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 ©2016 MFMER | slide-1 ©2016 MFMER | slide-2 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 ©2016 MFMER | slide-3 ©2016 MFMER | slide-4 1 4/19/2018 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 ©2016 MFMER | slide-5 ©2016 MFMER | slide-6 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 ©2016 MFMER | slide-7 ©2016 MFMER | slide-8 2 4/19/2018 Primary Progressive Aphasia (PPA) • A rare degenerative, neurological syndrome. • Insidious onset. • Progressive. • Primary and initial complaint of language Primary Progressive Aphasia (PPA) difficulties. ©2016 MFMER | slide-9 ©2016 MFMER | slide-10 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. ©2016 MFMER | slide-11 ©2016 MFMER | slide-12 3 4/19/2018 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 ©2016 MFMER | slide-13 ©2016 MFMER | slide-14 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. ©2016 MFMER | slide-15 ©2016 MFMER | slide-16 4 4/19/2018 PPA implies a disorder of language Primary Progressive impairment. Apraxia of Speech Aphasia may not even be present! ©2016 MFMER | slide-17 ©2016 MFMER | slide-18 PPAOS Insidious Progressive Motor planning difficulty ©2016 MFMER | slide-19 ©2016 MFMER | slide-20 5 4/19/2018 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 ©2016 MFMER | slide-21 ©2016 MFMER | slide-22 Motor processes of speech PPAOS Types Prosody PPAOS Articulation Resonance Phonation Prosodic Phonetic Respiration ©2016 MFMER | slide-23 ©2016 MFMER | slide-24 6 4/19/2018 Classification is not dichotomous, but a relative predominance Clinical Information Phonetic Prosodic • Demographics abnormalities abnormalities • Severity • Language • Cognition • Neurologic ©2016 MFMER | slide-25 ©2016 MFMER | slide-26 How does the initial presentation influence Some ideas… disease progression? Prosodic Phonetic ©2016 MFMER | slide-27 ©2016 MFMER | slide-28 7 4/19/2018 THE MAIN FOCUS: Differential diagnosis Apraxia of Speech Part Two Differential Diagnosis Progressive Aphasia ©2016 MFMER | slide-29 ©2016 MFMER | slide-30 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! ©2016 MFMER | slide-31 ©2016 MFMER | slide-32 8 4/19/2018 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 ©2016 MFMER | slide-33 ©2016 MFMER | slide-34 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 ©2016 MFMER | slide-35 ©2016 MFMER | slide-36 9 4/19/2018 Tests to Assess and Characterize AOS Apraxia of Speech Rating Scale (ASRS) • Apraxia of Speech Rating Scale (ASRS) • Articulatory Error Score (AES) ©2016 MFMER | slide-37 ©2016 MFMER | slide-38 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) ©2016 MFMER | slide-39 ©2016 MFMER | slide-40 10 4/19/2018 Third step • Is dysarthria present? ©2016 MFMER | slide-41 ©2016 MFMER | slide-42 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 ©2016 MFMER | slide-43 ©2016 MFMER | slide-44 11 4/19/2018 Case History Question Suggestions Case History: Speech • Any difficulties with speech or language as a • Speech