The Right Hemisphere and Disorders of Cognition and Communication

Theory and Clinical Practice

Theory and Clinical Practice

Margaret Lehman Blake, PhD 5521 Ruffin Road San Diego, CA 92123 e-mail: [email protected] Website: http://www.pluralpublishing.com

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Library of Congress Cataloging-in-Publication Data: Names: Blake, Margaret Lehman, author. Title: The right hemisphere and disorders of cognition and communication : theory and clinical practice / Margaret Lehman Blake. Description: San Diego, CA : Plural, [2018] | Includes bibliographical references and index. Identifiers: LCCN 2017028234| ISBN 9781597569620 (alk. paper) | ISBN 1597569623 (alk. paper) Subjects: | MESH: Communication Disorders | Cognition Disorders | Cerebrum--physiopathology | Brain Injuries--physiopathology Classification: LCC RC423 | NLM WL 340.2 | DDC 616.85/5--dc23 LC record available at https://lccn.loc.gov/2017028234 Contents

Preface vii Foreword ix Acknowledgments xi Reviewers xiii

1 The Right Hemisphere 1

2 Fundamentals of Clinical Practice 13

3 Pragmatic Aspects of Communication 29

4 Language Comprehension 63

5 Prosody 91

6 Attention 107

7 Unilateral Neglect 123

8 Executive Functions 153

9 Awareness 175

10 Memory 189

Appendix. Psychometrics for Select Assessments 207 References 235 Index 289

Preface

This book covers decades of work by The remaining chapters all begin with researchers in a variety of fields who all an overview of the construct and how that have been interested in what happens in construct is processed in the intact right the right side of the brain. It is designed hemisphere. This is followed by how the for advanced graduate students and prac- construct is affected by RHD and what ticing clinicians interested in neurogenic we currently know about assessment disorders of cognition and communica- and treatment. Given the current state of tion. The perspective is from the field the art and science in the area of RHD, of speech-language pathology, but the the assessment and treatment sections knowledge should be useful for a broad are relatively scarce in terms of concrete range of professionals interested in cogni- evidence-based practice. With this current tion and communication. reality, it is crucial to have a solid under- The first chapter provides an intro- standing of cognitive and communica- duction to right hemisphere brain dam- tion processes and the theories of how age (RHD) and some of the reasons why they are affected by RHD to guide clinical patients and clients with RHD often do not decision making. The treatment sections receive the same recognition or treatment build upon what we do know, and contain as survivors of left hemisphere strokes. many suggestions based on evidence from The second chapter provides a review the traumatic brain injury (TBI) literature of some fundamentals of clinical prac- and theoretically based expert opinion. tice, including the World Health Orga- For areas in which the research and the nization’s structure for viewing health theories are solid enough to support treat- and disability, cultural awareness, evi- ment approaches, I provide specific sug- dence-based practice, and practice-based gestions for approaching treatment (e.g., research. While it may seem odd to have language comprehension). For other areas two introductory chapters, they serve (e.g., anosognosia), explicit approaches very different purposes: one to introduce that go beyond the existing expert opin- the population, and the second to set the ion or evidence from TBI are not provided stage for working with that population. It because the theoretical support is not is important to approach assessment and strong enough for me to feel comfortable treatment with consideration of clients’ doing so. personal and environmental contexts, There are many possible ways in which their cultural background, and plans to the chapters could be organized, because assess treatment effectiveness all firmly in the areas of cognition and communica- the front of your mind. Thus, the review tion overlap and interact. Indeed, com- of these areas appears before the chapters munication is a cognitive process. They on the disorders. are divided here because in the field of

vii viii The Right Hemisphere and Disorders of Cognition and Communication: Theory and Clinical Practice

speech-language pathology we tend to discussed in the book, thus it is the first think of language and communication as “content area” following the introduc- separate from other cognitive processes. tory chapters. This is followed by lan- In this book, aspects of communication guage comprehension and prosody. The are presented first, followed by the other remaining chapters cover cognition: atten- cognitive areas. The pragmatics chapter tion and neglect, executive function and provides a model of social communication anosognosia, and finally memory. that sets the stage for all of the processes Foreword

About 23 years before I had the pleasure The entire book is terrific, but I par- of reading the prepublication chapters ticularly loved Chapter 1, in which Peggy of Margaret (Peggy) Blake’s wonder- astutely comments on how and why ful, informative new book, I had asked patients with right hemisphere disorders her to do the same thing for me. At that (RHD) often get “lost in the system.” She time I was a young(er) professor and was elaborates on the discrepancy in detecting thoroughly delighted to have convinced and intervening with the problems of right Peggy to come work with me as a PhD stu- versus left brain stroke patients, begin- dent. Fast-forward several years, past her ning with the earliest medical contacts assiduous work on several of my grants, and proceeding through various clinical a number of our joint publications, and assessment and management processes. multiple research projects of her own, and The rest of the chapter provides additional Peggy had become the only PhD gradu- important introductory material about the ate I know whose dissertation committee population of adults with RHD. Peggy required not even one change in her the- really connects with readers through fun sis document. The clarity of thought and thought experiments about vital right style connoted by this fact continue to be hemisphere contributions to communica- evident in the current volume. tion. This chapter also emphasizes essen- Peggy quickly developed into, and has tial issues such as patient/symptom het- remained, an influential sister-in-arms in erogeneity, thinking beyond the standard the pursuit and evaluation of knowledge clinical stereotypes, and common research about the nature, assessment, and man- problems. agement of cognitive/language disorders Chapter 2 helps to lay a strong foun- in adults with damage to the right side of dation for clinical work with the people the brain. There weren’t many investiga- who have RHD. It is an extremely useful tors interested in the topic 23 years ago guide to viewing the existing evidence and there still aren’t — a fact that makes me with an appropriately critical eye, and to even prouder of Peggy’s continued, sub- helping readers understand how they can stantive leadership through her research be involved in expanding this evidence. and publications, educational offerings, The chapter focuses in part on the nature and professional service roles. It has been of evidence and different sources of evi- a joy to collaborate with her and to learn dence, along with challenges to clinical from her over the years, having watched assessment and evidence-based practice. her grow into the expert who, among other It also offers some solutions to these chal- considerable contributions to the field, lenges. For example, Peggy calls the lack wrote the clear, engaging, and authorita- of data and investigation “a golden oppor- tive volume you have in your hands. tunity” to apply a practice-based evidence

ix x The Right Hemisphere and Disorders of Cognition and Communication: Theory and Clinical Practice

model with the RHD population, by gath- tory bodies of literature. In addition, they ering evidence in typical clinical situations offer the best clinical solutions currently to influence management practices. available, including borrowing from the The remaining chapters each tackle evidence about other populations with cognitive/language areas that are often similar disorders and theoretically based affected by RHD: pragmatics and social possibilities. Equally important, Peggy’s communication (including discourse pro- approach provides clinicians with remind- duction), discourse comprehension, pros- ers and tools that will help them find the ody, attention, neglect, executive functions, best solutions next week, next year, and awareness, and memory. In each chapter, many years from now. Peggy begins by introducing relevant the- This is a really opportune moment for ories and models and reviewing evidence Peggy’s book. The literature on normal on normal right hemisphere functioning. right hemisphere function has boomed — Each chapter ends with coverage of RHD much of it after investigators who were symptoms, assessment, and treatment con- interested in left brain functioning saw siderations. The coverage is typically com- the right hemisphere activation in their prehensive and always clear and under- investigations of the brain bases of nor- standable. Periodic sidebars help to clarify mal language and cognitive processes. difficult concepts, making the material even Theory and evidence about right hemi- more engaging. Tables and figures provide sphere disorders and their clinical man- useful summaries or illustrations, includ- agement have continued to grow since the ing, for example, the extremely helpful last book of this sort. And the literatures table that depicts manipulations that affect on evidence-based practice and practice- performance in the chapter on Neglect, based evidence have blossomed. Bringing and the excellent examples of the “contex- these literatures together in a comprehen- tualization process” for novel idioms in the sible and enlightening way does a real chapter on Language Comprehension. service for readers of all kinds. I enjoyed These chapters admirably bring to- reading every word and can’t wait to see gether vast, complex, and often contradic- what Peggy does next.

— Connie A. Tompkins, PhD, CCC-SLP, BC-ANCDS Professor Emeritus Communication Science and Disorders Center for the Neural Basis of Cognition University of Pittsburgh Pittsburgh, PA Acknowledgments

This book would not have been possible sphere. It also allowed me the opportunity without the many very smart, dedicated, to collaborate with Penny Myers and add amazing people who influenced my edu- a more direct clinical component to my cational journey through the “other side” views on RHD. Thanks also to the many of the brain. My first introduction to right colleagues who have been supportive of hemisphere brain damage (RHD) came my work throughout the years. in my graduate program at Arizona State While working on this book I received University when Dr. Leonard LaPointe valuable assistance with collecting, sort- suggested that I focus on RHD for my ing, and reviewing information from sev- Master’s thesis. I am forever indebted eral students at the University of Houston: to him for that suggestion, as it was the Natalie Ewing, Dionne Dias, Aaron Rodri- launching point for my career researching, guez, Kelly Tobey, and especially Jessica teaching, writing, and wondering about Connors, who spent many hours poring the right hemisphere. My path was solidi- over psychometric properties of cognitive fied when I spent several years working and communication assessments. Thanks with Connie Tompkins at the University of also to Jerry Hoepner and Rik Lemoncello Pittsburgh. She began as my mentor, and who lent their expertise to the chapters on became my colleague and friend. The year executive function and practice-based evi- I spent at the Mayo Clinic working with dence. Finally, thanks to Kalie Koscielak at Joe Duffy and Edy Strand strengthened Plural, who was so helpful at every stage of not only my clinical skills, but also my this process, and to the reviewers who took appreciation for other parts of the brain, the time to read the book draft and make like the basal ganglia and the left hemi- smart, thoughtful suggestions for changes.

xi 4 The Right Hemisphere and Disorders of Cognition and Communication: Theory and Clinical Practice

evidence to support this theory is incon- RH Function sistent (Rajah & D’Esposito, 2005); some studies do report asymmetrical changes Lateralization of function and lateralized related to aging (e.g., Dolcos, Rice, & asymmetries are not unique to humans; Cabeza, 2002; Goldstein & Shelly, 1981; they have been reported rather exten- Miller, Myers, Prinzi, & Mittenberg, sively in both vertebrates and inverte- 2009), but others indicate that age-related brates (Corballis, 2014). Most primates and changes in the size of structures are marine mammals show LH dominance for roughly equivalent across the hemispheres action dynamics, and all primates studied (Raz et al., 2005; Salat et al., 2004). While thus far show RH dominance for emotion. differential changes have been observed Some species (e.g., frogs, mice) also show in regions within the prefrontal cortex, LH dominance for vocalization. with greater changes in anterior and dor- The RH has long been thought to have sal regions of the RH compared with the greater interconnectivity than the LH. LH, these prefrontal changes occur in Early work supported this idea based on tandem with bilateral (and symmetrical) the greater amount of white matter in the changes in the ventral regions (Rajah & RH compared with the LH (Gur et al., D’Esposito, 2005). The RH may influence 1980). More recent studies employing a aging in other ways. Robertson (2014) variety of imaging techniques (magneto- suggests that the RH-biased networks for encephalography, near infrared spectrom- arousal, sustained attention, awareness, etry) have provided additional evidence and response to novelty may underlie the of differential white matter organization. construct of cognitive reserve, in which The RH appears to have greater functional individuals with higher education, higher interconnectivity than the LH (Gootjes, IQ, and more complex job responsibilities Bouma, Van Strien, Scheltens, & Stam, appear to have some “protection” against 2006; Iturria-Medina et al., 2011; Li et al., cognitive deficits related to brain injury 2014; Medvedev, 2014). The organizational and neurodegenerative disease. patterns suggest that the RH is better at general information processing such as integration processes, in contrast to the History of Understanding LH, which is more efficient at specialized RH Functions processing such as language and motor action (Iturria-Medina et al., 2011; Li et al., 2014). Historically, discovery of localization of It has been suggested that cognitive neurological function began with study- changes associated with aging reflect ing patients. This is true for RH functions. differential changes in the hemispheres. Much of our understanding of what the An early theory suggested that aging RH does comes from early studies of affected the RH more than the LH, result- patients with focal lesions and an explo- ing in RHD-like symptoms in older adults ration of their deficits. While there were (Goldstein & Shelly, 1981). Neuroimaging occasional descriptions of deficits attrib- 1. The Right Hemisphere 5 uted to RHD beginning in the late 1800s1 1990s, with Myers’ (1990) inference fail- (see review by Heilman, Bowers, Valen- ure hypothesis, Tompkins and colleagues’ stein, & Watson, 1986), it was not until the suppression deficit hypothesis (Tompkins, mid-1900s that specific functions of the Lehman, & Baumgaertner, 1999; Tomp- right hemisphere were explored in earnest kins, Baumgaertner, Lehman, & Fass- (see reviews in Blake, 2016; Heilman et al., binder, 2000; Tompkins, Lehman-Blake, 1986; and Searleman, 1977). Baumgaertner, & Fassbinder, 2001), and Case studies and experiments involv- Beeman’s (1998) coarse coding hypoth- ing visuoperceptual deficits, visuospa- esis. These are discussed in more detail in tial agnosia, and unilateral neglect began Chapter 4, along with the first treatments appearing in the 1940s (McFie, Piercy, & for language deficits associated with RHD Zangwill, 1950). While the early reports that were published in the 2000s. The clin- suggested that these disorders could not ical history of RHD thus is relatively new, be unequivocally linked to RHD, it was not beginning nearly a century after the dedi- long before the RH was considered “domi- cated interest in the LH. nant” for visuoperception. Language and communication were addressed in the 1960s, with the suggestion that RHD could Identification and affect abstract and complex language pro- Treatment of cessing (Critchley, 1991; Eisenson, 1962). Right Hemisphere Stroke In the 1970s there was a dramatic increase in the number of studies of emotion, prosody, visuoperception, and uni- RH and LH strokes occur at approxi- lateral neglect (Blake, 2016; Ross, 1984) mately the same frequency (RH 45%; LH that led to the current understanding that 55%) (Foersch et al., 2005; Hedna et al., the RH is dominant for these functions. 2013). However, there are stark differ- During that same time frame, descriptive ences in the recognition and treatment of studies of language and communication RH and LH strokes (Figures 1–1 and 1–2). supported ideas proposed by Critchley To fully grasp the issues, it is important (1991) and Eisenson (1962) that RHD to understand stroke treatment. Currently, resulted in changes to “extra-linguistic” the most effective medical treatment for or complex language, including interpret- ischemic stroke is tissue plasminogen ing connotative meanings, story morals or activator (tPA), a “clot-busting” drug. gist, comprehending sarcasm and humor, When administered within 4 hours after and other forms of nonliteral language the onset of a stroke, it can dissolve the (Blake, 2016; Perecman, 1983; Wapner, clot, restore blood flow, and substantially Hamby, & Gardner, 1981). Development reduce the amount of tissue damage and of theories to explain the language and resulting disability, thus significantly communication deficits occurred in the improving a patient’s prognosis. Beyond

1 Hughlings Jackson described visuoperceptual deficits in 1876; Babinski described anosognosia and changes to affect in 1914 (Langer & Levine, 2014). 6 The Right Hemisphere and Disorders of Cognition and Communication: Theory and Clinical Practice

Figure 1–1. Differences in age of onset and stroke type based on side of lesion (based on Foersch et al., 2005).

Figure 1–2. Timing of admission and pharmacological treatment based on side of lesion (based on Foersch et al., 2005). Note. tPA, the gold standard clot-busting treatment for ischemic strokes.

the 6-hour point, tPA has little effect. Thus, Adults with LH stroke are more likely early identification of stroke is critical to to get to an emergency department within receiving the best care. the critical time frame, are more likely to 1. The Right Hemisphere 7 get tPA, and tend to spend shorter amounts Table 1–1. Characteristics Influencing of time in acute care settings. In contrast, Timing of Arrival to an Emergency adults with RH stroke may not get to the Department hospital in time to receive the best medi- Early Arrival cal treatment and typically have poorer outcomes (Fink et al., 2002; Foersch et al., Good social network 2005; Hedna et al., 2013; Wee & Hopman, Severe stroke 2005). This is particularly evident for tran- Hemorrhagic stroke (versus ischemic or sient ischemic attacks (TIAs): of TIAs diag- TIA) nosed in the hospital, 63% are in the LH, Signs/symptoms include: and only 38% in the RH. The reason for sudden confusion this is not likely to be physiological, as RH speech/language problems strokes occur nearly as often as LH. Rather, hemiparesis it may be a difference in the rate of recogni- loss of consciousness tion of the mild signs or symptoms of LH Late Arrival versus RH TIAs (Foersch et al., 2005). Characteristics associated with early Live alone arrival to a hospital are provided in Mild stroke Table 1–1. The physical and somatosen- Right hemisphere stroke sory signs (hemiparesis or hemisensory Female* deficits) should occur equally as often from LH and RH strokes. However, in Note. *Females take 46% longer to get to an emergency department and wait 49% longer for treat- most cases aphasia probably is more obvi- ment at hospitals than males. ous than the cognitive-communication Sources: Foersch et al., 2005; Jorgenson et al., deficits associated with RHD and is more 1999; Maze & Bakas, 2004; Turan et al., 2005. likely to be recognized as a problem by patients or family members. Fink (2005) suggests that the presence of anosognosia, The bias in diagnosis and treatment of or reduced awareness of deficits, could stroke persists once an individual arrives be a major barrier to the recognition of at a hospital. Two of the most commonly RH stroke symptoms. Anosodiapho- used stroke scales are the National Insti- ria, or reduced concern for deficits, may tutes of Health Stroke Scale (NIHSS; Brott also play a role. A person who appears et al., 1989) and the Scandinavian Stroke to downplay any changes may be able Scale (SSS; Scandinavian Stroke Study to convince a spouse or family member Group, 1985). Both assess motor, sensory, not to seek medical attention for him/ and language functions and are used to her. While these suppositions make logi- assess severity of stroke. However, both cal sense, it is unknown how common are notably biased toward LH signs. Of anosognosia and anosodiaphoria are in the 42 points on the NIHSS, seven are initial presentation of stroke and if they related to language function to identify are actual contributors to recognition of aphasia. Only two points are related to stroke signs and symptoms. unilateral neglect, and those are based on 8 The Right Hemisphere and Disorders of Cognition and Communication: Theory and Clinical Practice

observation of performance on a picture ated with RHD. It is likely that aphasia, description task, not on a specific assess- which occurs in about 50% of adults with ment of unilateral neglect. The SSS has LH strokes, is one of the primary con- 10 of 58 points related to language, but cerns of neurologists. However, cognitive- none for any deficit related to RHD. In a communication deficits occur with about study examining the relationship between the same frequency — in about 50% of NIHSS scores and amount of tissue dam- adults with RH strokes (Blake, Duffy, age, for mild strokes (scores 0–5) individu- Myers, & Tompkins, 2002; Côté, Payer, als with RHD had twice as much tissue Giroux, & Joanette, 2007) — but may not loss (8.8 cc) as those with left hemisphere be considered at all. brain damage (LHD) (3.9 cc), with com- Unilateral neglect is arguably the best- parable NIHSS scores (Fink et al., 2002). known deficit related to RHD. Indeed, the Additionally the NIHSS is relatively insen- presence of neglect increases a patient’s sitive to cognitive deficits. In one recent chance of receiving tPA by approxi- study, approximately 40% of patients mately 40% (Di Legge, Fang, Saposnik, & with an NIHSS score of 0 (extremely mild Hachinksi, 2005). It is commonly assessed stroke) had at least one cognitive deficit by asking a patient to draw simple pic- (Kauranen et al., 2014). tures such as a butterfly or an analog clock. Physicians and researchers from Johns Such representational drawing tasks are Hopkins (Agis et al., 2010; Gottesman et al., not very sensitive and may identify only a 2010) have suggested several additions small percentage of individuals with uni- to the NIHSS to increase its sensitivity lateral neglect (Appelros, Nydevik, Karls- to RHD. One is to evaluate content units son, Thorwalls, & Seiger, 2003). Addition- (CUs) produced in response to the Cookie ally, according to a recent study of acute Theft picture description task. A variety stroke, unilateral neglect occurs in only of measures of CUs (CU/minute, ratio about 25% of patients, and the presence of CU from left and right sides of the of neglect alone identifies only 63% of RH picture, number of interpretive CUs [see strokes (Dara, Bang, Gottesman, & Hillis, Chapter 4]) were related to tissue loss in 2014). Thus, even a sensitive measure of various areas of the RH (Agis et al., 2010). neglect will not fix the imbalance in the The addition of visual extinction and recognition of LH and RH stroke. line bisection tasks (see Chapter 7) also Beyond the initial diagnosis and medi- increased the sensitivity of the NIHSS to cal treatment, the absence of clear patterns RH lesion size (Gottesman et al., 2010). of deficits and a standard label for “right In addition to the stroke scales, neu- hemisphere cognitive-communication dis- rologists and physicians have other ways orders” creates problems in both research to evaluate specific stroke-related defi- and clinical practice. In research studies, cits. While aphasia often is readily appar- often there are no a priori criteria to iden- ent after an LH stroke, it can be relatively tify and exclude the potential participants objectively screened using a set of easy-to- who have no cognitive or communication administer tasks. The same is not true for disorder. This adds to the heterogeneity of cognitive-communication deficits associ- participant samples, reducing the power 1. The Right Hemisphere 9 of the experiments and the strength of the and the presence of cognitive deficits conclusions that can be drawn. Addition- (Appleros, 2007; Gillen, Tennen, & McKee, ally, there are no standard clinical proce- 2005; Jorgenson et al., 1999; Kong, Chua, dures for determining the presence of a & Tow, 1998; Pedersen et al., 1996). Func- cognitive-communication disorder. This tional status at discharge has been linked is complicated by the limited options for to stroke severity, age, unilateral neglect valid, reliable, and sensitive assessment and anosognosia, depression, and pres- tools (see discussion in Chapter 2). ence of cognitive deficits (Meijer et al., The disparities continue after a patient is 2005; Paolucci et al., 1996; Pedersen et al., sent home, in regard to available resources. 1996; Vossel, Weiss, Eschenbeck, & Fink, General resources for stroke survivors 2012; Wee & Hopman, 2005). The likeli- obviously would be the same for RHD hood of being discharged to a dependent- and LHD. However, an individual with living environment is related to older age, aphasia has numerous resources for advo- anosognosia for illness, unilateral neglect, cacy groups, support groups, and sources and presence of cognitive deficits (Jehkonen of education (Aphasia Access, National et al., 2001; Kammersgaard et al., 2004; Aphasia Association, Aphasia Now, etc.). Paolucci et al., 1996; Wee & Hopman, 2005). A patient with “cognitive-communication The presence of cognitive deficits deficits” or some other vague diagnostic impacts a variety of outcomes. However, label will have a much harder time finding what constitutes a “cognitive deficit” is not resources or education sources specific to clear. While in speech-language pathology his/her deficits. aphasia is generally considered in its own category and deficits such as attention, memory, and executive function are put 2 Impact of Deficits into a “cognitive” category, many other Associated With RHD disciplines do not make this distinction. Thus, the outcomes linked to cognitive disorders described above are linked to A variety of studies have been conducted problems in attention, memory, executive to identify predictors of stroke outcome. functions, and/or aphasia. A second issue While there are many discrepancies across is how cognition is measured. Often, gen- studies, some general patterns are appar- eral screenings such as the Mini Mental ent in relation to deficits associated with State Exam (Folstein, Folstein, & McHugh, RHD (Table 1–2). The length of stay in 1975) are used as indicators of cognitive a medical setting, either in acute care deficits. Such tools are designed only to settings or acute and subacute settings screen for such deficits and are not sensi- combined, has been related to severity of tive measures of cognition (see discussion stroke, the presence of unilateral neglect, in Chapter 2).

2 Language is a cognitive function, and thus aphasia is a cognitive disorder. However, there is a long-standing tradition in speech-language pathology to think of language separate from the “other cognitive disorders.” x x x Deficits Visuoperceptual Visuoperceptual x x x Anosognosia x x x x x Neglect Unilateral x x x x x x Deficits Cognitive x x or Anxiety Depression Depression x x x x x x Age x x x x x x Stroke Stroke Severity Predictors of Stroke Outcomes of Stroke Predictors Mortality Long-term recovery Participation level outcomes Quality of life Independence in activities of daily living Discharge to Discharge dependent-living setting Length of stay (acute/subacute settings) Functional status upon discharge Table 1–2. Table

10 9 Awareness

Awareness of one’s own abilities, deficits, deficit in relation to symptoms of Korsa- strengths, and weaknesses can impact per- koff’s syndrome. Anton and Pick (known formance in daily life, vocational success, today as namesakes of types of cortical and participation in rehabilitation. Aware- blindness and frontotemporal dementia) ness can be impaired following brain also described aspects of reduced aware- injury, particularly when the RH is dam- ness in the late 1800s (Prigatano, 2010a). aged. This chapter will cover definitions, As with many disorders, there are in- types, and models of awareness, how the consistencies in terminology (Table 9–1). RH is involved, and how to assess and “Anosognosia” is commonly used for treat deficits of awareness. reduced awareness of specific impairments, most often hemiparesis and unilateral neglect. Anosognosia is the label Anosognosia used in research with stroke survivors, while “Impaired Self-Awareness” (ISA) is preferred in the literature on TBI. ISA The word “anosognosia” comes from is defined more broadly; in addition to Greek and means “without knowledge of referring to reduced awareness of a spe- disease.” In clinical practice it is used to cific deficit, it encompasses the functional refer to the reduced awareness of either implications of that deficit, the patient’s acquired deficits or the consequences expectations for recovery, differential of those deficits. The term was initially awareness for different domains, and coined in 1914 by Babinski in reference to adherence to treatment (Orfei, Caltagi- reduced awareness of hemiplegia (Babin- rone, & Spalletta, 2009). Some even use ski, 1914; translated by Langer & Levine, ISA synonymously with metacognition 2014). However, descriptions of reduced (Schmidt, Lannin, Fleming, & Ownsworth, awareness first appeared over 30 years ear- 2011), as both refer to one’s understanding lier when von Monakow described such a of one’s own strengths and limitations and

175 176 The Right Hemisphere and Disorders of Cognition and Communication: Theory and Clinical Practice

Table 9–1. Terminology Related to Anosognosia

Terms Description Anosognosia Reduced awareness of acquired deficits; typically used in relation to reduced awareness of specific impairments Denial of deficit Connotes that there is some awareness that allows for psychological refusal to acknowledge the deficit (conscious or unconscious) Impaired self- Commonly used in TBI literature to refer to reduced awareness awareness; can be used for specific impairments or general awareness; often includes insights about consequences and motivation to participate in therapy Lack of insight Connotes higher-level cognitive deficit implicating higher- level reasoning; reduced understanding/awareness of the consequences of an impairment Types of Unawareness Explicit awareness Ability to verbally report the presence of a deficit Implicit awareness Changes in behavior related to the presence of a deficit (e.g., to avoid failure related to a deficit) Related Disorders Alexithymia Reduced use of emotion-related words Anosodiaphoria Reduced emotional reaction to, or concern for, deficits Asomatognosia A form of disturbed sense of ownership in which a patient believes his impaired limb is missing or does not belong to him Misoplegia Hatred of one’s limbs or body part(s) Personification Refer to and treat a limb as if it were its own being (e.g., naming one’s arm) Somatoparaphrenia A form of disturbed sense of ownership in which a patient feels her impaired limb belongs to someone else

how those will impact performance on “Lack of insight” can be described as a daily activities. Prigatano and Morrone- component of ISA. It connotes a cognitive Strupinsky (2010) use the label “anosog- deficit implicating higher-level reasoning. nosia” to refer to a complete unawareness This may occur in some patients who have of a specific impairment, and use “ISA” to a reduced awareness of the consequences refer to partial unawareness that may be a of a deficit, even if they do have aware- stage of recovery from anosognosia. ness of the deficit itself. A patient who 9. Awareness 177 can describe his hemiparesis, but in the cies in research on anosognosia. First, as next sentence talk about how he plans to described above, there are inconsisten- resume his weekend bike rides with his cies in definitions and terminology. Sec- son once he is discharged from the hospi- ond is the distinction between explicit tal could be described as having reduced and implicit awareness (Fotopoulou, Per- insight. nigo, Maeda, Rudd, & Kopelman, 2010; The phrase “denial of deficit” often is Mograbi & Morris, 2013; Moro, Pernigo, used synonymously with anosognosia Zapparoli, Cordioli, & Aglioti, 2011). in clinical practice (Prigatano & Klonoff, Explicit unawareness is measured by ver- 1998). However, denial is very different bal responses to questions (e.g., is there from reduced awareness. In order to deny anything wrong with your arm?). Implicit that something exists, you must be aware unawareness, in contrast, is observed in of it and consciously reject it. Using the patients’ behaviors. A patient who does phrase “denial of deficit” may cause fami- not verbally acknowledge her hemiplegia lies to erroneously believe that the patient but who never attempts to get out of bed is being difficult or refusing to admit a without assistance might have implicit problem, when in reality the patient is not but not explicit awareness of her deficit. aware of the existence of the deficit at a Another example of implicit awareness conscious level. comes from studies that employ bimanual Another commonly used but not quite tasks (e.g., Cocchini, Beschin, Fotopoulou, accurate label is “unawareness.” While & Della Sala, 2010; Moro et al., 2011). Some some researchers and clinicians use “un- individuals with explicit anosognosia will awareness” synonymously with anosog- use strategies to complete bimanual tasks nosia, the former suggests a complete loss that suggest implicit awareness of upper of awareness and does not convey the limb paralysis. For example, when asked nuances of the disorder, in which a patient to lift a two-handled tray, they will lift may be aware of hemiparesis but not of with one hand in the middle of the tray cognitive deficits; have different levels instead of attempting to lift from the two of awareness of upper and lower extrem- ends. In studies of Alzheimer’s disease, ity weakness (Berti, Ladavas, & Della some patients may have emotional reac- Corte, 1996); demonstrate awareness that tions to failure despite not being able to appears to increase or decrease depend- explicitly acknowledge the poor perfor- ing on the questions asked; or may not mance (Mograbi & Morris, 2013). Not all verbally report hemiparesis but never try individuals with anosognosia have pre- to stand up unassisted (Mograbi & Mor- served implicit awareness. Evidence from ris, 2013; Nurmi & Jehkonen, 2014; Orfei priming studies (Fotopoulou et al., 2010; et al., 2007). For these and other reasons, Nardone, Ward, Fotopoulou, & Turnbull, Prigatano (2013) cautions that anosogno- 2007) suggests that some patients with sia should not be considered a unitary anosognosia show reduced activation of disorder. relevant disability-related words (e.g., Nurmi and Jehkonen (2014) highlight weakness, walk) compared with individu- some of the difficulties and inconsisten- als with hemiparesis but intact awareness, 178 The Right Hemisphere and Disorders of Cognition and Communication: Theory and Clinical Practice

indicating that explicit and implicit aware- can be aware of some deficits (e.g., hemi- ness can be affected differentially. paresis) but not others (e.g., unilateral Vocat and Vuilleumier (2010) suggest neglect) (Berti et al., 1996; Bisiach, Vallar, that the dissociation between implicit and Perani, Papagno, & Berti, 1986). Turnbull, explicit awareness could be due to two Fotopoulou, and Solms (2014) argue that separate monitoring systems. One is a such evidence does not spell the death subcortical system that provides implicit, knell for the idea that anosognosia may automatic monitoring of “affective rel- have an emotional component related to evance of a mismatch between a goal and a defense mechanism. They argue that the outcome” (p. 267). The other is a corti- emotional deficits associated with RHD cal system residing in frontal and parietal result in the person viewing the world as lobes which provides “conscious error he would like it to be, as opposed to the detection based on the quality of feedback reality. Thus, for some clients, emotion and on access to attentional and executive and motivation may play a strong role in networks” (p. 267). Damage to the former anosognosia. would cause a deficit of implicit aware- Geschwind (1965) suggested a discon- ness, and the latter would result in prob- nection model, in which verbal reports of lems with explicit awareness. awareness were disrupted by a disconnec- tion between the RH sensory and proprio- ceptive processing areas and the LH lan- Models of Anosognosia guage areas. If this were true, then there should be dissociations between verbal There have been a variety of theories of and nonverbal assessments of awareness. anosognosia over the years. Some of the These dissociations have not consistently earliest were motivational or psycho- been found. dynamic theories in which anosognosia More recent theories use anatomical was described as a form of psychological models and include different levels of denial that was used as a defense mecha- awareness. Higher-level, conscious aware- nism (Weinstein & Kahn, 1955). While the ness is thought to be controlled primar- terminology “denial of deficit” lingers, ily by the prefrontal regions. Low-level, strong versions of these theories have modality-specific awareness is localized been discarded in light of disconfirming posteriorly, in the temporal and parietal evidence. Anosognosia has been identi- lobes. Damage to either region could fied in acute stages of stroke recovery, result in reduced awareness. before patients have had a chance to expe- McGlynn and Schacter’s (1989) Con- rience their deficits (e.g., attempting to scious Awareness System (CAS) resides walk to the bathroom with a hemiparetic primarily in the prefrontal regions and leg) or the broader consequences of them works in concert with judgment, insight, (e.g., not being able to drive with hemi- and self-reflection processes. Damage to paresis). Without the experience of the the CAS may result in a global unaware- loss, there is no need for a psychological ness of self. The input from modality- defense against it. Other evidence against specific systems (visual, somatosensory) this theory is the fact that some patients is intact, but the signals are not processed 9. Awareness 179 correctly by the damaged CAS, thus result- in the comparison process. The intended ing in incorrect interpretation and self- (desired) and predicted results match, but monitoring of the sensory input. In this the “comparator” does not correctly iden- model, cognitive and affective states may tify a mismatch between these two states be part of the presentation of anosognosia. and the actual movement. If no mismatch In contrast to the CAS model are the is identified, then there is no awareness modality- or domain-specific accounts that the movement was incorrect or did of anosognosia (Bisiach, 1990). Damage not occur as planned. This model can to the temporal and parietal sensory pro- explain the phenomenon of illusory cessing areas may result in disruptions movement, in which patients report that to connections or signals sent to the fron- they felt a movement occur, even in the tal lobes for processing by the CAS. For face of contradictory visual and sensory example, an RH parietal lesion may result feedback (Feinberg, Roane, & Ali, 2000; in unilateral visual neglect. If information Fotopoulou et al., 2010). Jenkinson, Edel- about the incomplete visual representa- styn, Drakeford, and Ellis (2009) reported tion is not sent to the frontal lobes, or if that adults with anosognosia for hemipa- erroneous information is sent (e.g., the resis are impaired in determining whether visual representation is complete), then they had seen or imagined pictures or had the central processor will not detect a performed, observed, or imagined actions. problem, resulting in reduced awareness They tend to recall having seen pictures of the unattended visual field. or performed actions that had only been The theories with the most empirical imagined, indicating a deficit in reality support purport that anosognosia for monitoring. hemiparesis is caused by a disruption in the motor control system. The motor system is thought to control intention to Related Disorders move, the movement itself, and a comparison between the intended movement and There are several disorders that are related the actual movement based on sensory to, or commonly co-occur with, anosog- feedback. Disruption to either the inten- nosia (see Table 9–1). In some cases, the tion or the comparator system has been disorders are erroneously considered to be implicated. According to the feed-forward parts of the same problem. First is unilater- model (Heilman, 1991; Wolpert, 1995), al neglect. Some researchers appear to there is a loss of intention to move. In the equate unilateral neglect and anosognosia: intact system, the intended movement “right hemispheric stroke is usually asso- would be compared with the actual move- ciated with neglect, which reduces aware- ment, and discrepancies would be noted. ness of neurological deficits” (Foersch However, if there is a loss of intention, et al., 2005, p. 392). While patients with then there would be no discrepancy with unilateral neglect often have anosognosia an absence of actual movement. In the for neglect, the deficits are distinct disor- feedback model (Berti & Pia, 2006; Spin- ders that can be dissociated (Appelros, azzola, Pia, Folegatti, Marchetti, & Berti, Karlsson, & Hennerdal, 2007; Berti et al., 2008; Wolpert, 1995), the disruption occurs 1996; Bisiach et al., 1986; Vocat et al., 2010). 180 The Right Hemisphere and Disorders of Cognition and Communication: Theory and Clinical Practice

Additionally, some individuals with ownership” in which patients do not feel anosognosia for unilateral neglect can be that a paretic limb really belongs to them aware of other deficits, such as hemiplegia. (Karnath & Baier, 2010). One form of this Second is anosodiaphoria, which is a is asomatognosia, in which they believe reduced concern for deficits, or reduced their limb is missing. In another form, emotional expression related to those somatoparaphrenia, patients attribute the deficits (Babinski, 1914/Langer & Levine, impaired limb to someone else. 2014). This too has been dissociated from Other phenomena include misoplegia, anosognosia. Some patients may be aware in which patients develop a hatred for of their deficits and be able to identify and the impaired limb, and personification, in describe their hemiparesis but show no which patients develop a name and per- apparent concern over the loss of motor sonality for the impaired limb. For exam- control. It is not clear whether there is ple, a patient who names her hemiplegic reduction in emotional experience or if arm “Connie” and gives reports about the problem is in the expression of emo- how Connie is doing on a particular day tion. Related to the latter is alexithymia, a would be showing signs of personifica- reduced use of emotional words (Heilman tion. These delusional beliefs are produc- & Harciarek, 2010; Jorge, 2010). Again, tive deficits, in which there is an exacerba- these two deficits may co-occur, but just tion or production of additional function, because a person is not using many emo- while anosognosia itself is a defective dis- tional words does not mean that he or she order in which there is reduction of func- is not experiencing emotional responses. tion (Bottini et al., 2010). Third, some individuals with anosog- Finally, confabulation can be observed nosia develop delusional beliefs about in some individuals with anosognosia. their hemiparetic limbs (Bottini et al., 2010; The source of confabulations is not well Giacino & Cicerone, 1998). Several of these studied, but they are thought to be an fall under the category “disturbed sense of unconscious response to behaviors that

SIDEBAR

My first experience with anosognosia was with a patient who had been diagnosed with a right hemisphere tumor. His initial symptoms included getting lost in the hardware store in which he worked and bumping into the wall when walking down a hallway in his house. During a preoperative assessment in which he was asked about the latter problem, he explained: “My wife hangs too many pictures on the wall and I don’t like them. So when I’m walking down the hall, I hit them so they fall off the wall.” He confabulated an explanation for the symptoms because he was not consciously aware of the unilateral neglect caused by the RH tumor.