Targeting Autism: What We Know, Don't Know, and Can do to Help Young Children with Autism and Related Disorders - Softcover

Cohen, Shirley

Softcover

ISBN 10: 0520234804 ISBN 13: 9780520234802

Publisher: University of California Press, 2002

Synopsis

Targeting Autism reaches out to everyone who lives with, cares about, or is trying to understand and help a young child who has or may have autism. With special attention to recent advances in early identification and educational treatment, Shirley Cohen, a teacher of teachers, provides expert guidance for families, educators, students, therapeutic personnel, and all those interested in autistic spectrum disorders that may affect as many as one out of every 200 to 250 children. Cohen, who has no personal or professional stake in any of the various forms of treatment, leads us through an analysis of the approaches currently available, especially the educational interventions designed for young children. Readers will welcome this informed and humane combination of up-to-date research findings, personal observations, and narratives from parents and adults with autism. Targeting Autism also highlights advances in research that promise an answer to the puzzles of autism and the elimination of its devastating effects on the development of children.

In a new preface and updated appendix of resources Cohen includes information about new developments and new ways of thinking that have recently modified the map of the world of autism or may do so in the near future.

About this title

About the Author

Shirley Cohen is Professor of Special Education at Hunter College of the City University of New York. Her previous books include Special People: A Brighter Future for Everyone with Physical, Mental, and Emotional Disabilities (1977) and Respite Care: Principles, Programs, and Policies (1985).

From the Back Cover

"A realistic yet hopeful account of the current possibilities for autistic children. Parents and professionals alike will welcome its judicious survey of the variety of educational interventions, its useful listing of resources, and its extensive bibliography."―Clara Claiborne Park, author of The Siege: The First Eight Years of an Autistic Child

"This book contains a balanced discussion of the many different types of educational programs. It will help parents and professionals make wise choices. It needs to be in every library."―Temple Grandin, author of Thinking in Pictures and Other Reports from My Life with Autism

From the Inside Flap

"A realistic yet hopeful account of the current possibilities for autistic children. Parents and professionals alike will welcome its judicious survey of the variety of educational interventions, its useful listing of resources, and its extensive bibliography."—Clara Claiborne Park, author of The Siege: The First Eight Years of an Autistic Child

"This book contains a balanced discussion of the many different types of educational programs. It will help parents and professionals make wise choices. It needs to be in every library."—Temple Grandin, author of Thinking in Pictures and Other Reports from My Life with Autism

Excerpt. © Reprinted by permission. All rights reserved.

Targeting Autism

What We Know, Don't Know - Updated EditionBy Shirley Cohen

University of California Press

Copyright © 2002 Shirley Cohen
All right reserved.
ISBN: 9780520234802
1
Meeting Autism

There she moved, every, day, among us but not of us, acquiescent when we approached, untouched when we retreated, serene, detached. . . . Existing among us, she had her being elsewhere.
(Park 1982, 12)

I visit the home of parents I recently met. Their five-year-old son is standing on his head on the couch. I go up to him, turn my head to the side, and say, "Hello, Kenneth." "Hello, Kenneth," he echoes.

I enter a room at a hotel where an informal meeting is taking place. As soon as I step through the doorway, a handsome, nicely dressed young man of perhaps seventeen walks up to me and tells me his name. The following exchange then takes place.

"What is your name?"

"Shirley."

"What is your sister's name?"

"Which sister?"

"How many sisters do you have?"

"Two."

"What are their names?"

"Paula and Sandy."

"What is your brother's name?"

"How do you know I have a brother?"

After faltering for a second he continues, "You don't have a brother?"

His attention then immediately shifts to the person who entered the room after me, and the same questioning routine begins again.

I am at a national conference on autism. Walter, a man perhaps in his mid-twenties, draws my attention. He claps loudly when anyone is introduced, and as he does so his mouth opens, his head moves from side to side, and his eyes appear to focus at a point near the ceiling. Walter and his mother are sitting only a few feet from the bluegrass band that is to play at the conference reception. As soon as the loud and lively music starts, Waiter's hands begin to twist rapidly in arcs before him, the right one clockwise, the left counterclockwise. He keeps clasping his hands together, whether to stop their movement or to clap I don't know, but his hands keep breaking loose. His head turns faster and faster, keeping time with both his hands and the music. When the music ends, Walter's movements slow to a stop. He looks at the ceiling briefly and then sits quietly.

A woman who has a Ph.D. is making a presentation: Temple Grandin has written two books about herself as well as numerous articles on autism. She is also the subject of an intensive case study in the book An Anthropologist on Mars by the neurologist Oliver Sacks. Temple Grandin is a person with autism.

We glimpse here a few of the many faces of autism. One of the most striking aspects of the condition (or conditions) labeled "autism" is its variability. What then do people called autistic have in common? What does that term autism mean if it encompasses such heterogeneity? What is the concept behind the label? To answer this question we need to look across several perspectivesb those of researchers, clinicians, parents, and adults with autism. We can begin by studying the "bible" of diagnostic categories and labels, the American Psychiatric Association's Diagnostic and Statistical Manual of Mental Disorders (DSM-IV), now in its fourth edition.

Autism is classified by DSM-IV as a pervasive developmental disorder, a term meant to indicate "severe and pervasive impairment in several areas of development: reciprocal social interaction skills, com-

munication skills, or the presence of stereotyped behavior, interests, and activities" (APA 1994, 65). Let's look at what autism may mean through examples provided by parents.

Qualitative impairment in social interaction . Catherine Maurice describes the social isolation of her infant daughter.

Anne-Marie was not shy: she was largely oblivious to people, and would sometimes actually avoid them, including, a lot of the time, her own mother. She drifted toward solitary spaces: the corners of a room, behind the curtains, behind the armchair. If I was somewhere else in the apartment, she never sought me out. . . .

Worst of all, perhaps, was the lack of that primary connection: the sweet steady gazing into one's eyes that we began to see all around us in other toddlers. . . . Sometimes I would catch her gazing in my direction and would start up, eager to respond to her invitation, to meet her look. But her eyes, frighteningly, were focused upon some middle distance, somewhere between me and the wall behind me. She wasn't seeing me at all. She was looking right through me! (Maurice 1993, 31, 33)

Qualitative impairments in communication . Craig Schulze tells us about his son's speech without communication.

I then enter the room carrying Jordan's dinner. "Put it on the table," he blurts out when he sees his food. It's a remark he's been making four times a day (during meal times and his snack) for over two weeks. He doesn't seem to really care where I put the food since he darts away from me the minute I'm in the room. Like so many of his most recent utterances, this habitual response seems more a ritual than an attempt at communication. It's spoken in a loud monotone with almost no emotion. (Schulze 1993, 105)

Clara Park describes her daughter's language at age two and then at age twenty-three. At age two her daughter used words, but infrequently and not to communicate. "She had no idea of language as a tool that could cause things to happen" 1982, 74). At age twenty-three,

Anybody who hears Jessy speak more than a word or two realizes that something is wrong. She has learned English as a foreign tongue, though far more slowly, and she still speaks it as a stranger. The more

excited she is about what she has to say, the more her speech deteriorates; her attention cannot stretch to cover both what she is saying and how she is saying it. Pronouns get scrambled: "you" for "I," "she" for "he," "they" for "we." Articles and tenses are confused or disappear, verbs lose their inflections or are omitted altogether. (Park 1982, 292)

Restricted repetitive and stereotyped patterns of behavior, interests, and activities . "We watched him as he rocked his body and spun every round object he could find," writes Barry Kaufman in describing his not yet two-year-old son's repetitive behavior (1976, 62). Judy Barron writes of her infant son:

He was drawn to odd things. He'd crawl past a brightly colored selection of toys to get to the furnace register. Once there he would stick his fingers into the slots and watch his fingers move. There was a hole in the wooden floor of his bedroom that riveted his attention. He'd put his finger into that and wiggle it around for hours. (Barron and Barron 1992, 13)

Six-year-old Paul McDonnell became obsessed with light bulbs, his mother, Jane, wrote.

His light bulb collection had grown to include not only incandescent household light bulbs, but also fluorescent bulbs, black lights, infrared and ultraviolet bulbs, and flashcubes: three hundred seventy-two in all. He kept many of his light bulbs in a basket by his bed, and every night he tried out different bulbs. (McDonnell 1993, 154)

As an adolescent Temple Grandin became fixated on squeeze chutes for cattle, an obsession that she later transformed into a therapeutic device to calm herself and others.

All autistic children do not act exactly like the children described above. Not all are as cut off from people as was Anne-Marie Maurice; not all have to struggle as hard as did Jessy Park to use language for communication; and the stereotyped interests of children with autism vary widely. See Appendix A for the specific combinations of behavioral indicators that professionals use to arrive at a diagnosis of autism.

"There are, due to a tragic accident of nature, children with autism who live in society, but who for some as yet ill-understood reasons cannot

profit much from the social stimulation provided by loving and caring parents" (Volkmar 1993, 40). Who are these children? How many are there?

The Autism Society of America (ASA) estimates that there are about 400,000 people in the United States with some form of autism. For years, the generally accepted rate of occurrence was 4b 5 per 10,000, although individual studies sometimes reported higher figures. Recent studies have tended to support a higher prevalence rate, and estimates of 10b 15 per 10,000 people are now widely accepted. This shift may reflect an actual increase in the prevalence of autism, but an alternate possibility is that it reflects other factors such as better identification and modifications in diagnostic criteria. Autism used to be considered a low-incidence disorder. Now it is referred to as one of the most common childhood conditions within the category of developmental disorders.

"He" is the common personal pronoun in books on autism, but that usage does not reflect gender bias. Autism is largely a male disorder; for every autistic girl there are approximately four autistic boys. And yet girls diagnosed as autistic are generally more severely impaired and have lower IQs. At this time there is no explanation for this gender difference, although it may well reflect genetic mechanisms. Another pervasive developmental disorder, Rett syndrome (or Rett's disorder ), is virtually exclusive to females and shares many features with autism, thus possibly reflecting a common underlying neurobiological factor or factors.

Autism is more common in some families than in most others. Studies have shown that the prevalence of autism among siblings of autistic individuals is substantially higher than in the general population, and the concordance rate for autism in identical twins is much higher than in nonidentical twins; if one identical twin has autism it is likely that the other one does as well. Furthermore, parents with possible (mild) autism have been identified in some families of autistic children. These findings strengthen the hypothesis that at least some forms of autism have a genetic base. Even when no other person in a family has autism, there may be a higher than expected prevalence of cognitive and social

impairments that, while less severe than autism, may point to a common genetic base.

Most people, when they have heard anything at all about autism, have a particular image of it that represents only one of the faces of autism. While the full range of variations in autism may not be apparent in early childhood, one difference clearly is: some children with autism appear different almost from birth, while others appear to develop normally for a period of time and then begin to regress. By the time her daughter was one month old, Annabel Stehli (author of Sound of a Miracle ) was sure that something was wrong. Time only added confirmation. Georgie did not grasp her mother's finger or look at her or smile or snuggle against her when she was being held. Judy Barron reported a similar experience with her son Sean, who never seemed to want to be held, squirming, twisting, and pushing against her as if he felt trapped when she picked him up.

A very different infancy was experienced by other parents of autistic children, one in which the sense of loss was excruciatingly exacerbated by development suddenly halted and then reversed.

A clear-eyed boy of nineteen months is pointing to the gaily decorated tree in my living room. "Christmas lights, Daddy," he says as he reaches up to touch the bulbs. "This one is green. This one is blue." Jordan goes through all the colors, carefully pointing out the names of each that he touches. From across the room my wife, Jill, beams as she listens to these first words of the season from our first child. . . .

The here-and-now version of the happy toddler is the seven-year-old boy who, on this day alone, has bitten the school bus driver, flung himself on the floor in a fit of rage in the grocery store, spent nearly half an hour tapping toys on his teeth, and occupied himself in his last waking hour flushing the toilet over twenty times. (Schulze 1993, ib ii)

Autism is usually present within the first year of life, but about one-third of the time it appears to have its onset within the second or third year. However, experts have commented, what is taken to be late onset may sometimes reflect delay in detection owing to parental denial or lack of sophistication. There's no question of delayed detection creating a false appearance of late onset in Jordan Schulze's case. His early devel-

opment was too advanced and its reversal too sharp a contrast. But I think back to something the mother of my student Nellie said to me over thirty years ago. Nellie was doing all right until she went to nursery school, her mother told me, implying that something had happened at nursery school to cause her daughter's autistic behavior. Yet home films viewed by the clinical staff had shown a picture of two-year-old Nellie, before any nursery school experience, hiding behind furniture and people, not looking or smiling at or playing with anyone. A parent trying to account for unexplainable loss.

Why are there such differences in time of onset and the course of development during the first two or three years? The answer to this question will come only when we discover more about the causes of autism.

Perhaps the major factor accounting for the heterogeneity of the population called autistic is the presence or absence of mental retardation. Professionals report that more than half of all children with autism are mentally retarded, but it is often difficult to determine whether a young child with autism is mentally retarded. Aside from the broader issues of the meaning of IQ and the conceptualization of intelligence, how are we to assess intelligence in children who may have no functional speech or any other organized communicative system; who rarely look at people or follow verbal directions or imitate movements?

Occasionally a child will show unusual abilities that seem to give clues to an answer. Some young autistic children are "hyperlexic"b they learn to read at an early age without formal teaching, in spite of a general delay or deviance in language development, although this reading may be largely word naming with little comprehension. Other children may show different kinds of evidence of prodigious rote memory. Astounding abilities in art, music, and calendar calculations have been noted in autistic individuals who otherwise appear to be moderately to severely mentally retarded, and "islets" of competence in such areas as mathematics are common in autistic individuals who otherwise function at a level considered to indicate mild mental retardation. (Sometimes early signs of autism are not recognized because of such islets of competence in the young child.)

IQ in young children does seem to matter in a major way. A performance IQ approaching 70, combined with some functional language by age five, seems to predict a better developmental course for autistic individuals. But autistic children are idiosyncratic; they break all rules. Some autistic children are nonverbal until well beyond age five and are considered to be mentally retarded, and then they begin to communicate and prove everyone wrong. Yet the variability of intelligence among individuals with autism is unquestionable. Some young children with autism make slow and limited progress in learning even with skilled, intensive intervention, while others have gone from treatment programs to challenging educational programs, and on to college studies in mathematics or science.

What distinguishes autism from other pervasive developmental disorders? Young children who do not strictly meet the criteria for autism are often assigned the label PDD, which is a shorthand version of the term "pervasive developmental disorder not otherwise specified" (PDD/NOS ). Essentially what the PDD label indicates is that the child has some of the defining characteristics of autism but not enough of them, or that the child does not have the intensity of symptoms characteristic of autism. Sometimes well-meaning professionals use the term PDD with very young children when they feel that the label "autism" would be premature and they want to protect families from the frightening associations commonly linked to this term. "Autism is the cancer . . . of the developmental disorders," writes a neurologist explaining the reluctance of professionals to use this diagnosis (Rapin 2994, 2), while PDD is presented as a serious, multifaceted problem in development, but one that is not quite as severe as autism. At times the concern that leads to the use of the PDD label can have unintended negative effects, such as leaving parents confused or increasing the difficulty of obtaining intensive early intervention services for the child.

Another reason for use of the PDD label is parents' growing awareness of the importance of early identification of developmental disabilities and the availability of intervention services for young children with disabilities. While it is possible to identify many children with pervasive developmental disorders at eighteen to twenty-four months of age, at

such an age the distinction between autism and PDD/NOS is not always clear.

Childhood disintegrative disorder, or Heller's syndrome, is another pervasive developmental disorder, and it has a very poor prognosis. According to DSM-IV, its essential feature is "a marked regression in multiple areas of functioning following a period of at least two years of apparently normal development." The areas affected include language, social skills or adaptive behavior, play, motor skills, and bowel or bladder control; and this condition is "usually associated with severe mental retardation" (APA 1994, 73, 75). There are no known biological markers that can be used to differentiate childhood disintegrative disorder from autism.

Craig Schulze is an educator with a Ph.D. in human development whose son was diagnosed as autistic, but who came to question this label. In his 1993 book Schulze describes the deterioration of his young sonb "It is as if he has died from one existence and returned in another form" (42)b and his own fruitless struggle to return Jordan to his first life. "Psychologists, neurologists, self-styled gurus, teachers (American and Japanese), relatives and friends, even our own intuitions had joined in a continuing chorus of 'We Shall Overcome.' Now the harmony is gone, and the tune is flat, and the voices are silent" (147).

As a panelist at the 1995 convention of the Autism Society of America, Schulze questioned whether his son has autism or Heller's syndrome. Undoubtedly, this man was giving voice to despair over his son's meager response to all the efforts focused on him, but he was also raising doubt about the differential diagnosis of autism and another pervasive developmental disorder with a worse prognosis.

Higher functioning autism by another name is one way of thinking about Asperger's (or Asperger ) syndrome . Professionals often refer to this condition as a subtype of autism. DSM-IV treats this disorder as a pervasive developmental disorder separate from autism, although the two share some core characteristics, namely severe impairment in social interaction and restricted, repetitive patterns of behavior, interests, and activities. In contrast to autism, Asperger's syndrome is described in DSM-IV as not being marked by severe delays in the development of language, cognition, self-help skills, or curiosity about the environment

in childhood (77). Many children diagnosed with Asperger's disorder or syndrome show delayed motor development and have motor clumsiness that is reflected in difficulty developing such skills as riding a bicycle and catching a ball. They may also display awkwardness or oddness in posture and gait (Klin and Volkmar 1995).

The label "Asperger's syndrome" has recently come into wider use in the United States. Children and adolescents who in the past might have been diagnosed as having emotional disturbance or attention-deficit disorder are now sometimes being labeled or relabeled as having Asperger's syndrome. There also appears to be overlap between children and adolescents identified as having Asperger's syndrome and those identified as having schizoid personality or nonverbal learning disabilities. Individuals with Asperger's syndrome are often "loners." Although most show interest in making friends, their attempts to do so are often unsuccessful. Contributing to such failures are long-winded, one-sided "conversations" about their favorite interests, and insensitivity to other people's feelings and nonverbal communications.

Children with Asperger's syndrome often appear eccentric or emotionally disturbed. In spite of good academic performance, these children seem to lack common sense. This problem may be exacerbated in adolescence, as it was in Rita's case. Rita was not identified as needing special education services when she was a child; academically she functioned on grade level. But in adolescence her behavior appeared to become increasingly age-inappropriate and odd. At age eighteen Rita moved to a group home for adults with disabilities, where the staff tried to help her develop skills she would need to live independently and find work. On one of her weekend trips home Rita appeared to be trying to apply some of the skills she had been taught. She entered a fish store and, after a substantial period of consideration, indicated to the proprietor the particular fish she wanted. After the fish had been weighed and she had been given the price, Rita stated that she was now going home to ask her mother if she could buy it.

Many specialists in autism take the position that Asperger's syndrome is a mild variant of autism, part of a continuum or spectrum of autistic disorders involving impairments in social interaction, communication, and imagination along with rigid, repetitive activity preferences.

A higher than expected frequency of Asperger's syndrome has been found in the families of high-functioning individuals with autism, and Oliver Sacks refers to an entire autistic familyb the gifted parents and older son with Asperger's syndrome, the younger son with classic autismb who, "between the serious business of life," flapped their arms, jumped on a trampoline, and screamed (1995, 244).

If Asperger's syndrome is another name for high-functioning individuals with autism, the balance within the category of autism is tipped toward individuals with better language and academic achievement or potential. This would have "political" implications; it could influence the kind of behavioral research that is funded and the kinds of treatment and support services developed. A mother addresses this issue in her letter to the Advocate , the newsletter of the Autism Society of America. "As a mother of a severely-autistic self-injurious child, I am saddened by the continual neglect of the most severe cases of autismb and the increasingly dangerous glorification of the so-called high-functioning-curable-autistics" (Gilbert 1995, 4).

This mother raises another issue about classification and labeling, one that is not likely to be answerable until the condition that we now call autism is better understood. She questions whether high-functioning autistic individuals should be grouped with other autistic individuals whose behavior and prognosis is quite different. "It is about time that we start separating the primitive autistics who spend the majority of their day smashing their heads into the wall and biting themselves from the ever so interesting savant group that impresses Oprah Winfrey by playing Chopin." But are these really two distinct conditions or groups, or are they different points on a continuum? Do they have the same etiology, or are the underlying factors different, or is it a matter of a single impairment versus that same impairment plus others? What role does the nature of early environmental experience, including planned treatment, play in these differences? The answers to these questions are still unknown.

Questions can also be raised about the differential diagnosis of autism and other disabilities outside the spectrum of pervasive developmental disorders that may mimic the appearance of autism. Labels indicating

categories of disability are most useful when all individuals identified by a particular label exhibit the defining characteristics of that category, when there are clear boundaries between categories, and when an individual who fits into one such category will not meet the criteria for another one. Unfortunately, autism eludes this framework. Autism should be viewed as a working label. When more is known about the cause or causes of this disorder, the label autism may be replaced by several labels, or the way in which we think about this syndrome and related developmental disabilities may be dramatically altered.

Landau-Kleffner syndrome is a disorder that may mimic autism in some ways. The outstanding feature of this syndrome is loss of language after a period of normal development, usually of more than two years and sometimes as long as seven years. On March 9, 1995, an ABC news program called Day One included a segment on Landau-Kleffner syndrome. For weeks after that segment was shown, Thomas Jefferson University Hospital in Philadelphia was overwhelmed with telephone calls from parents for Gerry Stefanatos, director of the Center for Clinical and Developmental Neurophysiology, who had been featured in the television program. What had mobilized these parents was a rekindling of hope for the children lost to autism of late onset, children who had been developing as normally expected until perhaps sixteen, eighteen, or twenty-two months of age, when their development seemed to change course. Babies stopped using the words they had already acquired, stopped acquiring new words, stopped trying to communicate through smiles or gestures that they wanted to be picked up or played with, stopped snuggling up to their mothers to receive hugs and kisses, and stopped offering any of their own.

What parents heard and saw on the Day One program that gave them hope was a boy who had been labeled as autistic, but who was apparently helped to regain his developmental momentum after being rediagnosed as having Landau-Kleffner syndrome and treated for it. At around age two, T.J. Voeltz had "stopped talking and began to disappear" into his own world. His mother was told that there was nothing medicine could do for her son, who had incurable brain damage; she needed to get on with her life. But Kathy Voeltz did not heed these

words, and eventually at a conference on autism she heard a speaker say that anyone who knew a child who used to talk should look into Landau-Kleffner syndrome. She did. After testing, her son's diagnosis was changed, and he was treated with the drug ACTH. Two years later, eight-year-old T.J. Voeltz was in a second-grade class. He spoke; he played; he learned. He could do most of the things that a boy his age is expected to do. His parents believe that several months of treatment had given T.J. a second chance at a normal life. It is what thousands of parents of autistic children have dreamed of.

To parents, the saving grace of Landau-Kleffner syndrome is that medication can sometimes reverse its downward course, whereas no medication has been identified as effective in reversing the core features of autism. But we might question whether the steroid treatment used with T.J. did, in fact, cause his recovery: Landau-Kleffner syndrome is an odd condition; fluctuating progression and spontaneous remissions have sometimes been noted by physicians, and only controlled clinical studies will provide a definitive answer to this question. An even more basic issue has to do with the boundary between autism and Landau-Kleffner syndrome: are these totally distinct conditions, or can their boundaries overlap?

Landau-Kleffner syndrome, identified in 1957, has been considered a condition of very low incidence. As of the early 1990s the literature was still referring to "more than 170" reported cases of this syndrome, which is also referred to as acquired epileptic aphasia. Epilepsy is present in Landau-Kleffner syndrome about 70 percent of the time, and abnormalities in brain-wave patterns are central to the diagnosis of this condition. What complicates the differential diagnosis of late-onset autism and Landau-Kleffner syndrome is the fact that children with autism also have higher than normal rates of epilepsy and abnormal electroencephalograms (EEGs). Seizure disorders have been identified in 25b 40 percent of individuals with autism by early adulthood, with two peak periods of incidence, one in early childhood and one in adolescence (Lord and Rutter 1994). Furthermore, many children diagnosed with autism have not had EEGs; and even those who have had an EEG may not have had an extended-sleep EEG, which is often essential for detection of

the typical pattern of EEG abnormalities in Landau-Kleffner syndrome. Differentiation between autism and Landau-Kleffner syndrome may also be difficult because behavioral disturbances common to autismb for example, resistance to change and withdrawalb are sometimes found in children with Landau-Kleffner syndrome. Thus, it is possible that this condition is more prevalent than previously considered, and that a number of children diagnosed as having autism of late onset meet the criteria for Landau-Kleffner syndrome.

What are the prospects for successful treatment of children with Landau-Kleffner syndrome, and what does "successful treatment" mean in this context? Landau-Kleffner syndrome is usually treated with anti-convulsant medications and ACTH or corticosteroids such as prednisone. The number of children who have been treated is probably too small to yield a reliable estimate of successful outcomes, however we define such outcomes. The reported outcomes range from complete, dramatic remission of symptomsb including speech loss, poor language comprehension, and abnormal EEGsb to partial recovery with mild language disorder and continuing cognitive impairment. A surgical procedure, multiple subpial transection (brain surgery), has also been available since about 1990 for use in treating this disorder when medication is not effective. Neither treatment is to be undertaken lightly. Steroids have significant negative side effects, and brain surgery always involves serious risk.

Gerry Stefanatos and associates recently used prednisone to treat a six-year-old boy who had experienced severe regression in language and behavior at twenty-two months, and who had been labeled as PDD/NOS. This boy did not have the EEG pattern associated with Landau-Kleffner syndrome, although other signs pointed in that direction. Stefanatos reported that within several weeks the boy's spontaneous speech and social relatedness had increased significantly.

In some cases, months after apparently successful drug treatment was discontinued, abnormal EEGs and progressive language loss reappeared in children with Landau-Kleffner syndrome but went into remission when treatment was promptly reinstituted. Both the speed and extent of symptom remission appear related to the length of time from the

child's first symptoms to the initial treatment, as well as the adequacy of medication dosage and treatment length. Thus, Landau-Kleffner syndrome should be considered whenever a young child presents with what appears to be autism of late onset, and appropriate testing should be implemented to rule out this condition before a diagnosis of autism is confirmed.

Another condition that shares some distinctive features with autism is obsessive-compulsive disorder. The rituals of obsessive-compulsive individuals sometimes cannot be distinguished from the rituals and repetitive, fixed routines often found in children and adults with autism. Jeffrey's father describes his four-year-old son's behavior:

I went to father's night at my Jeffrey's pre-school. He was playing with a Fisher-Price toy, a schoolhouse, but his play was strange. He stood before the toy, jumped up and down, and flapped his arms as if excited by it. . . . When the jumping stopped, he would put his arms together and wiggle his fingers just above eye level. . . . He did this for 35 minutes. (Rapoport 1989, 29b 30)

The description of Jeffrey's stereotyped behavior makes it easy to begin thinking of autism, but Jeffrey did not have the severe difficulties in communication that are characteristic of autism. In this way he was more like a child with Asperger's syndrome, but that label didn't fit either because Jeffrey did not have a deficit in reciprocal social interaction. He was not only affectionate toward his parents, as are some young autistic children, but he also tried to act in ways that pleased them. "Mommy, why do I play with strings?" asked this child who would dangle strings in front of his eyes for four hours a day (31). Jeffrey was diagnosed as having obsessive-compulsive disorder, and his stereotyped behavior was controlled with medication.

A father who has guided the care of his severely disabled son for over thirty years said to me, "Yes, my son has autism, but that's just one of his problems." To understand what that father meant we have to

consider the concept of comorbidityb co-occurring conditions or behavioral symptoms.

Fragile X syndrome is a disorder defined by a particular genetic marker, namely a fragile site on the X chromosome. This syndrome is associated with mental retardation, and with social and communicative dysfunctions characteristic of autism. The prevalence of fragile X syndrome in the autistic population is higher than would be expected by chance alone.

Walter, the young man whose behavior at a conference reception caught my attention, has fragile X syndrome, a fact that his mother learned only after he was given the simple screening test for this condition that has been available since the early 1990s. As a child, Walter was diagnosed as having autism but no other condition was identified. Today, Walter is considered a man with fragile X syndrome and autism, or fragile X syndrome with autistic characteristics. Little in his life changed because of this revision in his diagnosis after he reached adulthood. He continues to work in a supported employment program operated by an autism center and his relationship to his family remains the same. What does now concern Waiter's mother is whether her daughter may be a carrier of fragile X syndrome and could transmit this genetic disorder to any children she may have.

Several other conditions are comorbid with autism, epilepsy among them. These conditions are not found in all individuals with autism, but they do occur at much higher rates than in the general population. From a parent's point of view such comorbidity implies the likelihood of more difficulties and a less optimistic developmental outlook. For the researcher comorbidity is a clue in the search for the causes of autism.

And so, after examining conditions difficult to differentiate from autism, associated with autism, and overlapping with autism we come to the question, what causes autism? As Marie Bristol, a speaker from the National Institutes of Health (NIH) said at a 1996 conference when she addressed this question, "The short answer is that we don't know." But, she added, we have some good hunches. We do know that autism does not come from something bad that parents did to their children, but it

may come from parents in a different way. One of the strong hunches among researchers today is that autism (or some subcategories of it) is a genetic disorder, or that a susceptibility to autism may be inherited and may be triggered by a variety of factors. Autism also appears to reflect a combination of abnormalities in both structural and functional aspects of the nervous system. Researchers are hard at work trying to identify these abnormalities and locate the genetic factors that may underlie them. We do not yet know the cause or causes of autism, but answers to critical issues surrounding its origins are beginning to emerge.

Continues...

Excerpted from Targeting Autismby Shirley Cohen Copyright © 2002 by Shirley Cohen. Excerpted by permission.
All rights reserved. No part of this excerpt may be reproduced or reprinted without permission in writing from the publisher.
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Edition details

• Publisher: University of California Press
• Publication date: 2002
• Language: English
• ISBN 10: 0520234804
• ISBN 13: 9780520234802
• Binding: Paperback
• Edition number: 1
• Number of pages: 238