Progressive Speech and Language Disorders

4/19/2018

Outline • Part One: Background • Primary Progressive Aphasias (PPA) • Semantic Variant of PPA Progressive Speech and Language • Logopenic Variant Disorders • Agrammatic/ non-fluent variant of PPA • Primary Progressive Apraxia of Speech Rene L. Utianski, PhD, CCC-SLP (PPAOS) [email protected] • Part Two: Differential Diagnosis/ Case Studies • Part Three: Treatment

Three General Components of Speaking

• Cognitive linguistic processing 1 Part One Primary Progressive Aphasias and Primary • Sensorimotor Progressive Apraxia of Speech 2 planning/programming

• Neuromuscular execution 3

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Three General Components of Speaking: What if something goes wrong? What if something goes wrong? • Brain lesion • Cerebrovascular Accident or Stroke (CVA) • Cognitive linguistic processing • Ischemic due to thrombosis or embolism 1 • Aphasia • Hemorrhagic due to aneurysm or AVM • Trauma (TBI) • Sensorimotor planning/programming • Tumor 2 • Apraxia of speech • Infection • Neuromuscular execution 3 • Dysarthria

Three General Components of Speaking: What if something goes wrong? What if something goes wrong? • Brain lesion • Cognitive linguistic processing • Cerebrovascular Accident or Stroke (CVA) • Dementia • Occlusive (ischemic) due to thrombosis or 1 • Progressive aphasia embolism • Hemorrhagic due to aneurysm or AVM • Sensorimotor planning/programming • Trauma (TBI) 2 • Progressive apraxia of speech • Neoplasm (tumor) • Infection/Toxin • Neuromuscular execution • Disease Process 3 •Dysarthria

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Primary Progressive Aphasia (PPA) • A rare degenerative, neurological syndrome. • Insidious onset. • Progressive. • Primary and initial complaint of language Primary Progressive Aphasia (PPA) difficulties.

Gorno-Tempini et al. Diagnosis Criteria (2011) Variants of PPA • Pattern of deficits are not accounted for by • Semantic variant other nondegenerative nervous system, • Logopenic medical, or disorder at onset. • Agrammatic/ Non-fluent variant • Absence of episodic memory, visual memory, and visuo-perceptual impairments at onset. • Absence of behavioral disturbance at onset.

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Semantic variant of PPA Semantic variant of PPA • Anomia with loss of single word meaning. • Fluent spontaneous speech is mostly retained. • Difficulty generating and recognizing familiar • Some patients have problems recognizing words. familiar objects and faces. • For rare words first and common nouns for later • The presence of this sign can help confirm stages. the diagnosis. • Verbs and abstract words are often spared. • Report difficulty with writing and spelling, particularly with irregularly spelled words. • During confrontation naming, will demonstrate low confidence for identifying target word. • Surface dyslexia

Logopenic variant of PPA Agrammatic/ non-fluent variant of PPA • A slow rate of speech, secondary to word • Speech is effortful and reduced in quantity. retrieval difficulties. • Sentences become shorter and word-finding • Phonologic errors may be present. hesitations become more frequent. • Sentence and phrase repetition is impaired. • Pronouns, conjunctions and articles are lost. • Repetition of single words is spared. • Word order may be abnormal, especially in • Reading and writing abilities may be preserved writing or e-mails (agrammatic). longer than speech. • Reversals (e.g., “he” for “she”; “yes” for “no”). • Trouble understanding long or complex verbal • Reduced comprehension for long and information. grammatically complex sentences.

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PPA implies a disorder of language Primary Progressive impairment. Apraxia of Speech

Aphasia may not even be present!

PPAOS

Insidious

Progressive

Motor planning difficulty

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PPAOS Diagnostic Criteria Patient Perceptions and Complaints • Initial complaint of speech difficulty. • “Pure” AOS: • “my speech won’t come out right” • Unequivocal AOS. • “know what I want to say but doesn’t come out right” • not as fluent as before • No evidence of aphasia. • mispronounce words • with mild or moderate AOS, patients report being • Normal neurological examination. surprised by errors that “sneak into” narratives • complaints usually center around articulation problems • Normal neuropsychological testing. • some patients report having to speak slowly or more carefully to prevent errors • predict errors on multi-syllabic or difficult to pronounce words • recognize errors and attempt to correct them • problems may be more obvious when stressed or fatigued

Motor processes of speech PPAOS Types

Prosody PPAOS Articulation

Resonance Phonation Prosodic Phonetic Respiration

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Classification is not dichotomous, but a relative predominance Clinical Information

Phonetic Prosodic • Demographics abnormalities abnormalities • Severity • Language • Cognition • Neurologic

How does the initial presentation influence Some ideas… disease progression?

Prosodic Phonetic

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THE MAIN FOCUS: Differential diagnosis

Apraxia of Speech Part Two Differential Diagnosis Progressive

Aphasia

First step • Is aphasia present?

Assessment and differential diagnosis

Step 1: Is aphasia present? Step 2: Is apraxia of speech present? If so, what dominates? Step 3: Is dysarthria present? Largely completed simultaneously!

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Variants of PPA: assessment tips Semantic dementia • Agrammatic variant • Loss of function words/ morphological markers • Assess spoken language AND writing Logopenic Agrammatic • Semantic variant PPA PPA • Comprehension • Omission of • Surface dyslexia difficulties articles • Loss of word meaning • Logopenic variant Sound • Anomia without loss of single word meaning errors • Poor sentence repetition Apraxia of Speech • Comprehension deficits • Phonological errors are present

Tasks for Assessing Motor Speech Second Step Programming Capabilities • Is apraxia of speech present? • General conversational ability • Imitation (sounds, words and sentences) • Narrative picture description • Automatic tasks (counting, days of the week, sentence completion) • Singing a familiar song • Reading aloud • SMRs • Writing sample

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Tests to Assess and Characterize AOS Apraxia of Speech Rating Scale (ASRS) • Apraxia of Speech Rating Scale (ASRS) • Articulatory Error Score (AES)

01 2 3 4 Not Infrequent Frequent but not Nearly always evident but Nearly always observed in pervasive not marked in severity evident and marked any task in severity No more Noted more than once Noted 20-50% of all Noted on many utterances Noted on most Articulatory Error Score (AES) than one (but less than about utterances, but not on on most tasks but not utterances on most occurrence 20%) most tasks or utterances enough to decrease overall tasks and severe intelligibility enough to impact • “Repeat each of the • “Repeat these sentences intelligibility following words three one time.” Phonetic Features (based on speech tasks only (i.e., ignore AMR & SMR performance) 1 Sound distortions (excluding distorted substitutions or distorted additions) times each.” • We saw several wild 2 Distorted sound substitutions . Cat animals. 3 Distorted sound additions (including intrusive schwa) . Snowman • My physician wrote out . Catnip a prescription. 4 Increased sound distortions or distorted sound substitutions with increased utterance length . Artillery or increased syllable/word articulatory complexity . Catapult • The municipal judge Phonetic Subscore (Add scores for items 1-4) . Statistics Prosodic Features (based on speech tasks only (i.e., ignore AMR & SMR performance) sentenced the criminal. 5 Syllable segmentation within words > 1 syllable . Catastrophe . Stethoscope (Brief silent interval between syllables and/or inappropriate equalized stress across syllables) . Aluminum 6 Syllable segmentation across words in phrases/sentences . Harmonica (Increased inter-word intervals and/or inappropriate equalized stress across words) . Rhinoceros 7 Slow overall speech rate (apart from pauses for word retrieval and/or verbal formulation) . Specific . Volcano 8 Lengthened vowel &/or consonant segments independent of overall slow speaking rate

Prosodic Subscore (Add scores for items 5-8)

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Third step • Is dysarthria present?

Differential diagnosis: Dysarthria Other tips for diagnosis • Oral mechanism examination • History is critical! • AMRs • Insidious onset • Progression over time • SMRs • Asking the right questions is crucial • Groping? • Distortions v. substitutions v. distorted substitutions

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Case History Question Suggestions Case History: Speech • Any difficulties with speech or language as a • Speech better at any time of the day? child? • Things that make it worse? • How would you describe the difficulties you • Does alcohol have any effect? have been having? • Any point at which it completely returns to • How long have the difficulties been occurring? normal? • Was the onset sudden? Or gradual? • Any situation in which people have a difficult • Any worse over time? time understanding you? • Difficulty producing words, despite knowing what you want to say? • Difficulty with longer/ harder words?

Case History: Language Case History: Other • Difficulty understanding? • Any changes in handwriting? • Difficulty thinking of words? • Any changes in behavior or personality? • Mean to say one words and another word • Laughing or crying more easily? At comes out? i.e. dog for cat or cone for comb? inappropriate times? • Hear a word and not know what it means? • Any difficulty chewing or swallowing liquids or solids? • Difficulty with yes/ no? • Difficulty with left/ right? • Ever leave words out in speaking or writing?

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Questions? Summary

Is the disorder progressive?

• Change over time

Is aphasia present?

• Agrammatism • Loss of word meaning • Phonologic errors • Reduced comprehension

Is apraxia of speech present?

• Articulatory errors • Segmentation

Case Study One Case Study Two • 81 year old • 63 year old • 2 year history of difficulty expressing himself • 2.5 years symptom duration (“executing”) and reading • Reports difficulty remembering names of • Silent reading is normal in rate, accuracy, and people/ recalling their faces comprehension • Reports word-finding difficulty and increasing • Occasional word finding difficulties difficulty with reading and spelling • He denies difficulty with spoken or written • Denies difficulty with comprehension language comprehension

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Case Study Three Case Study Four • 62 year old • 69 year old • 2.5 year history of speech difficulty (noticeable • 1.5 year history of “missing words,” confusing to others only in the last 6 months) tenses, word order, and pronouns • Longstanding lisp • Denies difficulty with comprehension • Denies difficulty with word retrieval • Reports changes in handwriting (more laborious; larger print)

Part Three Treatment Using a Theoretical Framework to Guide the What is an intelligibility disorder? Clinical Management of Intelligibility Disorders

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POSSIBLE SOURCES OF What is an intelligibility disorder? INTELLIGIBILITY BREAKDOWNS A breakdown in the reception of a spoken message.

Acoustic signal Transmission Reception

•Dysarthria • Noisy • Hearing loss • Stuttering environments • Auditory • Cleft palate • Cell phone comprehension • Non-native • Acoustic deficits speech reverberation • Lack of • Cochlear • Cocktail party experience or implant signal familiarity with distorted signal • Synthesized speech (AAC) Transmission (environment)

Acoustic signal (sender) Reception (listener)

Let’s take a look at Dysarthria Differential diagnosis  Motor speech disorder  Examination of oral structure and function  AMR, SMR  Results from neurological injury due to damage in the Central or Peripheral Nervous Systems  Sustained /a/  Measures of intelligibility  Affects one, many, or all speech subsystems (respiration, resonance, phonation, articulation)  …but largely, via the associated medical diagnosis ◦ Hyperkinetic dysarthria- Huntington’s Disease ◦ Hypokinetic dysarthria- Parkinson’s Disease ◦ Mixed spastic-flaccid dysarthria- ALS ◦ Ataxic dysarthria- cerebellar degeneration (Mayo Classification System- Darley, Aronson, and Brown)

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Mayo clinic classification approach But does it matter? Hyperkinetic • Does the “dysarthria subtype” dictate your clinical decision making?

Hypokinetic Spastic Harshness • Probably not. Strained-strangled Articulatory imprecision Hypernasal Hyponasal Monotone Monopitch Flaccid Ataxic

Mixed

Mayo Clinic approach What do I mean by that?

1) there is considerable overlap in speech symptoms • We identify what is wrong and we try and fix it! among the classification categories, and 2) speech symptoms within a given classification may differ along the severity dimension. • Theory will tell us what will have the largest impact on the listener.  This classification approach does not map well to the resulting communication disorders or to intervention targets.  It does help diagnose neurologic disease!  Bottom line: we can’t treat the diagnosis, we need to treat the resulting intelligibility disorder!

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Speech Perception Theory A theory is a set of interrelated principles and definitions that present a systematic view “Intelligibility is as much in the ear of phenomena by specifying of the listener as it is in the mouth relationships among variables with the purpose of explaining of the speaker.” natural phenomena. (Martin & Weismer, 1992)

Theoretical perspective How does it do that?  Dysarthria interferes with perceptual processes:  To understand this interference, let’s take a look ◦ Activation of an optimal lexical candidate pool at “normal” speech perception ◦ Competition among lexical candidates ◦ Application of strategies to lexically segment Namely: the connected speech  How do listeners understand single words? and connected speech?  How does dysarthria interfere with these processes?

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Models of Spoken Word Recognition Time Course (e.g. Logogen, Cohort, Trace, Shortlist, Neighborhood Activation, PARSYN) • Time course Commonly assume that at least two fundamental • Left-to-right processing of acoustic information processes underlie spoken word recognition: • Delay decision until whole word is spoken

lexical activation: When a listener encounters a spoken word, certain aspects of the acoustic signal serve to activate a set of possible lexical candidates.

lexical competition: This is followed by competition among these candidates for the best fit with the input, and the winner of this competition is the recognized word.

Dysarthric speech and tip word recognition models “dip” tip top tap Distortions Omissions dip dip dip dot Vowel Substitutions reductions dab

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“_ip” Acoustic information Word in frequency it effects _ip ill Single if is word intelligibility

Syntactic Semantic probabilities priming

There is very little predictive value in Listener Acoustic expectations single word intelligibility signal quality about the Perceiving connected speech is a very different beast! Connected message speech intelligibility

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Deciphering Connected Speech Lexical Segmentation  Lexical activation and lexical competition are • A fundamental process in deciphering degraded strongly influenced by the message speech that determines “word size frames” for  Semantics, syntactics, knowledge of topic lexical access. and speaker: prime the lexical candidates and facilitate efficient competition  The quality of the acoustic signal is not critical as listeners listen for words, not phonemes ◦ Phonemic restoration  Listeners perform “lexical segmentation”

Every thing I say is easy to under stand.

Metrical Segmentation Strategy (Cutler & Norris, 1987) Lexical Segmentation • Semantically anomalous, syntactically correct When listeners encounter degraded speech

They listen for STRONG (stressed) syllables

Treat them as WORD ONSETS

And they’ll most often be right in English

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Fundamental frequency variation

Cues to If you don’t “chop up” the acoustic stream Loudness Syllable/vowel variation syllable duration into words correctly… strength You have no chance at using all available acoustic and top- down information to make a best match for the words.

Strong versus reduced vowels

Regardless of “dysarthria type,” Listeners are flexible different types of degradation patterns • Use available acoustic cues, even when they’re degraded; (e.g. slow rate, reduced pitch and • Can switch the amount of reliance on various cues, loudness variation, consonant depending on which are most robust and systematic; • They use their “higher level” knowledge to facilitate and imprecision), guide speech understanding yield different perceptual errors. • Semantics, syntax, phonotactics, knowledge of topic/ speaker

THIS is the intelligibility disorder! Context

Acoustic

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“Signal-Complementary Information” Activating optimal lexical candidates Lindblom, 1990 • Alphabet cueing • e.g. Hustad, Jones, & Dailey, 2003; Hustad, 2005 • Providing topic cues Augmented • e.g. Hustad, Auker, Natale, & Carlson, 2003; Jones, Mathy, Azuma, & perceptual Liss, 2004; Utianski, Azuma, and Liss, 2010 processing • Speech Signal • Improved • Providing semantic context transmitted performance • Listener applies • e.g. Dongilli, 1994; Hammen, Yorkston, & Dowden, 1991 information Listener Increased manipulation intelligibility

Lexical competition Perceptual training • Providing a syntactic template or structure • Training material • Creating semantically and syntactically • Talker-specific predictable utterances • Disorder-specific • Using supplementary cues such as first-letter, • Feedback type and frequency word-class cues, or gestures • Passive exposure vs. training • Generalization of minimal exposure/training • Training regimen and intensity

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Different patterns yield different errors Intelligibility results • Equivalent intelligibility • Phonetic errors • Segmentation errors

Metrical Segmentation Strategy Quality of lexical segmentation

• Rhythmic structures of language to identify 0.7 word boundaries 0.6 Ratio • Strong syllable as potential word onsets 0.5 (Cutler & Carter, 1987) 0.4 Control • Based on the statistical probabilities of English Familiarization 0.3 Segmentation • More likely to see insertions before strong Topic Knowledge syllables and deletions before weak syllables 0.2

Metrical 0.1

0 Hypokinetic Ataxic

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Identifying the potential source of benefit Topic words correct 50% 45% 40% • Do listeners more correctly transcribe topic words, regardless of a lack of overall intelligibility 35% for hypokinetic speech? 30%

correct Control 25% Familiarization

words 20%

Topic Knowledge % • Does the dysarthria type difference disappear 15% when we look at the listener’s ability to use topic 10% knowledge? 5% 27%29%30% 26% 34% 39% 0% Hypokinetic Ataxic

Take away points • Topic knowledge and familiarization offer equal perceptual benefits for ataxic speech • Sources of benefits for each speaker group • Listeners of hypokinetic speech rely on syllabic stress to segment words Research-practice divide • Listeners of ataxic speech utilize lexical Current pitfalls in the remediation of intelligibility disorders access and acoustic- phonetic remapping to segment words

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Theoretical and Clinical Perspectives

Clinical Goal: Determines how Theoretical Goal: characteristics of Advance a particular Clinic knowledge about clinical basic population Theory mechanisms relate to and processes hypotheses generated from a theory or model.

(Bernstein & Weismer, 2000)

Treating Intelligibility Disorders Speaker-based approaches to remediation • Subsystem Approach • Respiration • Phonation Speaker Listener • Resonance • Articulation • Global Approaches

Intelligibility

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Respiration Phonation • Modify posture, tone, strength • Hyperadduction: relax • Improve control of exhalation • Hypoadduction: pushing/pulling, maximum performance tasks • Use biofeedback or visual feedback • Traditional voice therapy • Modify abnormal breathing pattern • Compensation (breath groups)

Resonance Articulation • Prosthetic device/surgery • Articulation drills • Tactile/ kinesthetic feedback • Contrast drills • Visual feedback • Oral strengthening • Contrast drills • Range-of-motion exercises • CPAP

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Typical Approaches to Characterizing Modification of speech signal Intelligibility • Reducing speaking rate • Speaking louder • Single-word intelligibility tests • Is reduced loudness the problem? • Sentence intelligibility tests • Modifying prosody • Estimates from reading passages or spontaneous • Reduced pitch loudness and variation? connected speech Monopitch and monoloud?

The estimates of single-word intelligibility are generally poor predictors of connected speech intelligibility, except at very mild and very severe ends of the continuum.

Why is this the case? Treating Motor speech disorders  Listeners don’t listen for phonemes in connected speech. They listen for words or even phrases.

 Listeners apply their knowledge of syntax, Speaker Listener semantics, and phonotactics, and knowledge of the speaker to problem-solve degraded stretches of speech.

Intelligibility

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Face-to-face communication • Face the listener • Get the listener's attention before speaking • Audio-visual enhancement for comprehensibility How can we use a theoretical framework to guide clinical practice?

Transmission: Activate optimal lexical pool: Minimize environmental interference Alphabet Supplementation • Optimize the environment • Use an alphabet supplementation board, pointing to the first letter of each word as it is • Reduce background noise (the dishwasher, too; spoken. not just the tv!) • Avoid communication over a distance

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Syllable segmentation • What if hypernasality is interfering with • Slow speaking rate activation of the target lexical item? • Pausing between words but without separating syllables within words

Influence of familiarity Is PPAOS a special case? • Measures of speech intelligibility are subject to bias. • Are speakers improving, or are you becoming a better listener?

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Ways to quantify change to overcome bias Praat • Rate • Segmentation • Loudness

• Digital advances

Cases? Questions? Comments?