Clinical Neurology and Neurosurgery 192 (2020) 105747
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Clinical Neurology and Neurosurgery
journal homepage: www.elsevier.com/locate/clineuro
Case Report
‘I see colors when I touch them’. Color agnosia with visuo-tactile facilitation
in a patient with posterior cortical atrophy
Andrea Zangrandia,*, Alessandro Miolia, Emma Falatoa,b, Federico Ranieria,b, Giovanni Di Pinoa,
Vincenzo Di Lazzarob
a
NeXT: Neurophysiology and Neuroengineering of Human-Technology Interaction Research Unit, Università Campus Bio-Medico, Roma, Italy
b
Research Unit of Neurology, Neurophysiology and Neurobiology, Università Campus Bio-Medico, Roma, Italy
1. Introduction
Posterior cortical atrophy (PCA) is a neurodegenerative syndrome
characterized by a progressive impairment of visuospatial and praxis
skills, in the absence of impaired visual acuity or ocular causes. Patients
manifest a series of symptoms including simultanagnosia, alexia/agra-
phia, dyscalculia, ideomotor apraxia, achromatopsia, environmental
disorientation, visual and color agnosia. Neuroimaging usually shows
evidence of atrophy in the occipitoparietal or occipitotemporal areas,
and/or decreased metabolism on positron emission tomography (PET)
[1]. PCA is associated with different pathologies of which Alzheimer’s
disease is the most common. As part of the diagnostic algorithm, a
formal neuropsychological assessment is mandatory to evaluate the
involvement of each cognitive domains and to confirm that the disorder
is relatively restricted to posterior regions [1].
2. Case report
We describe the case of a 69-year-old left-handed male, former
butcher, with five years of formal education. He had a family history of
dementia (mother), and his prior medical history was noteworthy for
low vision of the right eye due to unspecified trauma in childhood. He
had undergone a first neuropsychological assessment at the age of 63,
due to subjective memory complaints and spatial disorientation started
5 years before. Scores were pathological on tests for verbal memory
(short story delayed recall, word list with interference) and executive
functions (TMT-A, TMT-B, phonemic fluency). Most visuospatial tests
were impaired: he was unable to draw figures, to copy figures, and he
failed the clock drawing test. Both writing and reading skills were af-
fected (agraphia and alexia). Instead, tests for auditory-verbal com-
prehension (Token test), cognitive estimation (CET) and short-term
memory (digit span) were within the normal range. At that stage, there
were no signs of ideative or ideomotor apraxia. Despite his cognitive
profile, his behaviour was normal, without personality changes. Head
CT scan was normal.
During the following years, a PCA was suspected due to the
⁎
Corresponding author at: Via Álvaro del Portillo, 200, 00128, Roma, Italy.
E-mail address: a.zangrandi@unicampus.it (A. Zangrandi).
https://doi.org/10.1016/j.clineuro.2020.105747
Received 23 July 2019; Received in revised form 10 February 2020; Accepted 23 February 2020
Available online 24 February 2020
0303-8467/ © 2020 Elsevier B.V. All rights reserved.
T
pronounced visuospatial and visuoperceptual deficits with relative
sparing of other cognitive functions. Consistently, FDG-PET at the age
of 66 showed bilateral occipital and temporal hypometabolism (Fig. 1).
When he came to our attention, at age 69, the patient was unable to
ambulate without aid in the outpatient clinic room and even to sit on a
chair autonomously. Neurological examination showed impaired visual
fixation and difficulties in simple orders execution. In the attempt to
reach objects, he missed the target most of the times, and he behaved as
if he relied only on touch to catch objects. MMSE score had worsened
from 24/30 at age 64 to 9/30. The patient was not oriented in time and
space (he could not recall the name of the hospital and the city) and he
was not autonomous in activities of daily living. Brain amyloid PET
scan was negative. Brain MRI showed diffuse cortical atrophy.
A detailed neuropsychological evaluation showed a severe multi-
modal cognitive impairment. Speech was fluent but poor in content,
verbal comprehension was good, and he sporadically used paraphasias.
Short-term verbal memory (digit span) and some language domains
(grammatical comprehension and spontaneous production) were pre-
served with respect to the remaining cognitive functions. His mood
appeared good throughout the assessment.
Notably, a verbal memory test for object color was impaired (18/
30): the pipe was defined "light yellow", the banana "gray", the crocodile
"of many colors … gray … yellow", the traffic light "of three colors …
white, gray and red”. The patient had no previous history of color
blindness.
Furthermore, object naming from description was impaired (32/
48). Patient’s performance was worse when he was provided with se-
mantic cues based on the color (8 errors out of 12 items), e.g. “it is a
stone fruit, it grows in couples, it is red” (correct answer: cherry; patient
answer: pineapple) rather than when the description was based on
functional and/or other characteristics (8 errors out of 36 items), e.g.
“it is an animal, it lives in Australia, it has paws and pouch” (correct
answer: kangaroo; patient answer: camel). Patient’s performance in
objects naming improved when he was holding and manipulating them.
In addition, an ad-hoc brief color naming test was administered. In
this test, the patient had to say the color of real (e.g. telephone, pen,
A. Zangrandi, et al.
Fig. 1. 18F-FDG PET at age 69 showing diffuse bilateral occipital and temporal hypometabolism.
etc.) or abstract objects (paper squares of different colors), which were
visually presented. In case of errors, he could touch and manipulate the
object, before trying to answer again. With real objects, the perfor-
mance improved when he was allowed to manipulate the objects: e.g.,
the telephone was defined yellow (wrong answer) when it was presented
visually, and became "grey" (correct answer) when he was allowed to
manipulate it. Surprisingly, the same phenomenon occurred with ab-
stract objects (i.e. with black, green and brown squares)
(Supplementary videoclip).
3. Discussion
We reported the interesting case of a patient with PCA, who had a
severe cognitive impairment with prominent visuospatial and visuo-
perceptual deficits, and a degradation of semantic knowledge of colors.
Patients with PCA have difficulties in objects perception, in space
analysis, and in distinguishing the objects from the background [2]
Patient’s better performance in objects naming when holding and ma-
nipulating them, could be readily explained by the contribution of
tactile information. This may account also for improvement of color
naming for real known objects, in which manipulation facilitates object
recognition and, in turn, known objects have likely known colors.
Surprisingly, the performance in colors discrimination improved even
with manipulation of real objects without a prototypical color (e.g.
telephone) and abstract objects (colored squares), despite object re-
cognition did not increase the chance for that object to be of a parti-
cular color.
Different mechanisms could account for this unexpected finding.
One possible explanation is that by moving the object, the color con-
trast between the object and background increased, thus helping object
color identification. Another possible explanation may be related to an
impaired gaze orientation showed by the patient, who let his gaze
wander as in the attempt to focus the objects visually presented. He
could have taken advantage of his residual tactile and motor abilities to
relocate the object in a more favourable position that matched his gaze
direction. Also, some people can experience color perception by feeling
2
Clinical Neurology and Neurosurgery 192 (2020) 105747
a certain shape in object manipulation (touch-color synesthesia).
However, such phenomenon does not account for our patient's im-
proved performance during visuo-tactile manipulation of abstract ob-
jects, which had identical shape; nonetheless our observation might
subtend a common neurophysiological background with synesthesia
characterized by a cross-talking between the cortical pathways pro-
cessing tactile and visual stimuli.
Indeed, the most fascinating possibility is that haptic objects ex-
ploration facilitated color perception trough a cross-modal information
processing. While the importance of the ventral pathway is largely es-
tablished, it has been suggested that the dorsal stream from V1 to
parietal areas participates in color perception [3]. It may be speculated
that information coming from the dorsal stream could be sufficient to
partially compensate the compromised ventral pathway. This may en-
hance the possibility of object recognition through multisensory facil-
itation mechanisms that are likely mediated by parietal neurons [4].
While PCA patients often exhibit a wide range of manifestations of vi-
sual deficits, presumably there are areas of the occipital cortex that are
relatively preserved. In this way, under somatosensory guidance, the
information about the visual features of the object could have been
more easily accessed, and therefore processed by the functionally
spared cortical regions.
4. Conclusion
For PCA patients, several non-pharmacological interventions have
been proposed [5]. This case report should be taken as an interesting
clinical suggestion to be explored more deeply with further patients or
clinical population. If results will be replicated, new rehabilitative ap-
proaches based on visuo-tactile integration could be attempted.
Acknowledgements
The authors received no financial support for the publication of this
article.
A. Zangrandi, et al.
Appendix A. Supplementary data
Supplementary material related to this article can be found, in the
online version, at doi: https://doi.org/10.1016/j.clineuro.2020.105747.
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