book review

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Brain Fiction: Self-Deception and the Riddle of Confabulation By William Hirstein Cambridge, MA, MIT Press, 2005 288 pp, $35.00 Potential neurologists often get interested in the specialty be- cause they feel that its clinical problems raise profound ques- tions about the human condition, more so than perhaps al- most any other specialty. William Hirstein in Brain Fiction shows that this faith was not misplaced. He discusses con- fabulation, anosognosia, and many other forms of lack of awareness of deficits—some very familiar to clinicians, some quite rare—and asks the reader to consider not only what they might have in common, but also how these pathological states might even be exaggerations of our “normal” lack of appreciation for our own motives and actions. From this new perspective, it is also easy to wonder again just what the forms of memory are, and just what are the manifestations of consciousness. Hirstein is an experienced researcher in con- fabulation and related pathologies. Brain Fiction combines a wide-ranging yet scholarly detailed discussion of these condi- tions with up-to-date grounding in neuroanatomy, neuro- physiology, and neuroimaging. More specifically, chapters in Brain Fiction deal with the definition of confabulation, con- fabulation and memory, lying, mind reading and misidenti- fication, unawareness and denial of illness, unawareness and denial in split-brain patients, confabulation as an issue in epistemology, and self-deception. The strengths of Brain Fiction include its wide scope and its knowledgeable citations of the primary literature. Hirstein manages to inform the reader of telling details of the obser- vations, experiments, and results that make up our knowl- edge of confabulation and its related problems, everyday or unusual. For neurologists and neuroscientists, the weaknesses of Brain Fiction are perhaps its reviews of basic neuroanatomy and also its confounding between levels of psychological dis- order and neuroanatomic associations. It all too readily tries to “explain” the psychological disorders by pointing to neu- roanatomy, when, in fact, we have only the vaguest idea in many cases what functions the neuroanatomy actually serves, or, for that matter, what actual disorders of internal mental processing are actually responsible for the observed deficits. Also, Brain Fiction has a tendency to give a great deal of weight to various authors’ explanations without necessarily being critical of the underlying data upon which they base their conclusions. Much if not all of this weakness can be traced to the nature of the evidence itself—often fragmen- tary, inconsistent, and inextricably bound up with the each investigator’s pet hypotheses. The reader who appreciates these limits will also gain one of the great benefits of Brain Fiction, which is to appreciate how much research remains to be done to explain conditions that neurologists might other- wise treat as commonplace. Brain Fiction will reward all readers with a rekindling of the wonder they probably once felt for what are now “rou- tine” cases of left-sided neglect and other agnosias and will reward some of its readers with a roadmap for the research they might undertake. Barry Gordon, MD, PhD Baltimore, MD DOI: 10.1002/ana.20519 Neurodevelopmental Disorders: Cognitive/Behavioral Phenotypes Edited by D. Riva, U. Bellugi, and M. D. Denckla John Libbey Eurotext, Montrouge, France, 2005 151 pp, illustrated, $75.00 Neurodevelopmental Disorders: Cognitive/Behavioral Phenotypes is fact filled and easy to read, with 14 chapters mainly on specific neurodevelopmental disorders. The first three chap- ters briefly discuss the tools used to define cognitive and be- havioral phenotypes. These chapters, which could be the ba- sis of an entire book, are probably necessary but inadequate. Genetic approaches that study subgroups based on quantifi- able, specific cognitive, or behavioral features are not cov- ered, although issues of how a single gene mutation causes many phenotypes is broached with some simple insights. Clinicians at all stages of their careers, including adult and pediatric neurologists, psychiatrists, psychologists, and those involved in neurodevelopmental therapy and rehabilitation will find Neurodevelopmental Disorders: Cognitive/Behavioral Phenotypes very interesting, with many short, generally excel- lent and up-to-date descriptions of selected disorders. The descriptions are generally written by leaders in the field and include fragile X syndrome, Williams’ syndrome and a com- parison with Down’s syndrome, Turner’s syndrome, tuber- ous sclerosis, Duchenne’s muscular dystrophy, developmental dyslexia, specific language impairment syndromes, autism, at- tention deficit hyperactivity disorder (ADHD), mood disor- ders, and Gilles De La Tourette’s syndrome. For those who have not kept up with the many develop- ments in neurodevelopment, there are many things to learn in this tome that are a great return for the time spent. Dr Hagerman describes the genetics of the CGG trinucleotide repeat in fragile X syndrome along with the clinical pheno- types associated with mental retardation in male subjects, au- tism in a substantial subgroup of these patients, and learning disabilities in female subjects. They describe the CGG pre- mutation that occurs in older male subjects that causes an adult-onset tremor/ataxia syndrome that was recently recog- nized by this group. Rovet details defects of visuospatial function, visual memory, and social cognitive deficits in Turner’s syndrome. Curatolo and colleagues briefly describe the mental retardation, learning disabilities, ADHD, and au- tism that occur in tuberous sclerosis. Hinton outlines the deficits in verbal working memory, digit span, story memory, and multiword comprehension in Duchenne’s muscular dys- trophy. Dyslexia and specific language impairment are high- lighted as genetic disorders generally with preservation of in- telligence, social skills, and normal sensory function. Chapters on autism and Tourette’s are up to date and ade- quate for summarizing current knowledge. An unexpected jewel is chapter by Bellugi, Klima, and Ko- renberg summarizing recent studies/advances in the study of Williams’ syndrome, which is associated with specific cardiac abnormalities, hypersensitivity to sound, characteristic facial features, and what turned out to be characteristic behavioral BOOK REVIEWS © 2005 American Neurological Association 171 Published by Wiley-Liss, Inc., through Wiley Subscription Services

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Page 1: Book review

Brain Fiction: Self-Deception and the Riddle ofConfabulationBy William HirsteinCambridge, MA, MIT Press, 2005288 pp, $35.00

Potential neurologists often get interested in the specialty be-cause they feel that its clinical problems raise profound ques-tions about the human condition, more so than perhaps al-most any other specialty. William Hirstein in Brain Fictionshows that this faith was not misplaced. He discusses con-fabulation, anosognosia, and many other forms of lack ofawareness of deficits—some very familiar to clinicians, somequite rare—and asks the reader to consider not only whatthey might have in common, but also how these pathologicalstates might even be exaggerations of our “normal” lack ofappreciation for our own motives and actions. From this newperspective, it is also easy to wonder again just what theforms of memory are, and just what are the manifestations ofconsciousness. Hirstein is an experienced researcher in con-fabulation and related pathologies. Brain Fiction combines awide-ranging yet scholarly detailed discussion of these condi-tions with up-to-date grounding in neuroanatomy, neuro-physiology, and neuroimaging. More specifically, chapters inBrain Fiction deal with the definition of confabulation, con-fabulation and memory, lying, mind reading and misidenti-fication, unawareness and denial of illness, unawareness anddenial in split-brain patients, confabulation as an issue inepistemology, and self-deception.

The strengths of Brain Fiction include its wide scope andits knowledgeable citations of the primary literature. Hirsteinmanages to inform the reader of telling details of the obser-vations, experiments, and results that make up our knowl-edge of confabulation and its related problems, everyday orunusual.

For neurologists and neuroscientists, the weaknesses ofBrain Fiction are perhaps its reviews of basic neuroanatomyand also its confounding between levels of psychological dis-order and neuroanatomic associations. It all too readily triesto “explain” the psychological disorders by pointing to neu-roanatomy, when, in fact, we have only the vaguest idea inmany cases what functions the neuroanatomy actually serves,or, for that matter, what actual disorders of internal mentalprocessing are actually responsible for the observed deficits.Also, Brain Fiction has a tendency to give a great deal ofweight to various authors’ explanations without necessarilybeing critical of the underlying data upon which they basetheir conclusions. Much if not all of this weakness can betraced to the nature of the evidence itself—often fragmen-tary, inconsistent, and inextricably bound up with the eachinvestigator’s pet hypotheses. The reader who appreciatesthese limits will also gain one of the great benefits of BrainFiction, which is to appreciate how much research remains tobe done to explain conditions that neurologists might other-wise treat as commonplace.

Brain Fiction will reward all readers with a rekindling ofthe wonder they probably once felt for what are now “rou-tine” cases of left-sided neglect and other agnosias and willreward some of its readers with a roadmap for the researchthey might undertake.

Barry Gordon, MD, PhDBaltimore, MD

DOI: 10.1002/ana.20519

Neurodevelopmental Disorders: Cognitive/BehavioralPhenotypes Edited by D. Riva, U. Bellugi, and M. D.Denckla John Libbey Eurotext, Montrouge, France,2005151 pp, illustrated, $75.00

Neurodevelopmental Disorders: Cognitive/Behavioral Phenotypesis fact filled and easy to read, with 14 chapters mainly onspecific neurodevelopmental disorders. The first three chap-ters briefly discuss the tools used to define cognitive and be-havioral phenotypes. These chapters, which could be the ba-sis of an entire book, are probably necessary but inadequate.Genetic approaches that study subgroups based on quantifi-able, specific cognitive, or behavioral features are not cov-ered, although issues of how a single gene mutation causesmany phenotypes is broached with some simple insights.

Clinicians at all stages of their careers, including adult andpediatric neurologists, psychiatrists, psychologists, and thoseinvolved in neurodevelopmental therapy and rehabilitationwill find Neurodevelopmental Disorders: Cognitive/BehavioralPhenotypes very interesting, with many short, generally excel-lent and up-to-date descriptions of selected disorders. Thedescriptions are generally written by leaders in the field andinclude fragile X syndrome, Williams’ syndrome and a com-parison with Down’s syndrome, Turner’s syndrome, tuber-ous sclerosis, Duchenne’s muscular dystrophy, developmentaldyslexia, specific language impairment syndromes, autism, at-tention deficit hyperactivity disorder (ADHD), mood disor-ders, and Gilles De La Tourette’s syndrome.

For those who have not kept up with the many develop-ments in neurodevelopment, there are many things to learnin this tome that are a great return for the time spent. DrHagerman describes the genetics of the CGG trinucleotiderepeat in fragile X syndrome along with the clinical pheno-types associated with mental retardation in male subjects, au-tism in a substantial subgroup of these patients, and learningdisabilities in female subjects. They describe the CGG pre-mutation that occurs in older male subjects that causes anadult-onset tremor/ataxia syndrome that was recently recog-nized by this group. Rovet details defects of visuospatialfunction, visual memory, and social cognitive deficits inTurner’s syndrome. Curatolo and colleagues briefly describethe mental retardation, learning disabilities, ADHD, and au-tism that occur in tuberous sclerosis. Hinton outlines thedeficits in verbal working memory, digit span, story memory,and multiword comprehension in Duchenne’s muscular dys-trophy. Dyslexia and specific language impairment are high-lighted as genetic disorders generally with preservation of in-telligence, social skills, and normal sensory function.Chapters on autism and Tourette’s are up to date and ade-quate for summarizing current knowledge.

An unexpected jewel is chapter by Bellugi, Klima, and Ko-renberg summarizing recent studies/advances in the study ofWilliams’ syndrome, which is associated with specific cardiacabnormalities, hypersensitivity to sound, characteristic facialfeatures, and what turned out to be characteristic behavioral

BOOK REVIEWS

© 2005 American Neurological Association 171Published by Wiley-Liss, Inc., through Wiley Subscription Services