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The Neural Basis of Cognition

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A case of posterior cortical atrophy with vertical

neglect

Hilary Glazer, Leila Saadatpour, Leilani Doty & Kenneth M. Heilman

To cite this article: Hilary Glazer, Leila Saadatpour, Leilani Doty & Kenneth M. Heilman
(2017): A case of posterior cortical atrophy with vertical neglect, Neurocase, DOI:
10.1080/13554794.2017.1312692

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Published online: 07 Apr 2017.

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NEUROCASE, 2017
http://dx.doi.org/10.1080/13554794.2017.1312692

A case of posterior cortical atrophy with vertical neglect

Hilary Glazera, Leila Saadatpourb,c, Leilani Dotyb,c,d and Kenneth M. Heilmanb,c,d,e
a
Comprehensive Center for Brain Health and Memory, Memorial Neuroscience Institute, Hollywood, FL, USA; bDepartment of Neurology and Center for
Neuropsychological Studies, University of Florida, Gainesville, Florida, USA; cFlorida Department of Elder Affairs, Alzheimer’s Disease Initiative, University of
Florida Cognitive and Memory Disorder Clinics, Gainesville, Florida, USA; dDepartment of Elder Affairs, National Institutes of Health/National Institute of
Aging 1Florida, Alzheimer’s Disease Research Center, Gainesville, Florida, USA; eDepartment of Neurology, Malcom Randall Veterans Affairs Medical Center,
Gainesville, FL, USA

ABSTRACT ARTICLE HISTORY

Posterior cortical atrophy (PCA) is a syndrome caused by a neurodegenerative disease that often presents Received 16 May 2016
with visuospatial deficits, and can be debilitating. PCA is often characterized by elements of Balint’s Accepted 24 March 2017
syndrome and dyslexia. The most common underlying pathology has been found to be Alzheimer’s disease. KEYWORDS
Signs of horizontal neglect are frequently associated with PCA, but the presence of vertical (or altitudinal) Vertical neglect; posterior
neglect has not yet been reported in a patient with PCA or other forms of neurodegenerative dementia. In cortical atrophy; Alzheimer’s
this paper, we present a patient with PCA who on vertical line bisection and cancellation tests revealed disease; neurodegenerative;
upper altitudinal spatial neglect. Detection of this abnormality may have important implications for Balint’s syndrome
diagnosis, therapeutic interventions, and patient safety.

Introduction Case report

Vertical neglect has been described in stroke patients (Rapcsak,
Cimino, & Heilman, 1988; Shelton, Bowers, & Heilman, 1990) and
Parkinson’s disease patients (Lee, Harris, Atkinson, Nithi, & Fowler,
2002; Nys, Santens, & Vingerhoets, 2010). Neglect for the lower part
of visual space is often associated with parietal lobe lesions,
whereas neglect for upper visual space may be associated with
ventral occipitotemporal lesions (Rapcsak et al., 1988; Shelton et al.,
1990). In addition, limited upward gaze and a downward visuo-
motor bias have been hypothesized to be due, at least in part, to
upward vertical neglect in patients with Parkinson’s disease (Lee
et al., 2002; Nys et al., 2010). The presence of vertical neglect may
have important functional implications for patients with this
disorder.
Posterior cortical atrophy (PCA) is a visuospatial disorder
resulting from a neurodegenerative disease. The most common
underlying pathology is that of Alzheimer’s disease. PCA patients
are often severely debilitated and initially they may be more
impaired than patients in the early stages of typical Alzheimer’s
disease (Shakespeare, Yong, Foxe, Hodges, & Crutch, 2015). Their
disability may be caused by more severe visuoperceptual deficits
than those with typical Alzheimer’s disease. Though there is no
established therapy for PCA, detection of these deficits and
discussion of caregiver strategies to modify the home environ-
ment may significantly improve the ability of these patients to
function independently.
We describe a patient with PCA with signs of Balint’s syn-
drome, visual apperceptive object agnosia, and prosopagnosia
who was found to neglect the upper parts of visual space on
both line bisection and object cancellation tasks. To our
knowledge, this is the first report of a patient with a neurode-
generative dementia who demonstrated vertical neglect.
The patient was a 70-year-old right-handed man with an 8-
year history of progressive visuospatial problems. He could not
drive because he would stop too often due to thinking that
the car in front of him or a stop sign was too close. He
stopped playing cards and dominoes because of trouble see-
ing them. He had increasing difficulty recognizing familiar
people and had a decreased capacity to read. He also had to
be reminded about his daily schedule, and he could not take
care of his bills.
On his neurological exam, he was alert and in no distress.
On mental status testing, he had fluent speech with occasional
neologisms, hypophonia, and palilalia. Repetition was
impaired, and although he could comprehend one- and two-
step commands, he could not comprehend three-step com-
mands. Though he reported that he could not read, he could
read simple sentences and could write. He could not accu-
rately reach for a pen (optic ataxia) and could not accurately
saccade to the examiner’s finger (oculomotor apraxia). When
shown complex scenes such as pictures of a picnic or a school
and asked to name the entire picture with one word, he could
not and instead replied with elements of the picture, such as
“kids” and “family.” He had prosopagnosia, with an impaired
ability to recognize American presidents portrayed in photo-
graphs of their faces. He had no color anomia. He had no
right–left confusion, finger anomia, or acalculia. His horizontal
line bisection showed left pseudo-neglect (Bowers & Heilman,
1980). His vertical line bisection showed upward deviation on
three attempts. Previous studies on vertical line bisection
pseudo-neglect showed that normal people tend to have
upward vertical deviation, with the mean of 5.88 mm
(SD = 3.89 mm) up from the middle of the line. Our patient

CONTACT Hilary Glazer hglazer@mhs.net

© 2017 Informa UK Limited, trading as Taylor & Francis Group
2 H. GLAZER ET AL.
showed a mean downward deviation of 7.5 mm, which was 2
SDs from normal (Bowers & Heilman, 1980) (Figure 1). He also
demonstrated upward altitudinal neglect on a cancellation
task; when given a pen and asked to mark open squares on
a paper containing both open and closed squares, he did not
mark squares on the upper half of the paper (Figure 2). He had
no ideomotor apraxia.
He received a score of 21/30 on Mini-Mental Status Exam with
impairments of time orientation, spelling WORLD backwards,
recall of three words, and copying intersecting pentagons
(Folstein, Folstein, & McHugh, 1975) (Table 1). His auditory atten-
tion was intact, as he could repeat six digits forward. However, his
Figure 1. The patient’s performance on a vertical line bisection task illustrating
downward deviation and upward vertical neglect.
working memory was impaired, as he could repeat only two digits
backward. On the Montreal Cognitive Assessment, he was
impaired on performing TRAILS-B, copying a cube, and drawing
a clock (Figure 3). His “A” tapping was normal (Nasreddine et al.,
2005). On the Hopkins Verbal Learning Test, he learned a total of
13 words, recalled zero after a delay and his discrimination index
was 2 (two true positive and no false positive) (Brandt, 1991;
Vanderploeg et al., 2000). His performance on a phonemic fluency
test was normal, producing 15 words that began with the letter F
in 1 min; however, his category fluency was impaired. He named
only six animals in 1 min. On the Boston Naming Test, he only
named 39/60 objects correctly with evidence of a visual apper-
ceptive agnosia, as he could not recognize several of the drawings
by describing their use, their semantic category, or where they
were found (Kaplan, Goodglass, & Weintraub, 1983; Lansing, Ivnik,
Cullum, & Randolph, 1999). He was also unable to accurately copy
drawings. We asked him to fill in the blanks in an orally adminis-
tered Cinderella story). He was unable to verbally fill in the blanks
for key nouns and verbs in the story.
His general neurologic examination was normal.
Extraocular movements were intact including upgaze and
downgaze. He did not have any of the motor signs associated
with parkinsonism.
His laboratory investigation for reversible causes of cogni-
tive impairment due to abnormal serum levels, such as for
B12, folate, vitamin D, and TSH, was normal. His brain MRI
showed mild bilateral temporal lobe atrophy (Figure 4). It was
difficult to assess if there was atrophy of the occipital lobe. No
significant atrophy was present elsewhere.
Discussion
This patient has some of the features of Alzheimer’s disease,
including subjective memory complaints, impaired episodic
memory, and temporal lobe atrophy. Although, patients with
classic Alzheimer’s disease can also demonstrate visuospatial
impairments and elements of Balint’s syndrome (Pisella,
Rossetti, & Rode, 2016), this patient’s primary complaints cen-
tered around visuospatial disturbances and the degree of
visuospatial impairments including visual apperceptive agno-
sia, prosopagnosia, and upward visual neglect, suggestive of
more significant impairment of visual association area than
would be expected for classic Alzheimer’s disease. Though
there are various diagnostic criteria for PCA, we think that
this diagnosis best captures his overall clinical and functional
picture. Unfortunately, a more definitive diagnosis can only be
confirmed by neuropathological examination.
PCA (or Benson’s syndrome) is a progressive neurodegenera-
tive disorder characterized by features of Balint’s syndrome,
Gerstmann’s syndrome, dyslexia, agraphia, and visual agnosia,
with temporal-parietal-occipital atrophy (Benson, Davis, &
Snyder, 1988).
Though our patient did not have all of these features, he did
have the signs of Balint’s syndrome, visual apperceptive agno-
sia, constructional dyspraxia, and prosopagnosia with less pro-
minent memory deficits. Using a variable set of diagnostic
criteria for PCA (Crutch et al., 2012; McMonagle, Deering,
Berliner, & Kertesz, 2006; Renner et al., 2004; Tang-Wai et al.,
2004; Victoroff, Ross, Benson, Verity, & Vinters, 1994), several
 NEUROCASE 3

Figure 2. The patient’s performance on an object cancellation task. When asked to mark all of the open boxes, he only made marks on the open boxes on the lower
portion of the paper. He did not mark the open boxes on the upward portion of the paper, illustrating upward vertical neglect for focal objects.

researchers have reported that most frequently on post-mor-
tem examination, patients with PCA show the neuropathologi-
cal changes associated with Alzheimer’s disease; however,
tauopathies, subcortical gliosis, prion disease, and synucleino-
pathies have also been found to be associated with PCA. Since
our patient is still living, we do not have neuropathology and
therefore cannot confirm the form of pathology associated with
his neurodegenerative disorder.
The presence of vertical (or altitudinal) neglect suggests
that the human brain has attentional networks that can direct
attention to the vertical dimensions of extrapersonal space
(Rapcsak et al., 1988). Rapcsak et al. (1988) described the first
case of vertical neglect. Their patient had inattention to the
lower half of space due to bilateral parieto-occipital infarc-
tions. Based on this patient’s findings, these investigators
posited that the parietal lobes mediate the attention that is
directed toward lower vertical space. This hypothesis was
consistent with a prior report revealing that the receptive
field for most of the neurons in the parietal lobes is primarily
in the lower portions of the visual field (Robinson, Goldberg, &
Stanton, 1978). Subsequently, a case of upward vertical
neglect was described in a patient with bilateral inferior tem-
poral lobe infarcts with no involvement of the parietal lobes.
This observation led to the hypothesis that the inferior tem-
poral visual association cortex was responsible for mediating
attention toward the upper portions of visual space (Shelton
et al., 1990).
Our patient also had prosopagnosia and visual apperceptive
agnosia, providing further localizing value. Progressive prosopag-
nosia has been associated with right temporal lobe atrophy
(Evans, Heggs, Antoun, & Hodges, 1995), and functional imaging
studies have revealed that facial recognition is associated with
4 H. GLAZER ET AL.

Table 1. Neuropsychological assessments with raw scores, standardized norms, patient’s prosopagnosia and visual agnosia suggest that in him,
Z-scores, and percentiles.
the functions of his ventral temporal and occipital lobes may have
Norms also been disrupted. Though a definition naming task was not
Raw score from Z-
of patient; controls: score administered, his inability to name key nouns and verbs in the
number of mean of Percentile of Cinderella story suggests a possible deficit in semantic knowledge
Test test items ± SD patient patienta which may also involve functions of the temporal lobe (Lee et al.,
Mini-Mental 21/30 28.2 ± 2 −3.60 0.0159% 2002; Nys et al., 2010). Though these authors hypothesized that
Status Exam (70–74y.o.; ≥13 years
(Folstein et al., education). vertical neglect may be due to dopamine depletion in the right
1975; Bravo & side of the brain, we would like to propose that vertical neglect
Hebert,1997) may be better conceptualized as a localizing sign for an anatomic
Montreal 16/30 23.7 ± 4.1 −1.88 3.0054% = 3.01% or functional lesion of the ventral temporal–occipital lobes.
Cognitive Whereas horizontal neglect has been described in patients
Assessment
(Nasreddine
with Alzheimer’s disease and PCA (Renner et al., 2004; Tang-
et al., 2005) Wai et al., 2004), vertical neglect has not been described
Hopkins Verbal 3 4.8 ± 1.7 −1.059 14.48% previously to our knowledge in the setting of a neurodegen-
Learning Test,
Form 1 (Brandt,
erative dementia. The presence of vertical neglect may not
1991; only provide information about the localization of the neuro-
Vanderploeg nal dysfunction in patients with a neurodegenerative disease,
et al., 2000)
5 7.4 ± 2.1 −1.133 12.86%
but also the detection of the presence of vertical neglect may
Trial 1 5 8.4 ± 2.2 −1.545 6.117% = 6.12% have important functional implications. Unawareness or
Trial 2 13 20.6 ± 5.2 −2.714 0.33% impaired allocation of attention to a portion of space may
Trial 3 0 7.8 ± 2.7 −2.889 0.19%
Sum trials 1–3 2 11.3 ± 1.0 −9.30 0%
cause serious disabilities with impairments in the performance
Delayed recall 1 1.6 ± 1.5 −0.40 34.46% of activities of daily living, and neglect may even be respon-
recognition: 1 9.7 ± 1.9 −4.58 0.0002% sible for serious injuries. Though there are no known medica-
a. True positives
b. False positives
tions or therapies that have been shown to treat vertical
c. Discrimination neglect, there are some rehabilitation strategies used to treat
index horizontal neglect, such as prism adaptation (Rossetti et al.,
Boston Naming Test 21/60 52.4 ± 6.3 −4.98 0%
(Kaplan et al., (60-item
1998) and optokinetic stimulation (Karnath, 1996) that should
1983; Lansing long be tested in the treatment of vertical neglect.
et al., 1999) form) Patients and their caregivers should be informed about the
Applied “Z-Score to Percentile Calculator” from www.measuringU.com/pcalcz.
a
existence of vertical neglect and the best means by which
php (accessed 9/27/16). they can perform their activities of daily living, such as groom-
ing, bathing, and dressing, as well as performing household
chores. Misreaching for objects may result not only from optic
activity of the right ventral occipital-temporal networks (Rossion ataxia but also from vertical neglect and the placement (up or
et al., 2003). Visual object agnosia is most often caused by lesions down) of objects placed within their visual field. Most impor-
of the ventral posterior temporal and occipital lobes (Barton, tantly, vertical neglect can influence a patient’s safety, and
2014). Although some authors have proposed that PCA tends to caregivers must be informed about making these patients’
disrupt the dorsal visual streams (McMonagle et al., 2006), this environments safe. Depending on the presence of upward or

Figure 3. The patient’s cube copy and clock drawing on the Montreal Cognitive Assessment.

Figure 4. His MRI showing mild bilateral temporal lobe atrophy with left temporal atrophy worse than right.
downward vertical neglect, patient safety may be hindered
when climbing stairs, walking under doorways, near hanging
plants or fans, or obstacles on the floor. Based on our empiri-
cal data, we suggest that several strategies be employed, such
as environmental modification to accommodate these deficits,
as well as extra looking from side-to-side or up-and–down,
listening carefully for approaching people or pets, taking extra
time to move in a setting, and wearing good-fitting shoes with
flat soles and firm backing.
Finally, patients and caregivers cannot be informed about
safety and management, as well as possible rehabilitation, if
vertical neglect is not diagnosed. Most neuropsychological bat-
teries used to assess patients for neurobehavioral disorders do
not test patients for vertical neglect. Thus it is possible that due
to a lack of testing for vertical neglect in patients with neurode-
generative diseases, the prevalence of this disorder may be
underestimated. Given the implications for clinical localization
and for patient care, we propose that vertical line bisection and
cancellation tasks be given to patients being evaluated for a
neurodegenerative disease.
Acknowledgments
None
Disclosure statement
No potential conflict of interest was reported by the authors.
Funding
This work was partially supported by the Florida Department of Elder
Affairs, Alzheimer’s Disease Initiative, the National Institutes of Health/
National Institute of Aging 1Florida ADRC (Alzheimer’s Disease Research
Center), and the Research Service of the Malcom Randall Veterans Affairs
Medical Center, Gainesville, FL, USA.
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