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Oculomotor apraxia (OMA), also known as Cogan ocular motor apraxia or saccadic initiation failure (SIF) is the absence or defect of controlled, voluntary, and purposeful eye movement. It was first described in 1952 by the American ophthalmologist David Glendenning Cogan. People with this condition have difficulty moving their eyes horizontally and moving them quickly. The main difficulty is in saccade initiation, but there is also impaired cancellation of the vestibulo-ocular reflex. Patients have to turn their head in order to compensate for the lack of eye movement initiation in order to follow an object or see objects in their peripheral vision, but they often exceed their target. There is controversy regarding whether OMA should be considered an apraxia, since apraxia is the inability to perform a learned or skilled motor action to command, and saccade initiation is neither a learned nor a skilled action.
Currently there is no cure for dysmetria itself as it is actually a symptom of an underlying disorder. However, isoniazid and clonazepam have been used to treat dysmetria. Frenkel exercises treat dysmetria. There have also been numerous reported cases of chiropractic neurology as an effective holistic treatment for dysmetria. Cannabis has been used in trials in the U.K. and displayed some success, though it is not legal to use in some U.S. states.
Researchers now are testing different possibilities for treating dysmetria and ataxia. One opportunity for treatment is called rehearsal by eye movement. It is believed that visually guided movements require both lower- and higher-order visual functioning by first identifying a target location and then moving to acquire what is sought after. In one study, researchers used visually guided stepping which is parallel to visually guided arm movements to test this treatment. The patients suffered from saccadic dysmetria which in turn caused them to overshoot their movements 3. The patients first walked normally and were then told to twice review the area that was to be walked through 3. After rehearsal with eye movements, the patients improved their motor performance. Researchers believe that prior rehearsal with the eyes might be enough for a patient who suffers from motor dysmetria as a result of saccadic dysmetria to complete a motor task with enhanced spatial awareness.
Research has also been done for those patients who suffer from MS. Deep brain stimulation (DBS) remains a viable possibility for some MS patients though the long-term effects of this treatment are currently under review. The subjects who have undergone this treatment had no major relapse for six months and disabling motor function problems. Most subjects benefited from the implantation of the electrodes and some reported that their movement disorder was gone after surgery. However, these results are limiting at this time because of the small range of subjects who were used for the experiment and it is unknown whether this is a viable option for all MS patients who suffer from motor control problems.
OMA is a neurological condition. Although some brain imaging studies of people with OMA reveal a normal brain, some MRI studies have revealed unusual appearance of some brain areas, in particular the corpus callosum, cerebellum, and/or fourth ventricle. Oculomotor apraxia can be acquired or congenital. Sometimes no cause is found, in which case it is described as idiopathic
A person may be born with the parts of the brain for eye movement control not working, or may manifest poor eye movement control in childhood. If any part of the brain that controls eye movement becomes damaged, then OMA may develop. One of the potential causes is bifrontal hemorrhages. In this case, OMA is associated with bilateral lesions of the frontal eye fields (FEF), located in the caudal middle frontal gyrus. The FEF control voluntary eye movements, including saccades, smooth pursuit and vergence. OMA can also be associated with bilateral hemorrhages in the parietal eye fields (PEF). The PEF surround the posterior, medial segment of the intraparietal sulcus. They have a role in reflexive saccades, and send information to the FEF. Since the FEF and PEF have complementary roles in voluntary and reflexive production of saccades, respectively, and they get inputs from different areas of the brain, only bilateral lesions to both the FEF and PEF will cause persistent OMA. Patients with either bilateral FEF or bilateral PEF damage (but not both FEF and PEF) have been shown to regain at least some voluntary saccadic initiation some time after their hemorrhages. Other causes of OMA include brain tumors and cardiovascular problems.
The eye findings of Parinaud's Syndrome generally improve slowly over months, especially with resolution of the causative factor; continued resolution after the first 3–6 months of onset is uncommon. However, rapid resolution after normalization of intracranial pressure following placement of a ventriculoperitoneal shunt has been reported.
Treatment is primarily directed towards etiology of the dorsal midbrain syndrome. A thorough workup, including neuroimaging is essential to rule out anatomic lesions or other causes of this syndrome. Visually significant upgaze palsy can be relieved with bilateral inferior rectus recessions. Retraction nystagmus and convergence movement are usually improved with this procedure as well.
In adults, many of the symptoms diminish over time. Although it has been suggested that a similar diminishing of symptoms occurs in children as well, it appears more likely that most do not overcome their deficits, but instead simply learn to adjust.
There is no cure for Gerstmann syndrome. Treatment is symptomatic and supportive. Occupational and speech therapies may help diminish the dysgraphia and apraxia. In addition, calculators and word processors may help school children cope with the symptoms of the disorder.
Deep brain stimulation (DBS) is a technique that uses electrodes placed in the brain to modify brain activity by sending a constant electrical signal to the nearby nuclei. Treatment of muscle tone issues via deep brain stimulation typically targets the global pallidus and has shown to significantly improve symptoms associated with ADCP. The specific mechanism by which DBS affects ADCP is unclear. DBS of the globus pallidus interna improves dystonia in people with dyskinetic CP in 40% of cases, perhaps due to variation in basal ganglia injuries.
Medications that impede the release of excitatory neurotransmitters have been used to control or prevent spasms. Treatment with intrathecal baclofen, a gamma-aminobutyric acid (GABA) agonist, decreases muscle tone and has been shown to decrease the frequency of muscle spasms in ADCP patients. Tetrabenazine, a drug commonly used in the treatment of Huntington's disease, has been shown to be effective treating chorea.
Articulation problems resulting from dysarthria are treated by speech language pathologists, using a variety of techniques. Techniques used depend on the effect the dysarthria has on control of the articulators. Traditional treatments target the correction of deficits in rate (of articulation), prosody (appropriate emphasis and inflection, affected e.g. by apraxia of speech, right hemisphere brain damage, etc.), intensity (loudness of the voice, affected e.g. in hypokinetic dysarthrias such as in Parkinson's), resonance (ability to alter the vocal tract and resonating spaces for correct speech sounds) and phonation (control of the vocal folds for appropriate voice quality and valving of the airway). These treatments have usually involved exercises to increase strength and control over articulator muscles (which may be flaccid and weak, or overly tight and difficult to move), and using alternate speaking techniques to increase speaker intelligibility (how well someone's speech is understood by peers). With the speech language pathologist, there are several skills that are important to learn; safe chewing and swallowing techniques, avoiding conversations when feeling tired, repeat words and syllables over and over in order to learn the proper mouth movements, and techniques to deal with the frustration while speaking. Depending on the severity of the dysarthria, another possibility includes learning how to use a computer or flip cards in order to communicate more effectively.
More recent techniques based on the principles of motor learning (PML), such as LSVT (Lee Silverman voice treatment) speech therapy and specifically LSVT may improve voice and speech function in PD. For Parkinson's, aim to retrain speech skills through building new generalised motor programs, and attach great importance to regular practice, through peer/partner support and self-management. Regularity of practice, and when to practice, are the main issues in PML treatments, as they may determine the likelihood of generalization of new motor skills, and therefore how effective a treatment is.
Augmentative and alternative communication (AAC) devices that make coping with a dysarthria easier include speech synthesis and text-based telephones. These allow people who are unintelligible, or may be in the later stages of a progressive illness, to continue to be able to communicate without the need for fully intelligible speech.
Treatment consists of physical rehabilitation programs designed to improve overall function, increase strength and improve balance. The ultimate goal is to increase the patient's degree of independence, thus improving the patient's quality of life. Exercise typically begins with simple movements, gradually transitioning into more complex actions. Various aspects of treatment are assessed based on the individual patient's condition, utilizing many assessment tools:
- Functional Reach Test
- External Perturbation Test – Push, Release
- External Perturbation Test – Pull
- Clinical Sensory Integration Test
- Single Leg Stance Test
- Five Times Sit to Stand Test
Various scales are also utilized
- Brief Ataxia Rating Scale
- Friedreich's Ataxia Impact Scale
- Scale For Assessment and Rating of Ataxia
There are many potential causes of dysarthria. They include toxic, metabolic, degenerative diseases, traumatic brain injury, or thrombotic or embolic stroke.
Degenerative diseases include parkinsonism, amyotrophic lateral sclerosis (ALS), multiple sclerosis, Huntington's disease, Niemann-Pick disease, and Friedreich ataxia.
Toxic and metabolic conditions include: Wilson's disease, hypoxic encephalopathy such as in drowning, and central pontine myelinolysis.
These result in lesions to key areas of the brain involved in planning, executing, or regulating motor operations in skeletal muscles (i.e. muscles of the limbs), including muscles of the head and neck (dysfunction of which characterises dysarthria). These can result in dysfunction, or failure of: the motor or somatosensory cortex of the brain, corticobulbar pathways, the cerebellum, basal nuclei (consisting of the putamen, globus pallidus, caudate nucleus, substantia nigra etc.), brainstem (from which the cranial nerves originate), or the neuro-muscular junction (in diseases such as myasthenia gravis) which block the nervous system's ability to activate motor units and effect correct range and strength of movements.
Causes:
- Brain tumor
- Cerebral palsy
- Guillain–Barré syndrome
- Hypothermia
- Lyme disease
- Stroke
- Intracranial hypertension (formerly known as pseudotumor cerebri)
- Tay-Sachs, and late onset Tay-Sachs (LOTS), disease
The prognosis for individuals with apraxia varies. With therapy, some patients improve significantly, while others may show very little improvement. Some individuals with apraxia may benefit from the use of a communication aid.
However, many people with apraxia are no longer able to be independent. Those with limb-kinetic and/or gait apraxia should avoid activities in which they might injure themselves or others.
Occupational therapy, physical therapy, and play therapy may be considered as other references to support patients with apraxia. These team members could work along with the SLP to provide the best therapy for people with apraxia. However, because people with limb apraxia may have trouble directing their motor movements, occupational therapy for stroke or other brain injury can be difficult.
No medication has been shown useful for treating apraxia.
Treatment for individuals with apraxia includes speech therapy, occupational therapy, and physical therapy.
Generally, treatments for apraxia have received little attention for several reasons, including the tendency for the condition to resolve spontaneously in acute cases. Additionally, the very nature of the automatic-voluntary dissociation of motor abilities that defines apraxia means that patients may still be able to automatically perform activities if cued to do so in daily life. Nevertheless, research shows that patients experiencing apraxia have less functional independence in their daily lives, and that evidence for the treatment of apraxia is scarce. However, a literature review of apraxia treatment to date reveals that although the field is in its early stages of treatment design, certain aspects can be included to treat apraxia. One method is through rehabilitative treatment, which has been found to positively impact apraxia, as well as activities of daily living. In this review, rehabilitative treatment consisted of 12 different contextual cues, which were used in order to teach patients how to produce the same gesture under different contextual situations. Additional studies have also recommended varying forms of gesture therapy, whereby the patient is instructed to make gestures (either using objects or symbolically meaningful and non-meaningful gestures) with progressively less cuing from the therapist. It may be necessary for patients with apraxia to use a form of alternative and augmentative communication depending on the severity of the disorder. In addition to using gestures as mentioned, patients can also use communication boards or more sophisticated electronic devices if needed. No single type of therapy or approach has been proven as the best way to treat a patient with apraxia, since each patient's case varies. However, one-on-one sessions usually work the best, with the support of family members and friends. Since everyone responds to therapy differently, some patients will make significant improvements, while others will make less progress. The overall goal for treatment of apraxia is to treat the motor plans for speech, not treating at the phoneme (sound) level. Research suggests that individuals with apraxia of speech should receive treatment that focuses on the repetition of target words and rate of speech. Research rerouted that the overall goal for treatment of apraxia should be to improve speech intelligibility, rate of speech and articulation of targeted words.
Given the complexity of the medical problems facing ideomotor apraxia patients, as they are usually suffering from a multitude of other problems, it is difficult to ascertain the impact that it has on their ability to function independently. Deficits due to Parkinson's or Alzheimer's disease could very well be sufficient to mask or make irrelevant difficulties arising from the apraxia. Some studies have shown ideomotor apraxia to independently diminish the patient's ability to function on their own. The general consensus seems to be that ideomotor apraxia does have a negative impact on independence in that it can reduce an individual's ability to manipulate objects, as well as diminishing the capacity for mechanical problem solving, owing to the inability to access information about how familiar parts of the unfamiliar system function. A small subset of patients has been known to spontaneously recover from apraxia; this is rare, however. One possible hope is the phenomenon of hemispheric shift, where functions normally performed by one hemisphere can shift to the other in the event that the first is damaged. This seems to necessitate, however, that some portion of the function is associated with the other hemisphere to begin with. There is dispute over whether the right hemisphere of the cortex is involved at all in the praxis system, as some evidence from patients with severed corpus callosums indicates it may not be.
Although there is little that can be done to substantially reverse the effects of ideomotor apraxia, Occupational Therapy can be effective in helping patients regain some functional control. Sharing the same approach in treating ideational apraxia, this is achieved by breaking a daily task (e.g. combing hair) into separate components and teaching each distinct component individually. With ample repetition, proficiency in these movements can be acquired and should eventually be combined to create a single pattern of movement.
Bruns apraxia, or frontal ataxia is a gait apraxia found in patients with bilateral frontal lobe disorders. It is characterised by an inability to initiate the process of walking, despite the power and coordination of the legs being normal when tested in the seated or lying position. The gait is broad-based with short steps with a tendency to fall backwards. It was originally described in patients with frontal lobe tumours, but is now more commonly seen in patients with cerebrovascular disease.
It is named after Ludwig Bruns.
There is no cure for the condition. Management is through therapy.
Parinaud's syndrome, also known as dorsal midbrain syndrome, vertical gaze palsy, and Sunset Sign, is an inability to move the eyes up and down. It is caused by compression of the vertical gaze center at the rostral interstitial nucleus of medial longitudinal fasciculus (riMLF). The eyes lose the ability to move upward and down .
It is a group of abnormalities of eye movement and pupil dysfunction. It is caused by lesions of the upper brain stem and is named for Henri Parinaud (1844–1905), considered to be the father of French ophthalmology.
There is no cure for DVD/CAS, but with appropriate, intensive intervention, people with the disorder can improve significantly.
DVD/CAS requires various forms of therapy which varies with the individual needs of the patient. Typically, treatment involves one-on-one therapy with a speech language pathologist (SLP). In children with DVD/CAS, consistency is a key element in treatment. Consistency in the form of communication, as well as the development and use of oral communication are extremely important in aiding a child's speech learning process.
Many therapy approaches are not supported by thorough evidence; however, the aspects of treatment that do seem to be agreed upon are the following:
- Treatment needs to be intense and highly individualized, with about 3-5 therapy sessions each week
- A maximum of 30 minutes per session is best for young children
- Principles of motor learning theory and intense speech-motor practice seem to be the most effective
- Non-speech oral motor therapy is not necessary or sufficient
- A multi-sensory approach to therapy may be beneficial: using sign language, pictures, tactile cues, visual prompts, and Augmentative and Alternative Communication (AAC) can be helpful.
Although these aspects of treatment are supported by much clinical documentation, they lack evidence from systematic research studies. In ASHA's position statement on DVD/CAS, ASHA states there is a critical need for collaborative, interdisciplinary, and programmatic research on the neural substrates, behavioral correlates, and treatment options for DVD/CAS.
There is no curative treatment for this condition. Supportive management is helpful.
Developmental coordination disorder is a lifelong neurological condition that is more common in males than in females, with a ratio of approximately four males to every female. The exact proportion of people with the disorder is unknown since the disorder can be difficult to detect due to a lack of specific laboratory tests, thus making diagnosis of the condition one of elimination of all other possible causes/diseases. Approximately 5–6% of children are affected by this condition.
Many researchers are investigating the characteristics of apraxia of speech and the most effective treatment methods. Below are a couple of the recent findings:
Sound Production Treatment:
Articulatory-kinematic treatments have the strongest evidence of their use in treating Acquired Apraxia of Speech. These treatments use the facilitation of movement, positioning, timing, and articulators to improve speech production. Sound Production Treatment (SPT) is an articulatory-kinematic treatment that has received more research than many other methods. It combines modeling, repetition, minimal pair contrast, integral stimulation, articulatory placement cueing, and verbal feedback. It was developed to improve the articulation of targeted sounds in the mid-1990s. SPT shows consistent improvement of trained sounds in trained and untrained words. The best results occur with eight to ten exemplars of the targeted sound to promote generalization to untrained exemplars of trained sounds. In addition, maintenance effects are the strongest with 1–2 months post-treatment with sounds that reached high accuracy during treatment. Therefore, the termination of treatment should not be determined by performance criteria, and not by the number of sessions the client completes, in order to have the greatest long-term effects. While there are many parts of SPT that should receive further investigation, it can be expected that it will improve the production of targeted sounds for speakers with apraxia of Speech.
Repeated Practice & Rate/Rhythm Control Treatments:
Julie Wambaugh’s research focuses on clinically applicable treatments for acquired apraxia of speech. She recently published an article examining the effects of repeated practice and rate/rhythm control on sound production accuracy. Wambaugh and colleagues studied the effects of such treatment for 10 individuals with acquired apraxia of speech. The results indicate that repeated practice treatment results in significant improvements in articulation for most clients. In addition, rate/rhythm control helped some clients, but not others. Thus, incorporating repeated practice treatment into therapy would likely help individuals with AOS.
In terms of the specific rehabilitation of visuoperceptual disorders such as Bálint's syndrome, the literature is extremely sparse. According to one study, rehabilitation training should focus on the improvement of visual scanning, the development of visually guided manual movements, and the improvement of the integration of visual elements. Very few treatment strategies have been proposed, and some of those have been criticized as being poorly developed and evaluated.
Three approaches to rehabilitation of perceptual deficits, such as those seen in Bálint's syndrome, have been identified:
1. The adaptive (functional) approach, which involves functional tasks utilising the person's strengths and abilities, helping them to compensate for problems or altering the environment to lessen their disabilities. This is the most popular approach.
2. The remedial approach, which involves restoration of the damaged CNS by training in the perceptual skills, which may be generalised across all activities of daily living. This could be achieved by tabletop activities or sensorimotor exercises.
3. The multicontext approach, which is based on the fact that learning is not automatically transferred from one situation to another. This involves practicing of a targeted strategy in a multiple environment with varied tasks and movement demands, and it incorporates self-awareness tasks.
In cases of acute AOS (stroke), spontaneous recovery may occur, in which previous speech abilities reappear on their own. All other cases of acquired AOS require a form of therapy; however the therapy varies with the individual needs of the patient. Typically, treatment involves one-on-one therapy with a speech language pathologist (SLP). For severe forms of AOS, therapy may involve multiple sessions per week, which is reduced with speech improvement. Another main theme in AOS treatment is the use of repetition in order to achieve a large amount of target utterances, or desired speech usages.
There are various treatment techniques for AOS. One technique, called the Linguistic Approach, utilizes the rules for sounds and sequences. This approach focuses on the placement of the mouth in forming speech sounds. Another type of treatment is the Motor-Programming Approach, in which the motor movements necessary for speech are practiced. This technique utilizes a great amount of repetition in order to practice the sequences and transitions that are necessary in between production of sounds.
Research about the treatment of apraxia has revealed four main categories: articulatory-kinematic, rate/rhythm control, intersystemic facilitation/reorganization treatments, and alternative/augmentative communication.
- Articulatory-kinematic treatments almost always require verbal production in order to bring about improvement of speech. One common technique for this is modeling or repetition in order to establish the desired speech behavior. Articulatory-kinematic treatments are based on the importance of patients to improve spatial and temporal aspects of speech production.
- Rate and rhythm control treatments exist to improve errors in patients’ timing of speech, a common characteristic of Apraxia. These techniques often include an external source of control like metronomic pacing, for example, in repeated speech productions.
- Intersystemic reorganization/facilitation techniques often involve physical body or limb gestural approaches to improve speech. Gestures are usually combined with verbalization. It is thought that limb gestures may improve the organization of speech production.
- Finally, alternative and augmentative communication approaches to treatment of apraxia are highly individualized for each patient. However, they often involve a "comprehensive communication system" that may include "speech, a communication book aid, a spelling system, a drawing system, a gestural system, technologies, and informed speech partners".
One specific treatment method is referred to as PROMPT. This acronym stands for Prompts for Restructuring Oral Muscular Phonetic Targets, and takes a hands on multidimensional approach at treating speech production disorders. PROMPT therapists integrate physical-sensory, cognitive-linguistic, and social-emotional aspects of motor performance. The main focus is developing language interaction through this tactile-kinetic approach by using touch cues to facilitate the articulatory movements associated with individual phonemes, and eventually words.
One study describes the use of electropalatography (EPG) to treat a patient with severe acquired apraxia of speech. EPG is a computer-based tool for assessment and treatment of speech motor issues. The program allows patients to see the placement of articulators during speech production thus aiding them in attempting to correct errors. Originally after two years of speech therapy, the patient exhibited speech motor and production problems including problems with phonation, articulation, and resonance. This study showed that EPG therapy gave the patient valuable visual feedback to clarify speech movements that had been difficult for the patient to complete when given only auditory feedback.
While many studies are still exploring the various treatment methods, a few suggestions from ASHA for treating apraxia patients include the integration of objective treatment evidence, theoretical rationale, clinical knowledge and experience, and the needs and goals of the patient
Ideomotor Apraxia, often IMA, is a neurological disorder characterized by the inability to correctly imitate hand gestures and voluntarily mime tool use, e.g. pretend to brush one's hair. The ability to spontaneously use tools, such as brushing one's hair in the morning without being instructed to do so, may remain intact, but is often lost. The general concept of apraxia and the classification of ideomotor apraxia were developed in Germany in the late 19th and early 20th centuries by the work of Hugo Liepmann, Adolph Kussmaul, Arnold Pick, Paul Flechsig, Hermann Munk, Carl Nothnagel, Theodor Meynert, and linguist Heymann Steinthal, among others. Ideomotor apraxia was classified as "ideo-kinetic apraxia" by Liepmann due to the apparent dissociation of the idea of the action with its execution. The classifications of the various subtypes are not well defined at present, however, owing to issues of diagnosis and pathophysiology. Ideomotor apraxia is hypothesized to result from a disruption of the system that relates stored tool use and gesture information with the state of the body to produce the proper motor output. This system is thought to be related to the areas of the brain most often seen to be damaged when ideomotor apraxia is present: the left parietal lobe and the premotor cortex. Little can be done at present to reverse the motor deficit seen in ideomotor apraxia, although the extent of dysfunction it induces is not entirely clear.