neurology Board Review Manual Statement of Editorial Purpose The Hospital Physician Neurology Board Review Manual is a peer-reviewed study guide for residents and practicing physicians preparing for board examinations in neurology. Each manual reviews a topic essential to the current practice of neurology.
PUBLISHING STAFF PRESIDENT, Group PUBLISHER
Bruce M. White Senior EDITOR
Robert Litchkofski
Neuropsychological Evaluation in Clinical Practice: Case Interpretation and Treatment Editors: Alireza Atri, MD, PhD
Instructor in Neurology, Harvard Medical School; Assistant in Neurology, Massachusetts General Hospital, Boston, MA; Neurologist, Geriatric Research Education & Clinical Center, Veterans Administration Medical Center, Bedford, MA
Tracey A. Milligan, MD
Instructor in Neurology, Harvard Medical School; Associate Neurologist, Brigham and Women’s and Faulkner Hospitals, Boston, MA
Contributors: Lynn W. Shaughnessy, MA
Doctoral Student, Massachusetts School of Professional Psychology, West Roxbury, MA assistant EDITOR
Farrawh Charles executive vice president
Barbara T. White executive director of operations
Janet C. Sherman, PhD
Assistant Professor, Department of Neurology, Harvard Medical School; Clinical Director, Psychology Assessment Center, Massachusetts General Hospital, Boston, MA
Maureen K. O’Connor, PsyD, ABCN
Instructor, Department of Neurology, Boston University School of Medicine, Boston, MA; Director of Neuropsychology, Edith Nourse Rogers Memorial Veterans Hospital, Bedford, MA
Jean M. Gaul PRODUCTION Director
Suzanne S. Banish PRODUCTION assistant
Nadja V. Frist sales & marketing manager
Deborah D. Chavis
Table of Contents Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 Case 1: A 71-Year-Old Man with Memory Loss. . . . . . . . . . . . . . . . . . . . . 2 Case 2: A Man with Traumatic Brain Injury . . . . . . . . . . . . . . . . . . . . . . . 5 Case 3: A 66-Year-Old Man with Cognitive and Behavioral Difficulties. 8 Case 4: A 9-Year-Old Girl with Reading Difficulty. . . . . . . . . . . . . . . . . 12
NOTE FROM THE PUBLISHER: This publication has been developed without involvement of or review by the Amer ican Board of Psychiatry and Neurology.
Summary. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Cover Illustration by Nadja V. Frist
Copyright 2009, Turner White Communications, Inc., Strafford Avenue, Suite 220, Wayne, PA 19087-3391, www.turner-white.com. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, mechanical, electronic, photocopying, recording, or otherwise, without the prior written permission of Turner White Communications. The preparation and distribution of this publication are supported by sponsorship control over the design and production of all published materials, including selection of topics and preparation of editorial content. The authors are solely responsible for substantive content. Statements expressed reflect the views of the authors and not necessarily the opinions or policies of Turner White Communications. Turner White Communications accepts no responsibility for statements made by authors and will not be liable for any errors of omission or inaccuracies. Information contained within this publication should not be used as a substitute for clinical judgment.
www.turner-white.com
Neurology Volume 13, Part 4
Neurology Board Review Manual
Neuropsychological Evaluation in Clinical Practice: Case Interpretation and Treatment Lynn W. Shaughnessy, MA, Janet C. Sherman, PhD, and Maureen K. O’Connor, PsyD, ABCN
Introduction This manual is the second half of a 2-part review of neuropsychology. The first part presented an overview of the practice of neuropsychology, focusing on the goals of a neuropsychological evaluation, its methods, and the cognitive domains assessed. In clinical practice, there are 4 main goals of a neuropsychological evaluation: (1) to assess a patient’s cognitive status across a range of domains to determine areas of strength and weakness; (2) to provide assistance in addressing questions pertaining to differential diagnosis; (3) to monitor cognitive status over time through repeat neuropsychological evaluations; and (4) to provide recommendations regarding possible treatments and interventions that can help patients and their families or help determine competency for managing instrumental activities of daily living (eg, finances, medication, appointments, driving). The neuropsychologist accomplishes these goals through both qualitative observation and quantitative data obtained by administration of standardized tests. In this manual, we present clinical cases that illustrate essential concepts in neuropsychology and demonstrate how neuropsychological evaluations can contribute to a patient’s clinical care. In each case presentation, we describe the patient’s background, symptom presentation and history, behavioral observations, test scores, interpretation of results, and treatment recommendations. For each case, the test data have been converted from raw scores to percentiles. These scores reflect the percentage of people in the general population who attain a lower score; the scores can be interpreted as indicating that the patient scores “better than ‘x’% of similar individuals who take the same test.” To facilitate interpretation and comparison of these values, the percentile ranks are classified into descriptive ranges. The ranges used are as follows: 98th percentile and above = very superior 91st–97th percentile = superior
Hospital Physician Board Review Manual
75th–90th percentile = high average 25th–74th percentile = average 9th–24th percentile = low average 3rd–8th percentile = borderline impaired 2nd percentile and below = impaired
It is notable that 3 of the 4 patients described are male; however, this is not meant to imply that the presented conditions are gender specific or that males are more often referred for neuropsychological evaluation. Additionally, in an effort to provide clear and concise cases, we present cases involving individuals with average estimated premorbid levels of functioning. It has been suggested that individuals with above average premorbid intelligence may possess “cognitive reserve” and in turn exhibit signs of memory loss or cognitive decline later in the disease process.1 This is an important concept when examining individuals with higher estimated premorbid levels of functioning, as they may score within the “average range” but still be experiencing initial symptoms of neurologic dysfunction.
CASE 1: A 71-YEAR-OLD MAN WITH MEMORY LOSS CASE Presentation and History A 71-year-old right-handed man is referred for neuropsychological testing by his primary care physician for evaluation of memory and cognitive functioning, assistance with differential diagnosis, and recommendations regarding possible treatments and interventions. The patient reports experiencing “short-term memory loss” for the past 3 to 4 years. Specifically, he describes some difficulty keeping track of his score when playing golf, remembering conversations, and remembering what he has read in the newspaper. The patient says that he feels his memory problems are minor and are caused by stress, and he notes that occasionally he
www.turner-white.com
Neuropsychological Evaluation in Clinical Practice: Part 2 feels his memory is improving. In contrast, the patient’s wife, who is present during the interview, describes a clear progression of memory problems over the past 3 to 4 years, which she feels are considerable. She explains that on 2 occasions her husband became lost while driving in a familiar area. She notes that he is repetitive in conversation and asks repetitive questions. She also adds that he often forgets appointments and needs frequent reminders. The patient’s wife is particularly concerned about her husband’s functioning after watching her mother die from AD, and adds that because of this experience she is hypervigilant about any memory problems her husband might be displaying. Regarding mood, the patient denies mood disturbance or change in mood and describes numerous activities he enjoys outside of the home. However, his wife reports an increase in irritability, most notably when she reminds him to take his medications or tries to help him organize his office. Medical history is significant only for glaucoma, reportedly well controlled through medication. Medical records indicate that the patient has been seen previously by a neurologist for memory concerns, who told him that he suffers from “age-related cognitive decline” and subsequently reassured him that his memory difficulties are normal and not progressive. The patient graduated from college and reportedly completed school with no academic difficulties. He has been retired from his industrial managerial position for approximately 5 years. When asked about family history, the patient states he is an only child; his mother died at age 50 years from a motor vehicle accident, and his father died in his late 80s of “old age.” The patient reports no family history of dementia, but noted that his maternal grandmother “went crazy” in her elder years and lived in a nursing home until she died at age 80 years; he cannot recount the specific details. • What are the possible causes for this patient’s memory problems? A neurodegenerative dementia such as Alzheimer’s disease (AD) is a consideration given the patient’s age, his 3- to 4-year history of progressive cognitive difficulties with problems most notably in the memory domain, and his behavioral history, including repetitiousness and getting lost in familiar places. Normal aging is a possibility, given his wife’s admission that she is hypervigilant about her husband’s memory since watching her mother’s battle with AD and the discrepancy between her report of considerable memory difficulties and the patient’s report of only minor memory difficulties. If normal aging is responsible, this would be reflected on the neuropsychological evaluation by per-
www.turner-white.com
formance within normal limits compared to same-age peers and his premorbid estimate of functioning. CASE 1 Neuropsychological Evaluation Following the clinical interview, the patient is administered a battery of neuropsychological measures to assess cognitive functioning. A summary of the patient’s performance for each test is provided in Table 1. Behavioral observations during testing reveal euthymic mood and affect in full range. He is oriented to person and place; however, he is 5 years off in providing the date. He demonstrates intermittent word-finding difficulties during spontaneous speech but is articulate and fluent. He provides a detailed description of remote history, but his report about events within the past 2 to 3 years is vague and less descriptive. He is very pleasant, cooperative, and responsive during the evaluation. There is no evidence of inappropriate behavior, verbalizations, or jocularity. The patient works diligently with full effort throughout. The results of the evaluation are therefore considered an accurate reflection of his cognitive ability at the time of the evaluation. Premorbid intellectual functioning is estimated to fall within the average range based on his educational and occupational attainment and other demographic variables, and this estimate is consistent with his performance on a task of oral word reading. Compared to premorbid estimates, results reveal deficits within the memory domain, specifically on measures of delayed recall and recognition for both verbal and visual material, with minor benefit from structure or context. Testing also shows minor reductions in executive functioning and simple attention, while working memory is intact. Language skills are preserved on neuropsychological measures, specifically confrontation naming and fluency tasks, despite word-finding difficulties in interview. Minimal depressive symptoms are endorsed. • How does this cognitive profile inform the differential diagnosis? The differential diagnosis includes a degenerative dementia such as AD versus normal age-related cognitive decline. Examination of the cognitive profile reveals profound deficits within the domain of memory, specifically with retention of new information and minor reductions in attention and executive functioning. The patient’s profile in conjunction with his wife’s report of progressive decline, with difficulties remembering appointments and driving (becoming lost in a familiar area) is most consistent with a diagnosis of AD and is not what would be expected in normal aging.
Neurology Volume 13, Part 4
Neuropsychological Evaluation in Clinical Practice: Part 2 Table 1. Neuropsychological Test Findings for Case 1: Assessment Data Result/Percentile/ Classification
Domain/Test Administered
Evaluation 1 Evaluation 2
Mini-Mental State Examination2 Premorbid functioning Wide Range Achievement Test-33 Attention/executive functions WAIS-III4 subtests Similarities Digit Symbol-Coding Digit Span Forward Digit Span Backward Controlled Word Association5 FAS Animals Clock drawing Command Copy Trail Making Test A (letter sequencing) B (number-letter sequencing)
27/30
23/30
Average
N/A
50th, A 49th, A 21st, LA 38th, A
47th, A 31st, A 21st, LA 38th, A
49th, A 45th, A
40th, A 10th, LA
Borderline impaired Intact
Impaired
6
Language Boston Naming Test7 WAIS-III subtest: Vocabulary
Borderline impaired
34th, A 21st, LA
3rd, B < 1st, I
48th, A
40th, A
N/A
53rd, A
Result/Percentile/ Classification
Domain/Test Administered
Evaluation 1 Evaluation 2
Memory Verbal memory WMS-III8 subtest Logical Memory Recall I Recall II Recognition CLVT—short form9 Word List Recall Short delay Free Long delay Free Cued Recognition
30th, A 18th, LA 17th, LA
5/6/6/7, 54th, A 3/3/4/5, 1st, I
Visual memory Rey-Osterrieth Complex Figure10 Immediate recall Delayed recall
4 free, 7th, B
1 free, 1st, I
2 free, 7th, B 2 cued, 1st, I 6 hits/3 false positives
1 free, 7th, B 1 cued, 1st, I 4 hits/6 false positives
8th, B 3rd, B
1st, I < 1st, I
24th, LA 51st, A
18th, LA 16th, LA
4/30 within normal limits
2/30 within normal limits
Visuoperceptual/visuospatial/ visuoconstructional functioning Rey-Osterrieth Complex Figure Copy Hooper Visual Organization Test11 Emotional functioning Geriatric Depression Scale12
10th, LA 2nd, I 6th, B
A = average; B = borderline; HA = high average; I = impaired; LA = low average. CLVT = California Verbal Learning Test; WAIS = Wechsler Adult Intelligence Scale; WMS = Wechsler Memory Scale.
Case 1 Recommendations and Follow-up Evaluation The patient and his wife are provided with psychoeducation about AD during feedback and given information for local resources that provide programming to help them better understand the disease and the services available to them. A referral to a neurologist is made in order to follow the patient and consult regarding pharmacologic treatment with a cholinesterase inhibitor. Given reports of becoming lost while driving, in conjunction with the cognitive impairments noted on the evaluation, it is recommended that the patient either self-elect to discontinue driving or obtain a formal driving evaluation to determine whether it is safe for him to continue to drive. The severity of memory difficulties also suggests that the patient will need assistance with medication management and with important financial decisions. Also, it is suggested he may be a candidate for clinical trials.
Hospital Physician Board Review Manual
Participation in clinical trials not only provides another treatment option, but also can offer the patient a sense of accomplishment in contributing to research and potentially benefiting individuals suffering from AD in the future. Finally, it is recommended that he return for a follow-up evaluation in 1 year to monitor cognitive functioning, aid in confirmation of diagnosis, and tailor additional treatment recommendations. The patient returns for a follow-up neuropsychological evaluation approximately 2 years after initial testing. At this evaluation, the patient is being treated with a cholinesterase inhibitor, which was initiated following the first evaluation. Additionally, the patient’s wife now fully manages her husband’s medications, appointments, and finances. She reports progressive worsening of her husband’s memory difficulties, noting that he typically cannot remember what he had for breakfast and continues to be repetitious. In contrast, the patient shows little awareness of his difficulties, reporting, “I
www.turner-white.com
Neuropsychological Evaluation in Clinical Practice: Part 2 haven’t had too many problems.” Similarly, when asked about sleep, the patient describes getting a “perfect” 7 to 8 hours of sleep and denies taking daytime naps, while his wife reports that he sleeps “all the time,” from 9 pm to 10 am with frequent naps during the day. When asked about mood, the patient describes it as “just fine” and states that he is rarely down. His wife notes that he has been more irritable and agitated since the previous evaluation. She also reports an increase in apathy and a tendency to stay home rather than engage in social activities he previously enjoyed. A comparison of the results from the 2 evaluations is shown in Table 2. The results of the second evaluation indicate progressive memory impairment, specifically continued difficulty with retention of new information. Executive functioning is now impaired, including increased difficulty initiating and maintaining set, trouble with cognitive flexibility, and notable disinhibition. The patient also exhibits greater difficulty with semantic fluency than phonemic fluency, which was not the case at the initial evaluation. His confrontation naming is now marked by circumlocutions, confabulations, and semantic paraphasic errors; word-finding difficulties are also more obvious in spontaneous speech. There is no decline in simple attention and working memory. • How do these results contribute to our understanding of this patient’s difficulties? The pattern of rapid forgetting, which is considered the hallmark of AD, is even more marked during the second evaluation. Executive functioning has continued to decline. The patient’s greater impairment in semantic than phonemic fluency and impaired confrontation naming are consistent with the semantic memory loss that also often accompanies AD pathology.13 These findings, in addition to reported behavioral history of functional decline, worsening memory difficulties, and behavioral changes including increased agitation and apathy, confirm the original diagnosis. Case 1 Recommendations and Case Resolution The addition of memantine to the patient’s current cholinesterase inhibitor is suggested given that combination therapy has been shown to slow both cognitive and functional decline in individuals with AD.14 The patient’s wife is encouraged to attend local support groups and discuss legal matters with the family. It is also stressed that maintaining organization and structure within the patient’s environment will help to reduce confusion and overstimulation. Approximately 3 years after the second evaluation, the patient has significantly deteriorated both
www.turner-white.com
cognitively and physically and is moved to a nursing home. He eventually becomes unable to walk without assistance and his sight deteriorates. He is scheduled for surgical treatment of his cataracts, and during the preoperative evaluation it is discovered that he has suffered a silent myocardial infarction. Soon after, he develops pneumonia and his family and clinical team decide, based on his preferences when he was well, that in view of his clinical decline, multiple medical problems, and current pneumonia, he should not undergo any aggressive treatment. The patient is given comfort measures and dies peacefully. Brain autopsy confirms AD as the primary diagnosis.
CASE 2: A MAN WITH TRAUMATIC BRAIN INJURY CASE Presentation and History A 49-year-old left-handed man is referred for a neuropsychological evaluation by his psychotherapist to determine cognitive strengths and weaknesses and to assist in providing recommendations regarding possible treatments and/or interventions. The patient presents with a 10-year history of significant cognitive impairment since sustaining a traumatic brain injury (TBI) during a motor vehicle accident. Description of the injury indicates that the patient was unresponsive at the scene, with a documented 30-minute loss of consciousness. He was transported by ambulance to the hospital and discharged the same day after receiving medical attention, including a head computed tomography scan, which was normal. The patient experienced anterograde and retrograde amnesia for several hours surrounding the accident. Left-sided motor weakness caused by the accident has mostly subsided by the time of evaluation, although there is some reported residual weakness. He and his wife report that the current cognitive difficulties seem to fluctuate, with some days better than others. Since the injury, he has had difficulty remembering conversations and needs reminders from his wife to take his medications. His wife has also taken over managing the household finances and has taken away his credit card and checkbook because he was buying items they could not afford. The patient reports becoming easily distracted; for example, when running errands he becomes sidetracked and does not complete the errand. Similarly, he often leaves household chores halfway done because of distractions during the task. The patient reports misplacing items (keys, wallet) and being generally
Neurology Volume 13, Part 4
Neuropsychological Evaluation in Clinical Practice: Part 2 disorganized. He reports that he frequently forgets appointments and attempts to compensate for this by using a daily planner. Regarding mood, the patient reports feeling frustrated and explains that he has a significant amount of anger toward himself that he inadvertently directs toward others. His wife describes him as more irritable, unpredictable, demanding, and easily frustrated. The patient had no psychiatric history prior to the accident, but since his head injury he has experienced mood instability and an explosive temper. The patient also reports sleep disturbance, with trouble falling asleep and multiple night awakenings due to excessive worry. He denies appetite disturbance. Medical history is significant for hyperlipidemia and osteoarthritis. At the time of the evaluation, he is seeing the referring therapist for long-term care focused on family and individual psychoeducation regarding the effects of TBI and psychotherapeutic intervention aimed at addressing adjustment to life post-injury. The patient completed high school and 1 year of college with no reported difficulty. He worked as a mechanic prior to the accident. Although he is interested in returning to work, he has not returned due to his cognitive difficulties. The patient reports no significant family medical history and is the oldest of 6 siblings, all in good health. He and his wife have been married for 25 years and have 4 children. • What is the significance of anterograde and retrograde amnesia for events surrounding the accident in this patient’s diagnosis? Impaired ability to learn and remember episodic information learned after a neurologic insult is called anterograde amnesia, often referred to in TBI cases as post-traumatic amnesia (PTA).15 Retrograde amnesia refers to loss of information learned prior to the injury. Individuals with TBI may experience some extent of PTA and/or retrograde amnesia surrounding the traumatic event. When assessing TBI, determining the extent of loss of consciousness and amnesia surrounding the event informs the TBI severity classification (ie, mild, moderate, severe). Several scales have been developed to define and grade the severity of TBI, most notably the Glasgow Coma Scale; however, loss of consciousness and PTA are the most common injury characteristics referenced in most classification systems.16 Additionally, PTA is commonly the primary and most specific diagnostic indicator of injury, and the extent of presenting PTA is often correlated with recovery (ie, shorter duration of PTA is correlated with better cognitive and functional outcome).15,17 This patient reported less than 24 hours of PTA, which would suggest a clas-
Hospital Physician Board Review Manual
sification of mild traumatic brain injury and a relatively good prognosis for recovery following the incident. • What factors aside from TBI might account for the fluctuations in this patient’s cognitive performance? Fluctuations in cognitive performance are often due to situational variables such as mood disturbance and lifestyle factors. This patient reported feelings of frustration and depression, which can vary from day to day and exacerbate cognitive difficulties. Further, the patient reported significant trouble sleeping, which would also intensify difficulties with memory and thinking and increase mood disturbance. Case 2 Neuropsychological Evaluation Following the clinical interview, the patient is administered a battery of neuropsychological measures to assess his current cognitive functioning and determine strengths and weaknesses that will aid in treatment planning. A summary of the patient’s performance for each test is provided in Table 2. The patient arrives late and reports significant anxiety about his difficulty finding the office and perseverates about this throughout the evaluation. When upset, his voice becomes loud and he leans in toward the examiner. He recognizes this at times and apologizes for inappropriate behavior. During testing, he often becomes visibly disappointed or frustrated when he perceives that he is not doing well. Given his full level of effort and motivation, the test results are considered a valid indicator of his cognitive functioning. The patient’s premorbid intellectual functioning is estimated to lie within the average range based on his educational and occupational attainment and other demographic variables, and this estimate is consistent with his performance on a test of oral word reading. Results of the evaluation indicate impairment across several domains. Attention and working memory are reduced. Processing speed is intact for simple timed tasks but is reduced on more complex measures. Executive dysfunction is characterized by perseveration, cognitive inflexibility, and poor visuoconstructional planning and organization. The patient was asked to copy the ReyOsterrieth Complex Figure (Figure, part A), and his direct copy is displayed in part B of the Figure. Set-shifting ability is impaired due to perseverative responding. Additionally, there is a minor reduction in phonemic as compared to semantic fluency. In contrast, abstract reasoning is intact. An evaluation of verbal memory reveals intact new learning of information that is repeated over multiple trials, but new learning of lengthier narratives is
www.turner-white.com
Neuropsychological Evaluation in Clinical Practice: Part 2 Table 2. Neuropsychological Test Findings for Case 2: Assessment Data Domain/Test Administered Premorbid functioning Wechsler Test of Adult Reading18 Attention/executive functions WAIS-III4 subtests Similarities Digit Symbol-Coding Digit Span Forward Digit Span Backward Clock drawing Command Copy D-KEFS19 subtests Trail Making Visual scanning Number sequencing Letter sequencing Number-letter sequencing Motor speed Verbal Fluency Total letter (FAS) Total category Category switching total Color-Word Interference Condition Color naming Word reading Inhibition Inhibition/switching
Result/Percentile/ Classification Average
25th, A 2nd, I 12th, LA 10th, LA Borderline impaired Borderline impaired
91st, S 75th, HA 75th, HA 2nd, I 75th, HA 9th, LA 25th, A 37th, A
47th, A 50th, A 5th, B 9th, LA
Motor functioning Finger tapping Dominant hand (R) Non-dominant hand (L)
24th, LA 16th, LA
Language Boston Naming Test7
43rd, A
Domain/Test Administered Memory Verbal memory WMS-III8 subtest: Logical Memory Recall I Recall II Recognition California Verbal Learning Test-II9 Word List Recall Short delay Free Cued Long delay Free Cued Recognition Visual memory WMS-III Subtest Visual reproduction Recall I Recall II Recognition Rey-Osterrieth Complex Figure10 Immediate recall Delayed recall Visuoperceptual/visuospatial/ visuoconstructional functioning WAIS-III subtest Block Design Rey-Osterrieth Complex Figure Copy Hooper Visual Organization Test11 Emotional functioning Beck Depression Inventory20
Result/Percentile/ Classification
2nd, I 9th, LA 37th, A 3/8/7/8/12, 27th, A 5 free, 16th, LA 9 cued, 31st, A 8 free, 31st, A 8 cued, 16th, LA 11 hits/3 false positives
5th, B 2nd, I 37th, A 1st, I 1st, I
16th, LA < 1st, I 86th, HA Severe depression
A = average; B = borderline; HA = high average; I = impaired; LA = low average; S = superior. D-KEFS = Delis-Kaplan Executive Function System; WAIS = Wechsler Adult Intelligence Scale; WMS = Wechsler Memory Scale.
reduced, which is thought to be due to difficulty attending to the story. Retrieval is reduced but improved with cueing on yes/no recognition trials. Visual memory for the aforementioned complex figure is impaired after an immediate and long delay, which is attributed to his difficulty planning and organizing during encoding. However, as demonstrated in part C (immediate memory for the design) and part D (memory after a delay) of the Figure, the patient retains all of the information initially recalled over the delay and does not exhibit evidence of rapid forgetting. Intact recognition performance also suggests adequate retention over time. Language and visuospatial functioning are intact. Severe depression is endorsed. Despite these limitations, several strengths
www.turner-white.com
are noted. Specifically, he is able to retain information over time and learn effectively when information is presented in small amounts, repeated, and structured in ways that place low demands on executive functions of organization and planning. Case 2 Recommendations and conclusions In providing feedback to the patient, the neuropsychologist commends him for his efforts in working with his therapist and psychiatrist to find helpful compensatory strategies and to combat depression. Regarding recommendations, the patient is encouraged to continue this work, to participate in local support groups for head injury survivors, and to partake in pleasurable
Neurology Volume 13, Part 4
Neuropsychological Evaluation in Clinical Practice: Part 2 A
D
B
C
Figure. The Rey-Osterrieth Complex Figure (A) and the patient’s attempts to copy this figure, including a direct copy of the figure (B), a copy drawn after an immediate delay (C), and a copy drawn after a long delay (D). (Reprinted from Osterrieth PA. Le test de copie d’une figure complex: contribution a l’étude de la perception et de la mémoire. Archives de Psychologie. 1944;30:286–356.)
activities outside of the home. The patient is also encouraged to practice sleep hygiene (eg, maintain consistency in his hours of sleep each night; refrain from caffeine; exercise, but not directly before bedtime) since his sleep difficulties are likely exacerbating his cognitive difficulties. Additional recommendations provided to the patient and his providers include suggestions to help increase the effectiveness of his cognitive rehabilitation program by focusing on his strengths and using additional compensatory strategies, with the overall goal of returning to work at some level. First, the neuropsychologist suggests that he slow down when he attempts to complete a task, that he understand and accept that he may need more time to complete a task, and that he practice being more patient with himself. Ultimately, he will likely perform best at jobs that do not require speeded performance or multitasking. Second, results indicate that the patient performs best on over-learned, structured, simple, repetitive tasks. As test results also indicate that the patient learns most effectively when provided with repetition and helpful prompts, his wife and care providers are advised to repeat information several times to help facilitate encoding. While the patient displays trouble with retrieval of information, there is no indication of rapid forgetting; thus, he would be expected to retain this information
Hospital Physician Board Review Manual
once it is effectively learned. Third, the patient appears to benefit from learning through modeling and receiving positive feedback for his accomplishments. Fourth, friends, relatives, and providers are encouraged to provide small amounts of information at a time so as not to overwhelm him and to reduce and simplify encoding demands. Similarly, it is suggested he would benefit from frequent breaks throughout the day to avoid becoming overtaxed.
Case 3: A 66-YEAR-OLD MAN WITH COGNITIVE AND BEHAVIORAL DIFFICULTIES CASE Presentation and History A 66-year-old right-handed man is referred for a neuropsychological evaluation by his neurologist to assist with differential diagnosis and provide recommendations regarding possible treatments and interventions. The patient presents with a 1-year history of gradually progressive cognitive and behavioral difficulties. While the patient generally denies difficulties with memory (“I’m not aware of any changes.”), he explains that he used to be “good at fixing things” but
www.turner-white.com
Neuropsychological Evaluation in Clinical Practice: Part 2 recently has had some difficulty “figuring things out.” For example, he was unable to successfully set up a recently purchased television. In contrast, the patient’s wife reports that he often asks repetitive questions and occasionally inquires as to the date. She also notes that his handwriting is significantly less legible and is characterized by misspellings and that he has considerable difficulty keeping track of appointments. The patient formerly managed the household finances; however, his wife took this over approximately 4 months prior to the evaluation because he was forgetting to make payments and was having trouble managing money and calculating change. The patient continues to manage basic activities of daily living such as dressing and maintaining hygiene, although he is reportedly much slower to complete activities, sometimes stopping in the middle of carrying out an action. The patient’s wife recounts a “bizarre” incident that occurred the week before the evaluation, in which the patient was unable to get up from the toilet, remaining there from roughly 11:30 pm until the next morning. His wife was unable to lift him and had no choice but to call 911. After being lifted from the seat, he reportedly walked without difficulty. The event was not attributed to any evident sensory or motor dysfunction and was thought to be associated with his significant apathy. He continues to drive locally, despite several minor accidents (he was not able to provide details, but his wife has found dents on the car) and his neurologist’s urging that he discontinue driving. Other changes in driving ability include incorrect signaling, failing to notice traffic signs, and stopping in traffic for no apparent reason. The patient’s wife also reports that her husband engages in odd repetitive behaviors such as rubbing his knees (to the point of rubbing holes in his pants), jingling his keys repeatedly, and running his thumb back and forth on the handle of a coffee cup. She describes a significant change in his appetite and eating habits, with the patient having gained 50 lb over the past year due to constantly craving sweets and junk food (cookies, doughnuts, pies), when he had previously maintained a healthy diet. He also often plays with his food, “mashing it together.” In regard to mood, the patient reports that it is “just fine.” In contrast, his wife notes that he is apathetic and that he spends the majority of his time in front of the television “just sitting and staring.” She describes him as more socially withdrawn and less conversational, with diminished interest and participation in activities he once enjoyed. She also states that he occasionally makes inappropriate comments and gestures in public
www.turner-white.com
places, such as touching her inappropriately or making provocative comments to female store clerks, which she adds “just doesn’t seem like him.” The neurologic examination is notable for reduced spontaneous speech, reduced verbal fluency, poor planning when drawing a clock, inability to perform relatively simple working memory tasks, and significant impersistence throughout the motor exam. The examiner is also able to elicit positive snout and bilateral palmomental signs. Brain magnetic resonance imaging findings reveal marked cerebral volume loss, predominantly in the frontal and temporal regions. There is also asymmetric hippocampal formation volume loss, with loss on the right greater than on the left. There is no evidence of ventricular enlargement. The patient has experienced several episodes of incontinence over the 6 months prior to the evaluation. Additional medical history is unremarkable, with normal blood pressure and cholesterol level, no gait disturbance, and no history of head injury, substance abuse, or significant surgeries. The patient completed the eleventh grade, leaving school because he noted he was “always better with [his] hands than anything else.” He went on to work as a welder for most of his life, until he retired at age 55 years. The patient reports no significant family history of dementia and is the middle child with 2 siblings, one who died unexpectedly at age 52 years and another who is in good health at age 50 years. The patient notes that he had an “odd aunt” but is unable to provide details. He lives at home with his wife, eldest daughter, and her 2 sons. He has 5 children but is unable to provide their correct ages, and he reports having 17 grandchildren when there are actually 7. • What are possible causes of this patient’s cognitive and behavioral problems? Given the progressive nature of the patient’s difficulties, neurodegenerative disorders should be considered, including frontotemporal dementia (FTD) and AD. Characteristics such as a progressive course, lack of insight, reported memory impairment (forgetting appointments and bills, asking repetitive questions), difficulty managing finances, and apathy suggest AD. However, these symptoms also may be present in FTD, and the patient’s young age, significant behavioral symptoms (including disinhibition and repetitive behaviors), profound degree of apathy, and history of incontinence are more consistent with FTD. Despite the patient’s self report of mood being “just fine,” a psychiatric etiology also should be considered due to his apathy, anhedonia, and social withdrawal.
Neurology Volume 13, Part 4
Neuropsychological Evaluation in Clinical Practice: Part 2 Table 3. Neuropsychological Test Findings for Case 3: Assessment Data Domain/Test Administered
Result/Percentile/ Classification
Mini-Mental State Examination2
22/30
Premorbid functioning North American Adult Reading Test21 Attention/executive functions WAIS-III4 subtests Similarities Digit Symbol-Coding Digit Span Letter-Number Sequencing Clock Drawing Command Copy D-KEFS19 subtests Trail Making Visual scanning Number sequencing Letter sequencing Number-letter sequencing Motor speed Verbal Fluency Total letter (FAS) Total category Category switching total Language Boston Naming Test7 Memory Verbal memory WMS-III8 subtest Logical Memory Recall I Recall II % Retention Recognition
Average
9th, LA 5th, B 9th, LA 1st, I Borderline impaired Borderline impaired
16th, LA < 1st, I < 1st, I Discontinued 25th, A < 1st, I < 1st, I < 1st, I 5th, B
Result/Percentile/ Classification
Domain/Test Administered Hopkins Verbal Learning Test-R22 Total recall Delayed recall % Retention Recognition Free and cued selective reminding23 Free recall Free and cued recall Visual memory WMS-III subtest Visual Reproduction Recall I Recall II % Retention Recognition Visuoperceptual/visuospatial/ visuoconstructional functioning WAIS-III subtest Block Design Emotional functioning Geriatric depression scale12 Frontal Systems Behavior Scale24— family rating form* Apathy Disinhibition Disinhibition executive Total
3rd, B < 1st, I 16th, LA 9th, LA 17 points below cutoff 2 points below cutoff
2nd, I 9th, LA 25th, A 2nd, B
5th, B 5/30, within normal limits Before After symptoms symptoms 21st 99th 16th 96th 50th 99th 27th 99th
5th, B 2nd, I 9th, LA 37th, A
A = average; B = borderline; I = impaired; LA = low average. D-KEFS = Delis-Kaplan Executive Function System; WAIS = Wechsler Adult Intelligence Scale; WMS = Wechsler Memory Scale. *For informant report measures, higher percentiles indicate greater level of difficulty in these areas.
Case 3 Neuropsychological Evaluation Following the clinical interview, the patient is administered a battery of neuropsychological measures. A summary of the patient’s performance for each test is provided in Table 3. The patient is pleasant and cooperative throughout testing, and although he is somewhat quiet and does not spontaneously initiate conversation, he responds to questions asked directly. Throughout the evaluation, he engages in perseverative motor behaviors, rubbing his thumb and index finger together repeatedly and occasionally also rubbing his right pant leg. His presentation is also notable for difficulty initiating and persisting with task behavior. For example, on
tasks in which he is provided with instructions and then expected to provide continuous responses (ie, measures of fluency, counting, and reading), he tends to provide one response and then stop, requiring direct prompting from the examiner before he continues. Additionally, a fire alarm sounds in the building during the evaluation, and although it is quite loud, he appears unaware of the disturbance. When the examiner accompanies him to the stairs to exit, he needs prompting to start descending. The patient is generally appropriate throughout testing, but exhibits mild disinhibition, such as making 2 sexually provocative comments to the female examiner. Language is sparse but fluent, grammatically accurate,
10 Hospital Physician Board Review Manual
www.turner-white.com
Neuropsychological Evaluation in Clinical Practice: Part 2 and prosodic. Given the patient’s cooperative effort, the test results are considered a valid indicator of his functioning at that time. Premorbid intellectual functioning is estimated to fall within the average range as indicated by a test of word reading in addition to demographic and occupational history. The results of the evaluation demonstrate impairment in executive functioning, visuoconstructional skills, and memory encoding and retrieval. Executive dysfunction is characterized by reduced psychomotor processing speed, impaired set shifting with errors of impulsivity and perseveration, and difficulty with initiation and task persistence. An evaluation of memory reveals frontally mediated impairment, specifically with the patient’s ability to encode and retrieve information. His performance indicates that his retention of information is relatively more preserved; specifically, although he has difficulty freely recalling information, he is significantly aided by cueing and by yes/no recognition paradigms. Deficits are also seen within the visuoconstructional domain as he has trouble copying a complex figure and exhibits minor difficulty configuring patterned blocks. Confrontation naming is reduced. On an informant rating scale, his wife endorses significant mood disturbance (apathy), behavioral disinhibition, and dysexecutive symptoms, specifically, difficulty with problem solving. • How does this cognitive profile inform the differential diagnosis? This cognitive profile is suggestive of significant deficits in frontal/executive functioning and frontally mediated memory and together with the provided history is most indicative of a behavioral variant of FTD. Differentiation between FTD and a neurodegenerative disorder such as AD is often difficult; however, careful review of behavioral history in concert with neuropsychological findings can aid in this delineation. Behavioral symptoms associated with FTD include hyperorality, early loss of social awareness and insight, early signs of disinhibition, difficulties with initiation, and perseverative behaviors, often with insidious onset before the age of 70 years.25 This patient exhibited many of these behavioral symptoms in interview and during testing, including repetitive, perseverative motor behaviors, disinhibition with little to no awareness, trouble with initiation, lack of insight into cognitive difficulties, and apathy. Behavioral symptoms of AD are numerous and may include the mentioned behaviors, especially apathy. However, in AD behavioral symptoms do not predominate, and although they can occur early in the disease course, they usually do not significantly inter-
www.turner-white.com
fere with daily living until the later stages of the disease. In addition, the age of onset of difficulties in this patient is typical of FTD, which has a mean age of onset of 62 years and is more common in younger patients.25 The patient’s neuropsychological findings are consistent with FTD as these patients frequently exhibit greater impairment on measures of executive functioning (including measures of problem solving) than on memory measures, a pattern considered to be the opposite of that encountered in AD.26 Also, patients with FTD benefit more from cueing and typically do not exhibit the rapid forgetting associated with AD.27 In summary, features most suggestive of FTD in this patient are the medical history (including incontinence), neuroimaging findings, age of onset, the progressive course of the illness, the history of change in personality with marked apathy, and the cognitive profile signifying frontal systems dysfunction. The patient’s marked executive dysfunction, strategic memory disturbance, and initial symptoms of change in personality and behavior are not consistent with the pattern of deficits typical of AD. Finally, although the patient exhibited some symptoms of mood disturbance such as apathy and anhedonia, he did not report depressed mood state, and his neuropsychological profile as well as behavioral changes were far more pronounced than would be expected in a case of cognitive dysfunction secondary to mood disorder. Case 3 Recommendations First and foremost, the patient and his wife are educated about the diagnosis of FTD to help them better understand symptoms of the illness at the present time and to help them prepare for the future as the disease progresses. Second, they are provided with in-depth information surrounding safety in and out of the home. For example, the patient is encouraged not to cook or operate dangerous welding equipment (as he had been a welder and still has this machinery). The installation of safeguards is suggested as he may experience difficulties when alone and may have trouble calling for assistance. He is also encouraged to immediately cease driving, even locally, for his safety and the safety of others given his considerable difficulty with self-monitoring and his wife’s report of unsafe driving. The patient and his wife are also encouraged to keep him engaged in social and mental activities to the greatest extent possible, as withdrawal from activities is likely due not only to apathy but also to his trouble with initiation and task persistence. Additionally, compensatory strategies are reviewed with the patient and his wife,
Neurology Volume 13, Part 4 11
Neuropsychological Evaluation in Clinical Practice: Part 2 including providing cues to trigger memory, keeping lists to guide daily activities and routines, and maintaining an organized environment with items kept in the same place to enhance structure and cueing within the home. Last, a reevaluation is recommended in 1 year to monitor the patient’s cognitive functioning and modify recommendations as necessary.
CASE 4: A 9-YEAR-OLD GIRL WITH READING DIFFICULTY CASE Presentation and History A 9-year, 9-month-old right-handed 4th grade girl is referred for a neuropsychological evaluation due to a 2-year history of reading difficulty first noticed by her mother and teachers. The evaluation was requested in order to assess the child’s cognitive strengths and weaknesses, to address questions pertaining to differential diagnosis, and to provide recommendations regarding possible educational services and interventions. The patient is accompanied by her mother, who provides much of the history. She has been attending the same private school since prekindergarten and since starting school has received teacher evaluations that consistently comment on her lack of confidence and significant anxiety. Her anxiety interferes with test taking and reading aloud during class. She is anxious about many things such as the weather (eg, if it is cloudy, she fears a storm), and she became fixated on fears of death and dying after 2 family friends unexpectedly died within the past 2 years and the patient was reportedly “unable to let it go.” She has a history of depression, and her mother mentions that the family “had some bad years” where they moved numerous times over a span of 4 years. It is also indicated that the patient often becomes fearful and sad if her mother is away for any period of time. She had been tutored 2 times per week the year prior to the evaluation, and the patient’s mother states that her daughter was particularly anxious on those days. At the time of the evaluation, the patient is no longer being tutored because of the stress and self-esteem issues she experienced surrounding tutoring the previous year (tutoring had been provided within the school environment). The patient’s mother describes self-esteem as a major issue. For example, although the child is a wonderful athlete, her success in this arena does not seem to bolster her self-esteem. About twice a week, the patient experiences difficulty falling asleep due to thoughts surrounding death. Once she falls asleep, she is able to stay asleep, although she reportedly talks and walks in her
12 Hospital Physician Board Review Manual
sleep occasionally. Appetite disturbance is denied. The patient sees a therapist, although her mother notes that she has not received feedback from the therapist for a while. In general, her mother feels that her daughter seems happy. Goals of the evaluation include determining the patient’s level of academic achievement and the presence or absence of a learning disorder and assessing the impact of anxiety on cognitive functioning. Medical history is unremarkable, with no hospitalizations, significant illness, or head injury. The patient’s mother describes her pregnancy as relatively normal. The patient was delivered vaginally, and the cord was wrapped around her neck at birth, although there were no associated or further complications. The patient’s mother describes her as having been a “tough” baby, with trouble with sleep and as “extremely defiant,” describing incidents in which she had pulled down her dresser and threw everything out of her room. Her mother cannot remember when this ended but states that the patient is now much more agreeable. However, there are times when she continues to refuse to cooperate (eg, the summer prior to the evaluation, she refused to cooperate with a reading tutor). The patient’s mother describes significant family stressors that were especially salient when the patient was a baby. At that time, she was going through a divorce with her husband, who struggled with addiction, mental health problems, and learning disabilities. The patient currently lives with her mother, older brother, and stepfather, with whom she has a good relationship. At the time of the evaluation, she has not seen her biological father in over 2 years. Aside from the biological father’s learning disabilities, reportedly dyslexia, there is no other family history of learning disorders, and her older brother is doing well in school. Regarding educational history, the patient reportedly did well in preschool, where she was very social, kind, and inquisitive. She has no attentional or behavior issues, and has no difficulty making friends. Her mother states that the patient struggles when she reads aloud and describes her reading as nonfluent. She also states that the patient never picks up a book on her own to read. It is noted that the patient continues to spell phonetically. The patient enjoys school, where math is her favorite subject. However, she struggles to work independently and receives poor grades for effort other than in gym class. Completing homework is problematic, and within the past year she has become very anxious about it, telling her mother, “I can’t do this.” • What are the possible causes of this patient’s reading difficulty?
www.turner-white.com
Neuropsychological Evaluation in Clinical Practice: Part 2 Given the patient’s difficulty reading, significant fear of tutoring surrounding reading and reading aloud, continued phonetic spelling, and family history of learning disorders, a developmental learning disorder is considered as a primary etiology, specifically developmental dyslexia. Additionally, given her history of anxiety, depression, and low self-esteem, difficulties secondary to mood are also a consideration. CASE 4 Neuropsychological Evaluation Following the clinical interview, the patient is administered a battery of neuropsychological measures to assess functioning. A summary of the patient’s performance for each test is provided in Table 4. During the evaluation, the patient initially presents as somewhat lethargic and disinterested in testing, although this seems to be due to a lack of confidence and reticence on her part to be tested. Given her reluctance, her mother is invited to join her in the testing room, and the patient seems to feel more comfortable with her there. Following lunch, the patient’s affect is significantly brighter, and her confidence and level of cooperation appear to be greatly improved. She is tested without her mother present and appears to be much more comfortable and fully engaged in testing. However, the patient’s confidence significantly varies by type of test. She is far more interested and engaged in tasks that involve visuospatial (rather than verbal) processing. Also, on academic testing, her confidence level and demeanor differ significantly between math and reading measures. In general, her presentation suggests that her confidence level significantly affects her ability to fully engage and exert effort. This factor is taken into consideration when interpreting the test results. While the patient is able to sustain attention for the evaluation, behavioral presentation is notable for some motor restlessness as she frequently slides down in her chair and needs reminders to sit up. Evaluation results indicate that the patient has average to high average intellectual functioning. Cognitive skills are relatively evenly developed, with a relative strength in processing speed. In contrast, testing in the domain of academic achievement provides evidence of a less consistent pattern. While the patient exhibits a considerable strength in math skills, particularly in her conceptual understanding, she exhibits a relative weakness in her reading skills, with difficulties evident in her word reading, fluency, and an inconsistency in her reading comprehension. On measures administered to assess functioning within individual cognitive domains, she exhibits a relative strength in attention and executive functioning, although difficulties are evident when
www.turner-white.com
the information is unstructured or complex. For example, on a verbal list learning task and a task in which she is asked to copy a complex figure, she fails to structure the information on her own, which would have made it easier to learn. She also exhibits a mild weakness in visuomotor integration, evident in her copy of a complex figure and in her copy of simpler geometric figures. Finally, both her presentation and her mother and teacher’s report on behavior checklists indicate significant problems with anxiety. The patient’s anxiety is evident in both home and school environments and appears to significantly impact her confidence as well as her self-esteem. This is evident in testing, where her difficulties with reading appear to be compounded by her low level of confidence in her abilities within this domain. The patient’s teacher also endorses a number of significant difficulties with executive functioning, which appear to be more evident in the more complex and demanding school environment than at home. Her teacher’s responses indicate that she has significant difficulties with working memory, planning and organizing, and organization of materials, and has milder difficulties initiating and monitoring her behavior on tasks. • How does the patient’s cognitive profile inform the differential diagnosis? These findings support a diagnosis of developmental dyslexia, with the patient’s reading skills falling significantly below expectations based on her level of intellectual functioning and grade placement. Moreover, the nature of her difficulties, characterized by a weakness in phonologic decoding, is consistent with the core deficit in developmental dyslexia. These difficulties impact the patient’s fluency as well as her reading comprehension and also appear to impact and to be impacted by her anxiety. That is, the patient’s anxiety exacerbates her reading difficulties, and her reading difficulties foster her lack of confidence and her level of anxiety within the school environment. The patient’s anxiety, while clearly impacted by her learning difficulties, extends beyond this, with specific phobias and obsessions as well as a generalized lack of self-confidence. Case 4 Recommendations Recommendations provided to the patient, her mother, and teachers first and foremost highlight the importance of tutoring directed at improving her weak reading skills. The neuropsychologist suggests that tutoring be provided to the patient 2 times per week and that it be based on a direct, phonologic, multisensory, and sequential method in order to address her specific underlying phonologic weakness. It
Neurology Volume 13, Part 4 13
Neuropsychological Evaluation in Clinical Practice: Part 2 Table 4. Neuropsychological Test Findings for Case 4: Assessment Data Domain/Test Administered Intellectual ability Wechsler Intelligence Scale for Children-IV28 Verbal comprehension Perceptual reasoning Working memory Processing speed Full scale IQ Verbal comprehension subtests Similarities Vocabulary Information Perceptual reasoning subtests Block Design Picture Concepts Matrix Reasoning Working memory subtests Digit Span Letter-Number Sequencing Processing speed subtests Coding Symbol Search Academic achievement Gray Oral Reading Test-429 Rate Accuracy Fluency Comprehension Quotient Wechsler Individual Achievement Test-II30 Word reading Reading comprehension Pseudoword decoding Numerical operations Math reasoning Spelling Written expression Reading Mathematics Written language
Percentile
Classification
66 50 34 84 63
Average Average Average High average Average
84 50 63
High average Average Average
37 75 37
Average High average Average
37 37
Average Average
75 84
High average High average
37 25 25 63 42
Average Average Average Average Average
21 50 50 77 99 53 45 32 97 45
Low average Average Average High average Superior Average Average Average Superior Average
Domain/Test Administered Attention/executive functions D-KEFS6 subtests Trail Making Test Visual scanning Number sequencing Letter sequencing Number-letter switching Motor speed Verbal Fluency Total letter Total category Category switching total Category switching accuracy Total set-loss errors Total repetition errors Design Fluency Filled dots Empty dots Switching Total set-loss Total repeated Language Expressive Vocabulary Test-231 Peabody Picture Vocabulary Test-432 Visuoperceptual/visuospatial/ visuoconstructional functioning Developmental Test of Visual Motor Integration-533 Rey-Osterrieth Complex Figure Test10 Copy Memory California Verbal Learning Test— children’s version34 List A trials 1–5 total List A trial 1 List A trial 5 Learning Slope List A Trials 1–5 List A short delay free recall List A short delay cued recall List A long delay free recall List A long delay cued recall
Percentile
Classification
91 91 91 95 84
Superior Superior Superior Superior High average
75 99 84 84 50 50
High average Very superior High average High average Average Average
97 75 37 25 37
Superior High average Average Average Average
66 73
Average Average
18
Low average
