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Script Training in Nonfluent/Agrammatic-Primary Progressive Aphasia
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
59 Script Training in Nonfluent/Agrammatic-Primary
Progressive Aphasia
Michael de Riesthal and Sarah Diehl
59.1 Introduction
An evidence-based approach to managing communication deficits is critical to therapeutic success. In the current case, we
describe an approach to therapy for an individual with nonfluent/agrammatic-primary progressive aphasia (nf-PPA) based on
patient history, as well as his social, community, and vocational
environment, and best clinical evidence.
59.2 Clinical History and
Description
BH, a 64-year-old, right-handed man, was actively employed as
a pharmacist. He presented with a 4-year history of decline in
communicative function. Early in this decline, he was incorrectly diagnosed with Alzheimer’s disease. He then received a
thorough neurological assessment. On examination, BH was
alert and oriented to person, place, and time. He named all of
the pictures on the NIH Stroke Scale and followed a three-step
command. His speech was hesitant and groping, and he made
inconsistent errors when producing multisyllabic words. He
repeated “no ifs, ands, or buts” and was able to spell WORLD
forward and backward. When presented with words to remember, he was able to register 3/3 words and recalled all three
words after a 10-minute delay. He was able to copy the intersecting pentagon figure and drew a clock with properly placed
numbers and hands. BH’s pupils were equal and reactive to
light, his visual fields were full, and his extraocular movements
were full without nystagmus. His facial movements and palatal
elevation were symmetric, and his tongue protruded at midline.
BH did not demonstrate arm drift, focal weakness, or dysmetria.
He was able to walk on a narrow base and perform a tandem
walk without difficulty. Magnetic resonance imaging indicated
minimal ischemic white matter changes in the left periventricular regions. Based on this assessment, with the primar y findings
being issues with motor speech production, the neurologist
diagnosed BH with a primary progressive apraxia of speech
(AOS). He was referred for a speech and language evaluation
and treatment.
59.3 Clinical Testing
During speech and language evaluation, BH and his wife
reported that, at first, he had diffi culty “getting his words out,”
followed by progressive difficulty communicating in conversational interactions at home, in the community, and at work. At
the time of this assessment, BH was having more difficulty initiating speech. He denied difficulty comprehending language or
with memory function. According to BH and his wife, he was
still able to perform most aspects of his job, although he
reported increased difficulty communicating with customers
and pharmacy technicians.
BH participated in a motor speech evaluation and the Revised
Token Test,
Communication Disorders of Dementia (ABCD),
description task including the “cookie theft” picture from the
Boston Diagnostic Aphasia Examination,
Trees Test (PPT),
ency measure. His word fluency was examined for both spoken
and written generation of words beginning with “f,”“a,” and “s”
to determine the influence of a potential motor speech disorder
(e.g., AOS) on the efficiency of performance during a timed generative naming task.
On the motor speech evaluation, BH presented with slow
rate, inconsistent articulatory errors, distorted substitutions,
sound repetitions, voicing errors, and excess and equal stress.
His performance was consistent with a moderately severe AOS.
On language testing, his overall score on the Revised Token
Test was 13.35, which placed BH in the 80th percentile compared to individuals with left hemisphere lesions. He made a
few frank errors (e.g., selecting the wrong color or shape); however, primarily, he demonstrated “self-corrections” and “immediacy” responses (i.e., he initiated the gestural response to a
command before the command was completed). During the picture description, BH’s number of correct information units
(CIUs) and percent CIUs were consistent with nonaphasic performance as described by Nicholas and Brookshire
his CIUs per minute and percent CIUs per minute were consistent with the performance of individuals with aphasia. He was
able to recall 15 of 17 elements on the Story Retell subtest from
the ABCD immediately after presentation and following a 10minute delay, which was consistent with his report of little difficulty with memory. BH named 13 of 15 pictures on the BNT
and correctly answered all 52 items on the PPT, suggesting
naming and the ability to identify semantic associations were
relative strengths. Performance on the word fluency measure,
for both spoken and written naming, was impaired—17 and 19
total words, respectively—across the three letter categories. BH
spoke in complete sentences, with rare omission of function
words. He was able to write in complete sentences. The most
striking aspect of BH’s communication was the delay in initiating utterances, inconsistent articulatory errors (groping for
articulatory position, self-correction), and halting speech during motor speech and language testing. Based on this assessment and his history of a progressive decline in communication
function, he was diagnosed with nf-PPA.
1
Story Retell subtest from the Arizona Battery for
3
4
Boston Naming Test (BNT),5and a word flu-
the Pyramid and Palm
7
2
a picture
6
; however,
59.4 Questions and Answers for
the Reader
1. The results of BH’s testing suggest that memory is not
impaired. This finding is important because it potentially
rules out the presence of
a) Stroke.
b) Traumatic brain injury.
229
Script Training in Nonfluent/Agrammatic-Primary Progressive Aphasia
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
c) Dementia of the Alzheimer’s type.
d) Tumor
Answer: c is correct. Memory must be impaired to have a diagnosis of dementia of the Alzheimer’s type.
a is incorrect. One can have a stroke and not have an impair-
ment in memory.
b is incorrect. One can have a traumatic brain injury.
d is incorrect. One can have a tumor and not have an impair-
ment in memory.
2. Given BH’s primary deficit appears to be AOS, how would
you justify a diagnosis of nf-PPA?
a) The classification of the nonfluent/agrammatic variant of
PPA includes the presence of agrammatism and/or effortful halting speech with inconsistent speech sound er rors
and distortions.
b) AOS is a phonological deficit that is reflected in the
agrammatism that is associated with the nonfluent/
agrammatic variant of PPA.
c) The diagnosis is not the nonfluent/agrammatic variant of
PPA.
d) The classification of the nonfluent/agrammatic variant
excludes the presence of AOS as a significant sign or
symptom.
Answer: a is correct. AOS is considered one of two primary
characteristics of the nonfluent/agrammatic variant of PPA.
b is incorrect. AOS is a disorder of motor programming and
not a disorder of language or phonological encoding.
c is incorrect. The diagnosis of nonfluent/agrammatic variant
of PPA is accurate.
d is incorrect. The classification of nonfluent/agrammatic variant includes the potential presence of AOS as a significant sign
or symptom.
3. A 63-year-old patient presents with a 2-year history of pro-
gressive decline in communication. Upon exam, you observe
impaired confrontation naming and single-word comprehension and dyslexia. His repetit ion is intact and speech production is normal. What type of progressive speech and/or
language disorder would you diagnose the patient with?
a) Logopenic variant of PPA.
b) Semantic variant of PPA.
c) Primary progressive AOS.
d) Nonfluent/agrammatic variant of PPA.
Answer: b is correct. The semantic variant of PPA may result in
surface dyslexia, as well as preserved speech production.
a is incorrect. A primary feature of the logopenic variant of
PPA is impaired repetition, and sur face dyslexia is not an
expected feature.
c is incorrect. Primary progressive AOS would result in
impaired repetition and speech production.
d is incorrect. The nonfluent/agrammatic variant is characterized by impaired repetition and possible speech production
deficits.
4. A 58-year-old patient presents with a 3-year history of pro-
gressive decline in communication. Upon exam, you observe
impaired word retrieval in conversation and on naming tasks
and impaired repetition of sentences and phrases. Phonemic
paraphasic errors are noted in spontaneous speech. Single-
word comprehension and motor speech production are
spared. What type of progressive speech and/or language
disorder would you diagnose the patient with?
a) Nonfluent/agrammatic variant of PPA.
b) Semantic variant of PPA.
c) Primary progressive AOS.
d) Logopenic variant of PPA.
Answer: d is correct. A hallmark of the logopenic variant of PPA
is impaired repetition and the presence of phonemic paraphasias in conversational speech.
a is incorrect. Nonfluent/agrammatic variant of PPA is not
characterized by phonemic paraphasias and motor speech production is impaired.
b is incorrect. The semantic variant of PPA is not characterized by phonemic paraphasias and repetition is typically
spared.
c is incorrect. Primary progressive AOS, by definition, must
include impaired motor speech production.
59.5 Description of Disorder and
Recommended Treatment
Treatment planning focused on educating BH and his family,
exploring potential alternative and augmentative communication options for the future, and implementing treatment targeting his speech and language impairments and functional communication needs. A particular limitation for BH was that, due
to transportation issues and his work schedule, he could only
attend therapy every other week. Script training was selected
as one intervention because it permitted targeting BH’s relatively scripted daily work interactions and he could practice
with his wife at home. The purpose of script training is to
improve automatic natural language. Functional scripts are
developed by the individual with aphasia, his or her family, and
the clinician. The training protocol utilizes the principles of
motor learning, including the use of massed practice through
repetitive, cue-based drill of the individual utterances within a
script and the script in its entirety, and the distributed practice
of whole scripts in natural conversational contexts. The therapeutic effect of script training on speech and language production in individuals with aphasia has been reported in published
8,9
studies.
BH’s primary concern was difficulty communicating at work.
Seven scripts were developed: five related to workplace interactions and two related to social interactions. One or two scripts
were trained at a time using a multiple baseline design. A modified script training protocol based on work by Youmans and
colleagues
were trained individually. Training included the use of reading,
repetition, choral reading, and immediate and delayed spontaneous productions to practice accurate productions of each sentence. The script was then practiced in its entirety. BH was
expected to practice the scripts with his wife daily. In addition,
he had opportunities to use the scripts in the targeted home
and workplace interactions on a daily basis. An example of a
script for interacting with pharmacy customers is provided
▶ Fig. 59.1. For the three scripts that dealt with this type of
interaction, training included practice “cutting and pasting”
9
was employed. Initially, the sentences of a script
230
Script Training in Nonfluent/Agrammatic-Primary Progressive Aphasia
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
parts of the three scripts. That is, the clinician would merge elements from different scripts to build flexibility to use the
scripts functionally.
59.6 Outcome
During baseline and treatment probes, BH’s performance on the
scripts was scored online at the beginning of a session. The percentage of lines produced accurately within a script was measured. A response was considered accurate if it was initiated in a
timely manner, contained all words within the script, and all
words were produced accurately with the exception of mild distortions.
BH was seen for a total of 15 one-hour treatment sessions
over an 8-month period. His baseline performance on the first
three scripts (▶ Fig. 59.2), which targeted interactions with cus-
tomers, ranged from 20% to 40% accuracy. By the seventh session for each script, he reached 100% accuracy. Some variability
was noted during the maintenance phase of the first three
scripts. This variability was determined to be related to a
decrease in BH’s level of home practice during one period of
treatment. Scripts 4 and 5 targeted two specific social interactions with two visitors to his home during the summer. Scripts
6 and 7 targeted interactions with his pharmacy technicians
(▶ Fig. 59.3). Baseline performance ranged from 0% to 33% accuracy. He reached 100% accuracy on scripts 4 to 7 by the first session. Throughout treatment, BH and his wife reported that he
was communicating more effectively at work, both with
customers and staff members, and in the targeted social interactions. The first three scripts targeting interactions with customers were monitored for a longer period as they were
deemed to be the most important interactions.
59.7 Key Points
●
Treatments designed for individuals with nonprogressive
communication disorders may be applied successfully to
individuals with progressive speech and language disorders.
●
The data from this case report suggest that script training
may be successful in improving the communication of an
individual with nf-PPA.
●
When practiced in functional contexts, a patient’s ability to
utilize scripts in everyday communication will improve in
these contexts.
●
A combined treatment approach, including education, direct
language training, and augmentative and alternative communication is necessary for individuals with PPA.
Fig. 59.1 Example of script for interacting with pharmacy customers.
Suggested Readings
[1] Carthery, Goulart MT, da Costa da Silveira A, Machado TH, et al. Nonpharma-
cological interventions for cognitive impairments following primary progressive aphasia: a systematic review of the literature. Dement Neuropsychol.
2013; 7(1):122–131
Fig. 59.2 BH baseline, treatment, and maintenance data for scripts 1–3.
231
Script Training in Nonfluent/Agrammatic-Primary Progressive Aphasia
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
Fig. 59.3 BH baseline, treatment, and maintenance data for scripts 4–7.
References
[1] McNeil MR, Prescott TE. Revised Token Test. Austin, TX: Pro-Ed; 1978
[2] Bayles KA, Tomoeda CK. Arizona Battery for Communication Disorders of
Dementia. Tucson, AZ: Canyonlands Publishing; 1993
[3] Goodglass H, Kaplan E, Barresi B. Boston Diagnostic Aphasia Examination. 3rd
ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001
[4] Howard D, Patterson KE. The Pyramids and Palm Trees Test: A Test of Seman-
tic Access from Words and Pictures. Bury St. Edmunds, UK: Thames Valley
Test Company; 1992
[5] Kaplan E, Goodglass H, Weintraub S. Boston Naming Test. Pro-Ed; 2001
[6] Nicholas LE, Brookshire RH. A system for quantifying the informativeness and
efficiency of the connected speech of adults with aphasia. J Speech Lang Hear
Res. 1993; 36(2):338–350
[7] Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary
progressive aphasia and its variants. Neurology. 2011; 76(11):1006–1014
[8] Cherney LR, Kaye RC, van Vuuren S. Acquisition and maintenance of scripts in
aphasia: a comparison of two cuing conditions. Am J Speech Lang Pathol.
2014; 23(2):S343–S360
[9] Youmans G, Holland A, Muñoz M, Bourgeois M. Script training and automa-
ticity in two individuals with aphasia. Aphasiology. 2005; 19(3–5):435–450
232
Hyperkinetic Dysarthria
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
60 Hyperkinetic Dysarthria: Dystonic Features in a Patient
with a History of Brainstem Encephalitis
Heather M. Clark
60.1 Introduction
One role of the speech-language pathologist (SLP) is to differentially diagnose motor speech disorders to aid physicians, typically neurologists, in establishing a medical diagnosis.
case highlights several aspects of clinical decision-making: (1)
selection of assessment procedures commensurate with clinical
goals; (2) differential diagnosis based on patterns of speech features; and (3) exploration of behavioral interventions to inform
recommendations to the treating clinician. The case further
highlights the similarities and differences in clinical features
arising from underlying spasticity and dystonia.
1
This
60.2 Clinical History and
Description
RM was a 29 -year-old right-h anded man who, 8 months prior
to presentation, developed brainstem encephalitis t reated
with antibiotics followed by speech and physical rehabilitation (▶ Fig. 60.1). His symptoms had been stable for several
months when he developed new-onset dysphagia, jerking of
the right arm and face, blurred vision, and left temporomandibular joint pain during speech. Prior to his evaluation at a
tertiary medical center, RM had 6 months of speech therapy,
twice per day, 5 days per week, focusing on breath control
and or al motor exercises. He did not feel his speech improved
as a result of therapy.
A summary of RM’s assessment is provided in ▶ Table 60.1.
RM was evaluated by three neurology subspecialties over the
course of 3 days. The first neurologic examination was remarkable for dysarthria, described by the neurologist as spastic with
nasality, as well as several other subcortical features consistent
with the previous diagnosis of brainstem encephalitis. Imaging
revealed symmetric bilateral hypertrophic olivary degeneration. The movement disorder neurology specialist documented
pursing mouth movements, intermittent thrusting of the jaw
down and to the left, and intermittent jaw clenching. These
symptoms, along with other clinical findings, yielded a diagnosis of oromandibular and upper limb dystonia and palatal
tremor, consistent with hypertrophic olivary degeneration.
Multichannel electromyogram with multiple electrodes over
the face, jaw, and palatal region revealed intermittent 8-Hz
tremor in the upper orbicularis oris muscle bilaterally and
speech-induced high-amplitude tonic contractions of palatal
muscles. These findings were determined to be consistent with
palatal dystonia. The final diagnoses were brainstem encephalitis without active infection or inflammation and dystonia.
tasks, and speech during picture description, word and sentence repetition, and diadochokinetic tasks.
symmetric at rest. Volitional lip retraction and rounding were
normal. Intermittent lip pursing, which appeared unintentional, was noted at rest. The tongue protruded at midline and
moved laterally with full range, strength, and speed. No lingual
atrophy or fasciculations were noted. The velum was immobile
during phonation but moved with full range with elicitation of
gag reflex and cough. Cough was norm al with respect to sharpness. Suck, snout, and palmomental reflexes were negative.
Alternate and sequential motion rate (AMR/SMR) movements
were produced at a rate of approximately five repetitions per
second and with good regularity, but were imprecise and
associated with reduced pressure of articulatory contacts. RM
sustained phonation for 14 seconds with mild strain, but subsequent attempts were more normal with regard to vocal quality,
without evidence of strain or tremor.
RM’s connected speech was characterized by severe hyperna-
sality with intermittent audible nasal emissions, mild intermittent phonatory strain, and moderately imprecise articulation.
Speaking rate was equivocally reduced, associated with pauses
between words and phrases rather than prolonged segments.
Overall loudness and pitch variation were within normal limits.
Intelligibility was fair in conversation, but repetition and/or
clarification were required for most utterances without contextual cues.
Videofluorographic evaluation, conducted separately by
another SLP, revealed oropharyngeal swallowing within functional limits. Palatal elevation was mildly and inconsistently
reduced, without evidence of nasal regurgitation.
1
RM’s face was
60.4 Questions and Answers for
the Reader
1. The presence of adventitious movements of lips raised the
potential for dystonia. Several sensory tricks were introduced
to assess the potential to reduce adventitious movements.
Chewing gum had minor facilitative effects but did not
completely normalize speech. A small stick placed between
the lips or a finger resting on the lips resulted in equivocal
reduction in lip pursing during speech. Involuntary (or adventitious) movements are most closelyassociatedwith what
type of dysarthria?
a) Ataxic.
b) Spastic.
c) Hypokinetic.
d) Hyperkinetic.
Answer: d is correct. Hyperkinetic dysarthria is defined by the
60.3 Clinical Testing
RM underwent motor speech examination that included observation of orofacial structure and function during nonspeech
presence of involuntary movements. The features present in
hyperkinetic speech reflect the underlying movement disorder
and the speaker’sefforts to suppress or compensate for the
involuntary movements.
233
Hyperkinetic Dysarthria
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
Fig. 60.1 Timeline of illness and care prior to
presentation at tertiary medical center.
a is incorrect. Ataxic dysarthria is associated with uncoordi-
nated movements.
c) Oral weakness and absent pathologic reflexes.
d) Involuntary movements and pathologic reflexes.
b is incorrect. Spastic dysarthria is associated with slow
movements.
c is incorrect. Hypokinetic dysarthria is associated with
movements that are difficult to initiate and that are reduced in
range. Some speakers with hypokinetic dysarthria exhibit tremors in the jaw or tongue, and these movements are, in fact,
involuntary. However, the most prominent features of hypokinetic dysarthria arise from impairments other than involuntary
movements.
2. Which aspects of RM’s performance are consistent with
spastic dysarthria?
a) Breathy voice and slow rate.
b) Strained voice and hypernasality.
Answer: b is correct. The referring neurologist characterized
RM’s speech disorder as spastic dysarthr ia, which would indeed
1
be expected in the context of brainstem encephalitis.
The classic presentation of spastic dysarthria is strained vocal quality
(typically constant and consistent across speech tasks and sustained phonation), slow rate, monopitch and monoloudness,
imprecise articulation, hypernasality, and slow and regular
2,3
AMRs.
spastic dysarthria include orofacial weakness,
Nonspeech observations supporting the diagnosis of
4
slow oral move-
ments, reduced sharpness of glottal coup, pathologic oral
2
reflexes, and pseudobulbar affect.
Of these features, RM dem-
onstrated strained phonation (although this was mild, intermit-
234
Hyperkinetic Dysarthria
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
tent, and typically absent during sustained phonation) and
hypernasality, which was disproportionate to all other speech
features. Speaking rate was only equivocally slow, associated
with intermittent interword pauses rather than lengthened
segments. Articulatory precision was reduced in the absence of
weakness or slowness of the lips or tongue. Def ining features of
spastic dysarthria absent from RM’s speech were monopitch
and monoloudness and slow AMRs. Moreover, none of the supporting nonspeech observations typical of spastic dysarthria
were evident (▶ Fig. 60.2).
a is incorrect. Slow rate is a prominent feature of spastic
dysarthria, but RM’s speaking rate was only equivocally slow.
RM exhibited mild and intermittent strained vocal quality,
which may be observed in spastic dysarthria. Breathy vocal
quality would be very unusual in spastic dysarthria.
c is incorrect. Although speakers with spastic dysarthria may
demonstrate oral weakness, pathologic oral reflexes are more
often present than absent. RM demonstrated a normal nonspeech exam—no oral weakness or pathologic oral reflexes
were observed.
d is incorrect. RM demonstrated involuntary movements,
but such movements are associated with hyperkinetic dysarthria, not spastic dysarthria. Speakers with spastic dysarthria
often exhibit pathologic oral reflexes, but RM did not.
3. Which of the following statements about RM’s previous
therapy is false?
a) The intensity (frequency and duration) of therapy RM
received in the outpatient setting is typical of treatment
schedules for adults receiving speech therapy in the
United States.
b) The intensity of therapy may have been warranted given
the severity of RM’s dysarthria.
c) RM’s therapy included oral strengthening exercises even
though oral weakness is not typical of hyperkinetic
dysarthria.
d) RM’s recollection of the goals and activities of therapy
may have been inaccurate or incomplete.
Answer: a is correct. Treatment twice per day for several
months would be highly unusual in the United States. A growing literature is informing the development of guidelines for
treatment “dose.” For now, many decisions about treatment
dosage in the United States are dictated by third-party payer
policies and availability of clinical and financial resources.
b is incorrect. As mentioned, the literature does not yet provide clear guidance about whether or how dysarthria severity
might influence decisions about treatment intensity. However,
in the absence of limitations in clinical or financial resources, it
would be understandable for a speaker to seek intensive therapy for a communication disorder so disruptive to participation
and quality of life.
c is incorrect. Hyperkinetic dysarthria is not typically associated with oral weakness, yet RM completed oral st rengthening
exercises as part of his speech therapy. It is tempting to judge
negatively the decisions made by the treating clinician, but a
number of factors may have influenced the goals and activities
selected for RM. First, not all (and probably only a small percentage of) SLPs arrive at a differential speech diagnosis and
those who do will likely make occasional mistakes. In RM’s case,
it is quite possible that the diagnosis of hyperkinetic dysarthria
had not been made previously, so the logical conclusion that
strengthening exercises were not needed may have not been
obvious. Moreover, RM may have displayed different clinical
signs at the time of the e arlier therapy. It is possible that he
Table 60.1 Timeline of medical workup and speech pathology assessment/intervention
Date Subspecialty/test Key findings
1/13 General neurology Spastic dysarthria with nasality, myoclonic jerks of right arm
1/13 Speech pathology (dysphagia) Mildly and inconsistently reduced velar elevation without nasal regurgitation
1/14 Speech pathology
(communication)
1/14 MRI Symmetric bilateral hypertrophic olivary degeneration
1/15 Speech therapy Improvement in hypernasality with gum chewing
1/16 Speech therapy Extended and expanded RM's ability to sustain appropriate oral resonance to additional phonetic
1/17 Speech therapy Severe dystonia; sensory tricks less successful; reducing speaking rate exacerbates dystonic
1/17 Movement disorder consultation History of brainstem encephalitis
1/20 Speech pathology exit counseling
1/20 Movement disorders laboratory Palatal dystonia
1/21 EEG No EEG correlate for tremor and shiver
1/21 Neurology exit counseling Brainstem encephalitis
Normal language
Hyperkinetic dysarthria (dystonia)
Initial progress in eliminating nasal flow during production of “sh” and /s,z/
contexts and longer utterances; established normal intonation of short phrases
effects on hypernasality
Dystonia, with oropharyngeal/palatal and upper extremity involvement, secondary to
encephalitis
Tremor versus dystonia, secondary to encephalitis
Possible palatal tremor, secondary to encephalitis
Vertical nystagmus, secondary to encephalitis
Mild facial tremor
Dystonia
Anxiety
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Hyperkinetic Dysarthria
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
Fig. 60.2 Venn diagram illustrating similarities
and differences in features associated with spastic
dysarthria and hyperkinetic dysarthria associated
with dystonia. Features displayed by RM are
starred.
demonstrated oral weakness, perhaps from disuse or deconditioning associated with his illness; what was observed at the
tertiary medical center may have been, in fact, evidence of
effective treatment.
d is incorrect. Most people have at least slightly inaccurate or
incomplete recollection of most life events. Moreover, it is possible that RM never fully understood the rationale for the activities he completed during therapy. Finally, it is common for two
or more individuals involved in an interaction to have different
perspectives about the nature, reason for, and success of any
undertaking. For this reason, it is very helpful to have access to
the written reports related to previous assessments or therapy.
At the very least, differences in perspective can be identified,
and in many cases the information in the reports provides
insight into the current clinical picture.
60.5 Description of Disorder and
Recommended Treatment
The diagnosis of hyperkinetic dysarthria associated with dystonia
was informed by the following observations: (1) Hypernasality
disproportionate to other deviant speech features, with preserved
reflexive velar movements and effective velopharyngeal valving
in select nonspeech movements (blowing bubbles through a
straw). Hypernasality more severe than other speech features is
most typically observed in flaccid dysarthria affecting the 10th
cranial nerve; however, in that case, reflexive movements would
also be affected. In the absence of evidence of lower motor neuron impairment, focal dystonia best accounts for disproportionate
impairment of specific muscle groups. (2) Adventitious lip pursing at rest and during speech: focal dystonia may be speechinduced but can also be observed at rest and during vegetative
movements.
sensory tricks. RM reported that chewing gum improved oral
motor function (he attributed the benefit to “relaxing [of] the
jaw”). Moreover, introduction of sensory tricks during the examination improved hypernasality and articulatory precision. (4)
Mild and intermittent phonatory strain. Focal laryngeal dystonia
most commonly presents in the form of spasmodic dysphonia,
1
(3) Modest improvement in speech with the use of
but can be observed in the context of generalized dystonia as
1
It is not unusual for phonatory strain associated with
well.
laryngeal dystonia to vary across tasks or even across trials.
1
(5)
Normal nonspeech oral motor function (with the exception of
adventitious lip movements). Finally, the remaining speech
features displayed by this patient (slow rate, articulatory imprecision) were not incompatible with dystonia. In summary, hyperkinetic dysarthria secondary to dystonia accounted for nearly all of
the deviant speech features observed (▶ Fig. 60.2). Moreover , the
subsequent identification of limb, oral, and palatal dystonia by
the movement disorder specialist lent additional support to the
communication diagnosis.
The following recommendations were offered during the
postassessment counseling session:
●
Botulinum toxin (Botox) injection is a first-line treatment for
cervical,
been explored with mixed success for lingual dystonia.
5
laryngeal,6and oromandibular7dystonia and has
8
Although no literature was identified describing the use of
Botox for palatal dystonia, it has been used to treat palatal
9,10
tremor and palatal myoclonus.
SLPs do not administer this
treatment but can offer input regarding potential benefit and
risks for speech and swallowing. Risks of palatal Botox injection to the palatoglossus include reduced posterior oral seal,
placing RM at risk for aspiration before the swallow. Positive
indicators include normal baseline swallowing assessment;
postinjection assessment could identify potential compensatory strategies to mitigate effects of reduced posterior seal.
Potential benefits include direct improvement from relaxation of injected muscles, as well as possible secondary
benefits for noninjected muscles due to altered sensorimotor
function acting as a sensory trick. These potential risks and
benefits were reviewed with RM and family members.
●
Fitting with a palatal lift to address hypernasality. A palatal
lift could hypothetically improve speech via two mechanisms.
First, the lift could facilitate velopharyngeal closure and thus
improve oral resonance, as previously demonstrated for other
11
types of dysarthria.
Second, the lift had the potential to
serve as a sensory trick, alleviating the dystonia and allowing
the palate to elevate more freely. RM indicated that his local
SLP collaborated with an orthodontist who may have the
236
Hyperkinetic Dysarthria
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
necessary expertise to fit a palatal lift/prosthesis. He was also
provided with the name of a prosthodontist in his home city
with experience with palatal lifts.
●
Sensory tricks. Gum chewing and, to a lesser extent, placing
the finger on the lips, had some benefit for improving speech
production. RM was cautioned that the benefits of sensory
12,13
tricks can decline with their use
and that he should therefore use gum chewing expeditiously and only when it is most
important for his speech to be clear.
●
Speech therapy/oral motor exercises. No evidence of weakness
1
was detected in the speech mechanism; therefore, it was no longer necessary for him to continue with exercises targeting breath
support, vocal cord adduction, or movements of the speech
articulators outside of speech movements. Instead, it was recommended that the emphasis shift to speech production.
●
Speech therapy/speech production. A motor learning–bas ed
approach emphasizing RM’s auditory, tactile, and kinesthetic
awareness during relaxed phonation and oral airflow during
14,15,16
speech was recommended.
It was further recommended
that a speech subsystems approach be adopted, establishing
integrity of the systems in the following order: velopharyngeal
17,18
closure, phonation, articulation, and prosody.
The respiratory system was not impaired and was not recommended as a
target for intervention. Although categorized as independent
systems, the systems function interactively during speech production. The order of the targets is intended to reflect the area
of “attention” that will guide the focus of feedback during
speech tasks. Ideally,medical and/or prosthetic interventions
would facilitate speech movements so that speech production–
focused therapy would be more fruitful.
60.6 Outcome
As is often the case in tertiary medical centers, ongoing treatment was not sought as RM and his family returned to their
home country. The author had the opportunity to use RM as a
teaching case for neurology staff and trainees.
60.7 Key Points
●
Differential diagnosis of dysarthria requires consideration of
the pattern of speech features, and is supported by nonspeech
and neurologic findings.
●
Adventitious movements, as well as speech features that vary
in severity across tasks, should raise suspicion of hyperkinetic
dysarthria.
●
Diagnostic therapy can help conf irm or refute the differential
diagnosis.
Suggested Readings
[1] Esper CD, Freeman A, Factor SA. Lingual protrusion dystonia: frequency, etiol-
ogy and botulinum toxin therapy. Parkinsonism Relat Disord. 2010; 16(7):
438–441
[2] Sinclair CF, Simonyan K, Brin MF, Blitzer A. Negative dystonia of the palate: a
novel entity and diagnostic consideration in hypernasal speech. Laryngoscope. 2015; 125(6):1426–1432
References
[1] Duffy J. Motor Speech Disorders: Substrates, Differential Diagnosis, and
Management. 2nd ed. St. Louis, MO: Elsevier Mosby; 2013
[2] Clark HM, Duffy JR, Whitwell JL, Ahlskog JE, Sorenson EJ, Josephs KA. Clinical
and imaging characterization of progressive spastic dysarthria. Eur J Neurol.
2014; 21(3):368–376
[3] Darley FL, Aronson AE, Brown JR. Differential diagnostic patterns of dysarth-
ria. J Speech Hear Res. 1969; 12(2):246–269
[4] Clark H, Duffy J, Strand E, Hanley H, Solomon NP. Orofacial muscle tone &
strength across the dysarthrias. In: Annual Convention of the American
Speech-Language Hearing Association. Atlanta, GA; 2012
[5] Marsh WA, Monroe DM, Brin MF, Gallagher CJ. Systematic review and meta-
analysis of the duration of clinical effect of onabotulinumtoxinA in cervical
dystonia. BMC Neurol. 2014; 14(1):91
[6] Watts C, Nye C, Whurr R. Botulinum toxin for treating spasmodic dysphonia
(laryngeal dystonia): a systematic Cochrane review. Clin Rehabil. 2006; 20(2):
112–122
[7] Persaud R, Garas G, Silva S, Stamatoglou C, Chatrath P, Patel K. An evidence-
based review of botulinum toxin (Botox) applications in non-cosmetic head
and neck conditions. JRSM Short Rep. 2013; 4(2):10
[8] Budak F, Aydın E, Koçkaya A, Ilbay G. Botulinum toxin in the treatment of lin-
gual dystonia induced by speaking. Case Rep Neurol. 2013; 5(1):18–20
[9] Conill Tobías N, de Paula Vernetta C, García Callejo FJ, Marco Algarra J. Objec-
tive tinnitus from palatal myoclonus. Use of botulinum toxin: a case report.
Acta Otorrinolaringol Esp. 2012; 63(5):391–392
[10] Penney SE, Bruce IA, Saeed SR. Botulinum toxin is effective and safe for pala-
tal tremor: a report of five cases and a review of the literature. J Neurol.
2006; 253(7):857–860
[11] Yorkston KM, Spencer K, Duffy J, et al. Evidence-based practice guidelines for
dysarthria: management of velopharyngeal function. J Med Speech Lang
Pathol. 2001; 9(4):257–274
[12] Albanese A. The clinical expression of primary dystonia. J Neurol. 2003; 250
(10):1145–1151
[13] Loyola DP, Camargos S, Maia D, Cardoso F. Sensory tricks in focal dystonia and
hemifacial spasm. Eur J Neurol. 2013; 20(4):704–707
[14] Clark HM. Neuromuscular treatments for speech and swallowing: a tutorial.
Am J Speech Lang Pathol. 2003; 12(4):400–415
[15] Maas E, Robin DA, Austermann Hula SN, et al. Principles of motor learning in
treatment of motor speech disorders. Am J Speech Lang Pathol. 2008; 17(3):
277–298
[16] Verdolini K. Principles of skill acquisition applied to voice training. NCVS Sta-
tus Prog Rep. 1994; 6:155–163
[17] Dworkin JP. Motor Speech Disorders: A Treatment Guide. St. Louis, MO: Mos-
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[18] Yorkston KM, Beukelman D, Strand E, Hakel M. Management of Motor Speech
Disorders in Children and Adults. 3rd ed. Austin, TX: Pro-Ed; 2010
237
Exploring Clinician Readiness for Interstate Telepractice
Branski and Molfenter, Speech-Language Pathology Casebook (ISBN 978-1-62623-487-1),
copyright © 2020 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
61 Exploring Clinician Readiness for Interstate Telepractice
Ellen R. Cohn and Jana Cason
61.1 Introduction
Telepractice (i.e., telespeech and teleaudiology) is a rapidly
evolving service-delivery model. The American Speech-Language-Hearing Association (ASHA) defines telepractice as: “…
the application of telecommunications technology to the delivery of speech-language pathology and audiology professional
services at a distance by linking clinician to client/patient or
clinician to clinician for assessment, intervention, and/or consultation.”
different terms, including telemedicine, telehealth, and telerehabilitation. This case describes the process for speech-language pathologists (SLPs) and audiologists to determine the
appropriateness of this service-delivery model (▶ Fig. 61.1).
1
Other rehabilitation and medical disciplines employ
61.2 Clinical Description
RS, a school-based SLP with 15 years of experience, lives near
Toledo, Ohio, close to the border of Michigan. RS works full time
during the school year in a suburban public school district.
Because she is very new to telepractice, she joined ASHA’s Special Interest Group on Telepractice to learn more. She has no
other experience or training in telepractice. To earn extra
money, RS wishes to engage in private telepractice during the
summer months, especially when she spends time at the ocean
visiting relatives in New Jersey and Maryland. She decided to
focus on a school-aged caseload located in a neighboring state
(Michigan) that will not pose a conflict of interest with her fulltime employment in Ohio.
61.3 Clinical Scenario
It is currently mid-June, and RS intends to begin telepractice in
July. The following is in place for her to begin telepractice with
patients in Michigan:
1. RS holds a state license to practice in the state of Ohio and is
qualified to work in the public schools.
2. RS has access to a laptop computer equipped with a camera.
This laptop belongs to her husband and he intends to keep
using the computer when RS is not using it.
3. RS intends to use a free version of a popular videoconferencing software. She already employs this software to talk with
friends and relatives. The address is posted on her public
Facebook account. She uses the same password for all of her
communication technologies so that she does not forget.
4. RS intends to use her personal, free e-mail account for scheduling, billing, and to send communications about therapy.
5. RS has password-protected Internet service in her home. She
also intends to use free Wi-Fi provided at hotels or coffee
shops to maintain her practice when she travels.
6. RS owns assessment instruments for both articulation and
language. Because these are not yet adapted for use over the
Internet, she plans to hold them up to the camera, as needed.
She will follow the same practice for commercially available
therapy materials presented in kits or spiral notebooks.
61.4 Questions and Answers for
the Reader
1. What aspects of RS’s preparation and clinical readiness are
positive indicators for safe and successful telepractice with
a school-aged populat ion?
a) RS holds the ASHA Certificate of Clinical Competence and
is a school-based clinician with 15 years of experience.
b) RS has access to her husband’s laptop computer that is
equipped with a camera and she is experienced with a
free version of a videoconferencing program.
c) RS has a password-protected service to access the Internet
in her home and will use free Internet access when she
travels.
d) RS owns assessment instruments for both articulation
and language and has identified a strategy to adapt their
use for telepractice.
Answer: a is correct: RS holds the ASHA Certificate of Clinical
Competence, and is a school-based clinician with 15 years of
experience. She is qualified to provide services to a school-aged
population.
b is incorrect: RS must further evaluate the use of private and
secure technology and software before delivering services via
telepractice.
c is incorrect: Public Internet is not secure. RS must use password-protected Internet access when engaging in telepractice.
d is incorrect: RS must consult with the assessment publishers for their guidance on the use of the assessments when
delivered through telepractice.
2. What aspects of RS’s preparation and clinical readiness need
to be bolstered?
a) Selection and use of telepractice technology.
b) Knowledge of assessment tools and materials sanctioned
by their publishers for use with telepractice.
c) Acquisition of appropriate state licenses to practice speech
therapy.
d) All of the above.
Answer: d is correct: RS is not yet demonstrating the preparation and clinical readiness to engage in telepractice in a manner
that ensures privacy and security. She requires f urther training
in both the selection and use of telepractice technology. She is
not yet knowledgeable about what commercially available and
copyrighted assessment tools and materials are sanctioned by
their publishers for use with telepractice. She does not yet hold
a license in the state where her prospective clients are located.
The “location of practice” is identified as the location of the client; thus, in most cases, a practitioner must be licensed in the
state where the client is located.
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