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Dementia Case Study

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Case study and overview on Dementia

Case study and overview on Dementia

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  • 1. DementiaA FamilyTragedyMark GronowskiMonday, 29 April, 13
  • 2. What is Dementia?• A brain disorder that produces widespread deterioration of mental functionsand social capabilities• A chronic condition that is usuallyprogressive, although the term can also beapplied to static conditions• A tragedy for the victim and for family andfriendsMonday, 29 April, 13
  • 3. Forms of Dementia• Alzheimer’s Disease (AD)• Vascular Dementia (VaD)• Frontotemporal Dementia(FTD)• Pick’s Disease• Dementia with LewyBodies• Creutzfeldt-Jakob Disease(CJD)• Huntington’s disease (HD)• Parkinson’s disease (PD)• Wernicke-KorsakoffSyndrome• And more..Monday, 29 April, 13
  • 4. Types of Dementia• More then half of all dementias are Alzheimer’sDisease (AD), followed byVascular Dementia (VaD)• AD and other forms of dementia are irreversible,however there are some that can be reversed withthe appropriate treatment• The assessment process, many of the symptomsand effects of the illness on the victim and familyare similar whatever the type of dementiaMonday, 29 April, 13
  • 5. Alzheimer’s Disease• AD is the 4th most common cause of death after heart disease,cancer and stroke• Gradual onset and continuous cognitive decline• Late-onset AD occurs at 65 years of age and up (majority)• Early-onset AD can occur between age 40 and 65 years• There is no cure for AD, however there are several treatments tohelp slow down progress of AD• Interventions• Anti-depressants / Antipsychotics• Nutrition, meaningful activities, and a familiar environmentMonday, 29 April, 13
  • 6. Early stage deficits• Usually the first symptom to emerge in AD and many otherdementias is forgetfulness• Increasing impairment of learning and short term memory• Impoverished speech• Shrinking vocabulary and decreased word fluency• Patient is aware of these difficulties and may avoid newsituations to minimize embarrassment or failure• May become depressed as they realize what’s happening• Difficulty with perception (agnosia) or executive movements(apraxia)Monday, 29 April, 13
  • 7. Middle stage deficits• Difficulties with executive functions• Abstract thinking gradually becomes difficult• Difficulty comprehending novel situations and subtlenuances of language and nonverbal expression• Frequent incorrect word substitutions (paraphasia)• Delusions and hallucinations• Personality changes• Rapid changes in emotion / agitation• Impaired long-term memoryMonday, 29 April, 13
  • 8. Late stage deficits• Profound anterograde and retrograde amnesia• Poor judgement and impulse control• Disinhibited behaviors are common• Loss of insight (Anosagnosia)• Alleviates depression• Extreme apathy and exhaustion• Loss of spontaneous speech / Mute• “Vegetative state”• Complete dependence on caregiversMonday, 29 April, 13
  • 9. Diagnosis• In general a longitudinal assessment demonstrating aprogression of cognitive deficits would be required to make adiagnosis• Number of test batteries or Wechsler Scales can be givenover time to assess progressive dementia• Rating scales and questionnaires can also be given atregular intervals to assess daily functioning• A history, physical examination, lab tests and brain imagingshould be conducted• Assess whether a specific organic factor is present that isetiologically related to the specific type of dementia• Excludes other specific dementiasMonday, 29 April, 13
  • 10. Assessment• MMSE (Mini Mental State Examination) is the most common measureof global cognitive decline• Shows any decline in cognitive functioning, particularly in memoryfunction• Single-photon emission CT (SPECT) measures regional cerebralblood flow and perfusion defects• Good confirmation of AD diagnosis• Can be used to distinguish AD fromVaD• Post-mortem confirmation• Overdiagnosis and misdiagnosis• Delayed diagnosisMonday, 29 April, 13
  • 11. Case Study• Sophie• Journalist• Prided herself on her excellent memory and oftenconducted entire interviews without taking notes• Diagnosed with AD at age 51• Genetic predisposition• Not the first member of her family diagnosed with AD• Her Mom was diagnosed with the disease at age65 and had died at at the age of 70Monday, 29 April, 13
  • 12. Middle Stage Late StageEarly StageInitial AssessmentMonday, 29 April, 13
  • 13. Sophie’s case: Early Stage• Short term memory problems started at age 49• Found difficulties with reading• Aware of the history of AD in her family• Decided to see a psychologist - But told that her lowscores were probably attributed to fatigue and anxiety• ‘Imagining her problems’Monday, 29 April, 13
  • 14. Sophie’s case: Initial Assessment• Results of her initial assessment:• Wechsler Adult Intelligence Scale (WAIS) & WechslerMemory Scale (WMS)• Scored in the average range• Rey Complex Figure delayed recall• Scored 2 SD’s below the average score for awoman of her age• Showed mild symptoms of verbal and visuospatialmemory impairment and word-finding problemsMonday, 29 April, 13
  • 15. Middle Stage Late StageEarly StageInitial AssessmentFollowup Assessment & DiagnosisMonday, 29 April, 13
  • 16. Sophie’s case: Middle Stage• Over the next 18 months it became apparent even to herfamily that something was wrong• Sophie retired from her radio talk-back show• She was frequently unable to find the words shewanted• Repeated questions she had asked only minutesbefore and forgot the name of the person she wasinterviewing• Referred to a geriatrician to be assessedMonday, 29 April, 13
  • 17. Sophie’s case: Medical Assessment• MMSE (Mini Mental State Examination)• Sophie’s score fell in the moderately impaired range• Physical examination was normal• Sophie,“could have passed for 40 rather than 50”,“her voice wasstrong and clear”• No history of hypertension, strokes, heart problems, metabolic imbalancesor endocrine disease that could account for her symptoms of memoryimpairment and speech difficulties• No neurotoxic exposure or other physical effects from work• No history of psychiatric disturbance• No metabolic disordersMonday, 29 April, 13
  • 18. Sophie’s case: Brain• CT scan:• No areas of infarction (stroke) or any masslesions• Lateral ventricles slightly larger thennormal for her age• Minor cortical atrophy• Reversible causes of dementia were ruledout• However, irreversible dementia was not yetcertain, so further studies were conductedMonday, 29 April, 13
  • 19. Sophie’s case: Gradual Cognitive Decline• Important dates and events were recalled and explored• A gradual decline of memory, word-finding abilities and aproblem finding her way around were all evident• Onset consistent with a dementing process such as AD• Not consistent with vascular dementia• Tends to have a more distinct onset and a stepwiseprogression• A more abrupt onset of memory and cognitivedifficulties would increase likelihood of depression as acausative factor, which was not evidentMonday, 29 April, 13
  • 20. Sophie’s case: Executive Function• Wisconsin Card Sorting Task (WCST)• Grasped the first category after 4 card sorts• Preservation• After 8 incorrect sorts, she sorted to thecorrect category but then immediatelyreturned to the original category• Became increasingly angry with each failureto sort correctly• Difficulty making decisions involving abstract concepts• Difficulty modifying performance in response to verbal feedbackMonday, 29 April, 13
  • 21. Sophie’s case: Executive Function• Interpreted as indicative of frontal-lobe dysfunction• Consistent with the middle stages of AD• Demonstrated insight into her difficulties, indicating thatfrontal-lobe dysfunction was not yet advanced• These findings had implications for her care and treatment:• Treatment with antidepressants or counsellingwould help alleviate the depression• She still had the ability to think for herself andmake decisions about her treatment and futureMonday, 29 April, 13
  • 22. Sophie’s case: Premorbid IQ• National Adult Reading Test (NART)• A word pronunciation test• Good estimate of premorbid IQ for dementia• Correlates highly with the WAIS IQ• Since pronunciation of irregularly spelledEnglish words do not deteriorate at the samerate as other cognitive abilities in dementia• Sophie’s premorbid IQ estimated to bein the superior rangeMonday, 29 April, 13
  • 23. Sophie’s case: Longitudinal Assessment• Initial assessment, 18 months earlier, made longitudinal assessmentspossible• The second instance of the WAIS demonstrated Sophie’s scoresnow fell 1 or 2 SDs below average• Visuospatial abilities (block design, object assembly andpicture arrangement)• Abstract concepts (similarities and questions asking for anexplanation of proverbs)• Slowed responses (digit symbol subtest)• Word-finding problem (struggled to find the words to definea simple word that she was able to demonstrate bycircumlocution)Monday, 29 April, 13
  • 24. Sophie’s case: Longitudinal Assessment• Second instance of the Rey Complex figure• Dramatic deterioration from initial assessment• Showing further decline in visuospatial functionMonday, 29 April, 13
  • 25. Sophie’s case: Memory• Episodic memory• Informally assessed by comparing events she recalledto those recalled by her husband• Could spontaneously recall details of major eventsheld in the past year (i.e. her 50th birthday)• Unable to recall any details of a television seriesthey watched over a recent 6-week period• Vague feeling of familiarity but could not addany detailMonday, 29 April, 13
  • 26. Sophie’s case: Memory• Showed significant decrease in memory test scores (WMS)• Poor recall of logical memory passages• Paired-associates memory subtest• Recalled all obvious pairs (e.g. baby-cries), but none of theunusual pairs (e.g. school-grocery)• Recall of Rey figure after 45min consisted of a rectangle and nothingelse• Recognition Memory Test• Scored 2 SDs below average on word and face recognitionMonday, 29 April, 13
  • 27. Sophie’s case: Language• Aphasia Battery• Good comprehension• Difficulty repeating meaningless sentences (e.g.“The day, that the dream thought, jumpedcheaply”)• Descriptions of ‘actions’ in pictures wasimpoverishedMonday, 29 April, 13
  • 28. Sophie’s case: Diagnosis• Results support the evidence that her poor performancewas not of pseudodementia (i.e. depression)• Demonstrated a generalized cognitive decline• In relation to estimated premorbid IQ, decline waseven more significant• A diagnosis of probable AD was made with the results ofthe neuropsychological assessment and medicalinvestigationsMonday, 29 April, 13
  • 29. Middle Stage Late StageEarly StageInitial AssessmentFollowup Assessment & DiagnosisManagementMonday, 29 April, 13
  • 30. Sophie’s case: Management• Lived at home for 4 years, and eventually placed in a hospice as thedementia progressed• Sophie’s depressed mood improved as her insight decreased, andwithin a year of diagnosis she no longer experienced periods ofdepression• Made inappropriate/insensitive comments• Forgot or confused her children’s names• Became agitated for no apparent reason• Cognitive function deteriorated rapidlyonce placed in the hospice, within 6 weeksshe was mute and could recognize no one• Death followed soon afterMonday, 29 April, 13
  • 31. Sophie’s case: Conclusions• Post-mortem it was confirmed that shehad suffered from AD• Sophie’s assessment and diagnosis weremore straightforward than is often the case• Primarily because of her own knowledgeof the diseaseMonday, 29 April, 13
  • 32. Sophie’s case: Contributions• Sophie’s case has shown us that:• Longitudinal assessments are key to diagnosing AD, asthey show any trend of cognitive decline• Premorbid IQ is also an aspect that should not beoverlooked• Placement in an environment with no support leads torapid decline in cognitive function• A supportive family and familiar environment maybe the best way to help slow progression of ADMonday, 29 April, 13
  • 33. Sophie’s case:Additional Tests• Natural Environment Testing• Frequently exposing patient to “open experiences” ofnovel stimuli• Nature and city walks, social experiences withnew people..etc• Assess changes in attention (Trail Making Test),language abilities (Aphasia Battery) and executivefunction (Tower of London)Monday, 29 April, 13
  • 34. D.H.’s Case Study• Patient D.H.• Teacher, with 2 years of college education• Medically and cognitively healthy• Recruited as a control participant through the Alzheimer’sDisease Research Centre for a study of executive functions inolder adults• Annual neuropsychological assessments• Followed for 6 years, beginning at age 81• Diagnosed with probable AD in her 6th year ofassessmentJacobson et al. (2009)Monday, 29 April, 13
  • 35. D.H.’s case:Assessment• Premorbid IQ in the high average range• Mattis Dementia Rating Scale• Above average range• MMSE• Score remained within normal limits• Performance on most individual tests in most domains (e.g., language,psychomotor..etc) were above typical cutoff levels for impairmentsrelative to the normative group• CVLT long-delay recall score• No decline into an impaired range until her final assessmentprior to her AD diagnosisMonday, 29 April, 13
  • 36. D.H.’s case: Diagnosis• 5th assessment• Reported symptoms consisted with mild depression• Diagnosed with pseudodementia• Referred for neuroimaging studies• 6th assessment (age 86)• Complained of subjective changes in memory ability and somefunctional impairment in activities of daily living• Assessment revealed moderate impairment on memory testsand in multiple cognitive domains• Received a diagnosis of probable ADMonday, 29 April, 13
  • 37. D.H.’s case: Brain• MRI scan• Following 5th assessment, one year prior to diagnosisof probable AD• Asymmetric atrophy in medial temporal regions(particularly in left hemisphere)• Generalized cortical atrophy in temporal lobe regions• Enlarged ventriclesMonday, 29 April, 13
  • 38. D.H.’s case: Pre-AD Analysis• Annual assessments before diagnosis, during theasymptomatic preclinical stage of AD, haveprovided much data for pre-AD analysis• Discrepancy scores used to identify subtledeclines in cognitive skills relative to onesmore resilient to dementiaMonday, 29 April, 13
  • 39. D.H.’s case:Verbal vs Spatial tasksns: BNT, Boston Naming Test; BD, WAIS-R Block Design subtest; DS, WAIS-R Digit Span subtest; VS, VisualD-KEFS Trail Making; CVLT, California Verbal Learning Test Delayed Recall; HVRT, Heaton Visual Reproductsion (Russell, 1975); LF / CF / CSF, D-KEFS Verbal Fluency: Letter / Category / Category Shifting; MS / SS,ng Motor Speed / Sequencing and Shifting; CNWR, D-KEFS Color Word Interference Test combined Color aWIT, D-KEFS Color Word Interference Test-Interference condition; CWITS / Interference Shifting conditioocabulary subtest; CVLT1-5, California Verbal Learning Test Learning Trials 1–5.Visual Scanning versus Verbal Attention Span Discrepancies: D-KEFS Visual Scanning with Mildly Impaired Digit SpaVISUAL SCANNNG VERSUS VERBAL SPAN–1.5–1–0.500.511.5YEARS TO AD DXZ-SCORED-KEFS Visual Scanning Digit Span Subtest6 5 4 3 2 1Monday, 29 April, 13
  • 40. D.H.’s case:Verbal vs Spatial tasksCOBSON ET AL.suoconstruction versus Verbal Naming Discrepancies: Stable Block Design Scores with Declining Boston NaVISUOCONSTRUCTION VERSUS VERBAL NAMING–5–4–3–2–1012Years to AD DiagnosisZ-SCOREBlock Design Test Boston Naming Test6 5 4 3 2Monday, 29 April, 13
  • 41. D.H.’s case:Verbal vs Spatial tasksoconstruction versus Verbal Naming Discrepancies: Stable Block Design Scores with Declining Boston Na–5Years to AD DiagnosisBlock Design Test Boston Naming Testal Recall versus Verbal Recall Discrepancies: Increasing Discrepancy between HVRT and CVLT LongVISUAL RECALL VERSUS VERBAL RECALL–2–1.5–1–0.500.511.522.5Years to AD diagnosisz-scoreHVRT Delay Free Recall CVLT Long Delay free recall6 5 4 3 2 1Monday, 29 April, 13
  • 42. D.H.’s case: Executive function andMemory tasksCOGNITIVE DISCREPANCY CASE STUDY4. Verbal Fluency Discrepancies: Stable Letter Fluency Relative to Declining Category and Category Switching ScoVERBAL FLUENCY DISCREPANCIES–2–1.5–1–0.500.511.522.53Years to AD DiagnosisZ-SCOREDKEFS Letter Fluency DKEFS Category FluencyDEKFS Category Switching6 5 4 3 2 1Monday, 29 April, 13
  • 43. 4. Verbal Fluency Discrepancies: Stable Letter Fluency Relative to Declining Category and Category Switching ScoresYears to AD DiagnosisDKEFS Letter Fluency DKEFS Category FluencyDEKFS Category SwitchingTrail Making Test Discrepancies: Stable Basic Motor, Sequencing Skills with Variable Performance and Deficits in Shi.TRAIL MAKING TEST DISCREPANCIES–3–2.5–2–1.5–1–0.500.511.52Years to AD DiagnosisZ-SCOREDKEFS Trails Motor SpeedDKEFS Trails Average of Num & Letter SequencingDEKFS Trails Number/Letter Sequencing + Shifting6 5 4 3 2 1D.H.’s case: Executive function andMemory tasksMonday, 29 April, 13
  • 44. D.H.’s case: Executive function andMemory tasksOBSON ET AL.oop Discrepancies: Color/word Naming Scores and Traditional Stroop Scores with Impaired Interference/SwCOLOR WORD INTERFERENCE DISCREPANCIES–3.5–3–2.5–2–1.5–1–0.500.511.5Years to AD DiagnosisZ-SCORESDKEFS Color naming/Word Reading Baseline DKEFS Interference conditionDKEFS Interference + Switching condition6 5 4 3 2 1Monday, 29 April, 13
  • 45. D.H.’s case: Executive function vsMemory tasksDiscrepancies: Color/word Naming Scores and Traditional Stroop Scores with Impaired InterferencDKEFS Color naming/Word Reading Baseline DKEFS Interference conditionDKEFS Interference + Switching conditionLearning versus Vocabulary Discrepancies: Stable Basic-skill Word Knowledge Ability with DecliWORD KNOWLEDGE VERSUS VERBAL LEARNING–3.5–3–2.5–2–1.5–1–0.500.51Years to AD DiagnosisZ-SCOREVocabulary CVLT Learning6 5 4 3 2 1Monday, 29 April, 13
  • 46. Mean Discrepancy Scores: Verbal vs. Spatial Task Contrastsy = 0.3872x + 0.8046R2= 0.64300.511.522.533.5(a)(b)41 2 3 4 5 6yearsz-scoredifferenceMean discrepancy scores:Combined Complex vs. Basic Task Contrastsy = 0.3917x + 0.4275R2= 0.692700.511.522.533.51 2 3 4 5 6yearsz-scoredifferenceMonday, 29 April, 13
  • 47. D.H.’s case: Contributions• Cognitive impairment can be masked in certain caseswhere declining scores remain above norm-basedcutoff scores• Serial assessments over time may improvedetection of subtle cognitive changes in individuals• Cognitive-discrepancy analysis may be beneficial inidentifying normal-functioning elderly with preclinicalADMonday, 29 April, 13
  • 48. Questions?Monday, 29 April, 13
  • 49. References• Jacobson, Mark W., Delis, Dean C., Peavy, Guerry M., Wetter,Spencer R., Bigler, Erin D., Abildskov, Tracy J., Bondi, Mark W.and Salmon, David P.(2009) The emergence of cognitivediscrepancies in preclinical Alzheimers disease: A six-year casestudy, Neurocase, 15: 4, 278 — 293, First published on: 21April 2009 (iFirst)Monday, 29 April, 13