OT re-evaluation | Autism PDD

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My daughter's OT just did a re-evaluation and the report says she has agraphia, apraxia and hypotonia.  This is the first time I've seen those words in a report.  I looked up their meanings in Wikipedia and they all sound pretty dramatic.  Does anybody else have children described in this way?  Will any of this get better?

My son is apraxic and also mildly hypotonic in addition to being PDD-NOS.  He is 3 years and a few months.  Apraxia and hypotonia can go hand and hand...sometimes a therapist will call the two of them together dysphasia???  I think.... I don't want to google it right now, LOL.  I'm sure somebody will come along and correct me.  But if you google apraxia you will come up with several different types and there is one that affects different parts of the body besides just oral and speech.  Possibly this is where your OT is getting those two dx's from. 

Yes, these conditions can be intimidating and scary.  My sons apraxia actually scares me more than his autism.  He is progressing by leaps and bounds in the autism area, but in the speech articulation area, not so much.  He gets roughly 4 hours a week of speech between school and private, most of it 1x1 and including oral motor and we will be doing this for a long, long, long time I'm sure.  However, he IS talking alot, making sense and somewhat understandable to us (although not so much to strangers).  The hypotonia we are just starting PT for now so I don't know too much about it.  Only that for my son it seems to be a mild case so I'm not sure yet what they will do.

Did you ask your OT about these terms and for an explanation after you got the report?  Did she have any recommendations as far as therapy and exactly what you should do?

AGRAPHIA

AGRAPHIA
Disorders of Writing & Spelling

The inability and the loss of the ability to write and spell (when writing), is referred to as "agraphia" i.e. an inabiliy to form graphemes. Agraphia is a neurological disorder involving the loss of the ability to write.

The Neurology of Writing

There are a number of brain areas which interact and which are responsible for various aspects of the ability to write. Likewise, there are a number of theories which have been proposed to explain the ability to write, and the loss of writing ability.

Broadly considered, the principle structures include the left frontal lobe (Exner's Writing Area and Broca's Expressive Speech area), the left temporal lobe (Wernicke's receptive speech area), and the superior and inferior parietal lobe. Exner's and Broca's area are implicated in the expressive aspects of writing, whereas the temporal and parietal lobe are involved in the comprehension of written words. However, the parietal lobe is also believed to program the frontal motor areas and to supply the anterior region of the brain with the grapheme equivalents of auditory language; i.e. converting or visual images sounds into written symbols.

Presumably the parietal lobe constructs the written-word images (probably via interaction with Wernicke's area) which and assists in converting these into graphemes. These motor-graphemes (or written word/letter images) are then transmitted to the left frontal convexity (i.e. Broca's and Exner's area) for grapheme conversion and motoric expression.

It has also been proposed that there are at least two stages involved in the act of writing: a linguistic stage and a motor-expressive-praxic stage. The linguistic stage involves the encoding of auditory and visual information into syntactical-lexical units--the symbols for letters and written words. This is mediated through the angular gyrus which thus provides the linguistic rules which subserve writing. The motor stage is the final step in which the expression of graphemes is subserved. This stage is mediated presumably by Exner's writing area (located in the left frontal convexity) in conjunction with the inferior parietal lobule.

Because different regions of the brain contribute to the ability to write, damage to these different areas, therefore, affect different aspects of the ability to write. Thus, there are different subtypes of agaphia, depending on which areas of the brain have been damaged. These subtypes are referred to as Frontal Agraphia, Pure Agraphia, Alexic Agraphia, Apraxic Agraphia, and Spatial Agraphia.

EXNER'S WRITING AREA

Exner's Writing Area is located within a small area along the lateral convexity of the left frontal lobe, and is adjacent to Broca's expressive speech area, and the primary and secondary areas controlling the movement of the hand and fine finger movements.

Exner's area appears to be the final common pathway where linguistic impulses receive a final motoric stamp for the purposes of writing. That is, Exner's area translates auditory-images transferred from the posterior language areas, into those motor impulses that will form written words and sentences.

Exner's area is very dependent on Broca's area with which is maintains extensive interconnections. That is, Broca's area also acts to organize impulses received from the posterior language zones and relays them to Exner's area for the purposes of written expression.

FRONTAL LOBE AGRAPHIA

Lesions localized to the left frontal lobe and Exner's and Broca's area typically result in disturbances in the elementary motoric aspects of writing, i.e. Frontal Agraphia. Frontal lobe agraphia, however, is sometimes referred to as Pure Agraphia. On the other hand, pure Agraphia has also been attributed to left parietal lesions (Basso et al. 1978; Strub & Geschwind, 1983). The similarity in symptoms is probably due to the fact that the inferior parietal lobe (IPL) transmits auditory-motor impulses to Broca's and Exner's area, and assists in programing sensory-motor movements.

In general, with frontal agraphia, grapheme (letter) formation becomes labored, incoordinated, and takes on a very sloppy appearance. Cursive handwriting is usually more disturbed than printing, as cursive handwriting requires additional fine motor control. Cursive handwriting is also acquired at a later age, and is thus more likely to be more severely disrupted than printing which is (comparatively) an older and more ingrained skill.

With frontal agraphia, the ability to spell is often affected. Patient's may not be able to spell correctly even if given block letters--particularly if the left IPL is also damaged.

However, with frontal agraphia, primarily, it is the ability to properly form letters and words that is disrupted.

Predominantly, with frontal lesions, and thus frontal agraphia, there is a disturances in grapheme selection. That is, the wrong letters may be chosen, and the patient may seem to have "forgotten" how to form certain letters. They may write the wrong letter--which in turn makes it appear that they have forgotten how to spell. Those with frontal agraphia may also abnormally sequence or even add unnecessary letters when writing.

In general, with a left frontal lesion, spelling and writing with both the right and left hand is severely affected, and the left hand may be even more profoundly affected.

Patients with frontal agraphia, particularly if Broca's area is damaged, are unable to write spontaneously or to dictation. Writing samples are contaminated with perseverations (such that they write the same letter over and over) or the addition of extra strokes to letters (e.g. such as when writing an "m"=mmm).

Patients with frontal agraphia may be unable to even write their name.

PURE (PARIETAL LOBE) AGRAPHA

Pure agraphia is associated with frontal lesions, but is most commonly secondary to damage involving the superior and inferior parietal lobe (IPL). The IPL sits and the junction of the frontal, parietal, occipital, and temporal lobe, and assimilates and integrates complex auditory, visual, motor and tactile sensations so as to form those multi-modal images and concepts which are integral to the comprehension and expression of human language.

With IPL damage, patients have difficulty programming those movements necessary to form written words, such that there is a loss of the ability select, form, and express, in writing, words and sentences.

Patients with pure (parietal lobe) agraphia frequently misspell words, and may insert the wrong letters or place them in the wrong order or sequence when attempting to write. In contrast, reading, oral speech and the ability to name objects or letters are usually unimpaired.

Most commonly, pure agraphia is associated with lesions involving the superior and mid parietal regions of the left hemisphere; areas, 5 and 7 (Basso, et al. 1978; Vignolo, 1983), and/or the inferior parietal region (Strub & Geschwind, 1983). Patients are unable to write because the area involved in organizing visual-letter organization (i.e. the inferior parietal lobe) is cut off from the region controlling hand movements in the frontal lobe (Strub & Geschwind, 1983). However, according to Vignolo (1983), the posterior superior left parietal lobule (area 7) is crucial for the sensorimotor linguistic integration needed for writing.

It has also been argued that the sensory motor engrams necessary for the production and perception of written language are stored within the inferior parietal lobule of the left hemisphere. When this region is damaged, patients sometimes have difficulty forming letters due to an inability to access these engrams (Strub & Geschwind, 1983). Writing samples, therefore, are characterized by mispellings, letter ommissions, distortions, temporal-sequential misplacements, and inversions.

ALEXIC-AGRAPHIA

Alexic agraphia is a disturbance involving the ability to read (alexia) and to write (agraphia). Thus alexic-agraphia is a disturbance in the ability to decode and to encode written language.

Reading and writing may not be equally affected. Some patients may be able to recognize written letters, but are unable to form them. Some patients can form the letters, but are then unable to read them. However, even those who are able to read the letters or words they cannot write, began having difficulty as the words become longer or less familiar.

Patients with alexic-agraphia have generally have difficulty with spelling, even when block letters are employed. Thus the ability to spell, per se, is disrupted. (Marcie & Heilman, 1979).

Often alexia-agraphia is due to a lesion involving the left inferior parietal lobule and angular gyrus (Benson & Geschwind, 1969; Hecaen & Kremin, 1977). Because alexic-agrahia is associated with parietal lesions, it is also referred to as parietal lobe alexia. That is, rather than referred to as alexic agraphia, as patients who cannot spell are unable to write appropriately by definition, this condition could simply be referred to as alexia. However, what distinguishes alexic agraphia from alexia, is the disturbance of motor control which is not characteristic of alexia per se. That is, alexia is a loss of the ability to read, and is not related to motor abnormalities, as is the case with agraphia and alexic-agraphia.

APRAXIC AGRAPHIA

When a patient has difficulty sequencing their movements or performing skilled movements involving a series of steps, the disorder is referred to as Apraxia. There are subtypes of apraxia, and different aspects of this disorder are seen with frontal vs parietal lobe lesions--in which case the ability to write may also be severely disrupted. This latter condition is referred to as apraxic agraphia.

Because the patient is also apraxic, the ability to make gestures or complex patterned movements becomes abnormal such as those involved in writing is also deficient. That is, the ability to correctly temporally sequence hand movements is affected which interferes with the ability to write. The patient no longer knows how to correctly hold or manipulate a pen, or how to move their hand when writing. However, if given block letters, or if asked to spell out loud, they may be able to spell correctly.

SPATIAL AGRAPHIA

Right cerebral injuries can secondarily disrupt writing skills due to generalized spatial and constructional deficiencies. This is because the right half of the brain, although minimally involved in grammar and word selection, is dominant for almost all aspects of visual spatial functioning and orientation, including the ability to coordinate movements in space. This would include those fine motor movements involved in writing.

Hence, with right hemisphere lesions, particularly right temporal-parietal injuries, words and letters will not be properly formed and aligned even when copying. Patients may have difficulty writing sentences in a straight line, and/or letters may be slanted at abnormal angles. In some cases the writing may be reduced to an illigible scrawl. In addition, patients may write only on the right half of the paper such that as they write, the left hand margin becomes progressively larger and the right side smaller (Hecaen & Marie, 1974). That is, they ignore the left half of visual space including the left half of the page they are writing on. If allowed to continue patients may end up writing only along the edge of the right hand margin of the paper.

Patients with right hemisphere lesions may tend to abnormally introduce spaces between letters within the words they are writing, particularly when writing cursively (i.e. cu siv e ly ). This is due to a failure to perform closure as well as a release over the left hemisphere (i.e. left hemisphere release). That is, whereas the right hemisphere performs closure, the left introduces sequences. If the right hemisphere is damaged, the left hemisphere acting unopposed begins to abnormally temporally-sequence and thus produce sequences separated by spaces, when writing. However, abnormal sequencing can also result from left hemisphere lesions

APHASIA & AGRAPHIA

Aphasia is a disorder of receptive and expressive language, involving loss of comprehension, or loss of the ability to speak, or both.

Although every patient with agraphia does not necessarily suffer from aphasic abnormalities, every patient who has aphasia has some degree of agraphia. Thus patients with aphasia always suffer from some degree of agraphia, though patients with agraphia do not always suffer from aphasic disturbances

 

What is Hypotonia?

Hypotonia is a condition characterized by severely reduced muscle tone. It is seen primarily in children. When hypotonia affects adults, it may be due to cerebellar degeneration. Hypotonia is not the same as muscle weakness but it can co-exist with muscle weakness.

Hypotonia does not affect intellect. However, depending on the underlying condition, some children may take longer to develop social, language, and reasoning skills.

Hypotonia is also called Floppy, Decreased muscle tone, and Hypotonic infant.

Symptoms of Hypotonia?

The most common symptoms of hypotonia involve problems with mobility and posture, breathing and speech difficulties, lethargy, ligament and joint laxity, and poor reflexes.

What causes Hypotonia?

Hypotonia may be caused by trauma, environmental factors, or by genetic, muscle, or central nervous system disorders.

In some cases, the cause is not apparent. Sometimes it may not be possible to find the cause of the hypotonia. While most children tend to flex their elbows and knees when resting, hypotonic children hang their arms and legs by their sides. They also may have substantial weakness and little or no head control, giving them a "floppy" appearance.

Some common conditions that cause hypotonia are:

Can Hypotonia be Treated?

Treatment options depend on the cause. When hypotonia is caused by an underlying condition, that condition is treated first, followed by symptomatic and supportive therapy.

Physical therapy is often used to improve fine motor control and overall body strength. Occupational and speech-language therapy is beneficial in decreasing breathing, speech, and swallowing difficulties. Therapy for infants and young children may also include sensory stimulation programs.

What is apraxia of speech?

Apraxia of speech, also known as verbal apraxia or dyspraxia, is a speech disorder in which a person has trouble saying what he or she wants to say correctly and consistently. It is not due to weakness or paralysis of the speech muscles (the muscles of the face, tongue, and lips). The severity of apraxia of speech can range from mild to severe.

 

What are the types and causes of apraxia?

There are two main types of speech apraxia: acquired apraxia of speech and developmental apraxia of speech. Acquired apraxia of speech can affect a person at any age, although it most typically occurs in adults. It is caused by damage to the parts of the brain that are involved in speaking, and involves the loss or impairment of existing speech abilities. The disorder may result from a stroke, head injury, tumor, or other illness affecting the brain. Acquired apraxia of speech may occur together with muscle weakness affecting speech production (dysarthria) or language difficulties caused by damage to the nervous system (aphasia).

Developmental apraxia of speech (DAS) occurs in children and is present from birth. It appears to affect more boys than girls. This speech disorder goes by several other names, including developmental verbal apraxia, developmental verbal dyspraxia, articulatory apraxia, and childhood apraxia of speech. DAS is different from what is known as a developmental delay of speech, in which a child follows the "typical" path of speech development but does so more slowly than normal.

The cause or causes of DAS are not yet known. Some scientists believe that DAS is a disorder related to a child's overall language development. Others believe it is a neurological disorder that affects the brain's ability to send the proper signals to move the muscles involved in speech. However, brain imaging and other studies have not found evidence of specific brain lesions or differences in brain structure in children with DAS. Children with DAS often have family members who have a history of communication disorders or learning disabilities. This observation and recent research findings suggest that genetic factors may play a role in the disorder.

 

What are the symptoms?

People with either form of apraxia of speech may have a number of different speech characteristics, or symptoms. One of the most notable symptoms is difficulty putting sounds and syllables together in the correct order to form words. Longer or more complex words are usually harder to say than shorter or simpler words. People with apraxia of speech also tend to make inconsistent mistakes when speaking. For example, they may say a difficult word correctly but then have trouble repeating it, or they may be able to say a particular sound one day and have trouble with the same sound the next day. People with apraxia of speech often appear to be groping for the right sound or word, and may try saying a word several times before they say it correctly. Another common characteristic of apraxia of speech is the incorrect use of "prosody" -- that is, the varying rhythms, stresses, and inflections of speech that are used to help express meaning.

Children with developmental apraxia of speech generally can understand language much better than they are able to use language to express themselves. Some children with the disorder may also have other problems. These can include other speech problems, such as dysarthria; language problems such as poor vocabulary, incorrect grammar, and difficulty in clearly organizing spoken information; problems with reading, writing, spelling, or math; coordination or "motor-skill" problems; and chewing and swallowing difficulties.

The severity of both acquired and developmental apraxia of speech varies from person to person. Apraxia can be so mild that a person has trouble with very few speech sounds or only has occasional problems pronouncing words with many syllables. In the most severe cases, a person may not be able to communicate effectively with speech, and may need the help of alternative or additional communication methods.

 

How is it diagnosed?

Professionals known as speech-language pathologists play a key role in diagnosing and treating apraxia of speech. There is no single factor or test that can be used to diagnose apraxia. In addition, speech-language experts do not agree about which specific symptoms are part of developmental apraxia. The person making the diagnosis generally looks for the presence of some, or many, of a group of symptoms, including those described above. Ruling out other contributing factors, such as muscle weakness or language-comprehension problems, can also help with the diagnosis.

To diagnose developmental apraxia of speech, parents and professionals may need to observe a child's speech over a period of time. In formal testing for both acquired and developmental apraxia, the speech-language pathologist may ask the person to perform speech tasks such as repeating a particular word several times or repeating a list of words of increasing length (for example, love, loving, lovingly). For acquired apraxia of speech, a speech-language pathologist may also examine a person's ability to converse, read, write, and perform non-speech movements. Brain-imaging tests such as magnetic resonance imaging (MRI) may also be used to help distinguish acquired apraxia of speech from other communication disorders in people who have experienced brain damage.

 

How is it treated?

In some cases, people with acquired apraxia of speech recover some or all of their speech abilities on their own. This is called spontaneous recovery. Children with developmental apraxia of speech will not outgrow the problem on their own. Speech-language therapy is often helpful for these children and for people with acquired apraxia who do not spontaneously recover all of their speech abilities.

Speech-language pathologists use different approaches to treat apraxia of speech, and no single approach has been proven to be the most effective. Therapy is tailored to the individual and is designed to treat other speech or language problems that may occur together with apraxia. Each person responds differently to therapy, and some people will make more progress than others. People with apraxia of speech usually need frequent and intensive one-on-one therapy. Support and encouragement from family members and friends are also important.

In severe cases, people with acquired or developmental apraxia of speech may need to use other ways to express themselves. These might include formal or informal sign language, a language notebook with pictures or written words that the person can show to other people, or an electronic communication device such as a portable computer that writes and produces speech.

Just the hypotonia, not sure about the others, and that can get better with age. My understanding is it has something to do with the central nervous system, both my boys had/have it, and it has improved a lot. I always thought it was because of the premautirity and their central nervous systems just weren't completely developed yet. It has been the culprit for alot of the speech delay and feeding issues. But at the last appt the dr told  me neither child has it any longer, although I can still see it here and there in Andrew, but it is extremely mild now in him. I'd say Nikolas probably doesn't have it at all anymore. They both also drooled long past when they should have stopped, and Andrew still will occassionally. Nikolas hasn't drooled since he was 2.5. We just remind Andrew though and he wipes his mouth.
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