Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Cognitive Dysfunction In Patients with a Primary Brain Tumor: Exploring and Navigating Uncharted Waters Sandra A. Mitchell, CRNP, MScN, AOCN National Cancer Institute, Bethesda, MD Sherry W. Fox, PhD, RN, CNRN University of Virginia School of Nursing, Charlottesville, VA Margaret Booth-Jones, PhD Moffitt Cancer Center and Research Institute, Tampa, FL Objectives Analyze the domains of cognitive function. Identify and select tools/approaches for evaluating cognitive function in the clinic. Explain the indications for neuropsychological evaluation. Plan a program of support, accommodation, and rehabilitation for patients with a primary brain tumor who are experiencing cognitive dysfunction. Case #1: Janet 37 y/o right-handed, Caucasian married female Mother of 3 (ages 5, 9, and 10) 1 month s/p 80% resection of left frontotemporal oligodendroglioma (WHO grade 2, with elevated MIB-1 index) Considering XRT and/or chemotherapy vs. surveillance Partial motor seizures, controlled on Dilantin College educated, and working part-time at a public school No prior medical or psychiatric history Patient reporting depressed mood, increased tearfulness, reduced energy, and word-finding difficulties Husband is concerned about her mood and ability to accomplish daily tasks, including caring for their 3 children Case #2: Bernie 58 y/o ambidextrous Israeli male. Married to his second wife 2 adult children from first marriage 2 weeks s/p gross total resection of a right frontal Glioblastoma Multiforme (GBM) (WHO grade 4) Scheduled to begin treatment with XRT and concurrent temozolomide Currently prescribed Dilantin, Decadron and Anzemet No previous psychiatric history; history of HTN Has an MBA and is working as an executive in a major corporation – currently on sick leave Patient denies emotional distress or cognitive problems Wife and adult children are very concerned about his change in personality and decision making abilities Factors Contributing to Neurobehavioral Changes Associated with Brain Tumors Location of the tumor Pathologic type Patient characteristics FRONTAL •Personality changes (impulsivity, lack of inhibition, lack of concern) •Delayed initiation/apathy •Executive dysfunction •Diminished self-awareness of impaired neurologic or neuropsychological functioning (anosognosia) •Language deficits TEMPORAL •Auditory and perceptual changes •Memory and learning impairments •Aphasia and other language disorders PARIETAL •Somatosensory changes •Impaired spatial relations •Hemispatial neglect •Homonymous visual deficits •Agnosia (non-perceptual disorders of recognition) •Language comprehension impairments •Alexia (disorders of reading) •Agraphia (disorders of writing) •Apraxia (disorders of skilled movement) OCCIPITAL •Alexia (disorders of reading) •Homonymous hemianopsia •Impaired extraocular muscle movements •Color anomia •Achromatopsia (impairment in color perception) CEREBELLUM •Ataxia CORPUS CALLOSUM •Transmission of visual information •Integration of sensory input •Transmission of somatosensory information BRAINSTEM •Diplopia •Altered consciousness and attention •Cranial neuropathies (visual field loss, dysarthria, impaired extraocular muscle movements) Factors Contributing to the Neurobehavioral Changes Associated with Brain Tumors Pathologic type Low grade histology High grade histology Patient characteristics Age Physical co-morbidities Psychological co-morbidities Symptom experience Fatigue Pain Factors Contributing to the Neurobehavioral Changes Associated with Brain Tumors Adverse effects of treatment Side effects of adjunctive medications surgery radiation therapy chemotherapy corticosteroids, anticonvulsants psychoactive medications Medical complications endocrine dysfunction seizures infection anemia sleep disorders Effects of Cognitive Dysfunction on Patient, Family and Health Care Team Physical, psychological, social and vocational functioning Level of distress Quality of individual and family life Insight and self-appraisal Self care abilities, decisionmaking and treatment adherence Cognitive Function Cognitive function encompasses the processes by which sensory input is elaborated, transformed, reduced, stored, recovered and used. Domains of Cognitive Function Attention and concentration Visuo-spatial and constructional skills Sensory perceptual function Language Memory Executive function Intellectual function Mood, thought content, personality and behavior Source: Halligan, Kischka & Marshall, 2003 AttentionCapacity to Detect and Orient to Stimuli Prioritize signals from one spatial location Prioritize some forms of information and to suppress others on the basis of a functional goal spatial attention selective or focused attention Self maintain an alert and ready-to-respond state arousal/sustained attention ConcentrationDirecting Thoughts and Actions Toward a Stimulus Capacity- refers to the amount of information processing a person can do in a given time Control refers to an individual’s ability to direct concentration capacities. Concentration exists in three forms: sustained concentration focused concentration(selective) divided (alternating) Distractions environmental – external self – internal Visuospatial and Contructional Skills Apraxia Difficulty performing a planned motor activity in the absence of paralysis of the muscles normally used in the performance of that act. Can also be considered a disorder of language as many procedural tasks are verbally mediated. Visuospatial and Contructional Skills Apraxia Ideational apraxia Ideomotor apraxia Dissociation between the areas of the brain that contain the ideas for movements and the motor areas that actually execute the movements. Constructional apraxia Basic sequence of events and logical plan underlying a chain of simple actions is disrupted Inability to produce properly organized constructions such as drawings or simple building tasks Motor apraxia Not generally reported by patient, but family will often describe difficulty with using common objects (toothbrush, eating utensils). Visuospatial and Constructional Skills Loss of topographical memory Inability to find the way and tendency to become lost in familiar and unfamiliar environments Visuospatial and Constructional Skills Apraxic agraphia poor letter formation spatial distortions patient/family report illegible handwriting Visuospatial and Constructional Skills Alexia (difficulty reading) may occur as a result of an inability to perform the continuous and systematic scanning eye movements necessary for reading may also be considered a language deficit Visuospatial and Constructional Skills Acalculia (difficulty with calculation) may result from misplacement of digits, misalignment of columns, or aphasia for number symbols Sensory-Perceptual Function Distinction between sensation and perception: The senses capture information from the environment Subsequent elaborations and interpretations in different parts of the brain enable one to perceive or become aware of external stimulation The most common perceptual deficits are: auditory tactile visual Factors Influencing Evaluation of Sensory-Perceptual Function Underlying primary sensory deficit (eg. color blind at baseline, hypoacusis at baseline secondary to age-related hearing loss) Advancing age may diminish senses and dull perception The state of the perceiver(e.g. anxiety, physical discomfort) may influence the perception of a stimulus Severe language problems can impair a patient’s ability to respond appropriately to tests of sensory function Common Sensory-Perceptual Deficits in Patients with a Primary Brain Tumor Agnosia- literally, without knowledge, inability to know or interpret sensory experiences Tactile agnosia (inability to name common objects placed in one hand). Place a common object such as coin (dime, nickel, quarter), paper clip, pen, randomly in either hand. If patient is aphasic, they will have difficulty naming objects placed in either hand. When they have a specific difficulty in naming objects palpated with only one hand, tactile agnosia or astereoagnosis is present. Anosognosia Lack of awareness of impaired neurologic or neuropsychological function which is obvious to the clinician and other reasonably attentive individuals. Common Sensory-Perceptual Deficits in Patients with a Primary Brain Tumor Diplopia (double vision) Visual field deficits (hemianopia, quadrantanopia) Common Sensory-Perceptual Deficits in Patients with a Primary Brain Tumor Achromatopsia - impairments in color perception Color anomia - inability to name colors or to select a color from an array of colors when requested Common Sensory-Perceptual Deficits in Patients with a Primary Brain Tumor Visual hallucinations (photopsia) stars, dots, lines, fog, wavy lines Illusions (metamorphopsia) distorted objects, faces, scenes Common Sensory-Perceptual Deficits in Patients with a Primary Brain Tumor Alexia (reading difficulties) words or syllables missing change of lines, or reduced reading span (hemianopic alexia) Common Sensory-Perceptual Deficits in Patients with a Primary Brain Tumor Problems with figure-ground discrimination Problems in estimating depth on a staircase or reaching for a cup/door handle Bumping into obstacles or failure to notice persons on one side (hemispatialneglect, hemianopia) Difficulty detecting the movements of targets in space - visual scenes may appear as a series of static snapshots Language Aphasia/dysphasia language production (expressive aphasia/dysphasia) language comprehension (receptive aphasia/dysphasia) May be accompanied by alexia (loss or impairment of the ability to read) and/or agraphia (loss or impairment of the ability to produce written language) Language Dysarthria sensorimotor disorder affecting the respiratory and articulatory functions involved in speech sound production speech may be garbled, slurred or muffled, while grammar, comprehension, and word choice are intact Dysprosody interruption of speech inflections and rhythm (i.e. speech melody) resultant monotone or halting speech Evaluation of Spontaneous Speech Can communication be established? Does the patient produce speech at all? Is the patient's speech comprehensible (if not, is it because of semantic errors or because of dysarthria)? Is the patient's speech fluent or nonfluent? Are there semantic errors? Language Production Difficulties Pauses, hesitancy Restricted range of vocabulary Use of circumlocutions Discontinuation of a phrase Substitution of a presumablyintended word by another word (verbal paraphasia) Substitution of a presumablyintended word by a meaning related word (semantic paraphasia) Difficulty with grammatical construction Telegraphic speech style Language Production Difficulties Repetitive speech Automatisms Perseveration Stereotypy Language Comprehension Difficulties Difficulty following multistep commands Problems comprehending television or movies, difficulties reading, working on the computer or participating in conversation May be difficult to differentiate from problems with attention, and can overlap with stress and fatigue May lead to conflict and frustration in families Memory Remote memory (memories from childhood and early adulthood) usually preserved Recent memory Recall is uncued information retrieval Recognition is cued information retrieval in which the individual “remembers” by selecting from a number of pieces of information, including the target information Memory Loss Symptoms Examples of memory loss symptoms: Forgetting a message Losing track of a conversation Forgetting to do things Forgetting what has been read or events in movies/TV programs Inability to navigate in familiar places Memory Loss Symptoms Assess: Severity? Onset gradual or sudden? Memory impaired consistently or only on occasions? Fluctuation in severity? Is it an isolated symptom or are there other cognitive impairments? How is it affecting work or pastimes? Executive Function Adaptive abilities that enable us to: analyze what we want develop and carry out a plan Executive Function Establish new behavior patterns and ways of thinking about and reflecting upon our behavior Understanding of complex social behavior such as understanding how others see us, being tactful or deceitful. Burgess et al (2000) Executive Dysfunction Difficulties with abstract thinking, planning, decisionmaking Difficulty with goal formulation Difficulty with complex, multistage tasks Poor temporal sequencing Problems with reasoning and problem-solving Difficulty with carrying out everyday routine activities (eg. making a cup of tea, brushing teeth, dressing) Executive Dysfunction Lack of insight Distractibility Marked reduction in spontaneous purposeful activity Confabulation Perseveration Lack of concern Shallow affect, impulsiveness, disinhibition, aggression, unconcern for social rules Mood,Thought, Personality, Behavior Mood Thought content and processes Baseline personality and coping style Behavior Case #1: Janet 37 y/o right-handed female, status post 80% resection of left frontotemporal oligodendroglioma (WHO grade 2, with elevated MIB-1 index). Considering XRT and/or chemotherapy vs. surveillance. Partial motor seizures, controlled on Dilantin Patient reporting depressed mood, increased tearfulness, reduced energy, and word-finding difficulties. Husband is concerned about her mood and ability to accomplish daily tasks, including caring for their 3 children Case #1: Janet- Clinical Issues Cognitive function Short-term memory problems Frustrated by problems with expressive dysphasia Diminished initiative, feels somewhat apathetic Executive dysfunction: Problems with planning Overwhelmed by complexities of busy household Diminished mental concentration Overlay of: Fatigue Depression Side effects of anticonvulsants Case #2: Bernie 58 y/o ambidextrous male, status post gross total resection of a right frontal Glioblastoma (WHO grade 4). Scheduled to begin treatment with XRT and concurrent temozolomide Currently prescribed Dilantin, Decadron and Anzemet Family concerned about personality changes and decisionmaking capacities. Patient denies any current concerns. Case #2: Bernie- Clinical Issues Clinical Issues: Cognitive dysfunction: Mild short term memory problems Markedly diminished mental concentration Personality changes (impulsive, lacking tact, easily frustrated) Anosognosia (diminished awareness of impaired functioning) Overlay of: Cultural factors Side effects of steroids (patient is not sleeping) Situational anxiety Cognitive Screening: Clinical Context Evaluation of brain function Occurs with each verbal and non-verbal interaction with a patient Screening may be formal or informal Screening may also be conscious or unconscious Screening may be part of a professional or a social interaction Cognitive Screening: Clinical Context Cognitive impairment is common in persons with primary brain tumors (Fox, et al., 2004; Tucha et al., 2000) Cognitive impairment may have different patterns according to tumor types and treatment Caregivers or informant descriptions of cognitive decline, should be taken seriously and cognitive assessment and follow-up initiated (Guideline, 2001; Patterson & Glass, 2001) Nursing Implications for Cognitive Screening in the Clinic To identify issues in decision-making To identify ways to improve quality of life To identify best methods to assist caregivers To identify a changing illness trajectory To promote safety for the patient To improve the patient/nurse relationship To facilitate effective advocacy Evidence Supporting Cognitive Screening Patients with mild cognitive impairment should be recognized and monitored for decline due to their increased risk for subsequent dementia (guideline). General cognitive screening instruments should be considered for the detection of dementia (guideline). Interview based techniques may be considered in identifying patients with dementia, particularly in an at-risk population (option). American Academy of Neurology Guidelines on Early Detection of Dementia and Mild Cognitive Impairment, (2001) Patterson & Glass (2001) Screening for Cognitive Impairment and Dementia in the Elderly Linking Nursing and Neurocognitive Assessments Interview with client and observations of client during the interview are essential Identification of fund of knowledge based on age, culture, and education provide a basis for accurate evaluation and screening Obtaining a history of the person provides invaluable clues to future assessment of cognition Global Assessments During Interview and History Orientation - alertness and awareness of time, place, person and situation at all times Communication - ability to speak, understand, and respond appropriately, speech patterns Judgment - insight into self and situation direct decision making Global Assessments During Interview and History Appearance and Behavior General intelligence level of education, fund of knowledge Mood attire, grooming, appearance reactions to the topic being discussed general perspective about situation, i.e. sad? angry? Visuospatial ability attention given to visual cues attends to both right and left sides Factors Effecting Patterns of Cognitive Impairment Age Medical History Tumor progression and location Fatigue Depression Treatments, particularly radiation Drug therapy such as steroids, anticonvulsants, complementary therapies Goals of Clinical Cognitive Screening To assess multiple areas of cognitive function quickly To identify areas of cognitive dysfunction To screen in such a way that the results are reliable, valid and clinically relevant for patient care, safety and self-esteem To be practical about what is possible in the setting and the patient population Ideal Screening Instrument Characteristics Can be administered by clinicians at all levels and requires 5-15 minutes to administer to most patients Orientation, attention/concentration, executive, language, spatial, and memory functions included Acceptable sensitivity with disorders commonly encountered by neuroscience clinicians Mallory, et al., (1997) Evidence-Based Cognitive Screening Instrument Recommendation #1 Mini-Mental State Exam (MMSE) (Folstein, Folstein & McHugh, 1975) or Modified Mini-Mental State Exam American Academy of Neurology Guidelines on Early Detection of Dementia and Mild Cognitive Impairment (2001) Mallory, et al., (1997) Mini-Mental State Exam (MMSE) Tests orientation, registration, attention and calculations, recall and language Takes approximately 12-15 minutes to administer Scores added for a single number score Deals with communication Answers to individual questions may have more value than the single score Score of 23/24 out of 30 possible points suggests significant cognitive dysfunction or possible dementia Issues with the MMSE May be insensitive to mild cognitive impairments May be insensitive to impairments from lesions in the right hemisphere No measure of visual perceptual deficits False positives are reported in those of advanced age and low educational levels Evidence-Based Cognitive Screening Instrument Recommendation #2 Neurobehavioral Cognitive Status Examination (NCSE)(Cognistat)(Kiernam, et al., 1987; Mueller, 1984) Abdulwadud, (2002) Mallory, et al., (1997) Neurobehavioral Cognitive Status Examination (NCSE)(Cognistat) Provides data in ten areas including LOC, orientation, attention, communication, memory, constructional ability, calculations, reasoning, abstracting and similarities. Takes 30-45 minutes to administer Several questions are specific to screening Relies on communication and language skills Useful for evaluating ability to complete complex tasks Useful in identifying cognitive impairment in persons with focal neurologic lesions When to Refer for a Formal Neuropsychological Examination When patient requests assessment or expresses concerns When family members express concern When a physician or other health care provider needs a baseline or notices cognitive changes When a rehabilitation counselor or therapist needs a comprehensive baseline When documentation of disability or accommodation is required When competency is an issue When there are issues of placement in a rehabilitation or adult living facility Patient or Family Request Many patients are information seekers and are concerned about their brain function Subjective ratings of cognitive ability can be distorted and can lead to significant distress Patients and families may want a baseline to help make decisions regarding further treatment for their brain tumor Some brain tumor patients are unaware of their cognitive, emotional, and personality changes (anosognosia – related to frontal lobe dysfunction) Some family members want testing to assist with regaining a specific function: ability to drive, return to work, or live alone Health Care Provider Request pre-surgical assessments laterality and pre-surgical deficits language assessment prior to awake craniotomy post-surgical assessment - rehabilitation and recovery pre-chemotherapy baseline during chemotherapy if cognitive and / or emotional changes are observed pre-radiation therapy to obtain a baseline to address concerns for radiation induced dementia during or after radiation if delirium or cognitive decline observed Other Referrals for Testing Multidisciplinary rehabilitation may require neuropsychological assessment for admission Schools and employers may require neuropsychological testing and documentation to return to school or work and to receive necessary accommodations Brain tumors are not automatically considered a disabling condition and insurance companies and Social Security may require neuropsychological testing and documentation Competency to make treatment decisions may be in question and may require testing and documentation How to Present the Idea of a Neuropsychological Exam Some patients associate psychology or psychiatry with “being crazy” and resist the referral Some patients are concerned that they will appear stupid or be emotionally traumatized in some way Patients should be told that a neuropsychological evaluation is an assessment of brain function and a determination of strengths and weaknesses that will allow for more comprehensive treatment planning Neuropsychological testing is not painful or invasive Neuropsychological test is not an “IQ test” The Neuropsychological Exam Clinical interview with patient and with a family member when possible Behavioral observation Estimate of premorbid function Brief, repeatable battery of tests assessing cognitive domains Assessment of mood and quality of life Feedback to patient, family, and referring physician Documentation Referral to appropriate services Follow-up to determine change over time Testing Considerations Determine patient’s sensory limitations Determine patient’s language ability visual field cuts or diplopia hearing loss from aging or chemotherapy, peripheral neuropathy expressive – providing answers receptive – understanding the demands of the tasks Limit testing to 1-2 hours to minimize fatigue Neuropsychological Testing Brief well-validated measures: attention, concentration and vigilance verbal learning and verbal memory visuospatial function language – fluency, naming, reading executive function – problem solving, reasoning, susceptibility to interference psychomotor speed and stamina Appropriate psychosocial measures: emotional distress quality of life RBANS List Learning Immediate Memory Domain RBANS Story Memory Immediate Memory RBANS Figure Copy Visuospatial/Construction Domain RBANS Figure Recall Delayed Memory Domain RBANS Line Orientation Visuospatial/Construction Domain RBANS Picture Naming Language Domain RBANS Semantic Fluency Language Domain RBANS Digit Span Attention Domain RBANS List Recall Delayed Memory Domain RBANS List Recognition Delayed Memory Domain RBANS Coding Attention Domain Trailmaking Test – Trails A Executive Function Trailmaking Test – Trails B Executive Function Janet’s Test Profile Verbal memory (list learning and story memory) impaired Recognition better than recall for delayed memory Visuospatial/constructional ability intact Attention impaired characterized by slow responding but free of errors Language function significant for reduced fluency and impaired naming Executive function characterized by slowing and reduced effortful output Questionnaire information and clinical interview significant for symptoms of clinical depression Bernie’s Test Profile Verbal memory (list learning and story memory) mildly impaired and significant for intrusion errors and perseverations Recognition equivalent to recall for delayed memory Visuospatial/constructional ability impaired and figure is distorted Attention impaired characterized by increased distractibility and a high error rate Language function significant for loss of set during the fluency task and circumlocution errors on naming Executive function characterized by poor set shifting, loss of set, and increased susceptibility to interference Questionnaire information and clinical interview not significant for symptoms of clinical depression or clinical anxiety Providing Feedback Discuss findings with patient and family members at the end of the exam in real time Provide strengths and weaknesses in an educational, supportive manner Explain the findings in terms of the relationship to the tumor, the treatment and to activities of daily living Connect neuropsychological findings directly to brain function and brain location Provide appropriate treatment options and referrals Plan follow-up re-evaluation Neuropsychiatric Referral Neuropsychological evaluation may identify emotional and behavioral symptoms requiring medication Neurobehavioral slowing, problems with concentration, or apathy – consider stimulant medication such as methylphenidate (Ritalin) or modafinil (Provigil) Depressed mood – consider antidepressant Primary memory deficit – consider memory enhancing medication such as Aricept or Memantine Sleep disturbance, appetite decline, and behavioral changes from steroids require referral Unmodulated mood and behavioral irritability may require a mood stabilizer such as Depakote or Gabitril Principles of Cognitive Rehabilitation and Accommodation Systematically evaluate cognitive function at regular intervals (Meyers et al, 2000) Set specific goals for restoration, substitution or restructuring of environment (Lazar, 1998) Include rehabilitative disciplines (Lazar, 1998) Consider role for pharmacologic agents (Barton & Loprinzi, 2002; Chan et al, 2003; Meyers et al, 1998) Evaluate for and remediate co-morbidities, including fatigue, depression, anxiety, insomnia, and physiologic discomfort (Litofsky et al, 2004) Principles of Rehabilitation Three types of rehabilitative approaches are typically included: Restoration: cognitive training and exercises directed towards strengthening and restoration of function Substitution: compensatory devices and strategies directed towards substitution of lost functions and promoting conservation of affected brain functions Restructuring: environmental restructuring and planning to promote improved functioning by changing the demands placed on the individual by themselves and others Anticipatory Guidance Consider support group, online support, counseling resources available through the NBTF and ABTA, individual and family counseling Make of list of things that others can do to help the caregiver, and keep the list by the phone to consult when friends call to ask how they can help Expect that mood disorder, particularly depression is present and contributing to cognitive difficulties (Litofsky et al, 2004) Provide explanations and information that help link emotions, and changes in behavior and functioning to the tumor site and treatment Help the patient and family anticipate the trajectory of the illness, and plan for the next phases - end of life decision-making, articulate wishes, and fulfilling desired short term goals Help the patient maintain who they are and the roles that are important to them by suggesting alternatives, adaptation, accommodation and problem solving Maintain patient involvement and dignity, despite limitations Sherwood et al, (2004) Psychological Support Psychoeducation specific to cognitive and emotional changes associated with brain tumor and treatment Cognitive/behavioral strategies to help with relaxation, reduce frustration Compensatory strategies to enhance memory and concentration Activity pacing techniques to assist with fatigue and stamina issues Individual and family therapy to address adjustment and role issues Future Directions Practice More refined evaluation and description of the nature of cognitive dysfunction Deliberative intervention/remediation/support Timely referral to multidisciplinary experts Program Planning Advocate for improved access to neuropsychological evaluation, and cognitive rehabilitation Systematically evaluate patients at regularly scheduled intervals to document progress and adjust the plan Education Develop skills in assessing, describing aspects of cognitive functioning Expand the knowledge base of intervention techniques and approaches Research Agenda Instrument Refinement and Psychometric Evaluation- brief, clinically useful, valid and reliable measures of cognitive function Prevalence, incidence, correlates, and sequelae of cognitive dysfunction Evaluate the relative contributions of mood disturbance, insomnia, fatigue, and physiologic discomfort Research Agenda Develop, test and refine intervention approaches targeted to: remediate or substitute specific aspects of cognitive dysfunction (eg. language, memory) global aspects of cognitive dysfunction programs of patient and family support and adjustment Research Agenda Evaluate the effects of pharmacologic therapies for disorders of mood (anxiety, depression), attention, wakefulness, and memory on cognitive function and quality of life Research Agenda Evaluate the effects of complementary and mind-body therapies (relaxation, exercise, music, humor, nutrition, rest/sleep) on cognitive function