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  • Duffy - 2010
  • Duffy - 2010
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  • Duffy - 2010 Invol movements reflect “Impaired inhibition of competing motor patterns” (Mink, 2007, Arch Neurol). Hyperkinetic mvt disorders due to presumed reduction of the normal inhibitory BG output. Chorea – frequent, brief, sudden, twitchlike movements that flow from body part to body part in a chaotic manner, but often resembles fragments of normal movements; the invol movements are sometimes converted into motor patterns that appear voluntary. It exists as a spectrum of movements that can be proximal or distal, large or small in amplitude, and intermittent or nearly continuous. It is assoc w abn in striatum or STN. At present, not know if activity driving choreatic mvts originates in BG or in thalamocorticval or brainstem motor pattern generators. Dystonia – invol spasms producing twisting postures in different body parts. Assoc w focal lesions in putamen, GP, or thalamus. May reflect relative dopamine deficiency or dopamine type 2 receptor dysfunction. Abnormal cocontraction of agonist and antagonist muscles usually exacerbated by movement. Current hypothesis is that it results fro incomplete suppression of competing motor patterns due to insufficient surround inhibition of competing motor pattern generators. Deficient surround inhibition may also lead to “overflow” contraction of adjacent muscles. Decreased efficacy of the surround with or without expansion of the center causes inappropriate disinhibition of unwanted muscle activity.
  • Duffy - 2010
  • Duffy - 2010
  • Duffy - 2010
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  • Transcript

    • 1. Differential Diagnosis Among Acquired Dysarthrias Oklahoma Speech-Language-Hearing Assoc. Sept. 30, 2010 Joe Duffy, Ph.D., BC-ANCDS Mayo Clinic Rochester MN
    • 2. Motor Speech Disorders
      • Include:
        • Dysarthrias
        • Apraxia of speech
      • May be:
        • Congenital or acquired
        • Static, improving, worsening
        • Associated with lesions in variety of CNS & PNS structures
        • Caused by numerous diseases/conditions
    • 3. Why Attend To Motor Speech Disorders?
      • They occur frequently
      • Prevalence may increase
      • They can be treated/managed
      • They may announce neurologic disease
    • 4. Why Differential Diagnosis of MSDs?
      • Understanding nervous system organization for motor speech control
      • Management decisions
      • Localization & diagnosis of neurologic disease
    • 5.
      • “ In what must be one of the best kept secrets of our time, both speech-language pathology and medicine have ignored certain incontrovertible facts of major importance to the differential diagnosis of human illness --
        • voice, speech, and language undergo audible and visible change when people fall prey to certain organic and psychiatric illnesses...
        • such changes can be the first and only sign of a disease early in its evolution
        • the manner in which speech becomes defective logically reflects the pathophysiology and psychodynamics of the illness…
      • Aronson, ASHA, 1987
    • 6. A Skilled Neurologist’s Thinking
      • “ I will ask our speech pathologist to evaluate her speech because I am not quite certain about what kind of dysarthria this is and I need as many clues as I can get to know how to proceed.”
    • 7. Another Skilled Neurologist’s Thinking
      • “ I think it is important for the patient to visit with the speech consultants here. The two possibilities in my mind is that we are dealing with motor neuron disease or that we are dealing with a variant of multiple system atrophy. If …there is evidence of ataxia in his speech, then we may be dealing with MSA in which case it would be reasonable for the patient to undergo thermoregulatory sweat test and an autonomic reflex screen. However, if his speech is in keeping with motor neuron disease (ALS, ie flaccid or spastic dysarthria only) then I will have the patient undergo EMG… I think the diagnosis here will rest on the speech consultation.”
    • 8. How Did We get Good At This?
      • Darley, F. L., Aronson, A. E, & Brown, J. R. (1969a). Differential diagnostic patterns of dysarthria, Journal of Speech and Hearing Research, 12, 246-269.
      • Darley, F. L., Aronson, A. E, & Brown, J. R. (1969b). Clusters of deviant speech dimensions in the dysarthrias, Journal of Speech and Hearing Research , 12, 462-496.
      • Darley, F. L, Aronson, A. E, & Brown, J. R. (1975). Motor Speech Disorders , Philadelphia: WB Saunders.
    • 9. Dysarthria
      • A collective name for a group of MSDs associated with disturbed muscular execution or control of the speech mechanism due to CNS and/or PNS damage.
      • May include abnormalities in strength, speed, range, tone or accuracy of speech movements.
      • May affect respiration, phonation, resonance, articulation, & prosody.
    • 10. Dysarthria……..
      • Includes several different types , each corresponding to damage to particular part(s) of the nervous system, & presumably each having different underlying neuropathophysiology.
      • Each type has different auditory perceptual characteristics which can be distinguished clinically.
      • Accurate identification has implications for localization ; accurate description provides clues for management .
    • 11. Dysarthria Types Type Locus Primary Deficit Flaccid Lower motor neurons Weakness Spastic Upper motor neurons Spasticity Ataxic Cerebellar control circuit Incoordination Hypokinetic Basal ganglia control circuit Rigidity/ ROM Hyperkinetic Basal ganglia control circuit Invol. movements Unilateral UMN Unilateral UMN Weakness; incoord.; spasticity Mixed More than one More than one
    • 12. Evaluation Tools
      • Essential
      • - Ears
      • - Eyes
      • - Hands
      • Other
      • - Flashlight
      • - Tongue blade
      • - Mirror
      • - Stop watch
      • - Audio/video recording
      • - Acoustic & physiologic instrumentation
    • 13. Clinical Evaluation
      • History
      • Oral mechanism exam (confirmatory signs)
      • Vowel prolongation (quality, duration, pitch, loudness, steadiness)
      • AMRs (speed & rhythm)
      • SMRs (sequencing/programming)
      • Contextual speech (all valves & components, prosody)
      • Stress testing (fatigue)
    • 14. Dysarthria Types Type Locus Primary Deficit Flaccid Lower motor neuron Weakness Spastic Upper motor neuron Spasticity Ataxic Cerebellar control circuit Incoordination Hypokinetic Basal ganglia control circuit Rigidity/decr ROM Hyperkinetic Basal ganglia control circuit Invol. movements Unilateral UMN Unilateral UMN Weakness; incoord.; spasticity Mixed More than one More than one
    • 15. Flaccid Dysarthrias – Etiologies (N=154)
    • 16. Flaccid Dysarthrias Neuromuscular Bases
      • Weakness
        • Reduced force of muscle contraction
        • Reduced range of individual & repetitive movements
      • Confirmatory signs
        • Reduced muscle tone
        • Reduced reflexes
        • Atrophy
        • Fasciculations (only seen with LMN lesions )
    • 17. Confirmatory LMN signs associated with flaccid dysarthria
      • Trigeminal (V)
        • Jaw deviates to weak side on opening (unilateral)
        • Jaw hangs open at rest (bilateral)
      • Face (VII)
        • Facial droop – flat nasolabial fold
        • Reduced lip retraction, rounding, puffing
        • Chin & perioral fasciculations
        • Unilateral or bilateral
    • 18. Confirmatory LMN signs associated with flaccid dysarthria
      • Velopharyngeal (X, IX, XI)
        • Hangs lower on weak side @ rest
        • Elevates toward normal side on gag & phonation
        • Reduced gag reflex
      • Larynx (X)
        • Weak cough & glottal coup
    • 19. Confirmatory LMN signs associated with Flaccid Dysarthria
      • Tongue (XII)
        • Deviates to weak side on protrusion
        • Fasciculations & atrophy
    • 20. Flaccid Dysarthrias
      • RESPIRATORY-LARYNGEAL: Breathiness, hoarseness, diplophonia, stridor, monopitch, monoloudness, short phrases
      • RESONANCE: Hypernasality, nasal emission
      • ARTICULATION: Imprecision
      • PROSODY: Monopitch, monoloudness
      • DISTINCTIVE: Hypernasality, nasal emission, continuous breathiness, stridor, isolated muscle group involvement
    • 21. Dysarthria Types Type Locus Primary Deficit Flaccid Lower motor neuron Weakness Spastic Upper motor neuron Spasticity Ataxic Cerebellar control circuit Incoordination Hypokinetic Basal ganglia control circuit Rigidity/decr ROM Hyperkinetic Basal ganglia control circuit Invol. movements Unilateral UMN Unilateral UMN Weakness; incoord.; spasticity Mixed More than one More than one
    • 22. Spastic Dysarthria – Etiologies (N=144)
    • 23. Major abnormalities associated with UMN lesions - Neuromuscular bases of spastic dysarthria -
      • Typically reflect combined effects of direct & indirect pathway involvement
        • Spasticity
        • Weakness
        • Reduced range of movement
        • Slowness of movement
    • 24. Spastic Dysarthria
      • Confirmatory signs
      • Paresis/spasticity of other body parts
      • Hyperactive reflexes (e.g., gag)
      • Pathologic reflexes (e.g., suck, Babinski)
      • Dysphagia, drooling
      • Pseudobulbar affect
      • Meaning = Bilateral UMN dysfunction
    • 25. Spastic Dysarthria
      • RESPIRATORY -LARYNGEAL: Strained-strangled voice, harshness, pitch and loudness variability, pitch, pitch breaks and voice stoppages, short phrases
      • RESONANCE: Hypernasality
      • ARTICULATION: Imprecise, vowels distorted
      • PROSODY: rate, excess/equal stress, prolonged phonemes and intervals, short phrases
      • DISTINCTIVE: harsh/strained voice quality, slow rate, slow/regular AMRs, reduced pitch & loudness variability
    • 26. Dysarthria Types Type Locus Primary Deficit Flaccid Lower motor neuron Weakness Spastic Upper motor neuron Spasticity Ataxic Cerebellar control circuit Incoordination Hypokinetic Basal ganglia control circuit Rigidity/decr ROM Hyperkinetic Basal ganglia control circuit Invol. movements Unilateral UMN Unilateral UMN Weakness; incoord.; spasticity Mixed More than one More than one
    • 27. Ataxic Dysarthria – Etiologies (N=166)
    • 28. Ataxic Dysarthria
      • Neuromuscular Bases
        • Incoordination - Errors in speed, range, force, timing, & direction of movements
        • Reduced tone
      • Confirmatory Signs
        • Hypotonia, slow voluntary movements, jerkiness, wide-based gait, intention tremor
      • Meaning = Cerebellar dysfunction (paravermal, bilateral, or generalized)
    • 29. Ataxic Dysarthria
      • RESPIRATORY-LARYNGEAL: Monopitch, monoloudness , harsh voice
      • RESONANCE: Normal to variable, including hyponasality
      • ARTICULATION: Imprecision, irregular articulatory breakdowns, distorted vowels
      • PROSODY: Slow rate, excess/equal stress, prolonged phonemes and intervals, inappropriate silences
      • DISTINCTIVE: Irregular articulatory breakdowns, irregular AMRs, vowel distortions, excess/equal stress, dysprosody, excess loudness variations
    • 30. Dysarthria Types Type Locus Primary Deficit Flaccid Lower motor neuron Weakness Spastic Upper motor neuron Spasticity Ataxic Cerebellar control circuit Incoordination Hypokinetic Basal ganglia control circuit Rigidity/decr ROM; probs scaling mvts Hyperkinetic Basal ganglia control circuit Invol. movements Unilateral UMN Unilateral UMN Weakness; incoord.; spasticity Mixed More than one More than one
    • 31. Hypokinetic Dysarthria – Etiologies (N=167)
    • 32. Hypokinetic Dysarthria
      • Neuromuscular Bases
      • Slow individual movements
      • Fast or slow repetitive movements
      • Reduced range of individual & repetitive movements
      • Reduced force
      • Excessive tone (rigidity)
      • Confirmatory Signs
      • Rigidity False starts
      • Decreased ROM Resting tremor (perioral tremulousness)
      • Bradykinesia Masked facies
      • Slow or fast repetitive movements
      • Meaning = Basal ganglia control circuit dysfunction
    • 33. Hypokinetic Dysarthria
      • RESPIRATORY-LARYNGEAL: Breathiness, short phrases
      • RESONANCE: hypernasality
      • ARTICULATION: Imprecise
      • PROSODY: Monopitch, monoloudness, short rushes, short phrases, variable rate, reduced stress
      • DISTINCTIVE: Fast/accelerated rate, rapid/blurred AMRs, reduced loudness & stress, monopitch, monoloudness, breathiness, palilalia
    • 34. Dysarthria Types Type Locus Primary Deficit Flaccid Lower motor neuron Weakness Spastic Upper motor neuron Spasticity Ataxic Cerebellar control circuit Incoordination Hypokinetic Basal ganglia control circuit Rigidity/decr ROM Hyperkinetic Basal ganglia control circuit Involuntary movements Unilateral UMN Unilateral UMN Weakness; incoord.; spasticity Mixed More than one More than one
    • 35. Hyperkinetic Dysarthrias – Etiologies (N=141)
    • 36. Hyperkinetic Dysarthrias
      • Deviant characteristics are product of abnormal involuntary movements that occur on continuum of:
        • rhythmic irregular & unpredictable
        • rapid slow.
      • Present in any or all components of speech -- prominent effects on rate & prosody.
      • Abnormal movements sometimes activated only during speech (e.g., SD - focal, speech induced dystonia).
      • Highly variable across individuals as a function of
        • Type of abnormal movement
        • Loci of abnormal movement(s)
    • 37. Hyperkinetic Dysarthrias--- Neuromuscular bases & confirmatory signs
      • Too Much Movement
        • Chorea
        • Dystonias
        • Tremor
        • Palatopharyngolaryngeal myoclonus
    • 38. Chorea
      • Involuntary, rapid, nonstereotypic, unpatterned random, purposeless movements of a body part
        • at 1 or multiple levels at rest, during sustained postures, & during speech.
    • 39. Chorea--Speech Characteristics
      • Phonation - Respiration : sudden forced insp./exp.; excess loudness var.; strained-strangled voice; voice stoppages; transient breathiness; grunts
      • Resonance : intermittent hypernasality & weak pressure Cs
      • Articulation : distortions/irregular breakdowns; distorted vowels; slow & irregular AMRs; smacking noises
      • Prosody : prolonged intervals & phonemes; inappropriate silences; variable rate; excess/insufficient/variable stress
    • 40. Dystonia
      • Relatively slow waxing/waning involuntary postures resulting from excessive cocontractures of antagonist muscles
        • Tend to be slow & sustained but there may be superimposed quick movements
        • Can involve:
          • only one body segment
          • Contiguous regions (segmental)
          • Generalized
        • Can be present at rest or during sustained postures or can be movement induced (e.g., spasmodic dysphonia)
    • 41. Dystonia--General
      • Primary complaints : effortful; inability to get it out; involuntary movements; dysphagia
      • Nonspeech orofacial findings :
      • Normal size, strength, symmetry, reflexes
      • Occasional drooling, dysphagia, blepharospasm
      • Relatively slow waxing/waning movements @ 1 or multiple levels @ rest & during sustained postures, but sometimes only during speech
      • Occasional improvement with sensory tricks
    • 42. Dystonia--Speech Characteristics
      • Phonation-Respiration : voice stoppages; strained-harsh voice; audible inspiration; excess or alternating loudness; unsteady, tremor-like voice
      • Resonance : intermittent hypernasality
      • Articulation : distortions & irregular breakdowns; distorted vowels; slow & irregular AMRs; noises
      • Prosody : inappropriate silences; excess loudness variation; excessive-inefficient-variable stress
    • 43. Neurogenic Spasmodic Dysphonia (SD)
      • Technically a hyperkinetic dysarthria because many SDs associated with tremor or dystonia (e.g., Meige syndrome, focal cranial dystonias, torticollis)
        • Adductor, abductor, or mixed in character
        • Onset at 20-80 years ( M ~ 45-50)
        • M:F ~ 1:1 - 1:4
        • Begins insidiously; remission rare when neurologic
    • 44. Neurogenic SD (cont.)
      • Often associated with flu-like illness &/or psychologic stress, even when neurologic (worsened by stress, fatigue)
      • Singing, laughter, “emotional” speech may be normal
      • Usually speech induced
      • ? secondary to basal ganglia or cerebellar control circuit pathology
    • 45. Neurogenic SD -- Adductor
      • Primary Complaints :  ’d speaking effort & fatigue; ETOH responsive; tight, strained, voice & chest
      • Nonspeech Oral Mechanisms : normal unless tremor or dystonia elsewhere; abnormal laryngeal exam during phonation; jerky, arrhythmic thoracic/abdominal movements & facial grimacing secondary to laryngospasm
    • 46. Neurogenic SD -- Adductor (cont.)
      • Speech Characteristics
      • Continuous or intermittent strained, jerky, squeezed, effortful voice
      • Voice arrests
      • Tremor
      • Occasional hypernasality
      • Inappropriate silences, slow rate
    • 47. Neurogenic SD -- Abductor
      • Primary Complaints :  ’d speaking effort & fatigue; ETOH responsive; “run out of air”
      • Nonspeech Oral Mechanism : similar to adductor SD but “spasms” are abductor
      • Speech Characteristics
      • Brief, breathy or aphonic segments at utterance onset or in VL environment
      • Occasional hypernasality
      • Short phrases secondary to glottal air wastage
    • 48. Tremor
      • Rhythmic (periodic) movement of a body part
        • The most common involuntary movement
        • Can be resting, postural, action or terminal
        • Essential tremor occurs with sustained posture and action, usually in upper limbs, head, or voice (probably related to cerebellar control circuit dysfunction).
    • 49. Organic (Essential) Voice Tremor
      • A focally manifest hyperkinetic dysarthria
      • Present in ~ 20% with essential tremor (Jankovic ‘90)
      • 50% familial
      • Locus ? cerebellorubrothalamocortical circuit
      • Onset usually gradual; patient may be unaware of presence
      • Worse with stress/fatigue; may improve with ETOH
      • Often accompanied by head/extremity tremor but can be isolated
    • 50. Organic Voice Tremor (cont.)
      • Primary Complaints : shaky, jerky voice; worse with fatigue; ETOH responsive
      • Physical Findings : rhythmic vertical laryngeal movements & adductor/abductor oscillation of cords in synch with voice tremor; tremor possible in other speech structures
      • Speech Characteristics :
      • Quavering, rhythmic waxing/waning tremor (4-7 Hz)
      • Voice arrest possible
      • Articulation normal but rate may be slow
      • Restricted pitch/loudness variability
    • 51. Palatopharyngolaryngeal Myoclonus
      • Abrupt, rhythmic/semirhythmic unilateral or bilateral movements of palate, pharynx, larynx (& occasionally nares, lips, tongue, respiratory muscles)
        • Locus : Guillain-Mollaret triangle (dentate n., red n., inferior olive)
        • Etiology :
          • Usually brainstem or cerebellar stroke
          • Other: tumor, MS, TBI, encephalitis, degenerative CNS disease
          • Onset can be delayed following acute insult
          • Can be benign
    • 52. Palatopharyngolaryngeal Myoclonus
      • Primary Complaints : earclicks; often unaware of movements
      • Nonspeech Orofacial Findings : abrupt, rhythmic/semirhythmic unilateral or bilateral movements of palate, pharynx, larynx (& occasionally nares, lips, tongue, respiratory muscles)
    • 53. Palatopharyngolaryngeal Myoclonus - Speech Characteristics
      • Phonation-Respiratory : myoclonic “beats” @ ~ 1-4 Hz; voice arrests when severe
      • Resonance : occasional intermittent hypernasality
      • Articulation-Prosody : Usually normal* but brief silent intervals if myoclonus interrupts respiration, phonation, or articulation
      • *But usually assoc. with spastic, ataxic, or flaccid dysarthria
    • 54. Dysarthria Types Type Locus Primary Deficit Flaccid Lower motor neuron Weakness Spastic Upper motor neuron Spasticity Ataxic Cerebellar control circuit Incoordination Hypokinetic Basal ganglia control circuit Rigidity/decr ROM Hyperkinetic Basal ganglia control circuit Invol. movements Unilateral UMN Unilateral UMN Weakness; incoord.; spasticity Mixed More than one More than one
    • 55. Unilateral UMN – Etiologies (N=98)
    • 56. Unilateral Upper Motor Neuron Dysarthria
      • Neuromuscular bases
        • Weakness
        • Incoordination
        • Spasticity
      • Lesion foci
        • Frontal lobe
        • Corona radiata
        • Internal capsule
        • Brainstem
    • 57. Unilateral Upper Motor Neuron Dysarthria (cont.)
      • Confirmatory signs
        • Hemiparesis/plegia
        • Central face weakness
        • Central tongue weakness
    • 58. Unilateral UMN Dysarthria (cont.)
      • Primary speech characteristics
        • Imprecise articulation
        • Irregular articulatory breakdowns
        • Slow (-1) & ? irregular AMRs
        • Hoarseness
        • Reduced loudness
    • 59. Dysarthria Types Type Locus Primary Deficit Flaccid Lower motor neuron Weakness Spastic Upper motor neuron Spasticity Ataxic Cerebellar control circuit Incoordination Hypokinetic Basal ganglia control circuit Rigidity/decr ROM Hyperkinetic Basal ganglia control circuit Invol. movements Unilateral UMN Unilateral UMN Weakness; incoord.; spasticity Mixed More than one More than one
    • 60. Mixed Dysarthrias – Etiologies (N=406)
    • 61. Mixed Dysarthrias
      • Most Common Mixes (300 Mayo patients)
        • flaccid-spastic - 42%
        • ataxic-spastic - 23%
        • hypokinetic-spastic - 7%
        • ataxic-flaccid-spastic - 6%
        • hypokinetic-hyperkinetic - 3%
        • other - 19%
    • 62. Mixed Dysarthrias
      • Disease & Type (examples)
        • ALS - spastic-flaccid
        • M.S. - spastic, ataxic *
        • Wilson’s disease - hypokinetic, spastic, ataxic * *
        • Shy-Drager syndrome - hypokinetic, spastic, ataxic, flaccid * *
        • Progressive supranuclear palsy - hypokinetic, spastic, ataxic **
              • * any type possible
              • * * one or more possible

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