a way to support individuals affected by Huntington's Disease (HD)?

    <p>Focusing solely on detecting potential cures.</p> Signup and view all the answers

    What type of therapy is especially noted for having over 700 randomized controlled trials supporting its efficacy?

    <p>Acceptance and Commitment Therapy (ACT).</p> Signup and view all the answers

    What does the 'hot cross bun' model in CBT represent?

    <p>The relationship between thoughts, feelings, bodily sensations, and behaviors.</p> Signup and view all the answers

    What is a critical aspect of the approach taken by neuropsychologists when working with clients?

    <p>Accepting that clients may not recover and addressing their psychological needs.</p> Signup and view all the answers

    How can understanding mental wellbeing in patients with HD contribute to their care?

    <p>It helps improve care and provides confidence to mental health professionals.</p> Signup and view all the answers

    In working with individuals affected by HD, what aspect is essential for caregivers to learn?

    <p>Positive engagement and strategies for different coping mechanisms.</p> Signup and view all the answers

    What distinguishes the focus of ACT from traditional cognitive-behavioral therapy approaches?

    <p>ACT centers on valuing life and acceptance of difficulties rather than 'fixing'.</p> Signup and view all the answers

    When supporting families affected by HD, what is a crucial area to address?

    <p>Improving support through understanding their psychological needs.</p> Signup and view all the answers

    Which aspect of mental health care is crucial for non-specialized mental health professionals working with HD patients?

    <p>Providing confidence in their therapeutic approaches.</p> Signup and view all the answers

    Study Notes

    Applied Neuropsychology

    • Lecturer: Dr Sarah Gunn
    • Email: [email protected]
    • Contact details for student wellbeing service available

    Content Label

    • Neurological conditions causing painful/distressing symptoms (life-limiting)
    • Difficulties associated with poor mental wellbeing (including self-harm and suicidal thoughts)
    • Support available for distress during sessions

    Learning Objectives

    • Understand the context of neuropsychology (who and how it relates to work)
    • Recognize difficulties associated with neurological conditions and symptoms
    • Describe the impact of neurological issues in typical aging, functional neurological conditions, and neurodegenerative conditions
    • Explore examples of clinical interventions
    • Apply an understanding of systemic contexts to support people with neurological presentations

    Plan for Today

    • Welcome
    • What is neuropsychology?
    • Who do neuropsychologists work with and how?
    • What difficulties may people with neurological conditions experience?
    • Neuropsychology and typical aging, Huntington's disease, and functional neurological conditions
    • Examining a specific clinical intervention in more detail

    Why am I speaking?

    • Teaching neuropsychology, aging, long-term conditions, and research methods
    • Researching neurological conditions and impacts on wellbeing
    • Clinical psychologist in Medical Psychology, working with people with neurological difficulties in an NHS trust
    • Developing neurological therapies and working in residential/community care

    What is Neuropsychology?

    • Study of the brain and its impact on emotions, behaviour, and cognition
    • Supports people with neurological diagnoses/symptoms and personal adjustments
    • Provides support for family members/carers

    Who do Neuropsychologists work with?

    • People with acquired brain injury (trauma, stroke, tumor, or post-surgical injury)
    • People with degenerative neurological conditions (dementia, neurodegenerative conditions)
    • People with functional neurological disorders
    • Families, carers, and organisations supporting individuals

    Neurological Conditions and Distress

    • Severity varies (from prolonged disorder of consciousness to the maintenance of previous activities)
    • Distress can result from biological/physical changes, social changes, and psychological changes
    • Systemic bias in research/therapy needs addressing

    Neurological Conditions and Distress: From the Literature

    • Psychosocial and behavioural difficulties frequently follow acquired brain injury, lasting for a decade or more
    • Common difficulties include self-isolation, reduced social skills, emotional lability, reduced empathy, apathy, suicide attempts, and self-harming behaviour (often worsened with communication difficulties)

    Neurological Conditions and Distress: From the Literature (2)

    • In neurodegenerative conditions, psychological difficulties include depression, anxiety, irritability, aggression, apathy, perseveration, hallucinations, disorientation, paranoid thinking, and obsessive-compulsive behaviours
    • Cognitive symptoms can further complicate these issues; heritability, family conflict, and intergenerational trauma are important factors

    Possible Biological Changes

    • Brain damage to emotion centers (limbic system, amygdala)
    • Hormone imbalances (e.g. hypothalamus/pituitary)
    • Pain (sensory or physical)
    • Mobility changes (e.g. seizures)
    • Reduced energy/fatigue/sleepiness

    Possible Social Changes

    • Loss of roles/responsibilities
    • Financial implications
    • Lessened ability to understand and cope with social interactions – avoidance of others
    • Reactions from others worsening the situation

    Possible Psychological Changes

    • Shock (sudden change in self-identity, expectations, or narrative)
    • Frustration, anger, denial
    • Low mood (depression, hopelessness) from loss, lifestyle/role adjustments, and changing self-image or future plans
    • Anxiety
    • Low motivation

    The "Change Curve" with Multiple Sclerosis

    • Multiple sclerosis (MS) is a progressive neurological condition with no cure
    • Evidence from a small study suggests that the experience follows the Kubler-Ross model, with high anxiety initially, decreasing over the first year, followed by increased depression towards 20 months
    • However, the study highlights an ongoing need for further research

    All these difficulties are transdiagnostic...

    • These difficulties are commonly experienced by individuals with both physical health and neurological conditions
    • Therapy can be helpful in managing loss, adjusting function, and working through the process of adaptation

    "Typical" Ageing

    • Older adults experience increasing cognitive deterioration after age 50
    • In the UK, this affects large numbers of people (12.3 million in 2019), with expected numbers increasing even further
    • Cognitive skills decline, which significantly impacts wellbeing

    As We Age, We Start to Notice Difficulties

    • Memory issues, including remembering appointments and medication
    • Difficulty concentrating and filtering distractions, affecting multitasking
    • Slower processing speed, impacting conversations, driving, and daily tasks
    • Complex cognitive abilities like problem-solving and planning become more difficult

    How does that experience feel?

    • Understanding objective cognitive changes is better understood than the emotional experiences
    • This requires more research
    • Possible concerns include: fear, embarrassment, shame, or reminding of bad memories (e.g., concerning relatives or past scenarios).

    Shame and Stigma in Cognitive Ageing

    • Cognitive difficulties evident to others may cause issues
    • Participants in groups report embarrassment, with some displaying shame or avoiding potentially embarrassing situations
    • This links to individuals with dementia and views about themselves and their capabilities

    Shame and Stigma in Cognitive Ageing (2)

    • Stigma is an important concern for older adults with dementia, with a significant number (24%) expressing fear of stigma and 40% feeling excluded from society
    • Existing approaches to support older adult wellbeing are inadequate, and new models are needed

    "Typical" Ageing (Distressing)

    • People worried about their cognitive functions frequently present in neuropsychological settings
    • Feeling of being validated and respected is a priority, as a lack thereof may suggest an underlying issue of a non-diagnosable condition

    "Functional Neurological Disorder"

    • Synonym for functional neurological symptom disorder, functional movement disorder, conversion disorder, psychogenic seizures, dissociative seizures, and non-epileptic seizures

    Functional Neurological Issues

    • Physical difficulties (weakness, paralysis, tremors, spasms, etc)
    • Sensory disruption
    • Possible seizures, blacking out, fainting

    Possible Contributing Factors

    • Previous trauma
    • Emotion regulation difficulties
    • Expression of psychological distress as physical symptoms (somatisation)
    • Low mood/anxiety
    • Stressful life events
    • Experiencing epilepsy/family member's experience

    From the Client's Perspective

    • Clients often worry about the cause of symptoms that might relate to various potential conditions (including neurological issues)
    • Therapy aids in understanding the role of stressors, low mood, anxiety, and systemic difficulties in one’s wellbeing

    Stigma against people with these difficulties

    • Stigma against people with functional neurological difficulties can impact diagnosis, treatment, and research
    • People with these issues experience frustration with providers and distressing healthcare interactions
    • Further research is required to investigate the prevalence and context of this

    A Break and Short Mindful Exercise

    • A break is provided for participants in sessions
    • A mindful exercise is suggested

    Huntington's Disease (HD)

    • Rare, life-limiting neurological disease
    • Prevalence (about 10 in 100,000 people)
    • Caused by a CAG expansion on the HTT gene
    • Dominant gene, cannot be a carrier, passed from affected parent
    • Mutant huntingtin causes symptoms

    HD Onset

    • Motor symptoms (chorea, rigidity, bradykinesia) typically start between ages 30 and 50
    • Cognitive, behavioral, and emotional changes start far earlier than the physical symptoms

    Cognitive Changes in HD

    • Memory issues
    • Orientation problems
    • Processing speed issues
    • Executive function impairment (inhibition difficulties, inflexibility, working memory challenges)
    • Anosognosia

    A Bit More on Insight in HD

    • Individuals with HD may have wide-ranging mental health difficulties
    • Communication or personality issues could influence their insight, and patients may underestimate their difficulties, while carers overestimate abilities
    • Predicting the future abilities of people with HD may be better done using reports from informants than the patients themselves

    Insight in HD (2)

    • Research question: Does having an informant present during psychological assessment improve the quality and accuracy of the assessment? This is evaluated using psychological symptom reports from four different groups: premanifest HD, manifest HD post-motor onset, genotype negative, and family controls.
    • Significant differences observed in the psychological symptom reports, particularly for apathy.

    What We Found: Insight is Complicated

    • Assessments of apathy may underestimate symptom severity if no informant is present
    • Presence of an informant improves scores (people with HD may underestimate their apathy levels, but someone who knows them well can adjust); thus, it is important that an informant is present during assessments to obtain a more accurate diagnosis.

    What We Found (Part 3)

    • Higher scores for scores related to both manifest and pre-manifest and genotype negative groups when an informant was present compared to those alone.
    • These fit with previous findings on differing experiences of irritability, as well as the fact that social undesirability of irritability might be an issue in this particular group.

    So We Know That...

    • Cognitive difficulties can significantly impact patients and their families.
    • HD is more than just an individual issue; it is a family/systemic issue
    • Next steps are considerations on how systems around individuals with neurological conditions, like HD, impact their experiences

    Family and Context in HD: Does it Matter?

    • HD is entirely determined genetically.
    • Mental health difficulties are frequently observed relative to other neurological diagnoses; however, these symptoms are not related to progression in the same way as cognitive and physical issues.
    • Examining HD-affected families (rather than just individuals) yields valuable insights into wider systemic impact

    So What Else May Be Causing Distress in HD?

    • Changes in narratives and expectations
    • Grief and loss
    • Worries about relatives
    • Financial stressors
    • Impacts on relatives without HD

    The Heritable Component of Living with HD

    • Children of affected parents have a 50% risk of inheriting HD
    • Genetic testing is available from age 18 onwards

    One More Study of Ours: Briefly

    • Participants with HD (including different stages of the condition) show similar mental health difficulties as those without the condition
    • Mental health difficulties were grouped into four categories: manifest HD, premanifest HD, genotype negative group, and family controls

    Four Factors, Consistent Across Groups

    • Factor 1: Anxiety (Dread, Inability to relax, Panic)
    • Factor 2: Depression (Not enjoying life, Can't find fun, Not looking forward to the future)
    • Factor 3: Outward Irritability (Loss of temper, Slamming doors)
    • Factor 4: Self-harm (Suicidal ideation, Self-harming)

    OK, But What About the Severity of Symptoms?

    • No observable difference regarding anxiety
    • Depression/irritability differ only between manifest and genotype-negative groups
    • Self-harm differences were observed uniquely in the manifest group
    • In general, the severity difference between groups was less noticeable than anticipated

    Why Does This Matter?

    • Understanding mental wellbeing aspects in HD can be used to improve care and find appropriate treatment options
    • Such insights create confidence in mental health professionals even if they are not specialising in HD
    • HD-affected families benefit from recognizing psychological needs and improved support options

    Back to Neurological Conditions in General

    • Neuropsychological settings face challenges due to incurable and worsening conditions
    • Finding other ways to support patients and families is key.

    What Kind of Support Can We Offer?

    • Provide therapeutic support
    • Support relatives/carers emotionally and practically
    • Co-work with others in the interdisciplinary team
    • Support with behavioural strategies, and adjust staff attitudes toward patients
    • Modeling positive engagement encourages coping mechanisms.

    Acceptance and Commitment Therapy (ACT)

    • Third-wave CBT focuses on understanding interrelations between thoughts, feelings, body sensations, and behaviours
    • Emphasis shifts from identifying problems to adapting to difficult situations and promoting a more valued life
    • This is helpful for neurological conditions due to its acceptance-based nature

    ACT - Key Points for Therapeutic Work

    • Accepting distressing thoughts, emotions, and sensations as part of life
    • Identifying "clean" and "dirty" pain
    • Living a valued life despite difficult experiences

    ACT - Key Points for Therapeutic Work

    • Choosing values and directing behavior toward their fulfillment
    • Examples of values include being a hard worker, caring for loved ones, and achieving goals
    • This can be helpful for individuals facing major life changes

    ACT - Key Points for Therapeutic Work

    • Identifying and supporting a person in setting goals, which are specific, measurable, achievable, realistic, and timely
    • Values can guide adjustments in life behaviors
    • The person can experience a richer and fuller life despite neurological conditions

    Any Questions?

    • Contact information for the lecturer and student wellbeing service is provided

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    Description

    This quiz explores the cognitive domains affected by Huntington's Disease, including key concepts like anosognosia and the importance of informants in assessments. Participants will delve into the implications of cognitive flexibility and mental wellbeing in individuals facing neurodegenerative conditions.

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