CEREBRAL PALSY EXPLAINED
What is CEREBRAL PALSY ?
The term CEREBRAL PALSY refers to any one of a number of neurological disorders that appear in infancy or early childhood and permanently affect body movement and muscle coordination but don’t worsen over time. Even though CEREBRAL PALSY affects muscle movement, it isn’t caused by problems in the muscles or nerves. It is caused by abnormalities in parts of the brain that control muscle movements. The majority of children with CEREBRAL PALSY are born with it, although it may not be detected until months or years later. The early signs of CEREBRAL PALSY usually appear before a child reaches 3 years of age. The most common are a lack of muscle coordination when performing voluntary movements (ataxia); stiff or tight muscles and exaggerated reflexes (spasticity); walking with one foot or leg dragging; walking on the toes, a crouched gait, or a “scissored” gait; and muscle tone that is either too stiff or too floppy. A small number of children have CEREBRAL PALSY as the result of brain damage in the first few months or years of life, brain infections such as bacterial meningitis or viral encephalitis, or head injury from a motor vehicle accident, a fall, or child abuse.
Is there any treatment for CEREBRAL PALSY ?
CEREBRAL PALSY can’t be cured, but treatment will often improve a child's capabilities. Many children go on to enjoy near-normal adult lives if their disabilities are properly managed. In general, the earlier treatment for CEREBRAL PALSY begins the better chance children have of overcoming developmental disabilities or learning new ways to accomplish the tasks that challenge them. Treatment may include physical and occupational therapy, speech therapy, drugs to control seizures, relax muscle spasms, and alleviate pain; surgery to correct anatomical abnormalities or release tight muscles; braces and other orthotic devices; wheelchairs and rolling walkers; and communication aids such as computers with attached voice synthesizers.
What is the prognosis?
CEREBRAL PALSY doesn’t always cause profound disabilities. While one child with severe cerebral palsy might be unable to walk and need extensive, lifelong care, another with mild cerebral palsy might be only slightly awkward and require no special assistance. Supportive treatments, medications, and surgery can help many individuals improve their motor skills and ability to communicate with the world.
What CEREBRAL PALSY research is being done?
Researchers are investigating the roles of mishaps early in brain development, including genetic defects, which are sometimes responsible for the brain malformations and abnormalities that result in CEREBRAL PALSY. Scientists are also looking at traumatic events in newborn babies’ brains, such as bleeding, epileptic seizures, and breathing and circulation problems, which can cause the abnormal release of chemicals that trigger the kind of damage that causes CEREBRAL PALSY. To make sure children are getting the right kinds of therapies, studies are also being done that evaluate both experimental treatments and treatments already in use so that physicians and parents have valid information to help them choose the best therapy.
NIH Patient Recruitment for CEREBRAL PALSY Clinical Trials
At NIH Clinical Center
Throughout the U.S. and Worldwide
Organizations
United CEREBRAL PALSY (UCP)
1660 L Street, NW
Suite 700
Washington, DC 20036
national@ucp.org
http://www.ucp.org
Tel: 202-776-0406 800-USA-5UCP (872-5827)
Fax: 202-776-0414
Pathways Awareness Foundation [For Children With Movement Difficulties]
150 N. Michigan Avenue
Suite 2100
Chicago, IL 60601
friends@pathwaysawareness.org
http://www.pathwaysawareness.org
Tel: 312-893-6620 800-955-CHILD (2445)
Fax: 312-893-6621
March of Dimes Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
askus@marchofdimes.com
http://www.marchofdimes.com
Tel: 914-428-7100 888-MODIMES (663-4637)
Fax: 914-428-8203
Easter Seals
233 South Wacker Drive
Suite 2400
Chicago, IL 60606
info@easterseals.com
http://www.easterseals.com
Tel: 312-726-6200 800-221-6827
Fax: 312-726-1494
Children's Neurobiological Solutions (CNS) Foundation
1726 Franceschi Road
Santa Barbara, CA 93101
info@cnsfoundation.org
http://www.cnsfoundation.org
Tel: 866-CNS-5580 (267-5580) 805-898-4442
Children's Hemiplegia and Stroke Assocn. (CHASA)
4101 West Green Oaks Blvd., Ste. 305
PMB 149
Arlington, TX 76016
info437@chasa.org
http://www.hemi-kids.org
Tel: 817-492-4325
CEREBRAL PALSY International Research Foundation
1025 Connecticut Avenue
Suite 701
Washington, DC 20036
nmaher@cpirf.org
http://www.cpirf.org
Tel: 202-496-5060
Pedal with Pete [For Research on CEREBRAL PALSY ]
P.O. Box 274
Kent, OH 44240
petezeid@aol.com
http://www.pedalwithpete.com
Tel: 800-304-PETE (7383)
Fax: 330-673-1240
Wednesday, February 25, 2009
Monday, February 16, 2009
PROPRANOLOL - Promising cure for Fear
Imagine being able to decouple bad memories from the fear and anxiety they produce with just a pill.
As more effective brain-boosting pills are developed, demand for them is likely to grow among...
As more effective brain-boosting pills are developed, demand for them is likely to grow among middle-aged people who want youthful memory powers and multitasking workers who need to keep track of multiple demands, said one commentary author, brain scientist Martha Farah of the University of Pennsylvania.
That's the promise of a new report from Dutch researchers published in the Feb. 15 advance online issue of Nature Neuroscience.
Merel Kindt and colleagues used a beta blocker called propranolol (Inderal) to erase, at least in the short-term, the fear response induced by a laboratory-induced painful memory in humans.
Such findings could one day help individuals suffering from pathological anxiety disorders from the debilitating physiological effects of their fears. Yet many questions remain, experts note, such as how permanent the effect is, and whether it can affect traumatic memories that may be decades old.
"I think it's a very interesting and exciting study," said Jane Taylor, a professor of psychiatry at Yale University, who studies memory reconsolidation in rats. "It will be interesting to know how long-lasting this effect is, and whether it only works on recently consolidated memories."
Mark Bouton, a professor of psychology at the University of Vermont, echoed that sentiment. "This study is a solid step forward in our understanding of how to reduce fear," he said. "The big question is whether this treatment will reduce all forms of relapse, including the return of fear that can occur with the passage of time."
Human memory often is compared to computer storage. Some memories exist in a sort of neurological flash RAM, whereas others are stored for the long term, on the brain's hard disk. The analogy works to a point, but it isn't perfect, as it turns out to be quite difficult to permanently erase files in the brain's memory banks.
"Fear memories can be surprisingly resilient," Bouton explained.
To try to break at least the physiological hold these fears have over individuals, Kindt induced a kind of Pavlovian fear response in 60 undergraduate students at the University of Amsterdam.
The study lasted three days. On the first day, the subjects learned to associate images of spiders with a mild electrical shock. Fear was measured by assessing each individual's startle response -- how much their eyes blinked in response to the stimulus. That fear memory was then consolidated -- written to the hard disk, if you will.
According to Treffert, about half of persons with SAVANT SYNDROME have autistic disorder, while the other half have another developmental disability, mental retardation, brain injury or disease. He says, "... not all savants are autistic, and not all autistic persons are savants." Other researchers state that autistic traits and savant skills may be linked, or have challenged some earlier conclusions about SAVANT SYNDROME as "hearsay, uncorroborated by independent scrutiny".
What is SAVANT SYNDROME?
People with SAVANT SYNDROME are people who despite serious mental or physical disability have quite remarkable, and sometimes spectacular, talents. This is an exceedingly rare phenomena, although there are several well documented cases (see Sacks, 1986; 1995; Treffert, 1989), and recently the Academy Award winning movie Rain Man has led to the term SAVANT SYNDROME being much more widely known. SAVANT SYNDROME is perhaps one of the most fascinating phenomena in the study of human differences and cognitive psychology. It is often claimed that, because of the extraordinary abilities involved, we will never truly understand human memory and cognition until we understand the savant.
SAVANT SYNDROME was first properly recognised by Dr. J. Langdon Down, (n.b. he also originated the term Down’s syndrome). In 1887, he coined the term "idiot savant" - meaning low intelligence, and from the French, savoir, knowing or wise, to describe someone who had "extraordinary memory but with a great defect in reasoning power." This term is now little used because of its inappropriate connotations, and the term SAVANT SYNDROME has now been more or less adopted. Another term, AUTISTIC SAVANT, is also widely used, but this can be somewhat misleading. Although there is a strong association with autism, it is certainly not the case that all savants are autistic. It is estimated that about 50% of the cases of SAVANT SYNDROME are from the autistic population, and the other 50% from the population of developmental disabilities and CNS injuries. The estimated incidence of savant abilities in the autistic population is about 10%, whereas the incidence in the learning disability population (which is very much larger) is probably less than 1%. Nevertheless, in order to understand SAVANT SYNDROME, it is helpful to know something about autism, also it is important to realize that there is some confusion over these estimates of the incidence of the syndrome which stems from the different ways in which it is defined and described.
What is Autism?
Autism is a moderately rare condition resulting from a complex developmental disability that typically appears during the first three years of life. It is a neurological disorder that affects the functioning of the developing brain, resulting in sometimes profound communicative, social and cognitive deficits. Autism is estimated to occur in as many as 1 in 500 individuals, and is four times more prevalent in boys than girls and does not seem to be associated with any demographic features, such as economic, class, racial, ethnic, etc. Autistic traits are also sometimes observed in connection with other developmental disabilities, and CNS injuries.
The term autistic was first used by Eugen Bleuler in 1908, but the condition of autism was first named and described by the psychiatrist, Leo Kanner, in a landmark paper published in 1943. It is a condition in which children and adults typically have a lowered level of intelligence, together with difficulties in verbal and non-verbal communication, in the skills of social interaction, and in play activities. The disorder makes it hard for them to relate to the outside world, and there is a marked tendency to withdraw from human interactions and become preoccupied with attachment to objects. There is a failure in human intersubjectivity, characterized by difficulties in joint action, turn taking, and shared activities. Aggressive and/or self-injurious behaviour may well be present. Often there will be continuous repetition of body movements (hand flapping, rocking), a rigidity of actions, resistance to changes in routine, and a "desire" for sameness. Independently of Kanner, in 1944 Hans Asperger, an Austrian physician, described a very similar condition, although there were some subtle differences. In 1981, Lorna Wing adopted the term Asperger’s syndrome in referring to a group of people who did not fit the strict criteria for autism, and were relatively high functioning (see Happé, 1994 for a fuller account).
It is probably best to think of autism as a spectrum disorder. For example, DSM-IV includes autism, grouped together with several related disorders, under the broad heading "Pervasive Developmental Disorder (PDD)." This is a general category of disorders which is characterized by severe and pervasive impairment in several areas of development. There are no medical criteria for diagnosing autism, a specific diagnosis is made when a specified number of characteristics are noted as present, based on the presence of specific behaviours indicated by observation and through parent consultation. Individuals who fall under the PDD category in DSM-IV exhibit commonalties in communication and social deficits, but may differ in terms of severity. Defining autism as a spectrum disorder, recognizes that the symptoms and characteristics of autism can present themselves in a wide variety of combinations, which may also range from mild to severe. Clearly, there is no standard "type" or "typical" person with autism, and the terminology in use includes: autistic traits, autistic tendencies, autism spectrum disorder, high-functioning or low-functioning autism. However, this lecture is not concerned directly with autism, its definition or diagnosis.
Characteristics of SAVANT SYNDROME
SAVANT SYNDROME is exceedingly rare, but a remarkable condition in which persons with autism, or other serious mental handicaps, or major mental illness, have astonishing islands of ability or brilliance that stand out in stark contrast to their overall disability. The condition can be congenital or be acquired by an otherwise normal individual following CNS injury or disease. It occurs in males more frequently than in females in an approximate ratio of 6 to 1. The skills can appear suddenly, without explanation, and have been reported as sometimes disappearing just as suddenly. It is useful to put these special skills into the following three categories: Splinter Skills where the individual possesses specific skills that stand in contrast to their overall level of functioning, Talented Savants where the individual displays a high level of ability that is in contrast to their disability, and Prodigious Savants which involves a much rarer form of the condition, where the ability or brilliance is not only spectacular in contrast to the disability, but would be spectacular even if viewed in a non-disabled person. It is very likely that many savants do go unnoticed, and depending upon whether the three categories above are recognized, estimates of the incidence of savant syndrome can vary widely. In the case of prodigious savants it has been estimated that there may be fewer than 100 cases reported in the world literature in the past 100 years.
#Categories of SAVANT SYNDROME Skill
SAVANT SYNDROME skills occur within a narrow but fairly constant range of human mental functions. If they have anything in common it is that they all more or less involve considerable feats of memory. In some cases a specific skill might exist, while in others there may be several skills that co-exist simultaneously. An important observation is that the skills tend to be right hemisphere oriented: i.e. non-symbolic, artistic, concrete, directly perceived. Table 1 describes some of the striking abilities that have been found in savants.
Table 1: SAVANT SYNDROME Skills
(n.b. the focus here is on examples of prodigious savants)
Memorization - superior memory is a common feature of SAVANT SYNDROME, but it also can be a special skill in its own right. There are cases of savants who have memorized population statistics, telephone books, bus scheduals, and in one remarkable case the 9 volume edition of Grove’s Dictionary of Music and Musicians (The Walking Grove, Sacks, 1986).
Lightening calculation - this is exhibited in the instantaneous calculation of multiplications, square roots, etc, the determination of prime numbers, or subitizing (The Twins, Sacks, 1986).
Calender calculating - often involving the ability to identify the day of the week upon which a particular date falls, in one case any time in the last, or next, forty thousand years!! (The Twins, Sacks, 1986).
Musical ability - this is a relatively common savant skill, the co-occurrence of musical genius, blindness and learning disability is a striking feature here. Savants will have perfect pitch, and can play a complete piece of music after hearing it only once (see Hermelin, 2001).
Artistic ability - not as common as musical abilities, but there are savants with exceptional painting, sculpture and especially drawing skills. e.g. Nadia (Selfe, 1977) and Stephen Wiltshire (1987; 1991; see also Sacks, 1995; Hermelin, 2001). See also The Autistic Artist in Sacks (1986).
Language ability - this is fairly rare, but there is one well documented case of a savant with CNS damage since birth who could read write and translate 15 to 20 languages (Smith & Tsimpli, 1995; Hermelin, 2001). Hermelin also includes a case of a savant poet.
#Theories of SAVANT SYNDROME
The reason why some autistic and disabled individuals have savant abilities is not understood, however, the strong link with autism does offer a good starting point. There have been many theories, but it is clear that no one theory is sufficient. Theories include: Biological-Developmental - such as genetic, neurochemical, left hemisphere dysfunction, frontal and temporal lobe damage, and the DSM IV diagnostic category is Pervasive Developmental Disorder (PDD); Cognitive - such as deficits in executive function and abstract thinking; weak coherence theory; highly developed procedural memory and eidetic imagery (Happé, 1994; Schopler & Mesibov, 1995). Other theories include a deficit in theory of mind (Frith, 1989), compensation for sensory disabilities (especially blindness) and social isolation, and the modularity of mind hypothesis which proposes that particularly when executive cognitive functions are disrupted the mind exhibits a striking modular organization (see Smith & Tsimpli, 1995). However, any theory would need to explain the link with autism, the islands of exceptional ability, the bias towards male savants, and recent research that includes a finding of the emergence of savant abilities in fronto-temporal dementia patients, and the suggestion of a neurotoxic effect of circulating testosterone on the left hemisphere in the male fetus possibly related to autism.
#Some Management Issues
There are two necessary components of the SAVANT SYNDROME: (i) a remarkable ability to memorize, to record detail, or repeat an operation endlessly and efficiently, and (ii) a means of giving expression to this ability. The importance of (ii) should not be underestimated. Not only are savants noticed by this expression of their special abilities, but also savants like doing something, and doing it again, again and again. No one has any idea how many savants go unnoticed. In the case of prodigious savants it is possible that early recognition and careful encouragement are important contributory factors to how the talent develops. It has been proposed that helping the savant to achieve a higher level of general functioning may result in a loss of the special savant skills. However, there is little evidence for this, and it may well be that "training the talent" could be a valuable approach towards improving socialization, communication and self-esteem.
#A illustrative case example: Tim, age 40+
Tim has profound sensory and communicative disabilities (his identity has been concealed). He lives in a residential home with day care facilities for adults with learning difficulties, and has been in residential or institutional care since the age of 15. He has no hearing and consequently no speech. He has moderate physical difficulties and sometimes he requires a wheelchair. Tim has probably been disabled since very early childhood, and it is believed that he has been diagnosed as having "autistic traits." But, as far as it is known, his medical records have been destroyed. He has a previous history of challenging behaviour and mood swings, which has in the past been controlled with powerful anti-psychotic drugs. These have been greatly reduced over the 5 years that he has been living at his current residential home, during which time there have been striking changes in his behaviour, including a particularly marked reduction in his challenging behviour. The most likely reason for this is due mainly to communication barriers being greatly decreased. Despite Tim’s profound disabilities, he is relatively outgoing and is not withdrawn, and he shows a remarkable intelligence (although this would be very difficult to measure formally). He is strong willed, and will only do things that he wants to do. He is helpful, he values affection, and he is considerate to other residents, especially in being tolerant of younger residents. Tim has probably received very little education, he cannot read or write (although he can recognize his name and a few words, and copy any shape that he wishes), but he has been taught a system of alternative communication called Makaton. This is a visual and signing process, usually used alongside speech, which is widely used in the UK by people with learning disabilities. The Makaton Vocabulary was designed in 1972 by Margaret Walker, a UK Speech and language Therapist. She developed Makaton in response to the needs of deaf adults with severe learning disabilities, particularly who were residents in an institution, because other sign communication systems were not very satisfactory. Without Makaton, Tim would only be able to make himself understood with a few crude gestures, and his life could and would be very confusing and frustrating. Tim uses Makaton to initiate conversations, to ask questions, and clarify any situation.
Table 2: Some observations of Tim
Tim draws from memory, and from life
He draws with accurate perspective
He draws with attention to detail
He can draw a good likeness, and can draw a self-portrait
He can draw a "building plan" with a ruler
He finds "hair" very difficult
Tim’s drawing involves deliberate use of lines - "as if tracing an image"
He has a high level of concentration
He is reflective, pausing to think
He chooses his pencils, colours carefully (he knows which pencil/crayon he needs, which box it is in, and he will make a very special effort to match "eye colour")
When drawing from life he takes brief infrequent glimpses
Tim draws what he wants to draw
He likes to draw batteries, light bulbs and lifts
In the past, he did not share his drawings with others, he folded them up very small and put them in his pocket, but kept them all in his room
He has developed his own narrative style of drawing
In addition, he has excellent assembly skills (e.g. IKEA furniture)
His rigidity has relaxed with improved communication
When placed within the context of all these disabilities, Tim possesses extraordinary abilities which primarily are illustrated by his drawing and his photographic memory. As far as it is known, these extraordinary abilities have gone unnoticed, or unrecognized, for most of his life. It was my wife, Elaine, who was the first to recognize Tim’s special abilities. It seems very clear that Tim falls into the category of a savant. What is particularly interesting is that very few cases of savants who are profoundly deaf have ever been documented (the one exception seems to be the case of James Henry Pullen, see Treffert, 1989). I will demonstrate what I am talking about by showing you a selection of his drawings. I will point out a number of features that show how his abilities fit well with those usually attributed to savant syndrome (see Table 2). His drawings are deceptively simple, and it is easy to underestimate the level of his achievements. I will draw especially upon the work of David Hockney (2001) who has recently uncovered some of the techniques used by the old masters in their paintings. Tim has very little difficulty drawing images in accurate perspective that the old masters could only do with sophisticated technical aids. What most people, including skilled artists, would find very difficult to do "by eye", Tim can do with little effort, from memory, sometimes months later, and without any formal instruction or training.
At this time, Tim is clearly a talented savant, he may even be prodigious. His special skills and abilities are highly specialized, and are obviously conspicuous when viewed over against his over-all handicap, he can draw in ways that most professional artists would find impossible. Tim seems to fit with one view of savant syndrome as resulting from a compensation for a sensory deficit, i.e. his deafness, and the possession of a remarkable photographic memory. My wife has adopted the position that Tim’s drawing ability would not have become so apparent if the communication barriers had not been bridged. Tim has a need to be sure of, and trust, what is happening around him. Without this need being met, Tim’s exceptional abilities would not have had the chance to develop in the way that they have. Indeed, it is highly unlikely that they would ever have been noticed at all.
For More information on AUTISM
As more effective brain-boosting pills are developed, demand for them is likely to grow among...
As more effective brain-boosting pills are developed, demand for them is likely to grow among middle-aged people who want youthful memory powers and multitasking workers who need to keep track of multiple demands, said one commentary author, brain scientist Martha Farah of the University of Pennsylvania.
That's the promise of a new report from Dutch researchers published in the Feb. 15 advance online issue of Nature Neuroscience.
Merel Kindt and colleagues used a beta blocker called propranolol (Inderal) to erase, at least in the short-term, the fear response induced by a laboratory-induced painful memory in humans.
Such findings could one day help individuals suffering from pathological anxiety disorders from the debilitating physiological effects of their fears. Yet many questions remain, experts note, such as how permanent the effect is, and whether it can affect traumatic memories that may be decades old.
"I think it's a very interesting and exciting study," said Jane Taylor, a professor of psychiatry at Yale University, who studies memory reconsolidation in rats. "It will be interesting to know how long-lasting this effect is, and whether it only works on recently consolidated memories."
Mark Bouton, a professor of psychology at the University of Vermont, echoed that sentiment. "This study is a solid step forward in our understanding of how to reduce fear," he said. "The big question is whether this treatment will reduce all forms of relapse, including the return of fear that can occur with the passage of time."
Human memory often is compared to computer storage. Some memories exist in a sort of neurological flash RAM, whereas others are stored for the long term, on the brain's hard disk. The analogy works to a point, but it isn't perfect, as it turns out to be quite difficult to permanently erase files in the brain's memory banks.
"Fear memories can be surprisingly resilient," Bouton explained.
To try to break at least the physiological hold these fears have over individuals, Kindt induced a kind of Pavlovian fear response in 60 undergraduate students at the University of Amsterdam.
The study lasted three days. On the first day, the subjects learned to associate images of spiders with a mild electrical shock. Fear was measured by assessing each individual's startle response -- how much their eyes blinked in response to the stimulus. That fear memory was then consolidated -- written to the hard disk, if you will.
According to Treffert, about half of persons with SAVANT SYNDROME have autistic disorder, while the other half have another developmental disability, mental retardation, brain injury or disease. He says, "... not all savants are autistic, and not all autistic persons are savants." Other researchers state that autistic traits and savant skills may be linked, or have challenged some earlier conclusions about SAVANT SYNDROME as "hearsay, uncorroborated by independent scrutiny".
What is SAVANT SYNDROME?
People with SAVANT SYNDROME are people who despite serious mental or physical disability have quite remarkable, and sometimes spectacular, talents. This is an exceedingly rare phenomena, although there are several well documented cases (see Sacks, 1986; 1995; Treffert, 1989), and recently the Academy Award winning movie Rain Man has led to the term SAVANT SYNDROME being much more widely known. SAVANT SYNDROME is perhaps one of the most fascinating phenomena in the study of human differences and cognitive psychology. It is often claimed that, because of the extraordinary abilities involved, we will never truly understand human memory and cognition until we understand the savant.
SAVANT SYNDROME was first properly recognised by Dr. J. Langdon Down, (n.b. he also originated the term Down’s syndrome). In 1887, he coined the term "idiot savant" - meaning low intelligence, and from the French, savoir, knowing or wise, to describe someone who had "extraordinary memory but with a great defect in reasoning power." This term is now little used because of its inappropriate connotations, and the term SAVANT SYNDROME has now been more or less adopted. Another term, AUTISTIC SAVANT, is also widely used, but this can be somewhat misleading. Although there is a strong association with autism, it is certainly not the case that all savants are autistic. It is estimated that about 50% of the cases of SAVANT SYNDROME are from the autistic population, and the other 50% from the population of developmental disabilities and CNS injuries. The estimated incidence of savant abilities in the autistic population is about 10%, whereas the incidence in the learning disability population (which is very much larger) is probably less than 1%. Nevertheless, in order to understand SAVANT SYNDROME, it is helpful to know something about autism, also it is important to realize that there is some confusion over these estimates of the incidence of the syndrome which stems from the different ways in which it is defined and described.
What is Autism?
Autism is a moderately rare condition resulting from a complex developmental disability that typically appears during the first three years of life. It is a neurological disorder that affects the functioning of the developing brain, resulting in sometimes profound communicative, social and cognitive deficits. Autism is estimated to occur in as many as 1 in 500 individuals, and is four times more prevalent in boys than girls and does not seem to be associated with any demographic features, such as economic, class, racial, ethnic, etc. Autistic traits are also sometimes observed in connection with other developmental disabilities, and CNS injuries.
The term autistic was first used by Eugen Bleuler in 1908, but the condition of autism was first named and described by the psychiatrist, Leo Kanner, in a landmark paper published in 1943. It is a condition in which children and adults typically have a lowered level of intelligence, together with difficulties in verbal and non-verbal communication, in the skills of social interaction, and in play activities. The disorder makes it hard for them to relate to the outside world, and there is a marked tendency to withdraw from human interactions and become preoccupied with attachment to objects. There is a failure in human intersubjectivity, characterized by difficulties in joint action, turn taking, and shared activities. Aggressive and/or self-injurious behaviour may well be present. Often there will be continuous repetition of body movements (hand flapping, rocking), a rigidity of actions, resistance to changes in routine, and a "desire" for sameness. Independently of Kanner, in 1944 Hans Asperger, an Austrian physician, described a very similar condition, although there were some subtle differences. In 1981, Lorna Wing adopted the term Asperger’s syndrome in referring to a group of people who did not fit the strict criteria for autism, and were relatively high functioning (see Happé, 1994 for a fuller account).
It is probably best to think of autism as a spectrum disorder. For example, DSM-IV includes autism, grouped together with several related disorders, under the broad heading "Pervasive Developmental Disorder (PDD)." This is a general category of disorders which is characterized by severe and pervasive impairment in several areas of development. There are no medical criteria for diagnosing autism, a specific diagnosis is made when a specified number of characteristics are noted as present, based on the presence of specific behaviours indicated by observation and through parent consultation. Individuals who fall under the PDD category in DSM-IV exhibit commonalties in communication and social deficits, but may differ in terms of severity. Defining autism as a spectrum disorder, recognizes that the symptoms and characteristics of autism can present themselves in a wide variety of combinations, which may also range from mild to severe. Clearly, there is no standard "type" or "typical" person with autism, and the terminology in use includes: autistic traits, autistic tendencies, autism spectrum disorder, high-functioning or low-functioning autism. However, this lecture is not concerned directly with autism, its definition or diagnosis.
Characteristics of SAVANT SYNDROME
SAVANT SYNDROME is exceedingly rare, but a remarkable condition in which persons with autism, or other serious mental handicaps, or major mental illness, have astonishing islands of ability or brilliance that stand out in stark contrast to their overall disability. The condition can be congenital or be acquired by an otherwise normal individual following CNS injury or disease. It occurs in males more frequently than in females in an approximate ratio of 6 to 1. The skills can appear suddenly, without explanation, and have been reported as sometimes disappearing just as suddenly. It is useful to put these special skills into the following three categories: Splinter Skills where the individual possesses specific skills that stand in contrast to their overall level of functioning, Talented Savants where the individual displays a high level of ability that is in contrast to their disability, and Prodigious Savants which involves a much rarer form of the condition, where the ability or brilliance is not only spectacular in contrast to the disability, but would be spectacular even if viewed in a non-disabled person. It is very likely that many savants do go unnoticed, and depending upon whether the three categories above are recognized, estimates of the incidence of savant syndrome can vary widely. In the case of prodigious savants it has been estimated that there may be fewer than 100 cases reported in the world literature in the past 100 years.
#Categories of SAVANT SYNDROME Skill
SAVANT SYNDROME skills occur within a narrow but fairly constant range of human mental functions. If they have anything in common it is that they all more or less involve considerable feats of memory. In some cases a specific skill might exist, while in others there may be several skills that co-exist simultaneously. An important observation is that the skills tend to be right hemisphere oriented: i.e. non-symbolic, artistic, concrete, directly perceived. Table 1 describes some of the striking abilities that have been found in savants.
Table 1: SAVANT SYNDROME Skills
(n.b. the focus here is on examples of prodigious savants)
Memorization - superior memory is a common feature of SAVANT SYNDROME, but it also can be a special skill in its own right. There are cases of savants who have memorized population statistics, telephone books, bus scheduals, and in one remarkable case the 9 volume edition of Grove’s Dictionary of Music and Musicians (The Walking Grove, Sacks, 1986).
Lightening calculation - this is exhibited in the instantaneous calculation of multiplications, square roots, etc, the determination of prime numbers, or subitizing (The Twins, Sacks, 1986).
Calender calculating - often involving the ability to identify the day of the week upon which a particular date falls, in one case any time in the last, or next, forty thousand years!! (The Twins, Sacks, 1986).
Musical ability - this is a relatively common savant skill, the co-occurrence of musical genius, blindness and learning disability is a striking feature here. Savants will have perfect pitch, and can play a complete piece of music after hearing it only once (see Hermelin, 2001).
Artistic ability - not as common as musical abilities, but there are savants with exceptional painting, sculpture and especially drawing skills. e.g. Nadia (Selfe, 1977) and Stephen Wiltshire (1987; 1991; see also Sacks, 1995; Hermelin, 2001). See also The Autistic Artist in Sacks (1986).
Language ability - this is fairly rare, but there is one well documented case of a savant with CNS damage since birth who could read write and translate 15 to 20 languages (Smith & Tsimpli, 1995; Hermelin, 2001). Hermelin also includes a case of a savant poet.
#Theories of SAVANT SYNDROME
The reason why some autistic and disabled individuals have savant abilities is not understood, however, the strong link with autism does offer a good starting point. There have been many theories, but it is clear that no one theory is sufficient. Theories include: Biological-Developmental - such as genetic, neurochemical, left hemisphere dysfunction, frontal and temporal lobe damage, and the DSM IV diagnostic category is Pervasive Developmental Disorder (PDD); Cognitive - such as deficits in executive function and abstract thinking; weak coherence theory; highly developed procedural memory and eidetic imagery (Happé, 1994; Schopler & Mesibov, 1995). Other theories include a deficit in theory of mind (Frith, 1989), compensation for sensory disabilities (especially blindness) and social isolation, and the modularity of mind hypothesis which proposes that particularly when executive cognitive functions are disrupted the mind exhibits a striking modular organization (see Smith & Tsimpli, 1995). However, any theory would need to explain the link with autism, the islands of exceptional ability, the bias towards male savants, and recent research that includes a finding of the emergence of savant abilities in fronto-temporal dementia patients, and the suggestion of a neurotoxic effect of circulating testosterone on the left hemisphere in the male fetus possibly related to autism.
#Some Management Issues
There are two necessary components of the SAVANT SYNDROME: (i) a remarkable ability to memorize, to record detail, or repeat an operation endlessly and efficiently, and (ii) a means of giving expression to this ability. The importance of (ii) should not be underestimated. Not only are savants noticed by this expression of their special abilities, but also savants like doing something, and doing it again, again and again. No one has any idea how many savants go unnoticed. In the case of prodigious savants it is possible that early recognition and careful encouragement are important contributory factors to how the talent develops. It has been proposed that helping the savant to achieve a higher level of general functioning may result in a loss of the special savant skills. However, there is little evidence for this, and it may well be that "training the talent" could be a valuable approach towards improving socialization, communication and self-esteem.
#A illustrative case example: Tim, age 40+
Tim has profound sensory and communicative disabilities (his identity has been concealed). He lives in a residential home with day care facilities for adults with learning difficulties, and has been in residential or institutional care since the age of 15. He has no hearing and consequently no speech. He has moderate physical difficulties and sometimes he requires a wheelchair. Tim has probably been disabled since very early childhood, and it is believed that he has been diagnosed as having "autistic traits." But, as far as it is known, his medical records have been destroyed. He has a previous history of challenging behaviour and mood swings, which has in the past been controlled with powerful anti-psychotic drugs. These have been greatly reduced over the 5 years that he has been living at his current residential home, during which time there have been striking changes in his behaviour, including a particularly marked reduction in his challenging behviour. The most likely reason for this is due mainly to communication barriers being greatly decreased. Despite Tim’s profound disabilities, he is relatively outgoing and is not withdrawn, and he shows a remarkable intelligence (although this would be very difficult to measure formally). He is strong willed, and will only do things that he wants to do. He is helpful, he values affection, and he is considerate to other residents, especially in being tolerant of younger residents. Tim has probably received very little education, he cannot read or write (although he can recognize his name and a few words, and copy any shape that he wishes), but he has been taught a system of alternative communication called Makaton. This is a visual and signing process, usually used alongside speech, which is widely used in the UK by people with learning disabilities. The Makaton Vocabulary was designed in 1972 by Margaret Walker, a UK Speech and language Therapist. She developed Makaton in response to the needs of deaf adults with severe learning disabilities, particularly who were residents in an institution, because other sign communication systems were not very satisfactory. Without Makaton, Tim would only be able to make himself understood with a few crude gestures, and his life could and would be very confusing and frustrating. Tim uses Makaton to initiate conversations, to ask questions, and clarify any situation.
Table 2: Some observations of Tim
Tim draws from memory, and from life
He draws with accurate perspective
He draws with attention to detail
He can draw a good likeness, and can draw a self-portrait
He can draw a "building plan" with a ruler
He finds "hair" very difficult
Tim’s drawing involves deliberate use of lines - "as if tracing an image"
He has a high level of concentration
He is reflective, pausing to think
He chooses his pencils, colours carefully (he knows which pencil/crayon he needs, which box it is in, and he will make a very special effort to match "eye colour")
When drawing from life he takes brief infrequent glimpses
Tim draws what he wants to draw
He likes to draw batteries, light bulbs and lifts
In the past, he did not share his drawings with others, he folded them up very small and put them in his pocket, but kept them all in his room
He has developed his own narrative style of drawing
In addition, he has excellent assembly skills (e.g. IKEA furniture)
His rigidity has relaxed with improved communication
When placed within the context of all these disabilities, Tim possesses extraordinary abilities which primarily are illustrated by his drawing and his photographic memory. As far as it is known, these extraordinary abilities have gone unnoticed, or unrecognized, for most of his life. It was my wife, Elaine, who was the first to recognize Tim’s special abilities. It seems very clear that Tim falls into the category of a savant. What is particularly interesting is that very few cases of savants who are profoundly deaf have ever been documented (the one exception seems to be the case of James Henry Pullen, see Treffert, 1989). I will demonstrate what I am talking about by showing you a selection of his drawings. I will point out a number of features that show how his abilities fit well with those usually attributed to savant syndrome (see Table 2). His drawings are deceptively simple, and it is easy to underestimate the level of his achievements. I will draw especially upon the work of David Hockney (2001) who has recently uncovered some of the techniques used by the old masters in their paintings. Tim has very little difficulty drawing images in accurate perspective that the old masters could only do with sophisticated technical aids. What most people, including skilled artists, would find very difficult to do "by eye", Tim can do with little effort, from memory, sometimes months later, and without any formal instruction or training.
At this time, Tim is clearly a talented savant, he may even be prodigious. His special skills and abilities are highly specialized, and are obviously conspicuous when viewed over against his over-all handicap, he can draw in ways that most professional artists would find impossible. Tim seems to fit with one view of savant syndrome as resulting from a compensation for a sensory deficit, i.e. his deafness, and the possession of a remarkable photographic memory. My wife has adopted the position that Tim’s drawing ability would not have become so apparent if the communication barriers had not been bridged. Tim has a need to be sure of, and trust, what is happening around him. Without this need being met, Tim’s exceptional abilities would not have had the chance to develop in the way that they have. Indeed, it is highly unlikely that they would ever have been noticed at all.
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