Dysarthria
The dysarthrias are a group of motor speech disorders characterized by slow, weak, imprecise, or uncoordinated movements of speech musculature. Rather than a single neurological disorder, the dysarthrias vary along a number of different dimensions. As stated above, the neuroanatomical site of a lesion causing dysarthria can be one or a combination of the cerebrum, cerebellum, brainstem, and cranial nerves. The prevalence and incidence of dysarthria are not precisely known. Dysarthria can be a symptom of a neurological disease process with a constellation of other symptoms, or it can stand alone with a disease. Approximately one third of individuals with traumatic brain injury (TBI) may be dysarthric with nearly double that prevalence during the acute phase of recovery (
8,
9). Dysarthria is frequently seen in 50% to 90% of parkinsonian patients with the increased prevalence as the disease progresses (
10). Additionally, dysarthria is often a preliminary sign of amyotrophic lateral sclerosis or can become present as the disease progresses. In larger tertiary care medical centers, dysarthria was the primary communication impairment with acquired neurological disease seen for speech-language pathology evaluations over a 4-year period (
11).
The dysarthrias can be classified by time of onset, site of lesion, and etiology; however, the most widely used classification was first described by Darley et al. (
12,
13,
14). This is often referred to as the Mayo Clinic Classification System with each type representing a perceived and distinguishable grouping of speech characteristics with a presumed underlying pathophysiology or locus of lesion.
As a first step, differential diagnosis involves distinguishing the dysarthrias from other neurogenic communication disorders. The dysarthrias are distinct from aphasia in that language function (i.e., word retrieval, comprehension of both verbal and written language) is preserved in dysarthria but impaired in aphasia. Although both apraxia and dysarthria are considered motor speech disorders, they can be distinguished on the basis of several clinical features. In apraxia, automatic (i.e., nonspeech) movements are intact, whereas in most dysarthrias, they are not. Highly consistent articulatory errors are characteristic of dysarthria, whereas inconsistent errors are a hallmark of apraxia. Finally, in most dysarthrias, all speech systems, including respiration, phonation, resonation, and articulation, are involved; whereas in apraxia, respiratory or phonatory involvement is rare. It should be recognized that patients often can have elements of both dysarthria and apraxia, particularly those with bilateral brain damage.
Differential diagnosis among the dysarthrias seems to have received more systematic attention than any other aspect of the disorder.
Table 15-1 summarizes information related to the various dysarthrias (
15). In studies conducted at the Mayo Clinic, the perceptual features of the speech of seven groups of dysarthric patients were examined (
12,
13). These groups consisted of patients who were unequivocally diagnosed as having one of the following conditions: pseudobulbar palsy, bulbar palsy, amyotrophic lateral sclerosis, cerebellar lesions, parkinsonism,
dystonia, and choreoathetosis. Speech samples were rated along 38 dimensions that described pitch characteristics, loudness, vocal quality, respiration, prosody, articulation, and general impression. Results of these studies indicated that each of the seven neurological disorders could be characterized by a unique set of clusters of deviant speech dimensions and that no two disorders had the same set of clusters. Thus, differential diagnosis among the dysarthrias can be made, in part, on the basis that one type of dysarthria sounds different from the others. However, single features such as imprecise consonant production or nasal emission may not be sufficient to distinguish one type of dysarthria from another. Instead, differential diagnosis is made on the basis of clusters of features reflecting underlying pathophysiology and the findings on examination of the musculature. The following summarizes the major types of dysarthrias, their primary distinguishing perceptual attribute, and their presumed underlying localization and distinguishable deficit.
Flaccid Dysarthria
Flaccid dysarthria is due to weakness in cranial or spinal column nerve innervations to the speech systems. Its specific characteristics depend on which nerve is involved. Nerves affecting articulation include trigeminal, facial, or hypoglossal. The vagus nerve contributes to voice and resonatory dysarthria primarily seen with breathy, hoarse, and diplophonic speech. Spinal respiratory nerves can also be affected with deficits in breath patterning for speech, often causing production of short phrases. Most common speech characteristics include hypernasality, breathiness, diplophonia, nasal emission, audible inspiration (stridor), short phrases, and rapid deterioration and recovery with rest. Brainstem stroke or brain injury is a common cause of flaccid dysarthria (
14).
Spastic Dysarthria
Spastic dysarthria is usually associated with bilateral lesions of upper motor neuron pathways that innervate the relevant cranial nerve and spinal nerve. Its distinguishable characteristics are attributed to spasticity, and they often include a harsh, strained vocal quality, slow speech rate, pitch breaks, and variable loudness. All speech systems are typically affected with this classification (
14).
Ataxic Dysarthria
Ataxic dysarthria is associated with lesion’s disturbances of the cerebellum or its controls. As it is known for its incoordination as a characteristic, individuals are perceived with articulation and prosodic feature disturbances. Irregular articulatory breakdowns, distorted vowels, and inappropriate variations in pitch, loudness, and stress often are the classic features (
14).
This type of dysarthria has been coined with the term “drunken speech.” Ataxic dysarthria is often a result of cerebellar stroke or spinocerebellar ataxia.
Hypokinetic Dysarthria
Hypokinetic dysarthria is associated with basal ganglia control of the central nervous system. Its features are mostly related to reduce range of motion and rigidity. Additionally, reduced loudness, short rushes of speech, breathy-tight dysphonia, and monoloudness and monopitch are notable characteristics. Often dysfluency and word repetition are reported. Parkinson’s disease and its syndromes are the most noted disorder for hypokinetic dysarthria (
15).
Hyperkinetic Dysarthria
Hyperkinetic dysarthria is also associated with basal ganglia control; however, unlike hypokinetic dysarthrias, it is distinguished by abnormal involuntary movements that affect the intended speech movements. It is classified in many of the movement disorders and subcategorized into slow and fast hyperkinetic dysarthrias. Slow includes dystonia and athetosis, while fast includes tic and chorea. They may affect any of the speech systems and area usually distinguished by unpredictable variability in voice and articulation. Distorted vowels, excess loudness variations, sudden forced inspiration/expiration, voice stoppages/arrests, transient breathiness, intermittent hypernasality, and inappropriate vocal noises are just some of the common characteristics. Etiologies include Huntington’s chorea, Tourette’s syndrome, cerebral palsy, and side effects of neuroleptic drugs (
16).
Unilateral Upper Motor Neuron Dysarthria
This dysarthria has an anatomical rather than a pathophysiological label. It typically results from stroke affecting upper neuron pathways. Damage is unilateral; severity usually is rarely worse than mild to moderate. Often its characteristics overlap with flaccid, spastic, or ataxic dysarthrias (
16).
Mixed Dysarthria
Mixed dysarthria reflects combinations of two or more of the single dysarthria type. It occurs more frequently than any single dysarthria type. A common diagnosis involving mixed dysarthria is amyotrophic lateral sclerosis, which, in advanced stages, has features of both flaccid and spastic dysarthrias (
15).
Assessment
Traditionally, the assessment of dysarthric speech has mainly involved the use of perceptual evaluation measures. The inadequacies of this method of assessment have resulted in the development of a range of instrumental assessment techniques to provide more objective analyses of the underlying physiological impairments of the speech mechanism. Generally, the assessment of dysarthria relies on a combination of perceptual and instrumental analysis, including acoustic analyses. Most of the perceptual scales are based on the studies by Darley et al., using a number of speech dimensions to be rated on a severity rating scale (
14). Intelligibility scores has been one of the main measurement parameters to indicate communicative functional status. The interactive product of respiration, phonation, articulation, resonance, and prosody helps to define the speech impairment and requires full attention during assessments. Often, little attention is placed on connected speech intelligibility in the dysarthria population; however, it may
be a more valid indicator of functional communication than assessment at the word or simple phrase level (
17).
Studies have demonstrated acoustic variations in dysarthria; however, many of the studies have focused on one element in acoustic analysis rather than a broadly directed analysis of the speech output by systems, for example the articulatory, resonatory, phonatory, and respiratory components (
18,
19,
20,
21,
22).
A dysarthric speech assessment must involve perceptual acoustic description of all the systems. Clinicians must understand the interrelationship of the weakness, slowness, discoordination, or abnormal tone of the speech musculature and its effect on the systems of the speech mechanism, including respiratory, phonatory, velopharyngeal, or oral articulatory subsystems (
23). Both perceptual and instrumental tools are available for measuring speech performance (
24,
25). The perceptual tools are those that rely on the trained eyes and ears of the clinician, whereas instrumental approaches to assessment include devices that provide information about the acoustic, aerodynamic movement, or phonatory aspects of speech.
Assessment of the respiratory subsystem begins with perceptual measures, including ratings of the number of words produced per breath, the loudness of samples of connected speech, or visual observations of the presence of clavicular breathing. Instrumental approaches to the measurement of respiratory function may include acoustic measures of vocal intensity and utterance durations. Respiratory performance may also be assessed by estimating the subglottic air pressure generated by the speaker (
26,
27). Respiratory inductive plethysmography, commercially available as the Respitrace, is an instrument capable of obtaining information about the movements of the rib cage and abdomen during breathing and speech.
Phonatory (laryngeal subsystem) assessment typically begins with perceptual ratings of pitch characteristics (e.g., pitch level, pitch breaks, monopitch, and voice tremor), loudness (e.g., monoloudness, excess loudness, variation of volume), and voice quality (e.g., harsh voice, hoarseness, wet voice, breathiness, strained-strangled voice). Acoustic analysis can be performed by deriving vocal fundamental frequency and intensity (
28,
29). Measures of laryngeal resistance to airflow can also be obtained (
30) along with laryngeal visualization with endoscopy or stroboscopy (
30).
Assessment of the velopharyngeal mechanism can be made with perceptual judgments of hypernasality or the occurrence of nasal air emission. Nasalization also can be measured acoustically. Precise inferences can be made about the timing of velopharyngeal closure by obtaining simultaneous pneumatic measures of air pressure and air flow during selected speech samples (
31,
32). Movement of the velopharyngeal mechanism can be observed through cineradiographic techniques and/or endoscopic visualizations.
Assessment of oral articulation can be made by the rating of consonant and vowel precisions and coordination of articulatory movement. Although movements can be recorded using cineradiographic technique and myoelectric activity with electromyographic recordings, these techniques are not used in routine clinical practice.
Treatment Considerations
Clinical decisions regarding treatment of dysarthria should include behavioral objectives for reaching short- and long-term goals. An overall goal to improve functional communication for the patient is necessary and should be identified and described with the patient and his or her significant family members. Generally, treatment goals can vary with the severity of the speech impairment and the overall medical disorder.
For severely involved speakers, whose intelligibility is so poor that they are unable to communicate verbally in some or all situations, the general goal of treatment involves establishing an immediate functional means of communication. This may include use of augmentative approaches. The term communication augmentation refers to any device designed to augment, supplement, or replace verbal communication for someone who is not an independent verbal communicator. These augmentative or alternative communication (AAC) systems can be as low tech as writing or communication boards, or higher tech such as talk-back switches or computer-based speech synthesis. The selection of an appropriate augmentation system requires a thorough evaluation of the individual’s communication needs. To determine an individual’s needs, the clinician needs to consider the patient’s physical and cognitive capabilities, including cognition, language, memory, physical control, vision, hearing, and overall medical condition. This assessment may require additional co-evaluators, such as an occupational therapist or a rehabilitation engineer. Once the individual’s capabilities have been ascertained, augmentative system components can be selected, and appropriate system modifications can be made.
For those moderately involved speakers who are able to use speech as their primary means of communication but whose intelligibility is compromised, the general goal of treatment involves improving intelligibility. Use of compensatory strategies with speech production or augmentative systems is common. Achieving compensated intelligibility may take a variety of forms, depending on the speaker and the nature of the underlying impairment. For some with greater involvement, use of an alphabet supplementation system, in which they point to the first letter of each word as they say the word, assists in the transition to intelligible speech (
9). For others, the treatment involves an attempt to decrease the impairment by exercises that will improve performance on selected aspects of speech production. For example, exercises may involve developing more adequate respiratory support for speech (
33) or training to establish an appropriate speech rate (
34). For the mildly involved dysarthric speaker whose speech is characterized as intelligible but less efficient and less natural than normal, treatment planning should consider the patient’s needs in communication within his or her home or job setting. For some speakers, these mild reductions in speech efficiency pose no problems. For other mildly involved speakers, however, treatment is warranted. The general goals of treatment
for dysarthric people with mild disabilities include maximizing communication efficiency while maintaining intelligibility and maximizing speech naturalness. Maximizing naturalness is accomplished by teaching appropriate phrasing, stressing patterning, and intonation (
35).
Often partnerships with other medical professionals are appropriate. For example, a maxillofacial prosthodontist may assist with managing resonant disorders such as velopharyngeal incompetence. Fabrication of a prosthetic device such as a palatal lift can assist with management of impaired velopharyngeal closure. Assessment of candidacy for the device is a coordinated effort between the speech-language pathologist and the prosthodontist. An appropriately fitted palatal lift will allow certain dysarthric speakers to better produce speech sounds that require the buildup air pressure and can maximize intelligibility by improving prosody and contextual breath support.
Treatment approaches for patients with progressive disorders such as parkinsonism, multiple sclerosis, and amyotrophic lateral sclerosis are different from those used with the dysarthric speaker who is recovering from a single medical event (
36). Initially, the patients are encouraged to maximize the functional communication level by paying specific attention to the clarity and precision of their speech. At some point, the patients will need to modify their speaking patterns by controlling rate and consonant emphasis and by reducing the number of words per breath. Some patients with progressive dysarthria make the adjustments in their speech pattern without specific treatment; others may need to practice these modifications with a speech pathologist or trained family member until the changes become habitual. In severe cases, a communication augmentation system may be considered. These augmentation systems usually are chosen or designed to accommodate the lifestyle of the patient while serving his or her anticipated communication needs over the longest period of time.
Apraxia
Apraxia of speech (AOS) occurs in the absence of significant weakness and incoordination of muscles, with automatic and reflexive movements undisturbed. Lesions in the premotor cortex are a frequent finding (
37). AOS is characterized by labored and dysprosodic productions, resulting in errors of omission, substitution, and repetition. There is debate as to whether AOS is a pure motor or linguistic (i.e., phonemic) disturbance (
38,
39,
40). Patients have difficulty programming the positioning of the speech musculature and sequencing the movements necessary for speech. It is seen by some as a distinct condition that often coexists and complicates aphasia, whereas others regard the characteristics as part of the nonfluent Broca’s aphasia. AOS carries a negative prognosis for recovery when there is a moderate to severe aphasia in tandem. When it occurs without the concomitant language disturbance, therapy can focus on retraining the patient’s ability to program sound patterns, to shift from one sound to another, and to use preserved melodic and rhythmic patterns to facilitate speech.
Head and Neck Cancer
The diagnosis of head and neck cancer often presents with speech and/or swallowing impairments. The location of the malignancy often dictates the speech impairment being phonatory, resonance, articulatory, or a combination of the three. Treatment of the disease can be a sole modality such as surgery or radiation therapy or multiple modalities, including surgery, irradiation, and chemotherapy. Over the last 20 years, treatment has focused on organ preservation with the goal of maintaining function. This type of treatment is often recommended for tumors of the tongue, oral cavity, tonsil, base of tongue, and pharynx and larynx. Depending on tumor size, spread of disease, and nodal involvement, the treatment often involves radiation with or without chemotherapy. Speech impairments thus would typically be seen in the acute phase of treatment and in the immediate posttreatment acute phase (6 months posttreatment). Chronic effects are often seen with scarring and fibrosis greater than 6 months posttreatment.
Often surgical intervention is needed with advanced disease or recurring disease. This may involve removal of the larynx alone or with other organs. Removal of the larynx or total laryngectomy is a common procedure. With loss of the voicing component for speech production, there is an obvious need for speech rehabilitation, beginning with presurgical teaching and continuing with communication alternatives and rehabilitation postsurgery.
Several options for speaking are available to postlaryngectomy patients including artificial larynx, esophageal speech, or tracheal esophageal puncture, a voice restoration procedure. Artificial larynx use or electrolarynx offers individuals the opportunity to speak within days of surgery. Commercially available electrolarynges are designed to introduce air vibrations either directly into the oral cavity through a catheter or indirectly through the neck tissues. In each case, the tones resonate within the oral and pharyngeal cavities and are modified by articulation into audible, intelligible words. Often an intraoral electrolarynx can be used 2 or 3 days after surgery, providing the patient with an immediate means of communication. Intraoral devices can also be used a long term for patients with necks that are unsuitable for indirect transmission of vibration, usually because of pain, edema, or scar tissue. Experience suggests that good speech is slower to develop using an intraoral device than with a neck device. Therefore, care must be taken to help the patient avoid early frustration associated with not being understood immediately.
A second form of speech alternative is esophageal speech. Esophageal speech is accomplished by training the patient to move air from the oral and pharyngeal cavities into the esophagus by injection or inhalation methods. The air is then trapped within the cricopharyngeal segment and released. The vibration of the release from the segment is the phonatory sound that is then modulated with resonatory and articulatory modifications to produce speech. Accomplished esophageal speakers can speak clearly and effortlessly; however, many laryngectomy patients are unable to learn this technique. Failure to learn esophageal speech may represent insufficient or excessive
pharyngoesophageal segment tone, scarring, nerve damage, or reduced patient commitment for learning.
A third option for speech involves surgical intervention and placement of a one-way-valved prosthesis between the trachea and the esophagus. This is termed tracheoesophageal puncture (TEP). The procedure often is performed by head and neck surgeons as a secondary procedure; however, it can be performed with the total laryngectomy. This method of voice production uses the same anatomic vibratory site as the esophageal speech technique.
Tracheal-esophageal puncture procedures have been used since 1980 and are a relatively simple means of voice restoration (
41). A small, one-way-valved voice prosthesis is inserted through the TEP tract that is surgically created to maintain patency. Air is then shunted via digital or valve occlusion of the stoma into the esophagus without having esophageal contents enter the TEP. Air then passes through the prosthesis to the cricopharyngeal segment for vibration to produce the voicing component of speech. Early speech success following TEP and voice prosthesis fitting has been reported in almost 90% of cases (
42,
43). Long-term success is reported at between 93% for patients given primary TEP and 83% for those given secondary procedures (
44). Success largely depends on patient selection, and in some cases, success can be enhanced by surgical techniques that can prevent pharyngoesophageal segment spasm (
45) such as pharyngeal plexus neurectomy or cricopharyngeal myotomy or botulinum toxin injection. Other factors to consider in patient selection are motivation, intellect, dexterity, eyesight, stoma size and sensitivity, hand hygiene, surgical risk, and cost.
Fluency
Fluency disorders are characterized by a disruption in the ease and flow of connected speech. The most common and well-known type of fluency disorder is stuttering. Stuttering is a very complex, dynamic, and somewhat controversial disorder in that theories and opinions abound regarding etiology, diagnosis, and treatment. Theories regarding underlying cause include genetic, cognitive, psycholinguistic, neuromuscular, as well as multifactorial. It is generally accepted that primary characteristics of stuttering include blocks (absence of sound), repetitions (of sounds or words), and/or prolonged sounds. Secondary behaviors often observed include struggle (body movement, eye blinks, lip/jaw tremors) and avoidance of sounds, words, conversation partners or environments that trigger the dysfluency. The evaluating clinician must be very cautious with diagnosis for several reasons. One, stuttering resolves in 75% to 90% of small children who begin to stutter. Additionally, there is a spectrum of normal dysfluency that is reactive to environmental pressures but is not consistent with a stuttering diagnosis. Labeling one as a “stutterer” when he or she is within the normal spectrum of behavior can be quite damaging and can lead to exacerbated symptomatology.
Due to high variability of type, severity, response to treatment, and possibly cause, having a standard treatment strategy is nearly impossible. However, common approaches include environmental modifications, desensitization, fluency-shaping techniques (i.e., easy onset, continuous phonation, auditory/visual feedback), and possibly psychological intervention. One component that is quite consistently included is counseling and support by the speech-language pathologist with the goal of changing the patient’s mindset from anticipating stuttering to anticipating fluency. Goals may vary from absence of stuttering to reducing severity and eliminating avoidance behaviors so as to improve quality and effectiveness of overall communication. It is generally believed that any effective treatment program must also have transference into nonclinical functional environments and maintenance as a goal (
46,
47).