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Studies Provide New Insights into Brain-Behavior Relationships

Double Special Issue on Aphasia, Hemispatial Neglect and Related Disorders Published in Behavioural Neurology

January 16, 2013
Approximately half a million individuals suffer strokes in the US each year, and about one in five develops some form of post-stroke aphasia, the partial or total loss of the ability to communicate. By comparing different types of aphasia, investigators have been able to gain new insights into the normal cognitive processes underlying language, as well as the potential response to interventions. Their findings are published alongside papers on hemispatial neglect and related disorders in the January, 2013 issue of Behavioural Neurology.

The January issue of Behavioural Neurology, edited by the journal’s co-Editor in Chief, Argye E. Hillis, MD, of the Departments of Neurology, Physical Medicine and Rehabilitation, and Department of Cognitive Science, Johns Hopkins University, Baltimore, Maryland, features papers on two topics that have traditionally captured the interest of behavioral neurologists – aphasia and hemispatial neglect.

The first section on aphasia includes a number of papers that compare post-stroke aphasia with primary progressive aphasia (PPA), in which the predominant deficit is language (with or without apraxia).

Andreia V. Faria, MD, Department of Radiology, Johns Hopkins University School of Medicine, and colleagues from Johns Hopkins and University College, London, report patterns of dysgraphia (spelling impairment) in participants with primary progressive aphasia, and compare these patterns to those in participants with dysgraphia following stroke. They also report the areas of focal atrophy associated with the most common pattern of dysgraphia in PPA and suggest this can not only provide a better understanding of the neural substrates of spelling, but may also provide clues to more effective treatment approaches.

Matthew A. Lambon Ralph, FRSLT (hons), FBPsS, and colleagues from the School of Psychological Sciences, University of Manchester, UK; the Department of Psychology, University of York, UK; and the Stroke and Dementia Research Centre, St George’s University of London, UK, use a novel approach to explore nonverbal semantic processing to demonstrate the qualitative differences between semantic aphasia and semantic dementia. Their conclusions provide further support for the proposal that semantic cognition is underpinned by two principle components: semantic representations and regulatory control processes which regulate and shape activation within the semantic system.

Cynthia K. Thompson, PhD, and colleagues from the Department of Communication Sciences and Disorders, Department of Neurology, Cognitive Neurology and Alzheimer’s Disease Center, and Department of Psychiatry and Behavioral Sciences at Northwestern University, Evanston, Illinois, evaluate the distinct patterns of morphological and syntactic errors in the variants of PPA, and compare them with patterns of errors in post-stroke aphasia.

Other papers compare treatment results of spelling in one individual with logopenic variant PPA (lvPPA) with an individual with post-stroke dysgraphia, and results of a new method of assessment of verbal and nonverbal memory in PPA. The issue is completed by three Clinical Notes including a fascinating case of an unusual form of lvPPA that degenerated into jargon aphasia, a case of nonfluent agrammatic variant PPA due to Pick disease with (what is argued to be) concomitant incidental Alzheimer’s disease pathology, and a case of successful treatment of PPA.

“Together, these papers illustrate how investigating PPA and post-stroke aphasia can yield complementary insights about brain-behavior relationships as well as about potential response to interventions and the normal cognitive processes underlying language,” says Dr Hillis.

Hemispatial neglect is characterized by reduced awareness of stimuli on one side of space. It occurs only after relatively focal (or at least asymmetric) brain damage, most commonly stroke, but is occasionally observed in other syndromes. In this second group of seven papers, Jonathan T. Kleinman, MD, of Johns Hopkins University School of Medicine, and Stanford University School of Medicine, Stanford, California, and colleagues from Johns Hopkins University School of Medicine, report an investigation of perseveration versus hemispatial neglect, and the lesion sites associated with each in acute stroke. The issue also includes an important paper by Junichi Ishizaki, PhD, and co-workers at the Department of Geriatric Behavioral Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan, of impaired visual-spatial attention in Alzheimer’s disease, which shows how a symmetric neurodegenerative disease results in impaired shifting of visual spatial attention, but not hemispatial neglect.

“Hemispatial neglect remains one of the most remarkable syndromes investigated by behavioral neurologists,” comments Dr Hillis. “These novel studies of neglect and related disorders provide new insights into brain-behavior relationships on the basis of detailed analysis of patient performance – and in many cases, their lesion sites.“ 

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Primary Progressive Aphasia and Post-Stroke Aphasia: Some Complementary Insights into Brain-Behavior Relationships/Hemispatial Neglect and Related Disorders

Editor: Argye E. Hillis, MD, MA, Professor of Neurology, Departments of Neurology, Physical Medicine and Rehabilitation, and Department of Cognitive Science, Johns Hopkins University, Baltimore, Maryland

Behavioural Neurology, Volume 26, Issue 1-2 (January 2013). Published by IOS Press.

Full text of the articles in the issue is available to credentialed journalists. Contact Daphne Watrin, IOS Press, at +31 20 688 3355 or for additional information. Journalists wishing to interview the authors may also contact Dr. Argye E. Hillis at +1 410 614 2381 or

Table of Contents:
Deterioration or recovery of selective cognitive function can reveal the role of focal areas within networks of the brain
Argye E. Hillis

Demonstrating the qualitative differences between semantic aphasia and semantic dementia: A novel exploration of nonverbal semantic processing
Krist A. Noonan, Elizabeth Jefferies, Peter Garrard, Sheeba Eshan and Matthew A. Lambon Ralph

Degenerative jargon aphasia: Unusual progression of logopenic/phonological progressive aphasia?
Paolo Caffarra, Simona Gardini, Stefano Cappa, Francesca Dieci, Letizia Concari, Federica Barocco, Caterina Ghetti, Livia Ruffini and Guido Dalla Rosa Prati

Patterns of dysgraphia in primary progressive aphasia compared to post-stroke aphasia
Andreia V. Faria, Jenny Crinion, Kyrana Tsapkini, Melissa Newhart, Cameron Davis, Shannon Cooley, Susumu Mori and Argye E. Hillis

Syntactic and morphosyntactic processing in stroke-induced and primary progressive aphasia
Cynthia K. Thompson, Aya Meltzer-Asscher, Soojin Cho, Jiyeon Lee, Christina Wieneke, Sandra Weintraub and M.-Marsel Mesulam

Spelling intervention in post-stroke aphasia and primary progressive aphasia
Kyrana Tsapkini and Argye E. Hillis

Treatment for apraxia of speech in nonfluent variant primary progressive aphasia
M.L. Henry, M.V. Meese, S. Truong, M.C. Babiak, B.L. Miller and M.L. Gorno-Tempini

Degenerative jargon aphasia: Unusual progression of logopenic/phonological progressive aphasia?
Paolo Caffarra, Simona Gardini, Stefano Cappa, Francesca Dieci, Letizia Concari, Federica Barocco, Caterina Ghetti, Livia Ruffini and Guido Dalla Rosa Prati

Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick’s disease pathology
Francesca Caso, Benno Gesierich, Maya Henry, Manu Sidhu, Amanda LaMarre, Miranda Babiak, Bruce L. Miller, Gil D. Rabinovici, Eric J. Huang, Giuseppe Magnani, Massimo Filippi, Giancarlo Comi, William W. Seeley and Maria Luisa Gorno-Tempini

New insights from a not-so-neglected field: Hemispatial neglect
Argye E. Hillis

Motor extinction in distinct reference frames: A double dissociation
Jennifer Heidler-Gary, Mikolaj Pawlak, Edward H Herskovits, Melissa Newhart, Cameron Davis, Lydia A. Trupe and Argye E Hillis

Impaired shifting of visuospatial attention in Alzheimer’s disease as shown by the covert orienting paradigm: Implications for visual construction disability
Junichi Ishizaki, Kenichi Meguro, Nobuhiro Nara, Mari Kasai and Atsushi Yamadori

Disentangling the neuroanatomical correlates of perseveration from unilateral spatial neglect
Jonathan T. Kleinman, Jeffery C. DuBois, Melissa Newhart and Argye E. Hillis

What is mine? Behavioral and anatomical dissociations between somatoparaphrenia and anosognosia for hemiplegia
Paola Invernizzi, Martina Gandola, Daniele Romano, Laura Zapparoli, Gabriella Bottini and Eraldo Paulesu

How to differentiate hemianesthesia from left tactile neglect: A preliminary case report
Marco Pitteri, Annalena Venneri, Francesca Meneghello and Konstantinos Priftis

The “Altitudinal Anton’s syndrome”: Coexistence of anosognosia, blindsight and left inattention
A. Carota, F. Bianchini, L. Pizzamiglio and P. Calabrese

An International Journal with an Emphasis on Lesion and Imaging Studies that Explore Abnormal Human Cognition and Behaviour

Behavioural Neurology ( publishes original experimental papers and case reports dealing with disordered human behaviour and exceptional animal studies that have direct implications for understanding human behavior or neural mechanisms of cognition. These embrace the field of cognitive neurology, biological psychiatry, neuropsychology, rehabilitation, and cognitive neuroscience. The emphasis of the approach is on lesion and imaging studies that explore abnormal human cognition and behaviour.


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