to the superficial branch of the ulnar nerve at the References

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(see figure 1B). Indeed, the PB spasm could still be
evoked by slight compression against the pisiform
bone or by tactile and electrical stimulations of the
fifth finger, even when the finger showed complete
loss of sensation after wrist lidocaine block of the
nerve. In addition, paresthesia of the fifth finger was
intermittent and always associated with the PB
spasm, suggesting an irritative lesion of the corre-
sponding ulnar nerve branch.
Acknowledgment
10. Cowdery SR, Preston DC, Herrmann DN, Logigian EL. Electrodiagno-
The authors thank Ms. A. Collins for help with English and Ms. A.
Laffi for manuscript preparation.
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syndrome. J Neurol Neurosurg Psychiatry 1995;59:182–184.
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lent of hemifacial spasm? Arch Neurol 1976;33:706 –708.
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report. Neurology 1990;40:153–154.
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puter palsy. Lancet 1991;338:832.
9. Deleu D. Mouse-directed computers and ulnar sensory neuropathy.
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tional tests. Neurology 2002;59:420 – 427.

Art and the brain

The influence of frontotemporal dementia on an
accomplished artist
Joshua Chang Mell, BS; Sara M. Howard, BFA; and Bruce L. Miller, MD

Abstract—A talented artist developed a progressive aphasia syndrome associated with frontotemporal dementia (FTD).
As her disease progressed, language and executive skills declined, but her paintings became freer and more original. She
demonstrates that artistic development can occur in the setting of language-dominant types of FTD. The study of artistic
development in the setting of FTD suggests that language is not required for, and may even inhibit, certain types of visual
creativity.
NEUROLOGY 2003;60:1707–1710

A painting can be a window into the mindset, mood,
skills, perceptions, preoccupations, and training of
the artist. Patients with frontotemporal dementia
(FTD) can develop new artistic skills in the setting of
their illness.1 Typically, these patients have no prior
background in painting and have the semantic de-
mentia subtype of FTD.2 With the painter described
in this report, an accomplished artist who subse-
quently developed FTD, the issues are somewhat dif-
ferent. One must ask whether the changes in her art
represented a logical and natural artistic evolution
or were secondary to the influence of FTD on the
artistic process.
Case history. A 57-year-old right-handed woman
was evaluated for a progressive aphasia syndrome
that had progressed to dementia. She had immi-
grated to the United States from Asia as a teenager
and studied painting in college, eventually becoming
a high school art teacher. In the 1970s, she com-
pleted an MFA, combining training in Western rep-
resentational art and Chinese brush painting. By
1986, she was having difficulty with grading and
lesson planning and slowly transferred these respon-
sibilities to her teenage son. Language deficits in-
creased, and in early 1995, she retired when she
could no longer control the classroom or remember
her students’ names.
She withdrew from painting in association with a
depressive episode between 1995 and 1996 but began
again in late 1996. She produced some of her best
pieces in 1997 and 1998 at a time when she had

From the Genetics Graduate Group (J.C. Mell), University of California at Davis, and Department of Neurology (Dr. Miller, S.M. Howard), University of
California at San Francisco.
Supported by a Program Project Grant from the National Institute of Aging (PO1AG19724) and a State of California Grant from the AD Research Center of
California (0115420).
Received October 22, 2002. Accepted in final form January 20, 2003.
Address correspondence and reprint requests to Dr. B.L. Miller, 350 Parnassus St., Suite 706, San Francisco, CA 94143-1207; e-mail:
bmiller@memory.ucsf.edu

Copyright © 2003 by AAN Enterprises, Inc. 1707


Figure 1. (A) Quan Yin, watercolor on paper. This piece
is a typical example of the artist’s Eastern-style work from
her MFA. (B) Abstract, oil on paper. An experimental
Western-style piece completed in the early 1970s.
difficulty with written and spoken language. By the
end of 1997, language, social, and spatial skills had
diminished and her production of paintings slowed.
In 1999, she stopped writing and driving, and by
2000, she required caregiver support to help with her
activities of daily living.
In 2000, the patient was evaluated at the Univer-
sity of California at San Francisco. Social graces
were normal, and she had little spontaneous speech
1708 NEUROLOGY 60 May (2 of 2) 2003
output. Language was nonfluent and effortful, and
she failed frontal executive tasks. Comprehension
was normal for two-step commands. She named 10 of
15 words on the Boston Naming Test. Copying and
praxis were normal. There were slowing of move-
ment and subtle right hand incoordination. MRI
showed moderate bifrontal atrophy slightly worse on
the left and mild left temporal atrophy. Progressive
nonfluent aphasia was diagnosed.
Art history. Her first pieces were landscapes and
representational paintings. In the early 1980s, she
produced two 12-piece figurative series, one
Western-style life-size watercolors of female nudes,
the others classic Eastern brush paintings of women
from Chinese folklore (figure 1A). In the 1980s, she
also painted landscapes and a few experimental, ab-
stract pieces (see figure 1B). From 1990 to 1993, she
created an exquisite series of paintings based on the
Chinese horoscope (figure 2A) with the intention of
merging the Eastern and Western components of her
training.
In 1993, instead of working alone in her studio,
she took a portfolio of paper to cafés and concerts
where she drew people in their surrounding environ-
ment. She used an ink brush pen to produce hun-
dreds of gesture drawings, spending at most 30
minutes on any given sketch (figure 3A). Uncharac-
teristically friendly, the patient ignored social cues
and entered into the conversations of strangers. In
1997, she began a new series intended to mix East-
ern and Western styles of painting. She produced 12
large male nude figurative paintings, rich in color
and slightly sexual in theme (see figure 2, B and C),
and continued to use mythologic imagery, as in the
Horoscope Series. The detailed patterning was
moved to the background, whereas the male figures
were a single layer of color. She continued to sketch
with her brush pen, but figures became distorted and
less realistic (see figure 3B). Only two more color
pieces were completed: the Four Masks painting
from 1999 (see figure 3C) and a small watercolor
made in 2001. While no longer producing new art,
she continues to have excellent memories regarding
her pictures and the strategies that she used to cre-
ate her lifetime of work.
Discussion. The work of this talented artist
showed a remarkable evolution over the 15 years of
her slowly progressive aphasia syndrome.2 Originally
trained in Western watercolor and traditional Chi-
nese brush painting, she began to experiment with
her technique around age 49, producing highly pat-
terned paintings using the Chinese horoscope icons.
This impressive artistic growth coincided with a de-
cline in her ability to organize class lessons or grade
homework. As language declined, paintings became
wilder and freer. In a series of male nudes, intricate
designs and patterns of the horoscope figures were
replaced by large, intensely colored figures; complex
patterning was pushed to the background. Her
Figure 2. (A) Chinese Horoscope Figure, lithograph. This is an example of the artist combining the influence of Eastern
and Western art into one piece. This series was completed soon after the artist could no longer teach because of her cogni-
tive difficulties, at least 7 years into her illness. (B) Sumo Wrestlers, from the Male Nude Series, acrylic on paper. This
series was completed about 2 years after the Horoscope Series, when the patient was developing profound aphasia, at
least 9 years into her illness. (C) Male Nude Series, acrylic on paper. This is 1 of 12 male nudes produced by the artist
around the same time as the Sumo Wrestlers.

choice of colors changed, with large swatches of
red, turquoise, and purple now dominating the pic-
tures. Release from the constraints of formal train-
ing became clear, and her last pieces were no longer
realistic, reflecting an intensely emotional and im-
pressionistic style, with less detail.
There is an evolving literature on artistic creativ-
ity in the setting of dementia. The artist de Koon-
ing’s work became freer and less intricate in the
setting of AD,3 whereas Cummings and Zarit de-
scribed the evolution of an artist with AD who went
from precise realism to a more surrealistic, but ap-
pealing, style.4 We are not aware of a case report on
an accomplished artist who developed FTD, but FTD
patients without previous artistic abilities have de-
veloped a new interest in art, creating complex and
visually precise paintings.1,5,6 These cases have had
asymmetric left anterior temporal lobe degeneration,

Figure 3. (A) Early Café Drawing, ink on paper. This drawing is typical of the hundreds that were created, sketching in
cafés, around 7 years into her illness. (B) Later Café Drawing, ink on paper. This is a later drawing that clearly shows
the decline in the artist’s perceptual abilities, around 12 years into her illness. (C) Four Masks, acrylic on paper. This is
one of her two final paintings made 13 years into her illness.
May (2 of 2) 2003 NEUROLOGY 60 1709
whereas our patient had predominantly left frontal
disease.1,5,6
The ability to transcend ordinary social, physical,
and cognitive constraints is a feature of great artists.
It is tempting to attribute the imaginative, freer, and
visually complex paintings produced between 1996
and 1997, when our patient had aphasia and behav-
ioral disinhibition, to FTD. The nondominant poste-
rior right parietal and temporal cortices, regions
spared in FTD, appear to be critical for accurate
copying or for drawing of images that are imagined
internally. Release of these regions from language-
dominant patterns of thinking organized in the dom-
inant frontal and anterior temporal regions appears
to be a key factor in the emergence of artistic skills
with FTD. The release of frontal lobe functions in-
volved with social restraints may have also played a
role in her beautiful later works. Similar linguistic
and social deficits and artistic creativity have been
described in autistic artists.7,8
Whatever the mechanism, our patient represents
a remarkable example of how a truly talented indi-
vidual can continue to evolve and create in the face
of a degenerative brain disease. She demonstrates
that dementia is not a monolithic entity and that
each individual is affected differently. Asymmetric
left hemisphere degeneration may release previously
untapped cognitive abilities.9 Our brain wiring ap-
pears to be a major factor in the determination of the
nature of our creativity. Release of creativity and
originality represents an unexpected and unexplored
feature of dementia.
Acknowledgment
The authors thank Jeanne Houston and Isabel Espiritu.
References
1. Miller BL, Ponton M, Benson DF, Cummings JL, Mena I. Enhanced
artistic creativity with temporal lobe degeneration. Lancet 1996;348:
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ation: a consensus on clinical diagnostic criteria. Neurology 1998;51:
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3. Espinel CH. de Kooning’s late colours and forms: dementia, creativity,
and the healing power of art. Lancet 1996;347:1096 –1098.
4. Cummings JL, Zarit JM. Probable Alzheimer’s disease in an artist.
JAMA 1987;258:2731–2734.
5. Miller BL, Boone K, Cummings J, Read SL, Mishkin F. Functional
correlates of musical and visual talent in frontotemporal dementia. Br J
Psychiatry 2000;176:458 – 463.
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Emergence of artistic talent in frontotemporal dementia. Neurology
1998;51:978 –981.
7. Snyder AW, Thomas M. Autistic artists give clues to cognition. Percep-
tion 1997;26:93–96.
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76 – 85.
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A critical review. Brain 1996;119:1775–1790.

Treatment of slow-channel congenital

myasthenic syndrome with fluoxetine
C. Michel Harper, MD; Takayasu Fukodome, MD; and Andrew G. Engel, MD

Abstract—The authors found that fluoxetine significantly shortens at 5 ␮M/L and nearly normalizes at 10 ␮M/L the
prolonged opening bursts of slow-channel congenital myasthenic syndrome (SCCMS) acetylcholine receptors (AChR)
expressed in fibroblasts. Prompted by this observation, they treated two SCCMS patients allergic to quinidine with up to
80 to 120 mg of fluoxetine per day over 3 years (serum fluoxetine ⫹ norfluoxetine levels 8 to 11 ␮M/L). Both patients
showed marked subjective and objective improvement by quantitative muscle strength testing and electromyography.
NEUROLOGY 2003;60:1710 –1713

The slow-channel congenital myasthenic syndrome
(SCCMS) is an autosomal dominant disorder caused
by gain-of-function mutations in the acetylcholine re-
ceptor (AChR) that prolong the opening events of the
receptor. This causes cationic overloading of the
postsynaptic region, resulting in an endplate myop-
athy. Neuromuscular transmission is compromised
by loss of AChR from degenerating junctional folds,
widening of the synaptic space that reduces ACh
concentrations reaching the postsynaptic membrane,
and a depolarization block caused by staircase sum-
mation of the prolonged endplate potentials at phys-
iologic rates of stimulation.1
We previously reported that quinidine is a long-
lived open-channel blocker of AChR that in clinically
attainable concentrations normalizes the prolonged

From the Department of Neurology (Drs. Harper and Engel), Mayo Clinic, Rochester, MN; and Kawatana National Hospital (Dr. Fukodome), Japan.
Supported by grant NS6277 from the NIH (A.G.E.) and by a research grant from the Muscular Dystrophy Association (A.G.E.).
Received December 6, 2002. Accepted in final form January 28, 2003.
Address correspondence and reprint requests to Dr. C. Michel Harper, Department of Neurology, Mayo Clinic, 200 First St. SW, Rochester, MN 55905;
e-mail: harper.michel@mayo.edu

1710 Copyright © 2003 by AAN Enterprises, Inc.

Art and the brain: The influence of frontotemporal dementia on an accomplished artist
Joshua Chang Mell, Sara M. Howard and Bruce L. Miller
Neurology 2003;60;1707-1710
DOI 10.1212/01.WNL.0000064164.02891.12

This information is current as of May 27, 2003

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