Office-based Cognitive Testing: Cases Paul R. Solomon, PhD Professor of Psychology /Neuroscience Williams College Visiting Professor of Neurology Boston University School of Medicine Clinical Director Boston Center for Memory Clinical Director The Memory Clinic DEMENTIA: A Comprehensive Update Boston, June 7-10, 2017 Disclosure (past 12 months) Grant Support: AstraZeneca, AVID, Axovant, Biogen, Cambridge Cognition, Eli Lilly, Forum Pharmaceuticals, Hoffman-LaRoche, Neuronetrix, ONNIT Labs, TransTech Pharma Consulting:, Boehringer-Ingelheim, Eli Lilly, General Electric, Neuronetrix, Novartis Royalties: Elsevier(Saunders), Psychological Assessment Resources Steps in Diagnosis / Differential Diagnosis Decision that dementia is present Determination of cause of dementia (differential diagnosis) Steps in Diagnosis Decision that dementia is present Determination of cause of dementia (differential diagnosis) Cognitive Eval. Hx Cognitive Complaints Neuro Exam Lab Eval Cognitive Profile Imaging Approaching Differential Diagnosis Up to 75% of cases will include AD Start with the hypothesis that AD is the cause in full or in part Be aware of the signs/symptoms of other common causes of dementia Cases Real cases Focus on most common causes of dementia Other Common Causes Of Cognitive Impairment Dementias FRONTAL ~5% VASCULAR AD ~ 15 - 25% ~ 75% NONDEGEN DEM ~ 5% Medication Side Effects ~5-10% MCI ~3-22% LEWY BODY ~ 20% Depression •MDD ~3% •Subsyndromal ~ 15-27% Office Based Assessment Procedures Neuropsychological Tests Informant Completed Questionnaires Neuropsychological Tests Advantages Disadvantages Commonly used, Requires patient to many choices Requires only patient (not the caregiver) to be present be present Requires patient to be cooperative Requires staff time to administer Informant Questionnaires Advantages Disadvantages Does not require Requires caregiver patient to be present and / cooperative Requires minimal staff time to administer to be present Neuropsychological Tests: Montreal Cognitive Assessment (MOCA) Advantages Disadvantages Test and Instructions freely Takes 10 minutes to administer available on the web (www.mocatest.org) Clear Instructions and scoring Translated into 30 + languages Covers multiple cognitive domains (orientation, memory, attention, language, executive function, visuospatial function) Accuracy > MMSE for AD and MCI (Nasreddine et al. JAGS, 2005) Montreal Cognitive Assessment (MOCA) MOCA + 5 ≅ MMSE Informant Completed Alzheimer’s Disease Caregiver Questionnaire (ADCQ) Advantages Test and Instructions freely available on the web (bostonmemory.com) 18 item YES / NO questionnaire Sensitivity > 90%, Specificity > 85% Minimal staff time required Disadvantages Requires presence of caregiver Not validated for selfreport by patient Solomon et al. International Psychogeriatrics, 2003 Domains Evaluated MOCA ADCQ Visuospatial /Executive Recent Memory Naming Executive Function Memory Language Attention Visuospatial Abstraction Mood & Behavior Delayed Recall Progression Cued Recall (optional) Abstraction Case 1 Patient Profile 88-years old Female 19 years of education (2 bachelors, 1 masters degree) Taught at the public school and college level Plays the organ Plays golf Medical Medical History Current Medications Hypothyroidism Levoxyl Mild anemia multivitamin + iron Mild arthritis cortisone injection Physical / Neurological Exam Metamucil Unremarkable B6 Laboratory Results Within normal limits calcium Imaging Studies CT scan w/o contrast Impression: Moderate cerebral atrophy No evidence of acute cortical infarction or intracranial bleed History of Cognitive Complaints Onset: 3 years ago, insidious Initial symptoms: deficits in recent memory Progression: progressive - particularly in the last 1–2 years Current Complaints: Memory Repeats questions multiple times within same conversation Rapidly forgets conversations Executive Function Bills now disorganized Can no longer organize medications Language Word finding difficulties Other aspects of cognition intact Cognitive Assessment MMSE = 24 Missed 3/3 delayed item recall Disoriented time, place MOCA =19 Missed 5/5 delayed item recall Missed 4/5 with cues Trailmaking B impaired Verbal Fluency impaired (8 animals / 1 minute) Clock Drawing impaired (hands set incorrectly) Alzheimer’s Disease Caregiver Questionnaire (ADCQ) - positive Endorsed forgetting conversations / repeating questions Endorsed deficits in executive function Problems have been Progressive MOCA Cued Recall Function ADLs intact IADLs mildly impaired Living independently Difficulty paying bills Difficulty managing medications Impairment index = 15% Differentials Alzheimer’s disease Mild Cognitive Impairment Diagnosis Alzheimer’s disease - early stages Diagnostic Criteria – Probable AD Dementia Present Presence of significant cognitive decline documented by knowledgeable informant and neuropsych. testing Interferes with independence in everyday activities Impairment is in a minimum of 2 domains Probable AD Dementia Insidious onset (months / years) Clear cut worsening Initial deficits are in memory (amnestic) or other cognitive area ( non-amnestic) such as language, visuospatial, executive. No evidence for other dementing disorder Why is this not MCI? The Concept of MCI due to AD was introduced in the 2011 NIA-AA criteria DSM-5 refers to this as Minor Cognitive Disorder due to AD Both NIA-AA and DSM-5: Assumes that AD pathology is present and patient will eventually progress to clinical AD Recognizes that biomarkers will eventually be available (e,g., amyloid and Tau PET, volumetric MRI) and will add confidence to this diagnosis MCI due to AD AD early stages Differential Diagnoses Mild Alzheimer’s disease Mild Cognitive Impairment due to AD Cognitive complaints by Present patient or family Present Cognitive deficits Present, mild deficits Dementia Present Present, very mild deficits Absent Functional impairment Present – Absent – Interferes with Independence of independence in everyday function– activities although patient may take longer or experience more difficulty Case 2 Patient Profile 71 year-old Female Living independently 12 years education Retired Home Health Aide (1980s) Recent death of companion Medical Medical History Hypercholesterolemia Left hip replacement Status post cholecystectomy Arthritis in many joints L5 diskectomy Physical / Neurological Exam Parkinsonism Rigidity Current Medications Levoxyl multivitamine + iron cortisone injection Metamucil calcium B6 Laboratory Results Within normal limits Imaging Studies CT scan w/o contrast Impression Generalized atrophy prominent in presylvian area Old white matter ischemic changes Old right basal ganglia lacunar infarct History of Cognitive Complaints Onset: 2-3 years Initial symptoms: becoming lost in familiar setting Progression: gradual Current Complaints: Memory Mild deficits in recent memory Executive Function Difficulty managing checkbook Can no longer organize medications Attention Fluctuating Cognitive Assessment MMSE = 26 Disoriented to place Could not copy complex figure MOCA = 22 Missed 1/5 delayed item recall Missed 0/5 with cues Trailmaking B impaired Clock Drawing impaired, could not copy cube Impaired attention, digits forward Alzheimer’s Disease Caregiver Questionnaire (ADCQ) - positive Endorsed visuospatial problems (e.g., becoming lost) Endorsed deficits in executive function Problems are progressive Clock Drawing (from MOCA) Function ADLs and intact IADLs impaired • Impairment Index = 46% Differentials AD MCI Lewy Body Disease Diagnosis Dementia with Lewy Bodies (DLB) Diagnostic Criteria Central Features (essential) Dementia Present Impaired executive function, attention, and visuospatial ability often prominent Memory impairment may or may not be prominent initially Diagnostic Criteria Core Features (2 for probable, 1 for possible LBD) Fluctuating cognition with pronounced variation in attention and alertness Recurrent visual hallucinations, well formed and detailed -- Often or people or animals -- Often initially present around sleep/wakefulness transitions Spontaneous features of parkinsonism Differential Diagnoses Cognitive deficits Alzheimer’s disease Dementia with Lewy Bodies Multiple cognitive areas with memory disorder prominent Visual hallucinations, sleep disturbance later in disease or not at all Functional impairment Present More prominent deficits in visuospatial, executive, and attentional function Visual hallucinations, sleep disturbance often present early in the disease Present Dementia Present Present Motor Symptoms None until late stages Parkinsonian symptoms often present early in disease Behavioral Symptoms Case 3 Patient Profile 67 year-old male Retired truck driver with 12 years of education Premorbid IQ in average range Medical Medical History Current Medications Hypercholestremia Simvastatin Hypertension Lisinopril Enlarged prostate (not Metroprolol thought to be cancer) ASA 325 Physical / Neurological Exam Procardia Unremarkable Laboratory Results Within normal limits donepezil (10 mg) Imaging Studies MRI scattered T2 hyperintensities some atrophy PET Hypometabolism in frontal lobes Insert MRI scan History of Cognitive Complaints Onset: 6 years ago, insidious Initial symptoms: behavioral Progression: progressive - particularly in the last 2-3years Current Complaints: Memory Recent memory deficits, especially in past year Executive Function Difficulty with financial decisions – wife now manages finances Difficulty organizing meals (no longer cooks) and household projects Language Word finding difficulties Other aspects of cognition intact Cognitive Assessment MMSE = 25 Missed 2/3 delayed item recall Difficulty with WORLD backwards MOCA = 21 Missed 3/5 delayed item recall Missed 0/5 with cues Trailmaking B impaired Verbal Fluency impaired (6 animals / 1 minute) Alzheimer’s Disease Caregiver Questionnaire (ADCQ) - positive Endorsed forgetting conversations Endorsed deficits in executive function Problems are progressive Function ADLs mildly impaired Needs reminders IADLs impaired Inappropriate in social settings Difficulty managing finances Difficulty planning and organizing household tasks Impairment index = 49% Mood and Behavior Disinhibition Embarrassing comments in social situations De Novo high sex drive Made socially inappropriate comments toward female examiner Paranoia misplaces items and feels others have stolen these items Feels people on TV are speaking to him Wants to eat when anyone else is eating Differentials Alzheimer’s disease Frontotemporal dementia Psychiatric disorder Diagnosis Frontotemporal dementia Frontotemporal Dementia Core Diagnostic Features (all must be present) Insidious onset and gradual progression Early decline in social interpersonal conduct Early impairment in regulation of personal conduct Early emotional blunting Early loss of insight Frontotemporal Dementia Supportive diagnostic features: Behavioral variant Decline in personal hygiene and grooming Mental rigidity and inflexibility Distractibility and impersistence Hyperorality and dietary changes Perseverance and stereotyped behavior Utilization behavior Physical signs: primitive reflex, incontinence, akinesia, rigidity, tremor, low/labile blood pressure Differential Diagnoses Alzheimer’s disease Frontotemporal Dementia Behavioral Variant Age of Onset Typically > 65 Typically < 65 Cognitive Deficits Multiple cognitive areas with memory disorder prominent None early, executive dysfunction later in disease Behavioral Symptoms None early in disease, Socially inappropriate apathy, agitation, and behavior early in others as disease disease progresses Differential Diagnosis Alzheimer’s Disease Functional Impairment Absent Lack of Progression Consider MCI Dementia Absent Parkinsonism Present Visual Hallucinations Early in Disease REM Sleep Disorder Fluctuating Attention Age < 65 Behavioral Disorders Early in Disease Spatial Abilities Preserved Memory Abilities Variable Imaging Evidence of Vascular Disease Lack of Progression Consider Lewy Body Disease Consider Frontotemporal Dementia Consider Vasc Dem Office-based Cognitive Testing: Cases Paul R. Solomon, PhD Professor of Psychology /Neuroscience Williams College Visiting Professor of Neurology Boston University School of Medicine Clinical Director Boston Center for Memory Clinical Director The Memory Clinic DEMENTIA: A Comprehensive Update Boston, June 7-10, 2017
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