Hyperkinetic Dysarthria

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Hyperkinetic Dysarthria
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1. Lesion in basal ganglia in different areas or sometimes in cerebellar circuits and indirect pathways 2. Opposite of hypo-here there is too much involuntary movements 3. Hyper: extra, excess, but NOT FAST 4. Movements are always involuntary 5. Rhythmic/arrhythmic, regular/irregular 6. Movements occur when steadiness is expected 7. Typically worsen with tiredness, fatigue or stress 8. Abnormal movements disappear during sleep 9. These abnormal movements interrupt speech movements as well
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Types of Hyperkinesias
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1. Dyskinesia 2. Myoclonus 3. Tics 4. Chorea 5. Ballism 6. Athetosis 7. Dystonia 8. Spasm 9. Tremor
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Dyskinesia
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– Abnormal involuntary movement. – Orofacial dyskinesias, Tardive Dyskinesia (oro-buccal-lingual): caused by prolonged use of antipsychotic drugs. – Results in involuntary stereotyped, repetitive lip smacking, pursing, puffing and retraction, tongue protrusion, opening or closing, or lateral jaw movements. – Can affect respiration with subsequent affect on speech.
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Akathisia
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Inner sense of motor restlessness occurs in both TD and PD. (video: # 18 & 20)
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Myoclonus
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• Repetitive movements seen as brief jerks of muscle or body part • Rhythmic or nonrhythmic • Spontaneous or induced (sensory/movmt) • When induced by movement: AM • Myoclonic epilepsy, postanoxic myoclonus • Hiccups, PPM -Guillian-Mollaret triangle • Myoclonic movements cannot be inhibited consciously • (Video # 24)
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Tics
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• Rapid, stereotyped, coordinated, patterned movements • Under partial voluntary control • Simple tics difficult to distinguish from myoclonus or dystonia. • Complex tics are coordinated:, coprolalia, lip smacking, jumping, noises, sounds (throat clearing, grunting,barking etc) and touching, obscene gestures, echolalia, palilalia (Duffy video)
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Tics (Tourette’s Syndrome)
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• Gilles de la Tourette’s Syndrome (TS): coprolalia (5-15% of patients) M to F, 3 to 1 ratio • Body, facial movements (blinking, twitching, grimacing) -onset before 18 years • Complicated movements can briefly suppress the tics but not for very long • Some say if try to suppress, gets worse • TS- Tics occur at normal pauses and rarely occur in middle of the word (at boundaries) • TS co-occurs w/OCD, ADHD, stuttering, dyslexia, conduct disorder, panic attacks, multiple phobias, depression, and mania • Tics loci: Genetic, pathophysiology involves striatal dopamine receptor supersensitivity
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Chorea
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• Nonstereotypic movements, rapid & random, excessive, irregular, purposeless • Part of the body, hands, face, and legs • Can occur while stationary, or during sustained posture, and also during movmt • Cannot control or prevent • Degenerative (Huntington’s), infectious (Sydenham’s), or in response to drugs. (Duffy video)
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Ballism
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• Gross, abrupt contractions of distal and proximal muscles • Produces wild flailing movements • Unilateral: hemiballism • Typically lower parts of body: not speech • Lesions of subthalamic nucleus • Stroke is the most common cause
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Athetosis
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• Slow, writhing, purposeless movements that tend to flow into one another • Major category of Cerebral Palsy • Acquired athetosis: often a combination of chorea and dystonia • When chorea is predominant: choreoathetosis
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Dystonia
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• A very slow or sustained posture (involuntary, abnormal) with superimposed quick movements • Results from excessive contraction of antagonist muscles • Primary abnormal movements slow and sustained • Reflects combination of dopaminergic & cholinergic overactivity in basal ganglia • Typically affects one or multiple organs • Focal mouth dystonia/orofacial dystonia • Writer’s cramps/primary generalized dystonia (no associated other deficits) • Torticollis (cervical dystonia): tonic/clonic spasms causing deviation of head • Blepherospasm: forceful, spasmodic, sustained closure of eyes • (Focal dystonia-Duffy videos)
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Spasm
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• Muscle contractions that can be tonic (prolonged/continuous) or clonic (repetitive/rapid, brief) motion of muscle • Usually involuntary, often result in movement, but occasionally limit motion (avoiding back pain from movement) • Hemifacial spasm: rapid, irregular twitching of half of face (7th nerve)-no CNS
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Tremor
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Most common involuntary movement • Rhythmic movement of a body part • Resting tremor, postural tremor, action tremor, terminal tremor • Essential (familial) tremor: occurs with sustained posture and action. Commonly affects upper limbs, head, or voice-reduced by alcohol. (Video # 6)
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Hyperkinetic Dysarthria – Etiologies:
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1. Degenerative Diseases: Huntington’s 2. Toxic metabolic: drugs etc, TD 3. Infectious processes: sydenham’s chorea-strep or rhuematic fever, rubella, diphtheria, SLE, AIDS 4. Vascular disorders, diseases of BG 5. Neoplasms of BG & thalamus 6. Others: channelopathies
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Chorea- Huntington’s Disease
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• Huntington’s chorea is the former name • Inherited autosomal dominant degenerative neurologic disease • Child of an affected parent has 50% chance of inheriting it • Affects motor control, cognition, and personality
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Symptoms of Huntington’s Disease
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• Personality changes • Chorea • Dystonia • Incoordination • Dementia • Abulia (lack of motivation, apathy, decreased initiation of conversation, selective mutism if severe; not depression) • Dysarthria • Dysphagia • Weight loss
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Onset of Huntington’s Disease
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• Symptoms usually appear in 4th decade • After childbearing years • Doesn’t skip generations • 2 – 70 yrs onset! • Slow progression of disease • Death 15-20 yrs after onset • Progresses more rapidly in younger individuals • Men & woman affected equal proportions
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Neuropathology of Huntington’s Disease
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• Deficiency of GABA (“don’t go”) neurotransmitter and enkaphalin in basal ganglia • Atrophy of striatal bodies, degeneration of cortical neurons, frontotemporal atrophy-MRI • Leads to overactivity of dopaminergic systems • Parkinson’s disease patients may exhibit Huntington’s Disease symptoms if they overdose on L-dopa!
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Stages of Huntington’s Disease
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• Difficulty to stage severity of disease because so many factors can influence functional performance –> Which one(s) are most important? • Motor problems vs cognitive problems? • Current scales try to rate performance of daily functions rather than motor/cognitive status
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Diagnosing Huntington’s Disease
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– Positive family history – Occurrence of chorea or psychiatric disorders without other apparent cause – Schizophrenia may be diagnosed incorrectly – Pattern of progressive deterioration – Imaging studies may be helpful with more advanced disease (CT, MRI, PET)
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Medical Management of Huntington’s Disease
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– No cure – Treatment symptomatic – Medications can alleviate one symptom but aggravate others – PT and OT focus on how chorea interferes with daily functioning & pt compensation – ROM and strengthening exercises rec
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Communication Changes in Huntington’s Disease
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– Cognitive and language changes – Dementia common o Subcortical type – deficits in concentration & acquisition of new information o Different from Alzheimer’s & progressive supranuclear palsy – Do we treat it? What factors should we consider?
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Swallowing Problems in Huntington’s Disease
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o Hyperkinesia o Rigid-bradykinesia
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Hyperkinesia
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– Tachyphagia (fast swallowing; more belching from excessive air intake) – Respiratory Chorea – Eructation; excessive belching – Aerophagia due to lingual chorea
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Rigid-bradykinesia
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– Mandibular rigidity – Poor chewing – Slow oral transit
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Non Speech Characteristics-Huntington’s
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– OME: fairly normal – ROM & strength ok when not interrupted by choreaic movements – Reflexes: normal – Drooling & swallowing problems due to extraneous movements – Motor unsteadiness-inability to sustain posture
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Speech Characteristics in Huntington’s Disease
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– Hyperkinetic dysarthria – Characterized by variable speech patterns due to chorea associated with many speech muscle groups (Video # 23)
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Stages of Functional Limitations In Dysarthria- Huntington’s Yorkston et al (1999)
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*Stage 1:* No detectable speech disorder *Stage 2:* Obvious speech disorder with intelligible speech (use compensatory techniques such as overarticulation) *Stage 3:* Reduction in speech intelligibility (introduce AAC) *Stage 4:* Natural speech supplemented with augmentative techniques (start using AAC if appropriate) *Stage 5:* No useful speech
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DAB clusters-Huntington’s Disease
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A. Phonatory stenosis B. Resonatory Incompetence C. Articulatory-resonatory Incompetence D. Prosodic Excess E. Prosodic Inefficiency
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DAB clusters-Huntington’s Disease A. Phonatory stenosis
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– Due to abnormal choreaic movements in larynx, not hypermovement – Harsh voice – Excessive loudness variations – Strained/strangled voice quality (even voice stoppages)
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DAB clusters-Huntington’s Disease B. Resonatory Incompetence
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– Hypernasality – Short phrases (wastage of air through nose) – Imprecise pressure consonants
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DAB clusters-Huntington’s Disease C. Articulatory-resonatory Incompetence
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– Vowel Distortions – Imprecise Articulation – Irregular articulatory breakdowns (due to extraneous movements and not due to incoordination as in ataxic dysarthria)
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DAB clusters-Huntington’s Disease D. Prosodic Excess
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– Inappropriate silences – Prolonged intervals/phonemes – Excess/Equal stress
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DAB clusters-Huntington’s Disease E. Prosodic Inefficiency
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– Due to primarily the phonetic system – Monopitch/Monoloudness – Short phrases – Reduced stress
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DAB clusters-Huntington’s Disease F. Other
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– Sudden forced involuntary inhalation or exhalation
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Salient Features-Huntington’s:
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– Variability (transient & unpredictable) in all aspects of speech (artic, rate, resonance, phonation, and prosody) – Visual Features (of the other movements) – Bizarreness* – important feature of all hyperkinetic dysarthrias
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Dystonia:
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– Extrapyramidal disturbances but neural mechanisms not fully understood – Neurotransmitters may be involved (GABA, dopamine, acetylcholine, norepinephrine, & serotonin) – No paralysis or weakness but abnormal, involuntary movements & postures – Chorea= continuous movement. Dystonia= postural with slow, sustained movements
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Onset of Dystonias
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– Usually occurs at or before age 10 (85%) – only 4% have onset after age 20
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Childhood form of Dystonias
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– Between 4-6 yrs – Initial symptom nearly always flexion inversion of foot with progression to generalized dystonia within 4-6 yrs onset
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Adolescent form of Dystonias
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– Between 8-13 yrs – Initial symptom usually foot but occasionally arm – Rate of progression slower than childhood form
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Adult form of Dystonias
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– Initial symptom usually arm – Develops into trunk dystonia with sparing of extremities for most part
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Etiology of Dystonia
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• Ideopathic (unknown) or inherited primarily • Primary dystonias are slowly progressive disorders • Secondary: environmental (drug), associated w/parkinsonism, psychogenic • May plateau at any stage of the illness • Symptoms can be generalized or focal –> Segmental (limited involvement of extremities) –>Multifocal (several body areas)
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Medical Management of Dystonia
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– Medications can induce dystonia! –> Some secondary dystonias may respond to some medications – Surgical approaches sometimes considered – Botox injections to specific areas (arm, etc) to block acetylcholine release –> Usually wears off 3-4 months
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Speech Characteristics of Generalized Dystonia
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– Variable patterns –> May not exhibit dysarthria; others have severe communication & swallowing problems – Increased respiratory rate, decreased rhythmic breathing, decreased lung volumes with apnea-like periods (stop breathing) – Augmentative communication necessary if unintelligible
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Speech Characteristics of Focal Dystonia
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– Spastic torticollis – abnormal postures & movements of strap muscles of neck – Results in abnormal fundamental frequency, sequential & alternating movement rates, sibilant & vowel duration & phonation reaction time – Dysarthria not too severe – Spasmodic Dysphonia – spastic dysphonia former name
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Types of Spasmodic Dysphonia
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–> Action-induced disorder (intentional) 1. Adductor spasmodic dysphonia – irregular hyperadduction of vocal folds 2. Abductor spasmodic dysphonia – intermittent abduction of vocal folds
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Dystonia- Non speech Characteristics
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– OME= fairly normal, although patient may not look normal – Unwanted movements interrupt normal processes – Complains of= drooling, chewing, swallowing problems – Sensory tricks are used to make the symptoms disappear
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Dystonia- DAB clusters
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A. Phonatory Stenosis B. Articulatory Inaccuracy C. Prosodic Insufficiency D. Prosodic Excess
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A. Phonatory Stenosis
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– Harsh, strained/strangled – Voice stoppages, excessive loudness – Short phrases, audible inspiration
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B. Articulatory Inaccuracy
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– Imprecise consonants & vowel distortions – Irregular artic/articulatory breakdowns
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C. Prosodic Insufficiency
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– Monopitch/Monoloudness – Decreased stress – Short phrases & inappropriate silences
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D. Prosodic Excess
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– Prolonged intervals/silences – Slow rate – Equal/excess stress
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Palato-Pharyngo-Laryngo Myoclonus (PPLM/PM)
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– Abrupt, rhythmic movements of VP, laryngeal muscles (unil/bil.) – Often occurs in combination with other dysarthrias (flaccid, spastic, ataxic) – Lesion in brainstem & cerebellum= Guillian-Mollart traingle, includes the loop between dento-rubro-olivary (inferior olive) tracts/ Hypertropic degeneration of inferior olives.
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Etiology of PPLM/PM
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– Idiopathic – Vascular event involving the brainstem/cerebellum – Neoplasm, MS, or other degenerative diseases. – Acute event results in a delay of several months to years before symptoms appear
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Non speech characteristics of PPLM/PM
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– OME= normal, with added movements on top of it – Beating movements of palate visible – Can also see this on Posterior Pharyngeal Wall (PPW) – Can see movements of larynx on neck
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Speech Characteristics of PPLM/PM
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– Little effect on connected speech – May hear intermittent nasality/aphonia – On vowel prolongation, intermittent hyper nasality & rhythmic voice arrest is heard – Voice interruptions are 2 Hz, which is different from tremor (tremor is longer) – *NO DAB clusters*, rhythmic, hyper, irregular/intermittent voice stoppages
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Tx for PPLM/PM
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– Take focus away from the clonus and towards solid breath support, awareness of deep abdominal muscles, abdominal breathing, vocal exercises (video # 5)
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Organic (Essential) Voice Tremor
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– Most common voice disorder – Typically worsens with fatigue/stress; improves slightly with alcohol intake (for some people only) – Gradual onset, can begin at any age-often before 50 years and increases with age; hereditary – Tremor can be isolated to voice but is often accompanied by head, extremity or lingual tremor (precursor to dystonias)
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Lesions That Cause Organic (Essential) Voice Tremors
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– CNS oscillatory abnormality. Involvement of red nucleus, cerebellum, inferior olive and ventrothalamic nuclei – indirect pathway – Often idiopathic
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Non Speech Characteristics of Organic Voice Tremor
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• +/- lingual tremor with voice tremor • +/- tremor in jaw/lip • Palatal/pharyngeal tremor during vowel prolongation (in PM, tremor is at rest too) • Tremor in cerebellar disease is different-3Hz frequency and is slower
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Speech Characteristics of Organic Voice Tremors
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• 4-7 Hz tremor/quavering during vowel prolongation • Reduced rate of speech • To check where tremor is located, ask patient to prolong /s/ & /z/. No tremor in /s/= no tremor in respiratory system (voiceless), a tremor in /z/ only= localizes to larynx as it is a voiced sound
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Voice Tx
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See handout!
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Spasmodic (previously spastic) Dysphonia
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• Abductor, Adductor, or mixed type (laryngospasms) • Earlier thought to be psychopathological due to stress, psychological trauma, anxiety-past 2 decades neurological(basal ganglia? Imaging studies are inconlusive) • Lesion loci: unknown, spasm/dystonia (BG or cerebellar control circuits)-focal dystonia
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Etiology of Spasmodic Dysphonia
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• Neurogenic • Psychogenic • Idiopathic • History and other exam findings, not distinguishable (etiology) perceptually
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Onset for Spasmodic Dysphonia
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• Age= 45-50, anywhere from 30-80 • Male to Female, 1 to 1 -> 1 -> 4 • Can be sudden/gradual-over a year, flu-like illness/psych stress
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Perceptual Characteristics of Spasmodic Dysphonia
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• Adductor Spasmodic Dysphonia – More common. Voice quality strained-strangled with abrupt initiation and termination of voicing (intermittent closing of VFs, extremely effortful as if air is being forced through tightly adducted VFs). • Laryngoscopy= normal, occ. • Hyperadduction, strobe= difficulty w/sustained phonation • Abductor Spasmodic Dysphonia – breathy vocal quality with abrupt termination of voicing resulting in whispered/aphonic speech (intermittent opening of VFs)
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Adductor & Abductor Spasmodic Dysphonia
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• Both forms of SD can be affected by stress, fatigue, depression, and emotional factors • Either type can disappear while singing or laughing (don’t know what causes it)
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MTD vs. SD
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• Voiced vs. voiceless sentences, SD worse with voiced and with transition from voiced to voiceless phonemes, ok with whisper • SD is action induced. Only during speech and not during breathing. • Both types can be associated with vocal tremor. EVT & SD as a continuum?
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Speech Characteristics of Spasmodic Dysphonia (Adductor)
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• Voice strained/effortful • Can be either continuous/intermittent • In some fairly consistent and others it comes and goes; voice stoppages • Strained quality is decreased when pitch of the voice is increased, pitch breaks
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Non Speech Characteristics of Spasmodic Dysphonia
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• Rate, Speed, and ROM-normal, until you get to the larynx • Soft neurological signs such as facial or palatal asymmetry, mild weakness, or pathological oral reflexes might be present • As SD is action induced, evidence only during voluntary speech (involuntary-ok)
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Speech Characteristics Spasmodic Dysphonia (Abductor)
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• Brief, breathy or aphonic segments • Increased airflow results in patients having to use reduced or short phrases • Rate may or may not be reduced (conscious strategy by patients) • Mirror image of add-SD, difficulty transitioning from voiceless to voiced phonemes (see video & audio of ADD/ABDSD, OVT) • Hyperkinetic dysarthria involuntary movements interrupting normal flow of movements
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Treatment of Hyperkinetic Dysarthria
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• Depends on the underlying disease • Sometimes behavioral treatment, mostly diagnostic and referral to neurologist
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Hyperkinetic Dysarthria Tx – Respiration
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1. Muscle Relaxation/postural adjustments 2. Prolonged inhalation and exhalation 3. Breathing for connected speech production -appropriate breath groups
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Hyperkinetic Dysarthria Tx — Laryngeal component:
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*1. Recurrent laryngeal nerve resection* (1976-Dedo) – limited success, only done unilaterally as bilateral would result in adductor palsy, SD recurred despite unil.paralysis. *2. Botox injection* – At synapse of cranial nerve & vocalis muscle decreases spasms. Very successful and done by ENTs or Neurologists. Not as useful for Abd-SD as it is difficult to reach the PCA muscle anatomically. Xanax type anti anxiety meds for EVT occasionally helpful (changes threshold of neuro activity). *3. Voice Therapy* – For Add-SD – relaxation techniques such as yawn/sigh approaches. Not for everyone as there is no control, poor response to therapy. – For Abd-SD – beginning phrases with hard glottal attack, hold breath to work on sustained adduction. *4. Referral to ENT/neurologist for botox is the most effective* – Works for 2-6 months and then wears off, repeat injs – Botox paralyses laryngeal muscles, hence need to monitor for aspiration and breathy, weak voice for 1-2 weeks
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Hyperkinetic Dysarthria Tx – Articulation (Dystonias):
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1. Botox injections to limit hemifacial spasms, tongue dystonias 2. Medications to reduce Tourette’s tics, and to decrease choreic movements 3. Bite block (jaw dystonia): small sponge with a string (not for everyone) 4. Biofeedback: not clear how it works 5. Sensory tricks for temporary relief
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Hyperkinetic Dysarthria Tx – Rate of Speech:
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1. Slow rate to increase articulatory precision 2. Letter board -for listener cues 3. Visual cues

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