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Epilepsy & Behavior 17 (2010) 147–152

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Epilepsy & Behavior


journal homepage: www.elsevier.com/locate/yebeh

Review

A review of Savant Syndrome and its possible relationship to epilepsy


John R. Hughes *,1
Department of Neurology, University of Illinois Medical Center in Chicago, Chicago, IL, USA

a r t i c l e i n f o a b s t r a c t

Article history: The goal of this article is to review Savant Syndrome, characterized by outstanding islands of mental abil-
Received 29 October 2009 ity in otherwise handicapped individuals. Two forms exist: the congenital form and the acquired form.
Revised 17 December 2009 Among the many examples of the congenital form are the calendar calculators, who can quickly provide
Accepted 18 December 2009
the day of the week for any date in the past; the musical savants, who have perfect pitch; and the hyper-
Available online 13 January 2010
lexics, who (in one case) can read a page in 8 s and recall the text later at a 99% level. Other types of tal-
ents and artistic skills involving three-dimensional drawing, map memory, poetry, painting, and
Keywords:
sculpturing are also observed. One savant could recite without error the value of Pi to 22,514 places. Per-
Savant
Seizures
sons with the acquired form develop outstanding skills after brain injury or disease, usually involving the
Epilepsy left frontotemporal area. This type of injury seems to inhibit the ‘‘tyranny of the left hemisphere,” allow-
Calculators ing the right hemisphere to develop the savant skills. Another way to inhibit the left frontotemporal area
Frontotemporal dementia is to use transcranial magnetic stimulation in normal subjects; nearly one-half of these subjects can then
Right hemisphere perform new skills during the stimulation that they could not perform before. This type of finding indi-
Central coherence cates the potential in all of us for the development of savant skills in special circumstances. Explanations
Connectivity of congenital Savant Syndrome include enhanced local connectivity as a compensation for underconnec-
tivity of long-range fibers, but also weak central coherence, replaced by great attention to detail,
enhanced perceptual functioning, and obsessive preoccupation with specific interests.
Ó 2009 Elsevier Inc. All rights reserved.

1. Introduction 2. Method

Savant Syndrome (SS) is characterized by remarkable islands of The Medline Section on the Internet provided references to pub-
mental ability in otherwise mentally handicapped individuals [1]. lished studies on the ‘‘Savant Syndrome.”
Treffert and Wallace [2] have traced the history of SS, pointing
out that in 1789 Dr. Benjamin Rush, often considered the Father 3. Different forms
of American Psychiatry, described the outstanding mathematical
ability of one man, named Thomas Fuller. Nearly 100 years later 3.1. Congenital Savant Syndrome
in 1887, Dr. J. Langdon Down, well known for the syndrome with
his own name, and supervisor of the Earlswood Asylum in London, Treffert [1] has indicated that nearly one-half of those with the
referred to 10 patients with SS as ‘‘idiot savants.” An even earlier SS are individuals with autism and the other one-half have some
reference was added by Foerstl [3], who discussed a man named other type of developmental disability.
Jedediah Buxton, described in February 1751 in the magazine Gen-
tleman. Mr. Buxton could not write his own name, but could do
3.1.1. Calendar calculators
quick mathematical calculations, like multiplying a 39-digit num-
One of the most common forms of SS are the calendar calcula-
ber by itself.
tors (CCs). These individuals can readily identify the day of the
The goal of this study was to describe SS and to investigate the
week for any given date in the past. Different authors have re-
possible relationship of this syndrome to epilepsy, especially be-
ported various characteristics of the groups that they have studied.
cause SS often occurs in autistic patients who often have seizures.
One group [4] reported shorter reaction times and fewer errors for
present or past dates than controls, but no differences from con-
trols were found for future dates. Other investigators [5] described
Donny, a young autistic savant, ‘‘who is possibly the fastest and
* Fax: +1 312 996 4169.
E-mail address: jhughes@uic.edu
most accurate calendar prodigy ever described.” The title of this re-
1
University of Illinois Medical Center (M/C 796), 912 South Wood Street, Chicago, port likely justifies the latter statement: ‘‘The day of the week
IL 60612, USA. when you were born in 700 ms.” O’Connor and Hermelin [6] re-

1525-5050/$ - see front matter Ó 2009 Elsevier Inc. All rights reserved.
doi:10.1016/j.yebeh.2009.12.014
148 J.R. Hughes / Epilepsy & Behavior 17 (2010) 147–152

ported that calculating speeds of 10-year-old savants were similar rior chord segmentation. Two other authors [17] reported on a sa-
to those of adult savants. Performance could not be accounted for vant with perfect pitch, exceptional recall and performance of
by practice alone, and no improvement was detectable over time. structured music, and also increased speed of information process-
Other authors have pointed out various normal characteristics ing. Although this savant had high levels of concentration and
in this group. An example is that savants do not differ from con- memory, he had difficulties in verbal reasoning.
trols with respect to short- or long-term memory. However, sa-
vants show recall superiority for long-term retention of 3.1.3. Hyperlexia
calendrical material [7]. Others [8] have described in one savant The life of one of the most famous savants, Kim Peek, was dra-
poor explicit knowledge of calendar structure as well as an in- matized in the popular movie Rain Man, in which Peek was played
crease in error rate and response latency with temporal remote- by actor Dustin Hoffman. Kim reads the left side of a page with his
ness of the dates under consideration. Mottron et al. [9] also left eye and simultaneously the right side of the page with his right
dealt with errors, reporting that testing of all the dates in a year re- eye (without a corpus callosum). The time Kim took to read these
vealed a random distribution of errors that were not stable across two pages was usually 8 s and, on testing for retention, he was 99%
time. In addition, that particular savant was able to answer ‘‘re- correct on the material just read [2]. These values contrast with the
versed” questions that could not be solved with a classic algorithm. 45 s for reading and 45% correct on testing observed in a group of
Another group [10] described a Chinese calendar savant who normal individuals. In describing the condition of hyperlexia, Gold-
had exceptional proficiency in converting a date from the Grego- berg [18] stated that the skill arises without any practice, is not
rian calendar to the Chinese calendar. The results did not support integrated with other areas of knowledge, and is associated with
the hypotheses of rote memorization, eidetic imagery, high-speed a dysfunctional procedural memory system, but intact declarative
calculation and anchoring strategy, use of calendar irregularities, memories. Finally, two investigators [19] described two autistic
or monthly configurations. The authors proposed that this particu- children whose reading speeds were considerably faster than those
lar savant was familiar with 14 calendar templates and the knowl- of controls. To these investigators, efficient grapheme–phoneme
edge of matching these templates to every year. For dates beyond (written-sound) conversion is likely a modular component of the
the 20th century, the calculation was accomplished by regressing reading skill responsible for the hyperlexia in these savants.
the date to a corresponding year in the 20th century by adding
or subtracting 28 or 700 years. This explanation from the authors 3.1.4. Various other skills
may be difficult for the reader to follow, and it would seem even One group [20] mentioned hypermnesia as one of the special
more difficult to put the calculations into play to accomplish this skills found among those with SS. An excellent example is Kim Peek,
conversion of dates. who remembers nearly everything that he has read in 9000 books
Other explanations for the CCs have not been so complex, but [2]. That same group also mentioned several branches of the arts
neither have they been very informative. Two investigators [11] such as drawing, painting, and sculpting as part of SS. Mottron
concluded that savants adopt a cognitive style of weak central and Belleville [21] described one individual who could draw objects
coherence, protecting single representations from being retained rotated in three-dimensional space more accurately than controls.
in the form of stable enduring wholes. This strategy allows for In addition, this individual could detect a perspective incongruency
transformation, reorganization, and reconstruction of the relation- between an object and a landscape at a superior level. The authors
ship between single items of information. In other words, savants claimed that this skill was reached without the use of explicit or im-
are more likely to process details at the expense of global informa- plicit perspective rules. Two years earlier the same authors [22] de-
tion. One group [12] discussed the dysfunctional attention hypoth- scribed the same patient with the same outstanding ability for
esis to explain the skills of CCs by the ability to divide, shift, direct, three-dimensional drawing. Their complex conclusion (difficult to
and sustain attention. They concluded that a deficit in shifting understand) was that the performance was an ‘‘anomaly with hier-
selective attention from one stimulus to another was attributed archical organization of the local and global parts of a figure, a local
to an inability to disengage attention as a result of deficient orien- interference effect in incongruent hierarchical visual stimuli, a def-
tation and overselectivity. In other words, this latter explanation icit in relating local parts to global form information in impossible
may come down to focused attention. Finally, Hermelin and O’Con- figures and an absence of feature grouping in graphical recall.”
nor [13] concluded that these human calculators used rule-based O’Connor and Hermelin [23] discussed gifted artists who were
strategies. According to an ‘‘explanation” given by another group outstanding in the perception of a recognition memory for shapes.
[9], CCs are ‘‘solving the problems in a non-algorithmic way,” Results indicated that reproduction ability depended on artistic
meaning that they do not follow a logical set of rules in arriving ability, independent of intelligence. Somewhat related to the skill
at any answers. It may be helpful to indicate how the savants do of drawing was the talent of a 4-year-old boy [24] who had an
not perform their skills, as one step closer to understanding how exceptional memory for maps and spatial locations, although the
they do perform these same skills. boy had a low-average intelligence and impaired motor skills.
One author [25] mentioned a savant who was excellent on a block
3.1.2. Musical savants design test, but scored only 76 on a Performance IQ test. Another
Absolute or perfect pitch is the ability to properly identify any group [26] described a savant poet who referred more often to as-
musical note after hearing it. On a test involving absolute pitch, pects of self-analyses, whereas descriptions of other people not re-
investigated by one group [14], savants scored higher than nonmu- lated to the self were less frequently mentioned. This latter
sicians, but not statistically better than a group of professional emphasis on self is consistent with one of the basic core features
musicians. Also, in short-term memory tests for musical phrases, of autism. Another special talent is the mechanical ability to repair
savants and musicians performed indistinguishably. The authors everything and anything. Brink [27] described such a mechanical
concluded that the skills of musical savants are separate from gen- genius with a right-sided paralysis and transient loss of speech
eral intelligence, but also confirmed that these individuals can, in caused by a bullet into the left temporal area. Two other authors
fact, have absolute pitch. According to Heaton et al. [15], autistic [28] reported on a similar savant who could not speak until 20
savants performed in a highly superior way compared with con- years of age, but had great mechanical ability. Finally, other inves-
trols who had self-reported absolute pitch. Five years earlier, Hea- tigators [29] have described artists who were savants, commenting
ton [16] included other data, claiming that the savant group that their specific ability can occur at any level of intelligence, usu-
showed enhanced pitch memory and pitch labeling and also supe- ally between IQ scores of 30 and 75.
J.R. Hughes / Epilepsy & Behavior 17 (2010) 147–152 149

From all of these examples, it seems clear that nearly any spe- the kind of circumstance to release savant skills, and this reviewer
cific skill or talent can occur in SS. is not aware of any patients with a left temporal lobectomy who
later developed SS. However, a hypoexcitable left frontotemporal
3.2. Acquired Savant Syndrome area from a head injury, cerebrovascular accident, FTD, or other
cause seems sufficient to release the normal right hemisphere to
3.2.1. Without seizures develop SS, escaping the ‘‘tyranny of the left hemisphere.”
Treffert [30] has written extensively about the congenital form of One way to escape this ‘‘tyranny” is to stimulate the left fronto-
SS, likely present at around birth, but also has discussed the acquired temporal area with repetitive magnetic transcranial pulses. Snyder
form occurring later in life. In this instance ‘‘after some brain injury et al. [33] reported that 8 of 12 normal individuals, under magnetic
or brain disease, savant skills unexpectedly emerge, sometimes at a transcranial stimulation, improved their ability to accurately guess
prodigious level, when no such skills were present before injury or the number of discrete items that were placed together. In a study
illness.” Treffert provides many examples, including a 10-year-old by one group [34], 5 of 17 subjects developed savant types of skills,
boy knocked unconscious by a baseball who later could do quick cal- including declarative memory, drawing, and mathematical and cal-
endar calculations; an 8-year-old boy with similar talent after a left endar calculating during the stimulation. In another study pub-
hemispherectomy; a 3-year-old child who, after meningitis, was lished 3 years earlier, other investigators [35] reported that 4 of
considered a musical genius; and a 9-year-old boy who, after being 11 subjects displayed enhanced proofreading ability during the
shot in the left brain, resulting in a right-sided hemiparesis, later magnetic pulses. These findings argue that many of us, in certain
developed special mechanical abilities. Finally, two painters were circumstances, like during inhibition of the left frontotemporal
mentioned who manifested significant qualitative improvements area, may acquire savant types of skills! This possibility should
after strokes involving the left occipital lobe and thalamus. stimulate readers to consider the potential of their own cognitive
A surprising number of acquired cases had been earlier diag- powers or artistic talents.
nosed with frontotemporal dementia (FTD). These cases included
a 68-year-old man without any interest in art who became an
accomplished artist after FTD and also two other similar patients. 4. Possible causes of Savant Syndrome
Another case manifested ‘‘paradoxical functional facilitation ‘‘as a
release phenomenon ‘‘where loss of some skills permits emergence Takahata and Kato [36] discussed acquired SS, as exemplified by
of others,” like artistry. One investigator, mentioned by Treffert patients with FTD who then develop savant skills, and provided a
[30], had collected 12 cases of FTD with later emergent artistic few explanations to account for this phenomenon. First is the
ability. One particular patient was a 51-year-old artist whose skills hypermnesic model, according to which these skills develop from
surfaced for the first time after a subarachnoid hemorrhage associ- existing or dormant cognitive functions, like memory. Second is
ated with cerebral artery aneurysms. The frontal damage in this the paradoxical functional facilitation model, which emphasizes
latter patient was similar to that seen in FTD. Finally, a talented the role of reciprocal inhibitory interactions among cortical re-
art teacher, at the beginning of a progressive FTD process at age gions. Third is the autistic model, including the weak central coher-
49, later showed ‘‘impressive artistic growth.” ence theory, also emphasizing the underconnectivity or disruption
of long-range fibers, but with an enhanced local connectivity in gi-
3.2.2. With seizures ven areas. The authors speculated that the enhanced local connec-
Only very few, if any, examples can be found in the world liter- tivity results from the specialized and facilitated cognitive
ature of acquired SS with epilepsy. One possible example comes processes responsible for savant skills.
from Oliver Sachs [31], who described a person with ‘‘high fever, The hypermnesic model with skills developing from existing
weight loss, delirium and perhaps seizures.” Following this illness, functions has some support from Miller [37], who concluded that
this individual began painting extremely accurate scenes that col- skills exhibited by savants shared many characteristics of normal
leagues believed he could not have painted before the illness. subjects and that the skills are usually accompanied by normative
Patrick Obyedkov, a 35-year-old musical savant after the onset levels of performance in various subtests. Also, this investigator
of seizures, was a fictional character on the TV serial House. claimed that it is unclear that savants have distinctive cognitive
The example of Daniel Tammet (D.T.) should be mentioned, but strengths or motivational dispositions. Two authors [38] similarly
may not be relevant here, because his childhood seizures at 3 years concluded that there may be no differences between savants and
of age had always been considered as not related to SS but instead normal subjects regarding the nature of mental structures underly-
to his synesthesia. The latter condition occurred after his seizures. ing specific talents, which are independent of the level of general
Daniel was on antiepileptic drugs for only 3 years and had been cognitive functioning. The latter two studies do not therefore clar-
seizure free since early childhood [32]. He experienced a form of ify how savants perform their outstanding skills.
synesthesia in which every number had its own color, shape, and The third model (autistic) is based, in part, on the weak central
texture. At 4 years of age, he developed rapid calendar calculating coherence theory, which some authors [39] believe would predis-
ability, in addition to an incredible memory for numbers. On Amer- pose individuals to develop their talents with obsessive preoccupa-
ican TV, many viewers witnessed Daniel at 26 years of age, in front tion. Others [40] have drawn a similar conclusion, that the
of Oxford University dons, reciting (without a single mistake) the excessive use of cognitive processing would be ‘‘instigated by the
value of Pi to 22,514 decimal places over a 5-h period. Thus, this probable failure of central executive control mechanisms.” Still oth-
prodigious memory was not likely related to his early seizures at ers [11] have concluded that a ‘‘cognitive style of weak central
3 years of age. As nearly one-half of savants have autism, which coherence may protect single representations from being retained
is closely associated with seizures, it may be speculated that sa- in the form of stable enduring wholes.” Happe [41] also concluded
vants might have seizures, but usually do not have such attacks. that a cognitive style biased toward local rather than global infor-
Thus, only one case of possible seizures associated with either mation processing (called weak central coherence) may be involved
acquired or congenital SS can be found in the world literature. in SS. In addition, other authors [22] have referred to an anomaly in
On the other hand, many examples can be found of acquired SS the hierarchical organization of the local and global parts of the skill
without seizures, but with some damage or disturbance to the involved. The other part of the autistic model is the presence of
brain, usually the left frontal temporal area. Because a seizure con- long-range fiber underconnectivity, emphasized by Hughes [42],
dition involves a hyperexcitable brain, this condition is not likely also associated with enhanced short-range or local connectivity.
150 J.R. Hughes / Epilepsy & Behavior 17 (2010) 147–152

Many other explanations have been suggested with various [24]. O’Connor [55] added that all savants can abstract to some
depths of insight into the fascinating phenomenon of SS. One group degree.
[43] hypothesized that a mutation gives rise to the development of
the positive aspects of SS, but has a deleterious effect on the devel- 5.2. Memory
opment of other phenotypic traits, resulting in autism. Another
group [44] reported that chromosome 15q11–q13 involving the Memory for the specific talent was described as exceptional
GABRB3 gene provides a genetic contribution to a subset of af- [24], but general and verbal memory was normal, independent of
fected individuals with savant skills. Two years later, others [45] the measured Verbal IQ [56]. Also, skills were not based on efficient
failed to demonstrate a linkage to 15q11–q13. Casanova et al. rote memory [50]. Another characteristic is that skills of savants do
[46] had an interesting approach to explaining these skills: they not change with practice [50,55].
analyzed the morphometry of three distinguished scientists who
died and were autopsied. They reported significant differences in 5.3. Sensation and perception
both smaller minicolumn width and mean cell spacing between
the cortices of these scientists and those of comparison groups. As previously mentioned, one group [47] proposed ‘‘enhanced
These conditions would be expected to enhance neuronal process- perceptual functioning” in savants. Included are the possibilities
ing, as would be anticipated in SS. of locally oriented perceptions, use of a more posterior network,
Mottron et al. [47] presented the concept of ‘‘enhanced percep- enhanced perception of static stimuli, decreased perception of
tual functioning” to explain SS. Included were possibilities of lo- movements, and differences between perception and general intel-
cally oriented visual and auditory perception, enhanced low-level ligence. These conditions add up to increased perceptual process-
discrimination, and the use of a more posterior network in com- ing. On the other hand, Chen et al. [57] reported normal auditory
plex visual tasks. Also included were enhanced perception of static and visual evoked potentials in savants.
stimuli, decreased perception of complex movement, autonomy of
low-level information processing toward higher-order operations,
5.4. Focus
and increased perceptual expertise. Consistent with the complex-
ity of the latter explanations, other investigators [48] found no evi-
A few investigators [58] described one savant under discussion
dence that savants use short-cut strategies.
as having a ‘‘propensity to focus on local detail.” Casanova et al.
The ‘‘dysfunctional attention hypothesis” of Casey et al. [12] ad-
[46] used a similar phrase, ‘‘focused attention,” likely related to
dressed the deficiencies of savants in shifting selective attention
an ‘‘obsessive personality profile” [40]. Others [59] described this
from one stimulus to another. This hypothesis does not clarify
characteristic as a ‘‘focus on one topic at the expense of other inter-
the mechanisms used by savants to express their talents, except
ests,” similar to the ‘‘preoccupations” of O‘Connor and Hermelin
to imply that savants are locked into one stimulus. The three
[50,51,53].
hypotheses offered by Kehrer [49] were that savants perceive
and store impulses differently, that they have an abnormal mem-
ory process and storing function, and that these outstanding abili- 6. Anatomical relationships
ties are reinforced by the environment. However, these points do
not easily explain the positive aspects of SS. Two other authors One of the most fascinating and significant points in this review
[50] claimed that savants use strategies founded on ‘‘deduction has been emphasized by Treffert [29], who summarized the condi-
and application of rules governing the material upon which their tion of acquired SS. He stated that damage to the left frontotempo-
special ability operates,” also generating ‘‘novel examples of such ral area seems to be required to escape the ‘‘tyranny of the left
rule-based structures.” Perhaps, these comments are not too help- hemisphere” and, thus, to permit the development of a savant skill
ful in understanding SS, but another point—that these savants have by the right hemisphere. After studying all types of SS, Bujas-Pet-
an obsessional preoccupation with a limited section of the environ- kovic [60] concluded that ‘‘the functioning of the right cerebral
ment—is more helpful. Goldberg [18] theorized that savants have a hemisphere is most probably responsible for those abilities.” Treff-
dysfunctional procedural memory system (related to skills or pro- ert [1] summarized the situation in another way, by concluding
cedures) though their declarative memories (of facts) are intact. that left brain damage occurs with right brain compensation. As
Two investigators, 1 year earlier [51] than the later report [50], previously mentioned, Snyder et al. [33] was one of the investiga-
concluded that savants have a superior image memory and ready tors who reported that inhibition of the left anterior temporal area
access to a ‘‘picture lexicon.” The example of savants like Daniel by repetitive magnetic stimulation could result in the improve-
Tammet, who can quickly multiply a long number by itself, re- ment of various abilities. Others [59] agreed that ‘‘the anatomic
quires comment. It is as if the problem is presented to these sa- substrate for the savant syndrome may involve the loss of function
vants and they ‘‘read off” the answer as the brain ‘‘automatically” also in the left temporal lobe,” but added that there is also en-
does the calculation. While listening to a familiar opera, this re- hanced function of the posterior neocortex.
viewer often ‘‘automatically” knew the next note, but this knowl- The prefrontal area has also been emphasized in some reports.
edge derives from learning the sequence of notes after hearing Two investigators [36] referred to the paradoxical functional facil-
those same sequences many times. The parallel with SS is that they itation model in which reciprocal inhibitory interactions occur
also deal with sequences, but the major difference here is that the among adjacent or distant cortical regions, especially the prefron-
savants show these prodigious skills without any rehearsal of the tal cortex and the posterior regions of the brain. The same prefron-
problem just presented to them. tal area was mentioned by others [58] reporting on a celebrated
savant (D.T.) whose fMRI showed hyperactivity in the lateral pre-
frontal cortex when demonstrating one of his skills by encoding
5. Characteristics of savants digits.

5.1. Intelligence 7. Incidence

Intellectual functioning was viewed as ‘‘average” [52], or ‘‘lim- Treffert [1] summarized data on the incidence of SS. As previ-
ited” [5,48], ‘‘independent” [50,51,53,54], and also ‘‘low average” ously mentioned, he stated that approximately 10% of autistic per-
J.R. Hughes / Epilepsy & Behavior 17 (2010) 147–152 151

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