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Why Suspected Childhood Apraxia of Speech Requires Careful Assessment

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Recently I got one of those phone calls that speech-language pathologists often dread. It went something like this:

Parent: Hi. I am looking for a speech therapist who uses PROMPT [Prompts for Restructuring Oral Muscular Phonetic Targets] to treat my son’s childhood apraxia of speech. Are you PROMPT-certified?

Me: I am PROMPT-trained and I do treat motor speech disorders but perhaps you can first tell me a little bit about your child? What is his age? What type of speech difficulties does he have? Who diagnosed him and recommended the treatment?

Parent: He is turning 3. He was diagnosed by a neurodevelopmental pediatrician a few weeks ago. She recommended speech therapy four times a week for 30 minutes, using PROMPT.

Me: And what did the speech therapy evaluation reveal?

Parent: We did not do a speech therapy evaluation yet.

Sadly, I get these types of phone calls at least once a month. Frantic parents of toddlers ages 18 months to 3+ years call to inquire about PROMPT therapy based on a neurodevelopmental pediatrician’s diagnosis. The speech-language diagnosis, method of treatment and treatment were typically specified by the physician in the absence of a comprehensive speech language evaluation and/or past speech-language therapy treatments.

The conversation that follows is often uncomfortable. I listen to the parent’s description of the symptoms and explain that the child needs a comprehensive speech language assessment by a certified SLP before being treated. I explain to the parent that, depending on the child’s age and the findings, the assessment may or may not substantiate CAS because symptoms are similar in a number of other speech and communication disorders.

Parents react in a number of ways. Some hurriedly thank me for my time and resoundingly hang up. Some stay on the line and ask me detailed questions. Some request an evaluation and become clients. A number of them find that their child never had CAS! Past misdiagnoses have ranged from autism spectrum disorder (CAS was suspected because of imprecise speech and excessive jargon) to severe phonological disorder to dysarthria secondary to cerebral palsy.

CAS is a disorder that disrupts speech motor control and creates difficulty with volitional, intelligible speech production. Research indicates that while children with CAS have difficulty forming words and sentences at the speech level, they also struggle with areas of receptive and expressive language. In other words, “pure” apraxia of speech is rare.

This condition needs to be diagnosed by an SLP. In fact, due to the disorder’s complexity, it is strongly recommended that parents seek an assessment by an SLP specializing in assessment and treatment of motor speech disorders. Here’s why.

  • CAS has a number of overlapping symptoms with other speech sound disorders, such as severe phonological disorder and dysarthria.
  • Symptoms that may initially appear as CAS may change during the course of intervention, which is why diagnosing toddlers under 3 years of age is problematic. Instead, a “suspected” or “working” diagnosis is recommended in order to avoid misdiagnosis.
  • Diagnosis of CAS is nuanced, complex and challenging, though a new instrument—Dynamic Evaluation of Motor Speech Skill (DEMSS)—shows promise with respect to differential diagnosis of severe speech impairments in children.

When children with less severe impairments, SLPs need to determine where the breakdown is taking place by designing tasks assessing:

  • Automatic versus volitional control.
  • Simple versus complex speech productions.
  • Consistency of productions on repetitions of same word.
  • Vowel productions.
  • Imitation abilities.
  • Prosody.
  • Phonetic inventory before and after intervention.
  • Types and levels of cuing required for response.

Given the complexity of CAS assessment and treatment described here, you can see that the PROMPT approach may not even be applicable to some children. Thus, I strongly urge developmental clinicians to first refer a child for a speech language assessment—and refrain from making recommendations for specific types and frequencies of treatment—when difficulty with speech production is observed.

source;http://blog.asha.org/

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Apraxia: the rise, fall and resurrection to explain how the brain works

The purpose of brains is to make movement. A brain has to identify what is going on in the environment and in its attached body and generate movements to stay alive and procreate to keep the species going. Movements of a person, which includes speech, are all that other people know of another person. The repertoire of movements that people can make is very broad, and even most ordinary movements are complex. Eating and dressing, for example, involve multiple body parts making a series of coordinated multi-joint movements. The movements of highly skilled athletes and musicians are so well tuned that most persons cannot match them. These complex movements that form the behaviour of everyday life are praxis movements. Their failure is apraxia. To call such a failure apraxia, of course, requires that the deficit cannot be explained by a more basic abnormality such as weakness, sensory loss, ataxia or aphasia. The range of capability of the brain to make movement is so large that there can be many types of failures. This has generated different classification schemes with a variety of different terms, sometimes with the same term meaning something else in a different classification.

The book by Goldenberg on apraxia contains a comprehensive description of different manifestations of apraxia and addresses the different classifications. Chapters 6 through 14 deal with topics such as imitation, body part specificity, and use of single tools. Chapters 1 to 5 give the historical background and set the stage for the rest of the book. I found the historical part particularly interesting, both as relates to apraxia but also ideas as to how the brain works.

While there were some historical precedents, the first well described cases of apraxia and pathological explanation were from Hugo Liepmann from 1900 to 1908 (Fig. 1). On the basis of detailed clinical assessment, Liepmann predicted brain lesions that were subsequently verified. After a series of cases, he came up with a theory of praxis and a classification of apraxia that arose from that theory. The movement formula was generated in the region of the occipital and parietal cortices and kinetic memories were in the region of the motor cortex, and movement was generated by a signal from posterior to anterior. Damage in the posterior region produced ideational apraxia, in the anterior region, limb-kinetic apraxia, and damage to the connection between them, ideo-kinetic apraxia. Similar ideas were coming from the early studies of aphasia such as those of Wernicke. Manuscripts of the day included diagrams with relevant brain regions and their connections. This all seemed rather successful until the attack from a distinguished series of British neurologists that reached its peak with Henry Head.

Figure1

Head’s frontal attack on the ‘diagram makers’ came in the Hughlings Jackson Lecture for 1920, mainly dealing with aphasia, published in Brain in 1921 (Head, 1921). I looked back at that paper, and some quotations from that lecture make clear the strong feelings that he had.‘Bastian published his well-known paper in 1869 which had such an evil influence on the subsequent course of the discussion. He started from the a priori assumption that we think in words, and that words are revived in the cerebral hemispheres as remembered sounds. He talked of lesions of special fibres and centres, and set the points on the catastrophic road to schemas and diagrams.No one could write on aphasia without producing a new diagram of centres and the paths between them. Each author twisted the clinical facts to suit the lesions he had deduced from his pet schema.

Figure 1

The ‘imperial counselor’ was one of the first patients with apraxia to be described by Liepmann.When asked to pour water from a jug into a glass, the man’s left hand duly picked up the jug in order to pour, but the right hand interfered with the action, moving the empty glass to the mouth instead. The left hand then followed the right, bringing the jug to the mouth too. Reproduced from Liepmann, H., Das Krankheitsbild der Apraxie (motorische Asymbolie) auf Grund eines Falles von einseitiger Apraxie,Monatschrift für Psychiatrie und Neurologie, 8, p. 34 © 1900, Karger, with permission.

How far the writers of this period were compelled to lop and twist the clinical facts to fit the procrustian bed of their hypothetical conceptions is shown by the famous case published by Wernicke in 1903 as “A Case of Isolated Agraphia”.’

Head strongly supported the ideas of Jackson in this lecture with the notion of higher and lower levels of brain function, but that the brain was functioning holistically. It was not possible to identify brain centres for specific functions.‘We know that speech can be affected by destruction of the substance of the brain, but this does not show that “the faculty of speech” is localized in any area of the cortex. We should as soon expect a special centre for eating as for speech; both are complex acts which do not correspond to any specific group of functions. No lesion, however local, can affect speech and speech only.’

Despite Head recognizing that such a view wouldn’t make it easy for neurologists, the view prevailed to a large extent. However, basic science moved on gathering evidence for localization of function in the brain, and the tide was turned in neurology, also on the pages of Brain by Norman Geschwind in his important two-part paper on ‘Disconnexion syndromes in animals and man’ in 1965. As a quick personal aside, Geschwind and these papers were important in my own career. I was a freshman medical student in 1965, and Geschwind was a popular and influential lecturer at the time. I worked with Hubel and Wiesel in the summer of 1966 and introduced them to these articles, which they were impressed with. I then spent the summer of 1967 with Geschwind. Part I begins (Geschwind, 1965a):‘THE early successes of the views of Broca and Wernicke led the classical neurologists to a mode of analysis of the disturbances of the higher neurological functions subsequently to be labelled with the derisive term “diagram-making”. Starting from the picture of the brain as a collection of sets of more or less specialized groups of cells connected by relatively discrete fibre pathways, these classical neurologists deduced a series of symptom complexes.On the whole the period between the wars seems to have led to a loss of interest in analyses in terms of disconnexion. The criticisms of the holistically oriented neurologists, Head, Marie, von Monakow, and Goldstein probably contributed heavily to this decline of interest. The growth of holistic psychology under the Gestalt school and Karl Lashley and the rapid development of holistic schools of psychiatry probably all played a role, perhaps more by their effects on the general atmosphere of thought than by their specific critiques of the classical school.’

Geschwind then summarizes a huge body of animal and human data in support of localization of function including the importance of the connections between them in order to move information from node to node and carry out complex brain functions. He concludes with (Geschwind, 1965b):‘For the past forty years there have been schools of thought which have stressed the importance of thinking of the patient as a whole, of seeing his responses as those of an integrated unitary structure, even in the face of damage. The ramifications of this thinking in neurology, psychiatry, psychology and other fields must be well known to most readers. It should be clear from much of our discussion that this principle, while it may be useful in some cases as a stimulus, may be actively misleading when it is regarded as a philosophical law.’

Geschwind, as a neurologist in training, went to Queen Square to study higher cortical function with McDonald Critchley. He was so turned off by Critchley’s approach that he spent his time with Ian Simpson studying muscle disease. Perhaps it was these issues in part that led to his feelings.

This important battle in the history of neuroscience and neurology was played out on the pages of Brain, and has been won by Geschwind. These days, the localization of function and brain connectivity continue to be hot topics. MRI and EEG/MEG, as well as basic animal studies, are diving more deeply into anatomical and physiological connections with the development of progressively better techniques and mathematical tools such as graph theory.

Back to the book, in Chapters 6 to 14, the different clinical features are related to associated brain lesions, either by pathology or MRI. This core of the book is a valuable summary of the data. The story, however, remains complex, and, for example, post-publication is another large series with clinical–MRI correlation recently published in Brain (Buxbaum et al., 2014), with a commentary by Goldenberg (2014), giving rise to a slightly different model of different types of apraxia.

Chapter 16 reviews the sparse data on therapy of apraxia, and is a reminder that this area needs more attention. Chapter 15 is Goldenberg’s own conclusion about the physiology of apraxia. While recognizing specifically that all brain function must have an anatomical correlate, he appears to support the general Jackson-Head concept of higher and lower aspects of motor control. Higher is cognitive and lower is motor, and thus the title of the book, as the ‘cognitive side of motor control’. Is indeed the brain hierarchical in function or does it work as a synergistic whole with each piece playing its part? I think Goldenberg would like to find the mind in the brain (the cognitive side), and perhaps many others would like that too

source;http://brain.oxfordjournals.org/

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Sign language may be highly effective in treating children with apraxia of speech

Using sign language with intensive speech therapy may be an effective treatment for children with a rare speech disorder called apraxia of speech, according to Penn State College of Medicine researchers. They suggest further exploration of the results of a case study showing the effectiveness of using several therapies together in cases of early diagnosis.

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Childhood apraxia of speech occurs when children have difficulty saying words or sounds because the muscles involved do not coordinate properly. It is generally diagnosed in children at about two-and-a-half years of age and confirmed at age three or later. In the case study, the child was diagnosed earlier at 18 months.

“Because early intervention is beneficial in other developmental conditions, we wondered if a similar approach might also yield better progress in a child thought to have apraxia,” said Dr. Cheryl Tierney, associate professor of pediatrics. “Very little is scientifically proven when it comes to ‘best practices’ but there is a growing body of literature that is helping to guide treatment for this rare speech sound disorder.”

The child in the case study was taught sign language as a way to communicate while his verbal speech developed. While research has been done about using alternative methods of communicating while speech develops – like devices that provide an electronic voice – none has been done on using sign language exclusively.

“There is a common misperception that if you teach a child sign language or give them a device to speak for them that this will slow down their progress when learning to speak using their voice,” Tierney said.

Previous research has shown, however, that sign language can be a way to encourage attempts at verbal speech and reduce frustration.

In the case study, the patient underwent intensive speech therapy using two known therapeutic programs – one to develop mouth muscle control and the other to develop sounds. His mother was also given a home program to use to help with the patient’s tongue movement. Before the treatment, he had limited verbal communication skills. After treatment, he was able to hold conversations with his parents, who reported understanding at least 90 percent of what he said. His speech therapist understood at least 80 percent of what was said. The patient discontinued use of sign language on his own as his speech developed. Researchers reported the results in Pediatrics.

“We suspect that early introduction of sign language by the family proved to be a highly effective form of language development that, when used with sound therapy and therapy to improve the functioning of the mouth muscles, helped correct speech issues quickly,” Tierney said. “More research is needed to determine how much the use of sign language contributed to such rapid correction of apraxia of speech. However, our case highlighted that when we combined early detection, early treatment and the use of sign language we had an optimal outcome which suggests an area of further study.”

Future studies should be designed to determine which children may respond best to early intervention, use of several treatment methods at the same time and the use of sign language and other alternative communication techniques to promote more rapid resolution of symptoms.

source;http://www.news-medical.net/

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Autism and apraxia: the importance of screening for both

Researchers find otherwise rare speech disorder affects nearly 65 percent of children with autism; call for screening and treatment

A new study finds that the relatively rare speech disorder apraxia affects nearly 65 percent of children with autism. The finding is important because apraxia warrants a specific type of therapy not otherwise part of an autism intervention program.

“Children with apraxia have difficulty coordinating the use of their tongue, lips, mouth and jaw to accurately produce speech sounds, so that each time they say the same word, it comes out differently, and even their parents have difficulty understanding them,” explains developmental behavioral pediatrician Cheryl Tierney, of the Pennsylvania College of Medicine.

Dr. Tierney co-authored the new report, in the Journal of Developmental and Behavioral Pediatrics In it, she and her colleagues emphasize the importance of keeping both conditions in mind when evaluating a child for either one.

The researchers assessed 30 children, ages 15 months to 5 years, seen at their developmental communication clinic. Their follow-up testing showed that 64 percent of the children initially diagnosed with autism also had apraxia, and 37 percent of the children initially diagnosed with apraxia also had autism. By contrast, apraxia occurs in just 1 or 2 out of 1,000 children in the general population. Autism affects 1 in 68.

Developmental experts have long noted autism and apraxia frequently coincide. The new study, though small, underscores just how commonly this overlap may occur.

Both conditions can be improved with early intervention, though each warrants a different intervention. In particular, the researchers emphasize that nonverbal children diagnosed with either autism or apraxia should continue to be screened for the other condition until they start talking.

“Children with autism frequently present with communication challenges including delayed speech and language development,” notes speech-language pathologist Donna Murray, senior director of the Autism Speaks Autism Treatment Network (ATN). “Speech-language pathologists are trained to identify the signs and symptoms of apraxia and will be able to assist families of children with autism in understanding the nature of their child’s communication delays and develop an intervention plan to treat apraxia if needed.”

 

source;http://www.autismspeaks.org
Jennifer-Linck-the-mighty-05142015

My Son’s Apraxia Journey Taught Me Love Needs No Words

May 14 is Apraxia Awareness Day, a day to advocate and educate others about the speech disorder that steals the voices of so many kiddos, including our little guy.

I guess you could say we’ve been on our apraxia journey since Jackson was 18 months old; we just didn’t know what to call it back then. We knew he was behind in his expressive language, but we had no doubt he’d catch up — after all, he was a very busy little boy. And he understood everything we said to him.

When we started speech therapy, I prayed the words would come, but they didn’t. In January, we learned Jackson had childhood apraxia of speech (CAS), which is a motor speech disorder.

He knows what he wants to say but he can’t. Simply put, his brain and mouth have a hard time working together, and his words get stuck in his head.

This journey has been filled with joy and pain; fear and hope; and laughter and tears. It has strengthened my faith and made me believe a little more in miracles, because I see small ones every single day.

Each word is a miracle. 

A child with apraxia might have to hear a word approximately 3,000 times before it becomes part of their vocabulary. Our life is a constant game of repeat.Everything we do is a speech therapy session (we just don’t let Jackson know it).

I’ve learned there will be people who’ll never understand the hard stuff we face on a daily basis. They will blow it off, they will say heartbreaking things, they won’t understand why we parent the way we do.

There will be preschool directors who deem our kid difficult.

There will be church nursery workers who humiliate us in the hall.

There will be people who want to label him and give up.

But I’m slowly learning that it’s OK, because there are a handful of people — people walking alongside us — fighting for Jackson and loving him just was much as his dad and I do. They understand when his frustration causes him to hit. They listen when I just need to cry. They see past the lack of words for the amazing little boy that he is. They cheer him on and celebrate each miracle with us.

It’s the friend who whispers, “It’s OK,” and I see understanding in her eyes.

It’s the 13-year-old who rounds up her friends to wear blue in support of #TeamJackson.

It’s the mom sitting next to me in the speech therapy office who speaks to Jackson each day. The mom who I now call a friend.

It’s the grandmother Googling apraxia.

It’s the speech therapists who don’t give up.

It’s the church volunteer who encourages Jackson each Sunday while John and I worship without worry.

These are the people we want on our team. These are the people we cherish.

We are extremely grateful for our speech therapists who are helping Jackson find his voice; without them he wouldn’t overcome apraxia. Jackson looks forward to seeing Ms. Sarah and Ms. Angela each week. They are amazing advocates and incredible at what they do.

When you see your therapists four times a week, they become more than a therapist, they become part of your team. They become friends.

This journey has taught me that love needs no words.

Love is those big brown eyes sparkling. It’s his pouty lips kissing mine. It’s his little arms wrapped tightly around my neck.

These moments between my son and me are just as powerful as hearing those three little words fall from his lips.

I’ve been entrusted with this amazing little boy. I am honored to be his voice and to educate others about apraxia. This is just another chapter of our story, and it’s my prayer that through our experiences you will see his strength. That through the silence, you will hear his voice.

source;http://themighty.com