Topics in Behavioral Neurology and Neuropsychology. With Key References

252 Pages·1987·4.367 MB·English

by  Daniel B. Hier, Philip B. Gorelick and Andrea Gellin Shindler (Auth.)

Topics in
Behavioral Neurology
and Neuropsychology
With Key References
Daniel B. Hier, M.D.
Chairman, Department of Neurology,
Michael Reese Hospital and Medical Center, and
Associate Professor of Neurology,
University of Chicago Pritzker School of Medicine,
Chicago, Illinois
Philip B. Gorelick, M.D.
Director, Stroke Service,
Michael Reese Hospital and Medical Center, and
Assistant Professor of Neurology,
University of Chicago Pritzker School of Medicine,
Chicago, Illinois
Andrea Gellin Shindler, M.A., CCC-SP
Speech Pathologist and Research Associate,
Department of Neurology,
Michael Reese Hospital and Medical Center, Chicago, Illinois
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Library of Congress Cataloging-in-Publication Data
Hier, Daniel B.
Topics in behavioral neurology and neuropsychology.
Includes bibliographies and index.
1. Clinical neuropsychology. 2. Cerebral cortex—
Diseases. I. Gorelick, Philip B. II. Shindler,
Andrea Gellin. III. Title. [DNLM: 1. Nervous System
Diseases. 2. Neuropsychology. WL 100 H633t]
RC386.2.H54 1987 616.8 87-11719
ISBN 0-409-95165-X
Butterworth Publishers
80 Montvale Avenue
Stoneham, MA 02180
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Printed in the United States of America
This book is dedicated to our spouses
and children:
Myra and Benjamin
Bonnie, David, and Alissa
Michael, Adam, and Carey
Preface
Several comprehensive texts on neuropsychology are now available, including
Walsh's Neuropsychology (1978), Heilman and Valenstein's Clinical Neuro-
psychology (1985), and Hécaen and Albert's Human Neuropsychology
(1978). Mesulam's Principles of Behavioral Neurology (1985) and Kirshner's
Behavioral Neurology (1986) are excellent introductions to the new discipline
of behavioral neurology. These books should serve as a "core" library for
anyone interested in neuropsychology and behavioral neurology.
Our book is not intended to replace these standard texts. Rather, it is
meant to supplement them by providing a ready source of concise definitions
and brief discussions. In caring for patients with disorders of higher cortical
function and in teaching house staff and fellows about these disturbances, we
were struck by the need for a book that would permit rapid entry into the
burgeoning literature devoted to neuropsychology and behavioral neurology.
The book was written by developing a list of topics that we felt were
central to neuropsychology and behavioral neurology. We have sought to
provide concise discussions and abundant references for each topic, including a
sampling of both current and "classic" references.
The book is divided into broad chapters covering such subjects as De-
mentia, Aphasia, Apraxia, Amnesia, Recovery, and Stroke and Other Brain
Disorders. Some conditions defy easy classification (e.g., allesthesia, synkine-
sia); these are arranged alphabetically in the chapter entitled Miscellaneous
Disorders. Other disorders could appropriately have been assigned to more
than one chapter (e.g., constructional apraxia could be classified as an apraxia,
an agnosia, or a visual disorder; developmental dyslexia could be considered
an alexia or a childhood disorder). In cases where more than one classification
was possible, we have chosen what we felt was most appropriate. If in doubt,
the reader is directed to the index to find a topic of interest.
We hope this book will prove useful to neurology residents, psychiatrists,
neurologists, neuropsychologists, speech pathologists, rehabilitation medicine
specialists, neuroscientists, and all others who share an interest in disorders of
higher cortical function. We are all indebted to our teachers, especially Drs.
J.P. Mohr, Louis R. Caplan, and Norman Geschwind, for introducing us to the
field of behavioral neurology. We thank Miss Merryl Billingsley for editorial
assistance and manuscript preparation.
XI
Note to Readers
Within each section, terms may appear in either bold type or italics. Bold type
indicates that the referenced term is discussed elsewhere in the book; the reader
may consult the table of contents or the index. Italics indicate that the term is
not discussed further in other sections.
xni
1
Cerebral Organization
CLASSIFICATION OF DISORDERS OF HIGHER CORTICAL
FUNCTION
A large number of disorders of higher cortical function are now recognized,
but no completely satisfactory schema exists for categorizing them. Tradition-
ally, disorders of higher cortical function have been grouped according to
major behavioral disturbance:
Aphasias: disorders of language
Apraxias: disorders of skilled movement
Agnosias: nonperceptual disorders of recognition
Alexias: disorders of reading
Agraphias: disorders of writing
Acalculias: disorders of calculating
Dementias: global disorders of intellect
Amnesias: disorders of memory
Unfortunately this schema is not complete, and many disorders are difficult to
classify (e.g., right-left confusion, confabulation). Disorders may also be
grouped by the main modality affected (e.g., visual, auditory, or somesthetic),
by whether they are motor (executive) or sensory (receptive), and by whether
they are developmental or acquired. Unfortunately, none of these schémas
permits easy classification of all disorders. Classification of disorders by major
site of involvement (e.g., frontal, parietal) is useful (see below), but for many
disorders (e.g., amusia, anosognosia) the exact site of the injury responsible for
the disorder is uncertain.
Within both hemispheres, certain general patterns of intrahemispheric
organization exist. Brain mass anterior to the central sulcus (rolandic sulcus)
subserves motor (executive) functions, whereas brain mass posterior to the
central sulcus subserves sensory (receptive) functions. The frontal lobe is in-
volved not only in the execution of motor acts but in generating much of the
motivation that drives these acts. A variety of higher cortical deficits may
follow frontal lobe injury (Table 1.1).
1
2 Topics in Behavioral Neurology and Neuropsychology
Table 1.1 Frontal Lobe Disorders
Left Right Bilateral
Broca's aphasia Impersistence Abulia
Oral apraxia Neglect Perseveration
Dysprosody Extinction Confabulation
Transcortical motor Reduplication Moria
aphasia Rigidity
Callosal apraxia
Depression (?)
Parietal cortex subserves tactile sensation (somatesthesis). The inferior
parietal lobule (angular gyrus region) serves to integrate sensory information
(thereby permitting visual, auditory, and tactile cross-modal associations). The
left temporal-parietal-occipital junction, unlike the homologous area in the
right hemisphere, must also integrate linguistic with sensory information.
Hence, damage to this critical junction on the left produces a unique set of
deficits that are both quasi-linguistic and quasi-perceptual (right-left confu-
sion, alexia, agraphia, finger agnosia, and dyscalculia). Spatial and quasi-spa-
tial mapping of sensory input occurs in both the left and right parietal lobes;
however, the right parietal lobe appears more efficient for many of these
operations. Deficits associated with parietal lobe injury are summarized in
Table 1.2.
The temporal lobes have prominent olfactory and auditory functions
and, because of their proximity to the underlying limbic system, have impor-
tant modulating effects on emotion. The dominant left temporal lobe is the site
of much of the primary language cortex within the brain. Deficits associated
with temporal-lobe injury are shown in Table 1.3, and visual disturbances that
follow injury to the occipital lobes are listed in Table 1.4.
Table 1.2 Parietal Lobe Disorders
Left Right Bilateral
Constructional apraxia Constructional apraxia Ideational apraxia (?)
Dyscalculia Dressing apraxia
Agraphia Aprosody (?)
Alexia Neglect
Semantic aphasia Extinction
Anomic aphasia Topographical disorientation
Gerstmann's syndrome Anosognosia
Conduction aphasia Affective agnosia (?)
Ideomotor apraxia Left astereognosis
Right astereognosis
Cerebral Organization 3
Table 1.3 Temporal Lobe Disorders
Left Right Bilateral
Amnesia (verbal) Amnesia (visual) Cortical deafness
Amusia (?) Amusia (?) Auditory agnosia
Jargonaphasia Agitation (?) Klüver-Bucy syndrome
Wernicke's aphasia Confusion (?) Global amnesia
Anomic aphasia Synesthesia (?)
Catastrophic reaction
Synesthesia (?)
Agitation (?)
The role of the insula remains largely a mystery. Conduction aphasia
may follow injury to the left insula. Deep structures (especially the thalamus)
subserve alerting functions for both hemispheres and act as a conduit for
cortex-directed signals arising from the brain stem reticular-activating system.
The thalamus also plays an important role in memory functions. The limbic
system, which lies between the deep basal ganglionic nuclei and the surface
cerebral cortex, is involved in memory, emotion, and possibly motivation. The
pattern of déficits depends on the laterality of the brain injury. Memory deficits
depend on the side of damage; left temporal lesions produce greater verbal
memory deficits, and right temporal lesions produce greater nonverbal (visual-
spatial) memory deficits. Similarly, emotional reactions also depend on the
hemisphere damaged. Left-hemisphere damage is more likely to elicit depres-
sion or catastrophic reactions, while right-hemisphere damage tends to pro-
duce anosognosia (indifference reactions) and affective agnosia.
Mesulam (1981) has suggested that the two cerebral hemispheres may
use different but complementary organizational schemes. Higher cortical func-
tions appear to be localized to specific brain areas in the left hemisphere (e.g.,
Broca's, Wernicke's). In contrast, the right hemisphere appears to be organized
by a series of overlapping networks (e.g., one for attention, one for motiva-
tion). The size of the various cortical areas devoted to higher cortical functions
probably varies among individuals. For example, important differences may
exist in the size of language zone. The work of Ojemann (1979) suggests
Table 1.4 Occipital Lobe Disorders
Left Right Bilateral
Simultanagnosia Palinopsia Cortical blindness
Alexia Metamorphopsia Visual agnosia
Color anomia Left achromatopsia Prosopagnosia
Optic aphasia Anton's syndrome
Right achromatopsia Balint's syndrome
4 Topics in Behavioral Neurology and Neuropsychology
considerable variability among individuals in intrahemispheric organization of
higher cortical functions. Sex, handedness, and various factors (e.g., genetic,
experiential) may influence the precise site and size of brain areas subserving
higher cortical functions.
References
Brown JW. Aphasia, apraxia and agnosia. Springfield, IL: Charles C Thomas, 1972.
Critchley M. The parietal lobes. New York: Hafner Press, 1953.
Hécaen H, Albert ML. Human neuropsychology. New York: John Wiley & Sons,
1978.
Heilman KM, Valenstein E. Clinical neuropsychology. New York: Oxford University
Press, 1985.
Kertesz A. Localization in neuropsychology. New York: Academic Press, 1983.
Marie P. Existe-t-il dans le cerveau humain des centres innés ou préformes de langage?
La Press Médicale 1922;17:117-81.
McGlone J, Davidson W. The relationship between cerebral speech laterality and spa-
tial ability with special reference to sex and hand preference. Neuropsychologia
1973a;ll:105-13.
McGlone J, Kertesz A. Sex differences in cerebral processing of visuospatial tasks.
Cortex 1973b;9:313-20.
Mesulam M-M. A cortical network for directed attention and unilateral neglect. Ann
Neurol 1981;10:309-25.
Mesulam M-M. Principles of behavioral neurology. Philadelphia: F.A. Davis, 1985.
Nielsen JM. Agnosia, apraxia, aphasia. New York: Hafner Press, 1965 (Reprint of
1946 edition).
Ojemann GA. Individual variability in cortical localization of language. J Neurosurgery
1979;50:164-9.
Semmes J. Hemispheric specialization: a possible clue to mechanism. Neuropsycholo-
gia 1968;6:11-26.
Whitaker HA, Ojemann GA. Graded localisation of naming from electrical stimulation
mapping of left cerebral cortex. Nature 1977;270:50-1.
CEREBRAL DOMINANCE
The term cerebral dominance reflects the observation that language functions
are usually lateralized to the left hemisphere and visual-spatial functions are
usually lateralized to the right hemisphere. Although this propensity for lan-
guage to lateralize to the left hemisphere is well documented, its biological
basis is unknown. There is convincing evidence that left-handers are much less
well lateralized than right-handers. Although right-hemisphere dominance for
speech is rare in true right-handers, bilateral-hemisphere or right-hemisphere
speech representation is common in left-handers. Aphasia after right-hemi-
sphere injury in right-handers is rare (crossed aphasia). Transient aphasia with
good recovery is common in left-handers, regardless of the hemisphere injured.
As of 1976, Brown and Hécaen (1976) found only nine convincing cases of
Cerebral Organization 5
crossed aphasia in dextrals in the world literature. On the other hand,
Zangwill (1960) found that of 54 left-handers with left-hemisphere lesions, 24
(44%) had severe aphasia and 21 (39%) had transient aphasia. Of 39 left-
handers with right-hemisphere lesions, 13 (33%) had severe aphasia and 17
(44%) had transient aphasia.
Hemispheric dominance for language may be assessed by a variety of
techniques, including the Wada test (intracarotid amobarbital [Amytal] injec-
tion), dichotic listening tests, and the effects of unilateral electroshock therapy.
Branch et al. (1964) examined dominance for language using the Wada test in
119 patients with intractable epilepsy. Of 48 right-handers, 43 (90%) were
left-hemisphere dominant for speech and 5 (10%) were right-hemisphere dom-
inant. The unusually high incidence of right-hemisphere speech dominance
reflects the presence of brain injury in a high proportion of the epileptics.
Among 71 left-handers, Branch et al. (1964) found 34 (48%) with left-hemi-
sphere speech dominance, reflecting the presence of brain injury in a high
proportion of the epileptics. Among the left-handers, evidence of prior left
hemisphere injury dropped the frequency of left-hemisphere dominance for
speech from 64% to 22%. Thus, early-life left-hemisphere injury strongly
influences speech dominance to shift from the left to right hemisphere. How-
ever, this injury must occur before six years of age and involve the central
language zone for language dominance to reliably shift from left to right.
Dichotic listening studies suggest that speech is less firmly lateralized to
the left hemisphere in left-handers than in right-handers. Some evidence sug-
gests that women are less lateralized than men. Based on available evidence,
Roberts (1969) concludes that the left hemisphere is dominant for language in
at least 95% of right-handers and 66% of left-handers. The right hemisphere is
dominant for language in about 30% of left-handers. Bilateral speech repre-
sentation probably occurs in a small percentage of left-handers and rarely in
right-handers. Attempts have been made to relate functional asymmetries (i.e.,
lateralization of language) to morphological (anatomical) asymmetries of the
brain, but such hypotheses remain controversial.
References
Annett M. Hand preference and the laterality of cerebral speech. Cortex 1975;11:
305-28.
Benton AL. Historical notes on hemispheric dominance. Arch Neurol 1977;34:127-9.
Branch D, Milner B, Rasmussen T. Intracarotid sodium Amytal for the lateralization of
cerebral dominance for speech. J Neurosurg 1964;21:399-405.
Briggs CG, Nebes RD. The effects of handedness, family history and sex on the perfor-
mance of a dichotic listening task. Neuropsychologia 1976;14:129-33.
Brown JW, Hécaen H. Lateralization and language representation. Neurology
1976;26:183-9.
Bryden MP. Tachistoscopic recognition, handedness and cerebral dominance. Neuro-
psychologia 1965;3:1-8.
Dennis M, Whitaker HA. Language acquisition following hemidecortication: linguistic
superiority of the left over the right hemisphere. Brain Lang 1976;3:404-33.