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Epileptic Seizures
Hemisphere Specialization
Aphasia
Apraxia
Ideomotor Apraxia
Ideational Apraxia
Visuoconstructive Apraxia
Alexia (Dyslexia)
Agnosia
Callosal Syndrome
Visual Effects
Hemialexia
Unilateral (Left) Ideomotor Apraxia
Unilateral (Left) Agraphia
Unilateral (Left) Tactile Anomia
Left Ear Extinction
Prefrontal Lobe Syndrome
The Grasp Reflex
Forced Collectionism
Alzheimer's Disease
Balint's Syndrome
Gerstmann's Syndrome
Anosognosia (Denial Syndrome, Anton-Babinski Syndrome)
Anton's Syndrome
Kluver-Bucy Syndrome
Simultanagnosia
The Alien Hand (Limb) Syndrome
KEY CONCEPTS
1) Epileptic seizures are manifestations of synchronized discharges of groups of neurons.
2) The left hemisphere is specialized or dominant for comprehension and expression of
language, arithmetic, and analytic functions. The right hemisphere is specialized or
dominant for complex nonverbal perceptual tasks, emotion, and some aspects of visual and
spatial perception.
3) Aphasias are classified into two major categories based on whether repetition is intact or
not.
4) Apraxia is the inability to perform skilled, learned, purposeful motor acts correctly.
5) Alexia pertains to the inability to comprehend written language (reading disability). It can
be developmental or acquired. Two forms of acquired alexia are recognized: pure alexia
(without agraphia) and alexia with agraphia.
6) Agnosia pertains to the inability to recognize stimuli that were recognized formerly.
Agnosia is modality specific: visual, auditory, and tactile.
7) Callosal syndromes include hemialexia, unilateral (left) ideomotor apraxia, unilateral (left)
agraphia, unilateral (left) tactile anomia, and left ear extinction.
8) Prefrontal lobe syndrome pertains to a conglomerate set of signs and symptoms that
includes impairments in decision making, ability to plan, social judgment, conduct,
modulation of affect and of emotional response, and creativity.
9) Alzheimer's disease is the example par excellence of cortical dementia.
EPILEPTIC SEIZURES

E
pilepsy is a common clinical condition characterized by recurrent paroxysmal attacks of
motor, sensory, autonomic, or psychic symptoms and signs depending on the area of the
brain involved. Epileptic seizures are triggered by synchronized discharges of a group of
neurons in the cerebral cortex as a result of developmental abnormality, infection,
trauma, tumor, metabolic derangement, or stroke. Epileptic seizures may be focal or
generalized. When generalized, they are usually associated with loss of consciousness. The
most common generalized seizure type is the tonic-clonic seizure type known as grand mal
seizure. Focal seizures are manifestations of the function of the cortical area from which
epileptic discharges emanate. Epileptic discharges in the region of the central sulcus may give
rise to motor and sensory symptoms. Spreading of the epileptic discharge along the motor or
sensory homunculus produces the so-called Jacksonian seizures or Jacksonian march. In such a
patient, a focal motor seizure may start by shaking of the side of the face contralateral to the
cortical lesion in the precentral gyrus and spread to involve the thumb, hand, arm, and leg in
this order in a pattern consistent with the location of these body parts in the motor homunculus.
A similar pattern of sensory march is associated with lesions in the postcentral gyrus. An
epileptic discharge in the frontal eye field produces attacks consisting of contralateral turning
of eyes and head (adversive seizures). Occipital discharges are associated with visual
hallucinations. Discharges in the primary visual cortex produce contralateral flashes of light,
whereas discharges in the association visual cortex produce well-formed images. Epileptic
discharges from the uncus and adjacent regions of the temporal lobe (uncinate fits) produce a
 233 Functional Neuroanatomy
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combination of complex motor and autonomic symptoms (psychomotor seizures). The epileptic
attack in such patients consists of a dreamy state, olfactory hallucinations (usually of “bad”
odors), gustatory hallucinations, oral movements of chewing, swallowing, or smacking of lips,
visual hallucinations (déjá vu experiences), and possibly aggressive behavior. Complex acts
and movements such as walking and fastening or unfastening buttons may occur.
HEMISPHERE SPECIALIZATION
The concept of cerebral dominance has undergone significant modification in recent years,
primarily because of studies on patients with unilateral brain damage. The older concept,
introduced by Gustav Dax and Paul Broca in 1865, which assigned to the left hemisphere a
dominant role in higher cerebral function, with the right hemisphere being subordinate to the
dominant hemisphere, has been replaced by a new concept of hemisphere specialization that
implies that each hemisphere is in some way dominant for the execution of specific tasks.
According to this concept, the left hemisphere is dominant or specialized for comprehension
and expression of language, arithmetic, and analytic functions, whereas the right hemisphere is
specialized for complex nonverbal perceptual tasks and for some aspects of visual (e.g., face)
and spatial perception. The right side of the brain is also dedicated to mapping feelings, bodily
sensations linked to emotions of happiness, anger, and fear. Language is localized to the left
hemisphere in more than 90% of right-handed people and two-thirds of left-handers. Thus
lesions of the left hemisphere are associated with disorders of language (aphasia or dysphasia),
whereas lesions of the right hemisphere are associated with impairment of visuospatial and
visuoconstructive skills. Patients with right hemisphere lesions are more likely to show such
manifestations as constructional apraxia (inability to construct or to draw figures and shapes),
dressing apraxia, denial of the left side of the body (denial that their left side is part of their
body), and hemineglect (visual and spatial neglect of the left side of their space, including their
own body parts). Idiographic(pictographic) language (Japanese Kanji) may be processed by
the right hemisphere because of its pictorial features.
APHASIA
The term aphasia refers to an acquired disturbance in comprehension, formulation of verbal
messages (language), or both. It can affect the grammatical structure of sentences (syntax), the
dictionary of words (contained in language) that denote meanings (lexicon), or the combination
of phonemes that results in word structure (word morphology).
Aphasia, as a specific impairment of language, should not be confused with mutism, dysarthria,
aphemia, and speech apraxia. Mutism is a nonvolitional state in which the patient does not
attempt to initiate speech. Dysarthria is a speech articulation due to disturbance in the muscular
control of the speech mechanism associated with damage to the central or peripheral nervous
system. Aphemia is a condition in which no articulation occurs due to a central motor deficit.
Speech apraxia is an imprecisely defined condition of impaired articulation of speech in which
speech is phonetically and prosodically awkward compared to dysarthric speech. Aphasia is
encountered most frequently in cortical lesions in the left hemisphere, although it may occur in
subcortical lesions. The cortical area of the left hemisphere invariably involved in aphasia is a
central core surrounding the sylvian fissure. The perisylvian core area is surrounded by a
larger region in which aphasia occurs less frequently.
The sequence of complex cortical activities during the production of language may be
simplified as follows: When a word is heard, the output from the primary auditory area (Heschl's
gyrus) is conveyed to an adjacent cortical area (Wernicke's area), where the speech sounds are
processed into word form and the word is comprehended (see Figure 17-20). If the word is to
be spoken, the comprehended pattern is transmitted via the arcuate fasciculus from Wernicke's
area to Broca's area of speech in the inferior frontal gyrus (see Figure 17-20). If the word is to be
read, the output from the primary visual area in the occipital cortex is transmitted to the angular
gyrus, which in turn arouses the corresponding auditory form of the word in Wernicke's area
(see Figure 17-22).
For didactic purposes, aphasia is classified into Broca's, Wernicke's, conduction, transcortical,
anomic, and global.
The different varieties of aphasia can be classified into those with impaired repetition (Broca's,
Wernicke's, conduction, and global aphasias) and those in which repetition is preserved
(transcortical and anomic aphasias) (Table 18-1).
Broca's aphasia is also known as nonfluent, anterior, motor, or expressive aphasia. This type of
aphasia is characterized by a difficulty in initiating speech and a decreased and labored
language output of 10 words or less per minute, during which the patient utilizes facial
grimaces, body posturing, deep breaths, and hand gestures to aid output; characteristically,
small grammatical words and the endings of nouns and verbs are omitted, resulting in
telegraphic speech. The speech output is thus unmelodic and dysrhythmic (dysprosody).
Despite the preceding limitations in verbal output, the speech often conveys considerable
information. These patients are unable to repeat what has been said to them. Paraphasias are
common and usually involve omission of phonemes or substitution of incorrect phonemes (“ha”
for “hall,” “pem” for “pen”). Writing and confrontation naming are impaired. Although Broca's
aphasia is usually attributed to a lesion in Broca's area of the frontal lobe, recent correlations of
aphasic speech with lesions seen on imaging studies have shown that the lesion is frequently
larger than Broca's area and involves the anterior insula, frontal operculum and the underlying
white matter. Pure damage to Broca's area (Brodmann areas 44 and 45) produces mild transient
speech deficit. Since the temporal lobe is intact in these patients, comprehension of language in
aural and written forms is usually intact. Most patients with Broca's aphasia can sing.
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Table 18-1. Aphasias

Type Repetition Fluency Auditory Localization

Comprehen-
sion

Broca's X Broca's area, anterior insula,

frontal operculum,
underlying white matter

Wernicke's X Posterior and superior tem-

poral gyrus, planum tempo-
rale, lower parietal cortex

Conduction X X Posterior perisylvian region

Global Massive perisylvian or

separate Broca's and
Wernicke's areas

Transcortical X X Anterior or superior to

motor Broca's area; may involve
part of Broca's area

Sensory X X Surrounding Wernicke's area

Mixed X Border zone, watershed area

of middle and anterior
cerebral arteries

Anomic X X X Inferior or anterior temporal

Broca's aphasia occurs often as a result of stroke (infarcts) most commonly affecting the middle
cerebral artery territory. Such infarcts often involve the motor cortex; thus patients with Broca's
aphasia are often hemiplegic with the arm (middle cerebral artery territory) more affected than
the leg (anterior cerebral artery territory). Broca's aphasia is named after Paul Broca, the French
anthropologist-physician who studied the patient Leborgne (nicknamed “Tan” because the only
word he could utter was tan) with aphasia and localized the lesion to the posterior part of the left
inferior frontal convolutions. Pierre Marie, in 1906, examined Leborgne's brain and found that
the lesion was more extensive.
Wernicke's aphasia is also known as fluent, posterior, sensory, or receptive aphasia. In contrast
to Broca's aphasia, the quantity of output in this type ranges from low normal to supernormal,
with an output in most patients of 100 to 150 words per minute. Speech is produced with little or
no effort, articulation and phrase length are normal, and the output is melodic. Pauses to search
for a meaningful word are frequent, and substitution without language (paraphasia) is common;
this may be substitution of a syllable (literal paraphasia) (wellow for yellow), phonemic
substitution of a word (kench for wrench) (verbal paraphasia), semantic substitution (knife for
fork), or substitution of a meaningless nonsense word (neologism). If a word is not readily
available, the patient may attempt to describe it, and the description may necessitate yet
another description, resulting in a meaningless output (circumlocution). Very highly paraphasic
fluent speech is termed jargon aphasia. Paraphasias also may occur in Broca's aphasia, but
these are articulatory errors, in contrast to those in Wernicke's aphasia, which are true
substitutions. Despite the fluent nature of speech output in Wernicke's aphasia, little information
is conveyed (empty speech). As in Broca's aphasia, patients with Wernicke's aphasia are unable
to repeat what is said to them. In contrast to Broca's aphasia, comprehension of both aural and
written forms of language is severely impaired in Wernicke's aphasia. As in Broca's aphasia,
 235 Functional Neuroanatomy
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naming is impaired. Wernicke's aphasia is attributed to a lesion in Wernicke's area in the
posterior part of the superior temporal gyrus and adjacent areas in posterior temporal
(including the planum temporale) and lower parietal cortex of the left hemisphere.
Wernicke's aphasia is named after Karl Wernicke, a German neurologist who in 1874
designated the posterior part of the superior temporal gyrus (area 22) of the left hemisphere as
an area concerned with the understanding of the spoken word. Wernicke's aphasia is also
known as Bastian aphasia, after Henry Charlton Bastian, the English neurologist who described
it in 1869, five years before Wernicke.
Conduction aphasia is characterized by fluent paraphasic speech, intact comprehension, poor
naming, and repetition. Classically, patients with conduction aphasia cannot read out loud
because of paraphasic intervention. Patients with conduction aphasia cannot write to dictation,
but write better when copying text and in spontaneous composition. Pathology in these patients
is usually located in the posterior perisylvian region and interrupts the output from Wernicke's
area to Broca's area via the arcuate fasciculus.
Global aphasia is a severe form of aphasia in which all the major functions of language (verbal
output, comprehension, repetition, naming, reading, and writing) are severely impaired.
Global aphasics retain limited capacity for singing. Global aphasics are differentiated from
patients with mutism in that the former make an attempt to speak and communicate with other
means, whereas the latter do not make such an attempt. Pathol-ogy is invariably extensive,
involving most of the left perisylvian area including Broca's area, Wernicke's area, the inferior
parietal cortex, and underlying white matter. In rare cases, two separate lesions in Broca's and
Wernicke's area are found.
Transcortical aphasia has been subdivided into motor, sensory, and mixed types. All are
characterized by preserved repetition. In transcortical motor aphasia, verbal output is nonfluent
and comprehension is intact, but writing and reading are invariably abnormal. Pathology in this
type of aphasia is located in the dominant frontal lobe in the neighborhood of Broca's area. It
involves the premotor region or a limited part of the inferior frontal gyrus in the left
hemisphere. This type of aphasia has been also reported with a lesion in the left basal ganglia.
In transcortical sensory aphasia, speech output is fluent and paraphasic, comprehension is
poor, and there are associated difficulties in reading, naming, and writing. Pathology in such
cases is usually in the border zone between the temporal and parietal lobes in the
neighborhood of Wernicke's area. Mixed transcortical aphasia, also known as isolation of the
speech area, is characterized by nonfluent speech output, poor comprehension, and inability to
name, read, or write. Pathology in these patients usually spares the perisylvian core region but
involves the surrounding border zone or watershed area, which is supplied by the most distal
tributaries of the middle cerebral artery.
Anomic aphasia, also known as amnestic or nominal aphasia, is characterized primarily by
word-finding difficulty. Although naming defects are common in almost all aphasic syndromes,
anomic aphasia refers to an isolated severe impairment of confrontation naming without
concomitant other speech impairments. This type of aphasia is most commonly encountered
with lesions in the left inferior or anterior temporal cortex. Different types of naming
impairments have been associated with damage to different cortical areas. Selective noun
retrieval deficits have been associated with damage to the left inferior and left anterolateral
temporal cortex. Disproportionate difficulty in verb retrieval, on the other hand, has been
associated with damage to the left premotor-prefrontal cortex. Within the temporal lobe, lesions
in the ventral inferotemporal cortex have been associated with disproportionate difficulty in
naming natural entities (like animals), whereas damage in the left temporal pole has been
associated with disproportionate difficulty in naming specific persons.
Crossed aphasia refers to the rare development of aphasia in right-handed persons, with right
(instead of left) hemisphere lesion. The aphasic syndrome in crossed aphasia may follow the
classical pattern (Broca, Wernicke, etc) with lesions in the corresponding area in the right
hemisphere, or be anomalous. In the latter, Broca's area lesion may present with Wernicke's
aphasia and Wernicke's area lesion with Broca's aphasia.
Subcortical aphasia. Aphasia has been reported in left basal ganglia and thalamic lesions. Within
the basal ganglia, the left caudate nucleus is especially involved. Within the thalamus, the left
ventrolateral and anteroventral thalamic nuclei are invariably involved. Aphasia associated with
left basal ganglia lesions is characterized by relatively fluent, paraphasic, and dysarthric
speech. Comprehension and repetition are often impaired. Thalamic aphasia has the profile of
Broca's or transcortical motor aphasia.
Pure word deafness, also known as verbal auditory agnosia, is characterized by poor
comprehension of spoken language and by poor repetition with intact comprehension of written
language, naming, writing, and spontaneous speech. The lesion in this type of disorder either
affects the primary auditory area or disconnects this area from Wernicke's area. This syndrome
is “pure” in the sense that it is not associated with other aphasic symptoms.
APRAXIA
Apraxia is the inability to perform skilled, learned, purposeful motor acts correctly despite
intact relevant motor and sensory neural structures, attention, and comprehension. The concept
of apraxia and the first classification of apraxia are credited to Hugo Karl Liepmann, the German
neurologist. There are several types of apraxia: ideomotor, ideational, and visuoconstructive.
Ideomotor Apraxia
Ideomotor apraxia is the inability to carry out, on verbal command, an activity that can be
performed perfectly well spontaneously. It is implied that this “inability” is not due to compre
 236

hension, motor, or sensory defects. Thus a patient with ideomotor apraxia will not be able to
carry out a verbal command to walk, stop, salute, open a door, stick out the tongue, etc.
To appreciate the pathophysiology of ideomotor apraxia, it should be understood that for a
skilled task to be performed, several events must take place. For example, the command to
walk, if oral, reaches the primary auditory area and is relayed to the left auditory association
cortex (Wernicke's area) for comprehension. Wernicke's area is connected to the ipsilateral
premotor area (motor association cortex, area 6) via the arcuate fasciculus. The motor
association area on the left side is connected to the primary motor cortex (area 4) on the left
side. When the person is asked to carry out a command with the left hand, the information is
relayed from the left premotor area to the right premotor area (via the anterior part of the
corpus callosum) and from there to the right primary motor area, which controls movements of
the left side of the body (Figure 18-1). Based on the preceding anatomic connections, three
clinical varieties of ideomotor apraxia have been recognized: parietal, in which the lesion is in
the anteroinferior parietal lobe of the dominant hemisphere; sympathetic, in which the lesion is
in the left premotor area; and callosal, in which the lesion is in the anterior part of the corpus
callosum.
Ideational Apraxia
Ideational apraxia is an abnormality in the conception of movement so that the patient may have
difficulty sequencing the different components of a complex motor act. To mail a letter, for
example, one must seal it, stamp it, and place it in the mailbox. The lesion in ideational apraxia
is in the dominant temporoparietooccipital area.
Visuoconstructive Apraxia
Visuoconstructive apraxia, also known as constructional apraxia, is the inability of the individual
to put together or articulate component parts to form a single shape or figure, such as
assembling blocks to form a design or drawing four lines to form a shape. It implies a defect in
perceiving spatial relationships among the component parts. Visuoconstructive apraxia was
described originally in lesions of the left (dominant) posterior parietal area. Subsequently, it
was shown that this type of apraxia is more prevalent and severe in right hemisphere parietal
lesions.

Figure 18-1 Figure 18-2

Figure 18-1. Schematic diagram showing the pathways Figure 18-2. Schematic diagram showing the neural
in-volved in carrying out a motor skill in response to an substrate of the syndrome of pure alexia without
oral command. agraphia A and of hemialexiaB.

Figure 18-3. Schematic diagram illustrating the

mechanism of unilateral (left) ideomotor apraxia A and
of unilateral (left) tactile anomia B.

The term constructional apraxia was suggested by Kleist in 1923 and fully described by Mayer-
Gross in 1935. Lord Brain proposed the term apractagnosia.
ALEXIA (DYSLEXIA)
Alexia (dyslexia) is the inability to comprehend written language (reading disability). It may be
acquired (acquired alexia or dyslexia), as in stroke patients who lose the ability to read, or
developmental (developmental dyslexia), in which there is an inability to learn to read normally
from childhood. Acquired alexia is of two types: pure alexia (alexia without agraphia, pure word
blindness) and alexia with agraphia (parietal alexia).
In pure alexia, the defect in comprehension may manifest as an inability to read letters (literal
alexia) or words (verbal alexia) or may be global with a total inability to read either letters or
words (global alexia). The anatomic substrate of pure alexia is usually a lesion in the left
primary visual area coupled with an other lesion in the splenium of the corpus callosum (Figure
18-2). The lesion in the left visual area prevents visual stimuli entering the left hemisphere from
reaching the left (dominant) angular gyrus, which is necessary for comprehension of written
language. The lesion in the splenium of the corpus callosum prevents visual stimuli entering the
intact right visual area from reaching the left angular gyrus. Writing is normal in this type of
alexia, but the patient cannot read what he or she writes. Cases have been described of pure
alexia without a splenial lesion. In such cases, one deep lesion in the left occipitotemporal
region isolates both occipital cortices from the left speech area in the angular gyrus.
 237 Functional Neuroanatomy
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Most commonly, alexia without agraphia occurs as a result of infarction in the territory of the left
posterior cerebral artery that supplies neural structures involved. Usually, a right homonymous
visual field defect is present.
In alexia with agraphia, there is a defect in both reading comprehension and writing. The
reading disorder is usually verbal (inability to read words). The writing difficulty is usually
severe. The anatomic substrate of this type of alexia is a lesion in the dominant angular gyrus,
hence the name parietal alexia.
The concept of alexia as separate from other language disorders was developed in 1885 by the
German neurologist Ludwig Lichtheim. The two types of acquired alexia (without and with
agraphia) were introduced by Dejerine in 1891 and 1892.
AGNOSIA
Agnosia is the inability of the individual to recognize perceived sensory information. Implied in
this definition is an intact sensory processing of the input, clear mental state, and intact naming
ability.
Agnosia is often modality specific: visual, auditory, and tactile. Visual agnosias include visual
object agnosia (inability to recognize objects presented visually), visual color agnosia (inability
to recognize colors), prosopagnosia (i.e., inability to recognize faces, including one's own face,
cars, types of trees), picture agnosia, and simultanagnosia (inability to recognize the whole,
although parts of the whole are appreciated correctly).
Auditory agnosia is the inability to recognize sounds in the presence of otherwise adequate
hearing. It includes auditory verbal agnosia (inability to recognize spoken language or pure
word deafness), auditory sound agnosia (i.e., inability to recognize nonverbal sounds such as
animal sounds, sound of running water, sound of a bell), and sensory amusia (inability to
recognize music).
Tactile agnosia is the inability to recognize objects by touch. It is usually associated with
parietal lobe lesions of the contralateral hemisphere. Astereognosis is the loss of ability to
judge the form of an object by touch. It includes amorphognosia (impaired recognition of size
and shape of objects), ahylognosia (impaired discrimination of quality of objects, such as
weight, texture, density), and asymbolia (impaired recognition of the identity of an object in the
absence of amorphognosia and ahylognosia). Asymbolia is used by some authors to refer to
tactile agnosia.
CALLOSAL SYNDROME
The disconnection of the right from the left hemisphere by lesions in the corpus callosum results
in the isolation of each hemisphere in such a way that each has its own learning processes and
memories that are inaccessible to the other hemisphere. The following are some of the effects of
callosal disconnection. The effects of callosal transection are considerably less in younger
children compared with adults because of the continued reliance in this age group on ipsilateral
pathways.
Visual Effects
Each hemisphere retains its own visual images and memories, but only the left hemisphere is
able to communicate, because of the callosal disconnection, what it sees through speech or
writing.
Hemialexia
Patients are unable to read material presented in the left hemifield. This occurs when the
splenium of the corpus callosum is involved in the lesion. Such visually presented material
reaches the right occipital cortex but cannot be comprehended because the splenial lesion
interferes with transmission of the visual image to the left (dominant) angular gyrus (Figure 18-
2).
Unilateral (Left) Ideomotor Apraxia
In response to verbal commands, patients are unable to carry out with the left hand some
behavior that is readily carried out with the right hand. The verbal command is adequately
received by the left (dominant) hemisphere but, because of the callosal disconnection, cannot
reach the right hemisphere, which controls left hand movement (Figure 18-3).
Unilateral (Left) Agraphia
Patients with callosal lesions are unable to write using their left hand (Figure 18-3).
Unilateral (Left) Tactile Anomia
Patients with callosal disconnection are unable, with eyes closed, to name or describe an object
placed in the left hand, although they readily name the same object in the right hand. The object
placed in the left hand is perceived correctly in the right somatosensory cortex but cannot be
identified because of the callosal lesion that disconnects the right parietal cortex from the left
(dominant) hemisphere (Figure 18-3).
Left Ear Extinction
Patients with callosal lesions show left ear extinction when sounds are presented simultaneously
to both ears (dichotic listening). Sounds presented to the left ear reach the right temporal cortex
but, because of the callosal disconnection, are not related to the left temporal cortex (dominant)
for comprehension.
There is evidence to suggest functional specialization of different segments of the corpus
callosum. Thus lesions in the posterior part of the corpus callosum (splenium) are associated
with hemialexia, lesions in the anterior part are associated with left ideomotor apraxia, and
lesions in the middle part are associated with left-hand agraphia; lesions in the middle and
posterior parts result in left-hand tactile anomia.
PREFRONTAL LOBE SYNDROME
 238

The prefrontal lobe syndrome occurs in association with tumors, trauma, or degenerative
disease in the prefrontal and or bitofrontal cortices. The syndrome is characterized by a
conglomerate of signs and symptoms that include impairments in decision making, ability to
plan, social judgment, conduct, modulation of affect and of emotional response, and creativity.
Such patients lose spontaneity in motor as well as mental activities. They do not appear to
realize that they are neglecting themselves and their responsibilities at home and work.
Affected patients may sit for hours looking at objects in front of them or staring out a window.
They manifest loss of inhibition in social behavior and are usually euphoric and unconcerned.
They may become incontinent of stools and urine because of the lack of spontaneity. Patients
with prefrontal lobe syndrome exhibit inappropriate repetitive motor or speech behavior
(perseveration) because of their inability to disengage from a behavior that is no longer useful.
THE GRASP REFLEX
Some brain-damaged patients show, in response to tactile stimulation of their hands or to the
mere presentation of an object, a tendency to grasp at the object without any apparent intention
to use it in a purposeful manner. Two types of grasp phenomena have been described: (1) the
grasp reflex and (2) the instinctive grasp reaction.
The grasp reflex is generally considered an index of frontal lobe pathology, although the
evidence in support of this localization is not so compelling. The grasp reflex has been reported
with pathology in the basal ganglia, temporal lobe, parietal lobe, and parietooccipital region. In
the majority of cases, however, pathology is either in the frontal lobe or in subcortical
structures. Unilateral lesions usually result in bilateral grasping.
In contrast, the instinctive grasp reaction (forced groping) is usually ipsilateral to the focal
cerebral lesion and is seen more often with retrorolandic lesions of the right hemisphere,
suggesting that it is one of the right hemisphere behavioral syndromes caused by disturbances
of selective attention.
FORCED COLLECTIONISM
Forced collectionism is a rare prefrontal lobe syndrome characterized by involuntary,
irrepressible behavior of searching, collecting, and storing that is goal-directed and item-
selective. It results from inefficient or loss of frontal lobe inhibition. It is associated with bilateral
damage to the orbitofrontal and polar prefrontal cortices.
Pathologic patterns of collecting have been observed following frontal lobe injury. They range
from a tendency to grasp to the irrepressible seizure and storage of surrounding objects
(hoarding behavior). In contrast to forced collectionism, these behaviors are not planned and
not selective.
ALZHEIMER'S DISEASE
Alzheimer's disease is the example par excellence of cortical dementia. It was first described
by Alois Alzheimer, the German psychiatrist, in 1906–1907, based on pathological findings in
the brain of a patient (Auguste D) with memory impairment. It is characterized by relentlessly
progressive memory loss. Early on in the disease, patients lose recent memory (telephone
numbers, appointments). As the disease progresses, remote memory is impaired. In the end
stage, memory loss is nearly total. With advance in the disease, patients will be unable to
recognize their family members or their familiar surroundings. The pathologic hallmarks are
neurofibrillary tangles and senile plaques. The former are intracellular aggregates of twisted
cytoskeletal filaments and abnormally phosphorylated tau protein. The latter are abnormal
neurites surrounding an aggregated B-amyloid core in the neuropil between nerve cells. The
limbic cortex is most affected, the association cortices are heavily affected, the primary sensory
areas are minimally affected, and the motor cortex is least affected. Within the limbic cortex, the
entorhinal cortex (Brodmann area 28) is the most heavily affected, thus disconnecting the
hippocampus from association areas of the cortex. In Alzheimer's disease, about 50% of neurons
are lost.
Ninety percent of Alzheimer's disease is sporadic. The familial cases comprise 10% of cases and
are related to abnormal mutations in chromosomes 21, 14, and 1. Chromosome 21 mutation is in
the amyloid precursor protein and has been associated with abnormal quantities of amyloid
production. Mutations in chromosomes 14 and 1, on the other hand, are associated with protein
presenilins that are possibly responsible for build up of amyloid.
Risk factors for Alzheimer's disease have been associated with chromosome 19, which encodes
apolipoprotein, and chromosome 12, which encodes alpha 2 macroglobulin.
BALINT'S SYNDROME
This rare syndrome is named after the Hungarian neurologist Rudolph Balint. The syndrome is
also known as Balint-Holmes syndrome, optic ataxia, ocular apraxia, and psychic paralysis of
visual fixation. It is characterized by a triad of (1) simultanagnosia, (2) optic ataxia, and (3)
ocular apraxia. Simultanagnosia, also known as visual disorientation, is the inability of the
patient to perceive the visual field as a whole. Optic ataxia is the inability to reach for objects
under visual guidance. Ocular apraxia is the inability to direct gaze voluntarily to visual targets.
The associated cortical lesion is bilateral parietooccipital junction (Brodmann areas 7 and 19).
GERSTMANN'S SYNDROME
This syndrome is named after Josef Gerstmann, an Austrian neuro-psychiatrist who described
the syndrome in 1930. The syndrome is also known as the Badal-Gerstmann syndrome and the
angular gyrus syndrome. Antoine-Jules Badal, a French ophthalmologist, had reported some
features of the syndrome in 1888. The syndrome consists of the combination of right-left
disorientation, acalculia (reduced ability to perform simple calculations), agraphia (inability to
write), and finger agnosia (inability to recognize various fingers) due to a lesion in the left
angular gyrus. Asymbolia for pain and constructional apraxia are added features in some cases.
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ANOSOGNOSIA (DENIAL SYNDROME, ANTON-BABINSKI SYNDROME)
The term anosognosia was introduced by Josef-Franéois-Felix Babinski in 1912 for unawareness
of physical deficits or disease. This is seen most often with lesions of the nondominant (right)
parietal lobe, with unawareness of deficits of the left side of the body. The denial syndrome may
include denial that the paretic limbs belong to the patient. Hemispatial neglect often co-occurs
with anosognosia. In hemispatial neglect, the contralateral side of the body and visual space are
ignored but can be used if attention is drawn to them. Hemispatial neglect occurs after damage
to either hemisphere but is typically more common and severe after right parietal lesions. The
precise location of the lesion in the parietal lobe has not been determined, but most likely is in
the inferior parietal lobule and the adjacent intraparietal sulcus.
ANTON'S SYNDROME
Anton's syndrome traditionally refers to the clinical phenomenon of denial of blindness
(anosognosia for blindness) in a patient who has suffered acquired cortical blindness. The most
common setting is acute bilateral occipital cortex ischemia secondary to posterior circulation
insufficiency. Although classically a manifestation of cortical blindness, Anton's syndrome has
been reported in patients with blindness from peripheral visual pathway lesions (optic nerve,
chiasm). The syndrome is named after Gabriel Anton, an Austrian neurologist who described
the syndrome in 1899.
KLUVER-BUCY SYNDROME
The Kluver-Bucy syndrome was first described in 1939 in monkeys after bilateral temporal
lobectomy. The human counterpart was described by Terzian and Dalle Ore in 1955 after
bilateral removal of the temporal lobes. The syndrome consists of six main elements: (1)
blunted affect with apathy, (2) psychic blindness or visual agnosia with inability to distinguish
between friends, relatives, and strangers, (3) hypermetamorphosis with a marked tendency to
take notice and attend to fine and minute visual stimuli, (4) hyperorality, placing all items in the
mouth, (5) bulimia or unusual dietary habits, and (6) alteration in sexual behavior
(hypersexuality, sexual libertarianism).
SIMULTANAGNOSIA
Simultanagnosia is the inability to appreciate more than one aspect of the visual panorama at
any single time. Affected patients cannot experience a spatially coherent visual field because of
an inability to voluntarily control the shifting of attention or to disengage from a fixed target.
Objects in the visual field of affected patients appear and disappear erratically. Affected
patients fail to see a match flame held several inches away when their attention is focused on the
tip of a cigarette held between their lips. The term simultanagnosia was introduced by Wolpert
in 1924 to refer to a condition in which the patient is unable to recognize or abstract the
meaning of the whole (pictures or series of pictures) even though the details are appreciated
correctly. Simultanag-nosia is frequently a component of Balint's syndrome. Isolated
simultanagnosia is associated with lesions in the unimodal visual association cortex (Brodmann
area 19).
THE ALIEN HAND (LIMB) SYNDROME
The alien hand (limb) syndrome is characterized by the unwilled and uncontrolled actions of an
upper limb on either the dominant or nondominant side. The alien hand performs auton-omous
activity that the subject cannot inhibit and then often contrasts with voluntary actions performed
by the other hand. Patients often fail to recognize ownership of the limb and state that the alien
hand has a mind of its own. The alien hand syndrome was first described in 1908 by Goldstein.
Two forms of alien hand exist: (1) an acute, transient condition in the non-dominant hand due to
callosal lesion and (2) a chronic condition resulting from additional medial frontal lesions
involving the supplementary motor area. The combined callosal and medial frontal lesions
presumably release the lateral frontal motor system responsible for environmentally driven
activity. A sensory alien hand syndrome has also been described in which the right arm
involuntarily attacks the left side of the body, including choking movement.
TERMINOLOGY
Adversive seizures.
A variety of seizures in which there is deviation of eyes and/or head to one side secondary to a
stimulating lesion in the contralateral frontal eye field region.
Agnosia (Greek a, “negative” gnosis, “knowledge”).
Impairment of the ability to recognize stimuli that were recognized formerly despite intact
perception, intellect, and language. The term was coined by Sigmund Freud in 1891. The lesion
is usually in the posterior parietal region.
Agraphia (Greek a, “negative” grapho, “to write”).
Inability to express thoughts in writing due to a cerebral lesion. The first modern descriptions of
agraphia are those of Jean Pitres in 1884 and of Joseph-Jules Dejerine in 1891.
Alzheimer, Alois (1864–1915).
German neuropsychiatrist and pathologist. He described Alzheimer's disease in a lecture in
1906 and a publication in 1907. The term “Alzheimer's disease” was coined in 1910 by Ernst
Kraepelin, a German psychiatrist and co-worker of Alzheimer.
Anomia (Greek a, “negative” onoma, “name”).
Inability to name objects or of recognizing and recalling their names.
Anton, Gabriel (1858–1933).
Austrian neurologist who described visual anosognosia (anosognosia for blindness) in 1899.
The term anosognosia was coined by Babinski in 1912.
Aphasia (dysphasia) (Greek a, “negative” phasis, “speech”).
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Language impairment following cortical lesion in the left hemisphere. Either inability to speak
or to comprehend language or both.
Apraxia (Greek a, “negative” praxis, “action”).
Inability to carry out learned skilled movements on command despite intact motor and sensory
systems and good comprehension.
Babinski, Josef-Franéois-Felix (1857–1932).
French neurologist of Polish descent. Described the “phenomenon of the toes” in 1896, which
became known as the Babinski sign. Coined the terms anosognosia, dysdiadochokinesis, and
asynergia, among others.
Bastian, Henry Charlton (1837–1915).
English neurologist, who described Wernicke's aphasia in 1869, five years before Karl
Wernicke.
Circumlocution.
Convoluted, meaningless speech output, providing information rather than defining the objects
to be communicated. Characteristic of Wernicke's aphasia.
Dax, Gustav.
French physician, who, in 1865, published the observation of his father, Marc Dax, about left
hemisphere dominance for language, which was noted, but not published, by his father in 1836.
Déjá vu (French, “already seen”).
An illusion in which a new situation is incorrectly viewed as a repetition of a previous situation.
Usually an aura of a temporal lobe seizure.
Dysphasia (Greek dys, “difficult” phasis, “speech”).
Distur-bance in communication involving language.
Dysprosody (Greek dys, “difficult” prosodos, “a solemn procession”).
Disturbance in stress, pitch, and rhythm of speech. A feature of all types of aphasia, but
especially of Broca's aphasia.
Goldstein, Kurt (1878–1965).
German-American physician. Described the alien hand (limb) syndrome (la main etrangere,
anarchic hand).
Idiographic language.
Pictographic language such as Japanese Kanji.
Jacksonian seizures.
The spread of tonic-clonic seizure activity through contiguous body parts on one side of the
body secondary to excitation of adjacent cortical areas within the motor or sensory homunculus.
Also known as Jacksonian march and Bravais-Jackson epilepsy. L. Bravais described this
phenomenon in his graduation thesis in 1827 from the University of Paris but did not analyze the
etiology, which John Hughlings-Jackson did.
Lichtheim, Ludwig (1845–1928).
German neurologist and pathologist. Recognized alexia as a distinct condition separate from
other language disorders. He is credited with describing subcortical aphasia in 1885 and
subacute combined degeneration of the spinal cord in vitamin B12 deficiency (Lichteim
syndrome).
Liepmann, Hugo Karl (1863–1925).
German neurologist and psychiatrist, who introduced the concept of apraxia and proposed its
classification in 1900.
Marie, Pierre (1853–1940).
French neurologist who, in 1906, disagreed with Broca on the extent of the lesion that produced
aphasia in patient Leborgne. He and Charcot are credited with describing peroneal muscular
atrophy in 1886 (Charcot-Marie-Tooth disease).
Mayer-Gross, Willi.
German psychiatrist who provided full description of constructional apraxia in 1935.
Neologism (Greek neos, “new” logos, “word”).
A newly coined word either in response to a communicative need or as a result of brain
disorder. In the latter case, the newly coined word is a replacement of a desired word but
without meaning.
Paraphasia (Greek para, “to, at, from the side of” phasis, “speech”).
An aphasic phenomenon in which the patient employs wrong words or uses words in wrong
combinations.
Prosopagnosia (Greek prosopon, “face” gnosis, “knowledge”).
Inability to recognize familiar faces. The word was coined by Bodamer in 1947, although the
phenomenon had been recognized by Jackson and Charcot at the end of the nineteenth century.
Simultanagnosia.
The inability to comprehend more than one element of a visual scene at the same time or to
integrate the parts into a whole.
Uncinate (Latin uncinus,”hook-shaped”).
Pertaining to the uncus of the temporal lobe. Uncinate seizures are temporal lobe seizures in
which olfactory and gustatory hallucinations occur as part of the seizure. The name uncinate fits
was applied by Jackson in 1899.
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19  
  
Boundaries and Divisions
Preoptic Region
Suprachiasmatic (Supraoptic) Region Tuberal Region
Mamillary Region
Connections
Local Connections
Extrinsic Connections
Functions of the Hypothalamus
Control of Posterior Pituitary (Neurohypophysis)