Posterior Cortical Atrophy
D. Frank Benson, MD; R. Jeffrey Davis, DO; Bruce
\s=b\ Five patients had progressive de-
mentia heralded by disorders of higher
visual function. All eventually developed
alexia, agraphia, visual agnosia, and com-
ponents of Balint's, Gerstmann's, and
transcortical sensory aphasia syndromes.
Memory, insight, and judgment were rela-
tively preserved until late in the course.
Predominant parieto-occipital atrophy
was demonstrated on both computed
tomography and magnetic resonance
imaging in two of the patients; posterior
circulation was normal by angiography in
the three studied. To date, no pathologic
specimen is available for study; specula-
tions on the underlying pathologic condi-
tion include an atypical clinical variant of
Alzheimer's disease, a lobar atrophy anal-
ogous to Pick's disease, or some previ-
ously unrecognized entity.
(Arch Neurol 1988;45:789-793)
Pouring evaluation of large popula¬
a logical tests supplemented clinical exami¬
tion of patients with dementia, a nation in the three patients who were
few individuals with slowly progres¬
sive deterioration stood out because of
early visual dysfunction. Despite dis¬
abling visual problems, both visual
acuity and visual fields remained
intact until late when unilateral qua¬
drante visual extinction could be
demonstrated. Agnosia, alexia, anom¬
ia, Balint's syndrome (sticky fixation,
ocular dysmetria, and simultanagno-
sia), Gerstmann's syndrome (right-
left disorientation, finger agnosia,
acalculia, and agraphia), and trans-
cortical sensory aphasia eventually
developed. Memory, insight into their
problems, and motivation to improve
themselves were preserved until late
in the course.
PATIENT SELECTION AND EVALUATION
Four patients from neurologic and psy¬
chiatric clinics at UCLA were noted to
have a history of slowly progressive
dementing illness, with the most promi¬
nent cognitive and functional deficits
referable to visual processing. A similar
Accepted for publication Feb 15, 1988.
From the Department of Neurology, UCLA
School of Medicine; and the Neurobehavior Unit,
West Los Angeles Veterans Administration Med-
ical Center (Brentwood Division) (Drs Benson
and Davis); and the Department of Neurology, St
Paul-Ramsey Medical Center, St Paul, Minn (Dr
Snyder).
Reprint requests to UCLA Department of Neu-
rology, 710 Westwood Plaza, Los Angeles, CA
90024-1769 (Dr Benson).
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D. Snyder, MD
case was discovered by one of us (B.D.S.).
None had hypertension, diabetes, hyperlip¬
idemia, clinical encephalitis, heavy metal
exposure, or known medical cause. Prima¬
ry motor and sensory modalities, including
visual fields, were intact. Causes other
than a primary degenerative process were
sought. All patients underwent a routine
complete blood cell count; thyroid, liver,
and renal function tests; glucose, serum
calcium, and phosphorus determinations;
and determinations of sedimentation rate
and folate and vitamin B12 levels. Twenty-
four-hour urine samples were collected to
determine the levels of heavy metals and
other toxic substances. All patients under¬
went lumbar punctures. No abnormalities
were demonstrated.
Computed tomography (CT) and mag¬
netic resonance imaging (MRI) were per¬
formed in all patients, and three under¬
went angiography of the carotid and verte¬
brobasilar systems (Table 1). An electroen¬
cephalogram (EEG) was performed on all
patients and a full battery of neuropsycho¬
testable.
REPORT OF CASES
Case 1.—A 64-year-old former bank
executive presented with episodes of anxi¬
ety that complicated a slowly progressive
disturbance of vision and language. About
eight years earlier, he had noted difficulty
reading; he remained at his job, but his
secretary had to read for him. Although
still able to write, he could not read what
he produced. Eventually, he also lost the
ability to write and had difficulty finding
his way in familiar areas (environmental
agnosia) and in performing visually medi¬
ated tasks. The problems slowly pro¬
gressed; he stated that what he saw disap¬
peared before he could sense what it was.
Progressive worsening of his expressive
ability was observed, though his family
remained convinced that he understood
what was said.
On examination, he was alert, oriented,
attentive, and in reasonably good physical
health. His manner was gracious and his
insight was painfully apparent. Conversa¬
tional speech was hesitant, with word-
finding pauses and occasional semantic
and verbal paraphasic substitutions. Artic¬
ulation and prosody were intact and he
readily repeated and comprehended simple
sentences; with complex or abstract
descriptions of objects or actions, however,
comprehension became faulty. Confronta¬
tion naming was markedly impaired but
improved with palpation of the object.
When offered multiple choices, he selected
the names of colors better than objects or
simple shapes. He could not read words,
letters, or numbers. He wrote his name and
one three-word sentence to dictation, but
paraphasic substitutions intruded into
other attempts. Simple calculations were
poor and not aided by paper and pencil.
Right-left orientation was almost perfect
for his own body but was otherwise ran¬
dom. His ability to name and recognize
fingers was severely impaired. Registra¬
tion of new information could not be strin¬
gently assessed because of word-finding
impairment and visual problems, but
recall of verbal material was nearly per¬
fect if multiple choice responses were pro¬
vided. Behaviorally, a considerable degree
of preserved memory function was indi¬
cated by the content of his continued
attempts at interpersonal discourse.
Despite language problems, he correctly
interpreted similarities, differences, and
proverbs. He easily performed verbally
commanded praxis tasks unless visual
mediation to an outside object was
required. For instance, he easily oriented
the telephone receiver to his ear but could
not replace the receiver on the hook. He
could not copy, trace, or perform line orien¬
tation tasks. He drew a circle, square, and
triangle on command, but the productions
were poor and he failed more complex
figures, su&h as a bicycle. He competently
tapped out rhythms and performed recip¬
rocal rhythm tasks.
The only problem noted on basic neuro¬
logic examination was an inconsistent and
questionable diminution of visual acuity in
the right lower quadrant, best demon¬
strated by double-simultaneous stimula¬
tion. He accurately counted fingers at 45
cm, but was slow and uncertain. There was
neither paresis nor alteration of sensation.
He walked as though blind but could navi¬
gate the room without colliding into any¬
thing. When offered a chair, he had diffi¬
culty finding it and when he did, sat on the
arm before correctly seating himself.
A strong tendency to fix his gaze on a
single object (sticky fixation) was present.
Full lateral and vertical gaze were possible
if he followed a moving object, but jerky,
hypometric saccades and tendency to over¬
shoot were noted. When asked to touch a
stationary object held in front of him, he
initially missed by 7.5 or 10 cm (ocular
dysmetria). With repeated attempts, his
performance improved but remained
uncertain. He routinely failed to find all
objects in an array (simultanagnosia).
Meaningful neuropsychological testing
could not be performed because of the
severe impairment present at the time of
the initial visit. Computed tomography
and MRI of the head revealed bilaterally
symmetrical atrophy, most prominent in
the parieto-occipital region, with enlarge¬
ment of both the ventricles and the cortical
sulci (Fig 1). An EEG showed a generalized
6- to 7-Hz pattern without evidence of focal
 dysfunction.
Case 2.—A 59-year-old bookkeeper
sought help for "increasing memory diffi¬
culty" but presented his own history with
only minimal additions from family mem¬
bers. His problem had surfaced the previ¬
ous year with progressive difficulty in fol¬
lowing a line of print. Problems in calcula¬
tion, even in keeping the family checkbook,
developed and he became lost while driving
in his community. He was unable to main¬
tain his job.
Mental status examination demon¬
strated an alert man with a digit span of
six and an intact memory for the chronolo¬
gy of his symptoms and for general infor¬
mation concerning both remote and cur¬
rent events. Comprehension and repetition
of spoken language were normal. Confron¬
tation naming of high-frequency items was
intact, but less common items were missed. Fig 1.—Magnetic resonance imaging scan (case 1) demonstrating ventricular enlargement,
Both reading and writing were limited to particularly of occipital horns, and cortical atrophy, most pronounced in parieto-occipital
simple statements (eg, "The weather is areas.
nice"). Calculations were totally failed.
Right-left orientation was poor, but finger
identification was adequate. Neurologic Table 1.—Patient Data*
examination revealed no focal abnormali¬
ty; visual acuity was normal, visual fields Patients
were full to confrontation, and the extra¬
ocular movements were intact.
Over the next three years, his mental age of onset, y 56 54 67 53
Approximate
state progressively deteriorated. Although Duration of disorder, y
able to feed himself, he could not identify Anomia
the food until it was in his mouth. Similar¬ Alexia
ly, although able to dress himself, he Memory impairment
needed guidance and encouragement. Sim¬ Gerstmann's syndrome
ple tasks, such as opening a jar or using a Agraphia_
tool such as a hammer or screwdriver,
Acalculia
became impossible. He developed total
Finger agnosia
alexia, agraphia, and acalculia plus com¬ R-L disorientation
plete right-left disorientation and finger
agnosia (Gerstmann's syndrome). The Balint's syndrome
aphasia worsened considerably, particu¬ Sticky fixation

larly naming, which was almost totally Ocular dysmetria

failed. Some difficulty with language com¬ Simultanagnosia
prehension developed, but he still repeated Visual field extinction RLQ RHH LLQ LLQ
well. A full Balint's syndrome could be Environmental agnosia
demonstrated. Emotional symptoms
Neuropsychological testing on two occa¬ Full scale IQ NT 83
sions early in the course demonstrated a Verbal IQ NT 91 85 NT
depressed full scale IQ, with a significant Performance IQ NT 63 63 NT 44
difference between verbal (VIQ) and per¬
Wechsler memory quotient NT Impairedt 59 NT 79
formance (PIQ) subscores (VIQ 91; =

Computed tomography PA GA NEG PA

PIQ 63). Serial EEGs consistently dem¬
=

onstrated slow (6- to 7-Hz) background
without focal abnormalities. Repeated CT
scans and a single MRI scan showed only a
mild, progressive atrophy.
Case 3.—A 57-year-old woman com¬
plained of slowly progressive difficulty
with vision. Three changes of glasses over
a 2!/2-year period had not helped. She could
not drive, had difficulty reading, and com¬
plained of forgetting where she had placed
her belongings.
Examination revealed a healthy individ¬
ual without basic medical or neurologic
difficulty. There was no paresis, sensory
loss, or visual field disturbance, and
extraocular movements were intact. She
was alert but not fully oriented. Verbal
output was fluent but empty, with circum¬
locutions and occasional paraphasic sub¬
stitutions. Comprehension and repetition
were normal. On confrontation naming,
she had difficulty with low-frequency
words (eg, the "bridge" of her glasses, the
NEG
Magnetic resonance imaging scan PA GA PA PA
Angiography NL NL NL
Electroencephalography SL SL SL SL SL
*NT indicates not testable; GA, generalized atrophy: PA, posterior atrophy: SL, slow; NL, normal: ?, not
reported; +, present; ±, mild; —, absent; RLQ, right lower quadrant; RHH, right homonymous hemianopsia;
LLQ, left lower quadrant; NEG, negative.
tNumbers not given in report and raw data subsequently lost.
"thread" used to sew a button). She could cal tests was most remarkable for its dem¬
comprehend written material only at the onstration of visually oriented impair¬
level of a three- or four-word sentence. ments reflected in a 22-point difference in
Writingwas severely impaired; only her the VIQ and PIQ subscores. A CT scan was
own name was correctly spelled. She easily read as normal, but EEGs were abnormal,
learned and retained new verbal informa¬ with generalized slowing and disorganized
tion and retrieved information from the background frequencies.
past. She was almost totally unable to Evaluation at several additional diag¬
calculate. Right and left were discrimi¬ nostic centers with repeated laboratory
nated with fair accuracy, but mistakes of tests produced similar results. Five and
finger identification were consistently not¬ one-half years following the original
ed. She could not copy drawings, except for examination, she was reevaluated. She now
a circle. acted as though blind and had to be led.
A complete battery of neuropsychologi- Visual acuity was normal, however, and

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 visual fields were full to confrontation
testing although an inconsistent right
visual extinction was noted. Her eyes
moved in all directions when following a
finger, but hypometric saccades were
prominent. Neither paresis nor primary
sensory loss was evident. She was now
severely aphasie with a fluent, anomic
output, significantly disturbed comprehen¬
sion, almost total inability to name on
confrontation, and total alexia and
agraphia but intact repetition of spoken
language (transcortical sensory aphasia).
The severity of the mental deterioration
made neuropsychological testing impossi¬
ble. Gerstmann's syndrome and a full Bal¬
int's syndrome were present. Repeated CT
and MRI scans demonstrated atrophy,
greatest in the occipital and parietal
regions bilaterally, without evidence of a
mass lesion or focal lucency (Fig 2). Angi¬
ography showed normal vessels in both
anterior and posterior vascular systems.
The EEG remained generally slow. Despite
marked disability, she retained consider¬
able insight, frequently manifested by cry¬
ing and despondency concerning her fear
of progressive disability.
Case 4.—A 75-year-old, college-educated
author was evaluated for progressive intel¬
lectual impairment of seven years' dura¬
tion. She first noted that her typing slowed
and typographical errors increased. Her
handwriting deteriorated, eventually be¬
coming illegible. She continued typing a
novel, which was published, and then
wrote another. Grammatical structure and
the general content were satisfactory, but
the more recent work was not accepted for
publication. By then, she also noted diffi¬
culty with many daily tasks but remained
independent.
On examination, she was alert and
expressed herself clearly, although some
word-finding pauses and circumlocution
were present. Comprehension, though gen¬
erally intact, broke down with complex
grammatical constructions. Repetition was
intact to an extended span length. She
exhibited a profound confrontation anom¬
ia, failing even the first few items on the
Boston Naming Test. She appeared unable
to recognize objects visually but also had
difficulty providing a name when the
item's function was described. Phonemic
cues did not help. When an object was
placed in her hand, she could pick its
function from multiple-choice offerings
but could not select the name from several
choices. Her only writing was a barely
legible signature. Memory testing was
complicated by the severe anomia, but
when offered multiple-choice responses
concerning current news, she was fully
correct, even for obscure details. Calcula¬
tion was profoundly impaired, right-left
orientation was totally disturbed, and she
could not differentiate fingers. She failed
to copy even the simplest geometric draw¬
ing and even had difficulty finding the
model on the page. She distinguished
between a thick and a thin line but could
not name or choose a color from multiple
choice. Ocular fixation was intense; visual
recognition was so limited that simul-
tanagnosia could not be tested. No visual
field defect could be demonstrated
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(case 4) demonstrating striking enlargement of occipital
Fig 2.—Computed tomographic scan
horns bilaterally.
although extinction in the left lower quad¬
rant with double simultaneous stimulation
was noted late in the course. The advanced
state of deterioration at the time she was
first seen in our clinic precluded meaning¬
ful neuropsychological testing. A CT scan
was considered normal for her age, but an
EEG showed diffuse slowing.
Case 5.—A 54-year-old housewife com¬
plained that "my vision is hazy and fades
in and out." Ophthalmologic examination
demonstrated normal visual acuity, but
she noted increasing difficulty in sewing,
playing cards, and in playing the piano.
She found herself uncertain about which
direction to take in her own home and
became lost in previously familiar sur¬
roundings. She could no longer perform the
simple arithmetic required to balance her
checkbook. Despite these difficulties, she
held telephone conversations, entertained
guests, performed most household chores,
planned meals, and attended a dance
class.
On mental status examination, she was
alert and oriented, articulated clearly, and
showed good verbal reasoning. She was
fluent and repeated and comprehended
well. She read aloud, but with difficulty.
Knowledge of current events was mildly
impaired and both registration and
retrieval of verbal and nonverbal material
was imperfect. The line bisection task was
performed slowly but correctly and with¬
out omission. She drew a clock but could
not set the specified time. Her copy of a
cube lacked three-dimensional quality.
Neurological examination revealed no
abnormalities of the cranial nerves, motor
strength, sensation, or coordination.
Visual acuity was normal, eye movements
were full, optokinetic nystagmus was
equal, and no visual neglect was demon¬
strated with double simultaneous visual
stimulation. An EEG demonstrated theta
activity. Computed tomography without
and with contrast showed only mild sulcal
widening. Neuropsychological testing
showed a clear discrepancy between VlQ
and PIQ scores (Table 1).
Two years later, she could still articulate
a detailed description of her problems with
only an occasional paraphasic substitution.
Despite intact functional speech, she could
write nothing but her name. All visual
tasks were severely impaired, particularly
attempts to integrate a complex visual
picture into a meaningful whole (simul-
tanagnosia). Spelling aloud was severely
impaired. New learning was limited and
she could perform only the simplest arith¬
metic.
After three years, her speech became
increasingly circumstantial. She no longer
handled money and could not perform seri¬
al threes. Her neighborhood was now unfa¬
miliar and she became lost if unaccompa¬
nied. New learning was profoundly
impaired. Although she experienced diffi¬
culty getting clothing on correctly, she still
attended ballet class for exercise, applied
her own makeup, and managed some
household duties. She remained socially
appropriate and retained insight into the
nature of her problems with no change in
personality or mood.
On examination three years after initial
examination, calculations and right-left
discrimination were clearly impaired and
her ability to find objects in space was
poor. Visual fields were intact to confron¬
tation, but she showed greater difficulty
localizing objects in the left visual field,
and fairly consistent extinction to double
simultaneous stimulation could be demon¬
strated in the left inferior quadrant. Cra¬
nial nerves were otherwise intact, there
was no dysarthria or motorimpairment,
reflexes were brisk and symmetrical, and
toes were downgoing. Sensation, balance,
and coordination testing were unreveal-
ing.
Neuropsychological testing was per¬
formed on several occasions, providing
supportive data for the observations of far
greater visual than verbal disorder. Com¬
puted tomographic scanning showed ven¬
tricular size to be somewhat larger than
average for age, particularly in the posteri¬
or-superior parietal regions bilaterally. An
MR scan demonstrated atrophy that was
most pronounced posteriorly. Brain-stem,

Table 2.—Posterior Cortical
Course
Clinical findings
Laboratory findings
in thesepatients deserves comment.
Atrophy*
Although first described in 1909,2 the
Slowly progressive syndrome was rarely reported until
Not stepwise recent years. While still considered
Initial symptoms visual
rare, reviews by Hécaen and Albert3
Visual agnosia, constructional disturbance, environmental agnosia and Damasio4 document a number of
Transcortical sensory aphasia (alexia, agraphia, anomia, and
decreased comprehension but normal repetition) examples and present pertinent theo¬
Gerstmann's syndrome (acalculia, agraphia, R-L disorientation, and ries concerning the underlying mech¬
finger agnosia) anism. Damasio4 stressed obligatory
Balint's syndrome (ocular dysmetria, sticky fixation, and bilateral parieto-occipital abnormali¬
simultanagnosia)
Decreased verbal learning (late) ty, usually with accompanying inferi¬
Normal primary visual, motor, and sensory systems or quadrantanopia, and suggested two
Retention of insight, appropriate affect cortical areas of greatest importance:
EEG: diffuse slowing in mid to late stages _

(1) Brodmann area 7 of the parietal

WAIS: PIQ 25-30 points lower than VIQ cortex appeared essential for direct¬
CT: posterior > anterior atrophy
MRI scan: posterior > anterior atrophy ing gaze to novel stimuli; (2) the supe¬
rior occipital cortex was thought nec¬
*EEG indicates electroencephalogram; WAIS, Wechsler Adult Intelligence Scale; CT, computed tomogra¬
phy; PIQ, performance IQ; MRI, magnetic resonance imaging; and VIQ, verbal IQ. essary to structure the full visual
field. Hécaen and Albert3 agree with
the observation of a mandatory bilat¬
eral, parieto-occipital pathologic con¬
Table 3.—Differential Diagnosis of Cortical Dementia dition but note the presence of frontal
Posterior
cortex or occipital-frontal pathway
Alzheimer's Pick's lesions in some cases. It has been
Features Disease Disease Cortical Atrophy
Amnesia Early Late Late
posited that a combined parieto-occip-
itofrontal dysfunction underlies the
Constructional disturbance Early Late Early full syndrome.5 All of the neuroana-
Environmental agnosia Early Early tomical areas said to be associated
Acalculia Early Late Early with Balint's syndrome are either
Anomia Early Early Early
association cortex or cortical-cortical
Alexia Moderately late Moderately late Early
Agraphia Moderately late Moderately late Early
connections, not primary visual cortex
or its efferent connections, a feature
Gerstmann's syndrome Moderately late Late Relatively early consistent with the lack of visual field
Balint's syndrome Absent Absent Present
defect in our cases. The three compo¬
Klüver-Bucy syndrome Absent or late Early Absent
nents essential for the diagnosis-
Loss of insight Early Early Late
Behavioral response Unconcerned Disinhibited Appropriately concerned
sticky fixation (also called optic
apraxia, oculomotor apraxia, ocular
apraxia, psychic paralysis of gaze, or
spasm of fixation), ocular dysmetria
visual, and somatosensory evoked re¬ for this dramatic disorder.1 Four (optic ataxia or misreaching), and
sponses were normal. An EEG showed patients developed a full Balint's syn¬ simultanagnosia (visual disorienta¬
intermittent (4- to 6-Hz) slowing superim¬ drome (sticky fixation, ocular dysme¬
posed on a 6- to 8-Hz background.
tion)—were fully present in four cases
tria, and simultanagnosia) and the and only partially present in one
COMMENT fifth showed optic ataxia and ocular (naming and pointing were too severe¬
dysmetria. All eventually developed ly disordered to prove simultanagno¬
Each of the patients showed a pro¬ all four components of Gerstmann's sia). The syndrome was incomplete in
gressive mental deterioration without syndrome (finger agnosia, right-left the early stages (cases 2, 3, and 5) but
evidence of basic motor or sensory disorientation, agraphia, and acalcu¬ developed into the complete clinical
dysfunction. The earliest clinical find¬ lia) and also developed the findings of picture over a course of several years.
ings (eg, alexia or visual agnosia) transcortical sensory aphasia (anom¬ When features of Balint's syndrome
implied visual-association malfunc¬ ia, comprehension disorder, alexia are present, a bilateral parieto-occipi¬
tion. Despite the early presence of and agraphia, and good repetition). As tal pathological condition is implied;
visual disturbances that progressed to an early finding, all had severe the progression into a full syndrome
severe disability, visual acuity re¬ impairment of the ability to copy may indicate additional involvement
mained intact and the only visual- drawings. Memory function was of frontal connections.
field discrepancies were extinction to abnormal but only to a limited degree Computed tomography and MRI
double-simultaneous stimulation and until late in the course. Each could revealed a degenerative process that
hemineglect noted late in the course present his or her own history, was appeared more prominent in the pos¬
of the illness, long after severe visual aware of current events, and showed terior cerebral hemispheres in three
disability was evident. Two showed considerable insight into his or her of the patients. There was no evidence
right- and two left-sided extinction predicament. While use of specific of vascular, neoplastic, or inflamma¬
and in none was it a consistent find¬ names was difficult, true abnormality tory disease. The EEGs of all patients
ing. An early complaint of each in memory function was mild until showed diffuse theta activity without
patient was a tendency to get lost in late in the course. No patient devel¬ focal slowing or epileptiform dis¬
familiar areas, such as an inability to oped a primary motor, auditory, or charge, patterns that resemble those
find their way in their neighborhood somatesthetic disturbance. Table 1 reported in the midstages of both
or difficulty finding their way from outlines the more important clinical Pick's and Alzheimer's disease.6·7 The
room to room in their own home. findings of the cases presented. neuropsychological test results of all
Environmental agnosia is a term used The presence of Balint's syndrome patients who could be tested showed a

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 marked discrepancy between PIQ and
VIQ scales, probably reflecting the
sensitivity of the PIQ subtests to
visual dysfunction.
The findings in these patients fit
current definitions of dementia.8·9 The
constellation of findings suggests dis¬
turbance of association cortex or con¬
nections, with sparing of the primary
sensory and motor pathways. The ear¬
ly clinical picture suggests that the
disorder initially involved occipital
and parietal association cortices, par¬
ticularly Brodmann areas 18 and 19.
Later findings suggest anterior
spread to involve the angular gyrus or
its connections bilaterally. Table 2
lists the major clinical features noted
in these patients.
The possibility that the visual
symptoms presented by these patients
could be the result of a disconnection
disorder deserves consideration. Most
reports of alexia,10 visual agnosia,11
prosopagnosia,4 environmental agno¬
sia,1 and Balint's syndrome2'4 describe
structural defects that effectively dis¬
connect the intact primary visual cor¬
tical areas from various association
cortices. Numerous brain-imaging
studies in these patients revealed no
such structural defects. Demyeiin¬
ating disorders, such as multiple scle¬
rosis or Schilder's disease, can
produce visual symptoms; again, MRI
scans in these patients revealed no
evidence of demyelination. On the
basis of these observations, degenera¬
tion primarily affecting posterior
association cortex appears most prob¬
able.
In the proposed division between
cortical and subcortical dementia syn¬
dromes,9 this disorder presents with
predominantly higher-function ab¬
normalities that appear to originate
almost exclusively from the posterior
association cortex. A descriptive term,
1. Landis T, Cummings JL, Benson DF, et al:
Loss of topographic familiarity: An encironmen-
tal agnosia. Arch Neurol 1986;43:132-136.
2. Balint R: Die seelenlahmung des 'Schauens.'
Monatschr Psychiatr Neurol 1909;1:25-51.
3. H\l=e'\caenH, Albert ML: Human Neuropsy-
chology. New York, John Wiley & Sons Inc,
1978.
4. Damasio A: Disorders of complex visual
processing: Agnosias, achromatopsia, Balint's
syndrome and related difficulties of orientation
and construction, in Mesulam M-M (ed): Princi-
ples of Behavioral Neurology. Philadelphia, FA
Davis Co Publishers, 1985, pp 259-288.
5. H\l=e'\caenH, de Ajuriaguerra J, David M, et al:
Paralysie psychique du regard de Balint au cours
de l'evolution d'une leuco-encephalite type Balo.
Rev Neurol 1950;83:81-104.
6. Gordon EB, Sim M: The EEG in presenile
dementia. J Neurol Neurosurg Psychiatry 1967;
30:285-291.
7. Harner RN: EEG evaluation in the patient
with dementia, in Benson DF, Blumer D (eds):
Psychiatric Aspects of Neurologic Disease. New
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posterior cortical atrophy, is suggest¬
ed to identify the disorder until more
definitive causative data become
available.
Several causative considerations
can be suggested. In that the disorder
begins as a progressive derangement
of the association cortex, it resembles
Alzheimer's disease. Differences from
the typical clinical features of Alz¬
heimer's disease12 are obvious, howev¬
er. Memory and personal insight are
relatively well retained in these
patients until late in the course, while
visual agnosia, alexia, and acalculia
are severe at early stages. Even if the
neuropathologic features of Alzheim¬
er's disease are eventually demon¬
strated, the early clinical findings are
strikingly atypical. Nonetheless, Alz¬
heimer's disease is said to show con¬
siderable variation in the initial pre¬
sentation and these cases may well
represent an atypical Alzheimer dis¬
ease clinical pattern.13
A degenerative disorder with lobar
predilection, such as Pick's disease,
offers a tempting alternative origin.
Pick's disease has long been described
as primarily affecting anterior struc¬
tures, particularly frontal and anteri¬
or temporal cortex.9·1415 The patients
in this report also appear to show a
lobar dysfunction, but one that pri¬
marily affects occipital and posterior
parietal cortex. Could these cases rep¬
resent a posteriorly localized variant
of Pick's disease? The answer must
await neuropathologic studies.
The recent reports of progressive
aphasia1618 deserve note. These cases
are also awaiting histological verifica¬
tion but are suspected to represent
variations in the onset of traditional
dementing disorders. Alterations in
the early clinical picture of degenera¬
tive dementia are well recognized,
apparently dependent on the site of
References
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the initial cerebral involvement.19 The
syndrome described here may well
represent an aberrant onset of a more
common disorder. Table 3 lists clinical
findings that appear to distinguish
the syndrome reported here from the
two currently recognized types of cor¬
tical dementia.
Among other causative possibilities
considered in these cases, focal vascu¬
lar disease deserves strong consider¬
ation. Most reported cases of Balint's
syndrome have been based on multi¬
ple vascular infarcts and the promi¬
nence of transcortical sensory apha¬
sia, and the Gerstmann syndrome in
these cases suggests a posterior bor¬
der-zone abnormality. However, no
evidence of a cerebrovascular disorder
was uncovered by CT, MRI, or angio¬
graphie studies.
One remote causative possibility
was suggested by the history that two
of these patients had received exten¬
sive (seven to eight years) tetracycline
therapy and a third had bronchial
problems treated with a variety of
antibiotics over a period of years. The
other two did not receive such drugs,
however, and, at most, any relation¬
ship between antibiotic therapy and
this syndrome must be considered
tenuous.
The possibility that each of these
individuals suffers from the same dis¬
order appears high. The findings and
course, particularly the presence of a
combination of rare clinical disorders
such as Balint's, Gerstmann's, and
transcortical sensory aphasia syn¬
dromes, appear unique. These cases
are sufficiently similar to each other
and the findings are sufficiently dif¬
ferent from those typical for either
Alzheimer's or Pick's disease to war¬
rant classing them separately until
definitive pathologic information be¬
comes available.
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