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Visual perception. In: Wet mind. New
Boone, PhD; F. Prince, PhD;
M. Ponton, PhD; and C. Cotman, PhD
describe the clinical, neuropsychological, and imaging features of five patients with
frontotemporal dementia (FTD) who acquired new artistic skills in the setting of dementia.
Creativity in the
setting of dementia has recently been reported. We describe five patients who became visual artists in the setting of FTD.
Sixty-nine FTD patients were interviewed regarding visual abilities. Five became artists in the early stages of
FTD. Their history, artistic process, neuropsychology, and anatomy are described.
On SPECT or pathology, four of
the five patients had the temporal variant of FTD in which anterior temporal lobes are involved but the dorsolateral
frontal cortex is spared. Visual
were spared but language and social skills were devastated.
function in the anterior temporal lobes may lead to the “facilitation” of artistic skills. Patients with the temporal lobe
variant of FTD offer a window into creativity.
Previously learned skills or talents can be conserved
in the setting of dementia, and demented individuals
have been described who maintained card-playing
skills,l musical skills,’ painting
game skills.’ Even more remarkable
man who became an artist in the setting of dementia
and whose paintings steadily improved despite pro-
gressive cognitive decline.8 The anatomic substrate
these unique patients is unknown, although
dence is emerging that indicates that enhancement
musical abilities is more common with
newly characterized subtypes of frontotemporal de-
of the presenile
dementias,‘j and has anatomic and chemical deficits
that differ from those seen with
and other de-
mentia~.~ The clinical manifestations
FTD are di-
verse in part because the regional distribution of
heterogeneous. Many patients show bi-
lateral frontotemporal degeneration6a9J0; but, in
asymmetric and it affects the
or the right side.15 The temporal lobe variant
subtype in which anterior temporal and
basal frontal lobes atrophy slowly while dorsolateral
frontal areas remain inta~t.~ We describe the clinical,
neuropsychological, and imaging features of five
FTD patients who acquired new artistic skills in the
setting of dementia.
From the Departments of Neurology
Miller and Cummings), Radiology
Mishkin), and Psychiatry (Drs. Boone and Ponton), University
Los Angeles School
Medicine; and the Department of Psychobiology
Prince is in private practice.
Supported by the University
Angeles Alzheimer Disease Center
the Christine Risse Award, and the Sidell-Kagan Research
Foundation through the University
Angeles Medical School.
Accepted in final form June
Address correspondence and reprint requests to
Bruce Miller, Professor of Neurology UCSFMt. Zion Hospital,
by the American Academy
This painting of horses was made at a time
when the patient was disinhibited. She was approximately
This church was painted by the artist
He remembered the building from his childhood.
was described previo~sly.~,~
age, a left-handed man with a
tenth-grade education and no prior interest in art took a
short course in drawing at a local park and began to paint.
Soon afterward he was dismissed from a job changing car
stereos and began to have trouble speaking. By age
was disinhibited and irritable. He developed a compulsion
to find coins and searched
money in the street. His first
drawings were simple still lifes of vases and bridges. His
precision improved and he began painting pictures of Indi-
ans and buildings recalled from his youth (figure
final paintings were churches and haciendas remembered
from childhood. In the year before evaluation, after
of intense artistic activity, he stopped painting. At
years he was pleasant but smiled inappropriately. His
Mini-Mental State Examination
He was oriented to the date, and copied the intersect-
ing pentagons correctly. He was unable
name any words
on the Boston Naming Test. He generated seven novel
designs (novel designs touching four clots in five-dot
minute) and two
ing proper nouns produced in
minute). He showed multi-
ple perseverations on the Wisconsin Card Sorting and
Stroop tasks, and his modified Rey-Osterrieth Complex
Figure Copy results were normal. Bulbar weakness, dysar-
thria, hypophonia, and a weak gag reflex were present.
Muscle fasciculations and wasting of the hand muscles
were noted. SPECT showed bitemporal hypoperfusion (left
more than right) and mild left frontal hypoperfusion.
with motor neuron disease was diagnosed.
age, a left-handed housewife
without previous artistic training took art classes. She be-
came uncomfortable coping with social engagements and
withdrew socially. At age
she completed her first paint-
ings, which depicted rivers, ponds, and rural scenes re-
called from childhood (figure
age, she produced realistic copies
paintings. At age
she gained weight, became incontinent, and spoke in
disinhibited fashion. Brown and yellow became dominant
colors in her paintings (figure
Her speech became repet-
itive and rambling. By age
she was expelled from her
art class due to wandering, and she stopped painting. Her
last paintings were slightly distorted family portraits. At
she died of advanced dementia. Autopsy showed
This rural scene was painted by the artist
years after she started taking art classes.
She was approximately
This is a photograph taken by the patient
Guatemala. It represents work early in the course of his
gliosis and microvacuolization of the cortex, most severe in
the right temporal lobe. Both inferior temporal
severely atrophic. There was mild frontal lobe atrophy and
little posterior atrophy.
plaques or tangles were found.
FTD was diagnosed.
57-year-old, right-handed man developed
a successful advertising agency between the ages of 29 to
35 years. At age 35 he decided to write a novel and quit
significant writing was produced, and he was
supported by his wife’s income. Between the ages of
he left his family
trips to remote areas of Central
America, where he photographed buildings and people.
The locations were dangerous; on one occasion he was ar-
rested by the military. Pictures were acquired in a compul-
sive manner, and he photographed them from multiple
achieve the “perfect” image (figure
44 and 48 years of age this patient carved wax miniature
animals using candle drippings. He photographed until the
At 53 he had several traffic accidents, started
cheating at card games, stole, berated his mother-in-law,
and masturbated in public.
age 54 he was fidgety and
stared inappropriately at strangers. His MMSE score was
Comprehension, fluency, naming, and constructions
were normal. His executive abilities were impaired, as ev-
idenced by his inability to perform the Wisconsin Card
Sorting, Stroop, or Trails tasks. His basic neurologic exam-
ination was normal. SPECT showed bifrontal hypoperfu-
sion (right worse than left) with mild right temporal
age 56 he was placed in a nursing facility.
Fourteen years earlier, a 59-year-old woman
divorced her husband and remarried. Always interested in
art, she quit her job as a business manager and started a
handicraft business with two friends. She sold gourds with
Santa Claus and sculpted small wooded
scenes. Her work steadily improved for
years and then
age 53 she stopped working and became apa-
thetic and inactive. She complained of fatigue and with-
drew socially. She showed mild disinhibition, stopped
flushing the toilet, and lost interest in her hygiene. She
gained approximately 27 kg and shunned vegetables, pre-
ferring sweets. She had odd compulsions-after meals she
folded paper plates into small triangles. Her ability to use
appropriate words worsened and she stopped talking. On
examination she was passive and stared inappropriately.
She giggled frequently and responded
don’t know” to
many questions. Her MMSE score was
She could not
name a pencil, but got
point for the intersecting penta-
gons. Digit span was six forward. She correctly named one
items (“bed) and was not helped with clues. Her
comprehension was mildly impaired. She failed memory
testing, but found her way easily back to her car. There
was mild impairment on three-dimensional drawing. She
could produce no
novel designs. Her basic
neurologic examination was normal. MRI showed moder-
ate left and mild right frontal atrophy with dilatation of
the ventricles anteriorly. The temporal lobe on the left was
slightly atrophic. SPECT showed left frontal and bitempo-
68-year-old right-handed man was seen
for a dementing illness of a 12-year duration. Previously a
successful businessman without interest in art, at age 56
describe “open” and “closed” periods. When
“closed,” he was dysphoric, and experienced lights and
sounds as exquisitely intense. When “open,” lights and
sounds produced a pleasant feeling that allowed him to
think creatively. He painted images experienced during
“open” and “closed” periods. At 58 years he became anomic
and disinhibited. Language and memory deteriorated, but
he showed heightened visual and auditory awareness. Odd
compulsions developed, and despite
wealth, he cajoled his caregivers to walk with him to look
56 years he began painting. During the next
decade he created paintings with increasing precision and
detail. The first featured brightly colored ellipses. Soon his
work became realistic, and he drew animals. Later works
were crafted with care, and he took hours to complete
single lines. Between 63 and 66 years his paintings won
several art show awards. By age
he drew oddly shaped
doll-like figures. On examination he was remote and irrita-
ble, showing little facial emotion. He displayed heightened
interest in his environment, commenting extensively on
color and sound. His MMSE score was 15, and his verbal
output was fluent.
mild comprehension deficit and a
semantic anomia were present. Word list generation was
two “d” words but
novel designs were produced.
showed bitemporal atrophy (worse on the left than the
and SPECT revealed bilateral temporal hypoperfu-
sion (worse on the right than the left). Frontal perfusion
was normal. Regions with highest perfusion were the right
primary visual cortex and the right posterior parietal lobe.
is more common than
FTD,‘j all patients we have seen who developed
maintained artistic talents carried a diagnosis of
FTD. The artistic products were diverse but shared
many features. The creativity was visual but never
verbal. Similarly, the paintings, photographs, and
sculptures were realistic copies lacking an abstract
symbolic component. The painters remembered re-
alistic landscapes, animals,
people; one woman
hand-crafted small figures; and a photographer cap-
tured scenes in rural Guatemala. The painters
seemed to recall images that were then mentally
pictures without the mediation
despite progressive cognitive and
cial impairment, they showed increasing interest in
the fine detail of faces, objects, shapes, and sounds.
Lastly, there was an intense preoccupation with
in all patients, and their willingness
perfect product was obtained enhanced
causes selective anterior frontotemporal cor-
tical degeneration, whereas in
involves the posterior parietal and medial temporal
visuoconstructive abilities dissipate in
conjunction with the progressive
of function in
posterior parietal and posterior temporal regions-
the brain areas responsible for visuoconstructive
In contrast, in FTD, copying skills are often
n0rma1,6.~ and posterior pathology is minimaL9J5
Sparing of these brain areas seemed to allow our
subjects to paint and photograph despite the ad-
their dementia. Yet even though copying
skills are preserved in FTD,16 diminished-not en-
hanced-creativity is more common with this ill-
ness,6 suggesting that those who become artistic are
atypical or unusual.
pathology, four of the five patients
had the temporal variant of FTD.7 Planning, organi-
zation, and motivation are mediated through dorso-
lateral and medial frontal lobe~’~-areas that are
preserved in the temporal lobe variant. The painters’
draw scenes remembered from the past
suggests that components of episodic and working
memory are spared. In contrast, semantic memory
(which relies on the integrity of the left inferior tem-
poral 10bel~~~) was devastated,
evidenced by ab-
normal performance on word recall and naming.
Goldman-Rakic observed that “Visuospatial pro-
cesses engaged in humans by activities such as
painting and drawing from memory.
rely on the
dorsolateral prefrontal convexity
The dorsolateral prefrontal cortex is
spared in the temporal lobe variant of FTD.
of patients with dominant-hemisphere dysfunction.
Nondominant-hemisphere injury devastates artistic
skills, even in previously talented artists,lg but in-
the dominant hemisphere can leave drawing
relatively intact.20 KaczmarekZ1 described
painter who suffered a left-hemisphere stroke associ-
ated with aphasia who lost the ability “to create the
highly symbolic pictures” painted before the stroke,
yet he continued drawing realistic pictures without
flaw. Kaczmarek noted that there was “impairment
of symbolic processing with fairly well preserved
technical skills” (reference 21, p. 370). This ability to
draw realistically without symbolism or abstraction
was true of our artists.
recent discussion of the unexpected occur-
behavioral improvement following brain in-
jury, KapurZ2 used the term “paradoxical functional
facilitation.” He noted, “in normal subjects, inhibi-
tory and excitatory mechanisms interact in
The role of inhibitory processes
may be critical in mediating specific restorative par-
adoxical functional facilitation effects” (reference 22,
p. 1783). It is well accepted that talent in one area,
may be accompanied by dysfunction in
other spheres, such
social skills.23 The loss of
cial skills and inhibitions may have facilitated the
of our patients. Our photographer traveled to
dangerous areas where he obtained unique photo-
graphs, whereas the willingness of Patient
social norms led him to take up painting and quit his
work. We hypothesize that selective degeneration
the anterior temporal and orbital frontal cortex de-
creased inhibition of the more posteriorly located visual
systems involved with perception, thereby enhancing
these patients’ artistic interest and abilities.
There is an association between bipolar disease
and the visual arts,24 although none of our patients
history suggestive of mania.
cial conventions is common among artists and can
a freer more creative output. Van
and Goya’@ work blossomed in midlife, when they
spurned conventional rules of society. “Outsider
ists” create distinctive paintings despite receiving lit-
tle formal training. Some manifest severe mental
illness and are hospitalized in mental institutions.
Like our patients, they are directed internally with
little interest in the teachings
others. Some have
history suggesting schizophrenia, but others develop
both talent and
behavioral disorder later in life.
Disinhibition, hallucinations, and aphasia are re-
ported in outsider artists. Some may have suffered
from the temporal lobe variant of FTD.27 The mecha-
nism for the enhanced
skills in these pa-
tients and outsider artists reported previo~sly~~
mystery. FTD is an unexpected window
into the artistic process.
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Direct genetic evidence
tau in progressive supranuclear palsy
P. Bennett, PhD;
Bonuccelli, MD; C. Colosimo, MD; M. De Mari, MD; G. Fabbrini, MD;
Meco, MD; D.J. Nicholl, MRCP;
Vanacore, MD; P. Vieregge, MD; and
A.C. Williams, MD, for the European Study Group on Atypical Parkinsonism Consortium*
confinrt whether a dinucleotide repeat sequence in an intron of the microtubule-
associated protein tau is associated with progressive supranuclear palsy
in an independent study population and to
establish an improved methodology for allelotyping. Background: It has recently been reported that a genetic variant
tau, known as the
allele, was represented excessively in PSP patients when compared with control subjects. Methods:
multicenter study, the authors examined the allelic distribution
this dinucleotide repeat marker in a set of
301, multiple system atrophy
and matched control
70). Individuals were allelotyped using automated analysis
fluorescently labeled PCR products. Results:
allele was significantly overrepresented in the PSP patients (93.3% versus 76.4%;
0.0067; odds ratio [OR]
homozygotes were overrepre-
sented in the PSP group (86.7% versus 61.1%;~
4.14; 95% CI, 1.19 to 14.48) compared with control subjects.
Conclusions: The findings
this study, which is the largest to date, support those of a previous investigation that used
patients. These data provide additional strong evidence that genetic variation at or near the
tau gene plays an important role in the pathogenesis of
Progressive supranuclear palsy
scribed by Steele et
progressive neurodegenerative disorder presenting
in persons older than
years as an akinetic, rigid
syndrome with vertical gaze palsy a,ssociated vari-
ably with dysarthria, dysphagia, axial rigidity, evi-
dence of pyramidal tract dysfunction, and
the frontal lobe qype). Patholog-
typically shows generalized cerebral atro-
phy with pallor of the substantia nigra and
the globus pallidus. There is neuronal
loss and gliosis, as well
the presence of numerous
neurofibrillary tangles (NFTs) and neuropil threads.
This occurs predominantly in the superior colliculus,
periaqueductal gray matter, pretectal areas, the
the substantia nigra, and the pal-
lidosubthalamic complex.2 In PSP, the NFTs consist
of clusters of straight filaments, compared with those
comprised of paired helical filaments seen in
tients. However, there
considerable overlap with
other tau-related disorders such as
postencephalitic parkinsonism and the ALS-PD de-
mentia complex (APDC) of Guam.3
Because of the frequency of tau pathology in neu-
been proposed in an attempt to explain how tau
could lose its normal affinity for microtubules and
recently by Conrad et
and indicates that a defect
gene itself may be involved,
PSP. This study
repeat in intron nine of the
gene, and subse-
highly significant overrepre-
sentation of one allele (AO) of this marker in a series
pathologically confirmed patients with
*See the Appendix on page
for a listing of additional members of the ESGAP Consortium.
From the University Department of Clinical Neurology (Drs. Bennett, Nicholl, and Williams), Queen Elizabeth Hospital, Birmingham, UK, the Department
of Neurosciences (Drs. Bonifati, Colosimo, Fabbrini, Meco, Stocchi, and Vanacore), “La Sapienza” University, Rome, Italy; the Institute of Neurology (Dr.
Bonuccelli), University of Pisa, Italy; the Institute of Neurology (Dr. De Mari), University of Ban, Italy; the Neurology Section (Dr. Marconi), “Misericordia”
Hospital, Grosseto, Italy; and the Department of Neurology (Dr. Vieregge), Medical University of Lubeck, Germany.
Supported by the Italian Ministry for University, Scientific and Technological Research grants to G.M.
Accepted in final form June
Address correspondence and reprint requests
Dr. Phil Bennett, University of Birmingham, Department of Psychiatry, Queen Elizabeth Psychiatric
Hospital, Mindelsohn Way, Edgbaston, Birmingham,
by the American Academy of Neurology