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Differential Diagnosis Among Acquired Dysarthrias

Oklahoma Speech-Language-Hearing Assoc.Sept. 30, 2010

Joe Duffy, Ph.D., BC-ANCDSMayo Clinic

Rochester MN

Motor Speech DisordersInclude:

• 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

Why Attend To Motor Speech Disorders?

They occur frequently

Prevalence may increase

They can be treated/managed

They may announce neurologic disease

Why Differential Diagnosis of MSDs?

Understanding nervous system organization for motor speech control

Management decisions

Localization & diagnosis of neurologic disease

“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

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.”

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.”

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.

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.

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.

Dysarthria TypesType 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

Evaluation Tools Essential

- Ears- Eyes- Hands

Other- Flashlight- Tongue blade- Mirror- Stop watch- Audio/video recording- Acoustic & physiologic instrumentation

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)


Flaccid Lower motor neuron Weakness

Spastic Upper motor neuron Spasticity



Rigidity/decr ROM


Invol. movements



Flaccid Dysarthrias – Etiologies (N=154)

Degenerative14%

Vascular4%

Surg.trauma29%

Undet.26%

Muscle dz8%

M.G.6%

Nonsurg.trauma4%

Tumor5%

Infectious2%

Anat.malform.2%

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)

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

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

Confirmatory LMN signs associated with Flaccid Dysarthria

Tongue (XII) Deviates to weak side on protrusion Fasciculations & atrophy

Flaccid Dysarthrias

RESPIRATORY-LARYNGEAL: Breathiness, hoarseness, diplophonia, stridor, monopitch, monoloudness, short phrases

RESONANCE: Hypernasality, nasal emissionARTICULATION: ImprecisionPROSODY: Monopitch, monoloudness

DISTINCTIVE: Hypernasality, nasal emission, continuous breathiness, stridor, isolated muscle group involvement






Rigidity/decr ROM


Invol. movements



Spastic Dysarthria – Etiologies (N=144)

Degenerative40%

Vascular29%

Traumatic10%

Undet10%

Demyelinating4%

Tumor3% Infectious

1%

Multiple2%

Inflammatory1%

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

Spastic Dysarthria

Confirmatory signsParesis/spasticity of other body partsHyperactive reflexes (e.g., gag)Pathologic reflexes (e.g., suck, Babinski)Dysphagia, droolingPseudobulbar affect

Meaning = Bilateral UMN dysfunction

Spastic Dysarthria

RESPIRATORY -LARYNGEAL: Strained-strangled voice, harshness, pitch and loudness variability, pitch, pitch breaks and voice stoppages, short phrases

RESONANCE: HypernasalityARTICULATION: 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






Rigidity/decr ROM


Invol. movements



Ataxic Dysarthria – Etiologies (N=166)

Degenerative36%

Vascular13%Traumatic

5%

Undet.15%

Demyelinating17%

Tumor3%

Multiple1%

Toxic/Metabolic5%

Inflamm.4%

Other1%

Ataxic DysarthriaNeuromuscular Bases

Incoordination - Errors in speed, range, force, timing, & direction of movements

Reduced toneConfirmatory Signs

Hypotonia, slow voluntary movements, jerkiness, wide-based gait, intention tremor

Meaning = Cerebellar dysfunction (paravermal, bilateral, or generalized)

Ataxic DysarthriaRESPIRATORY-LARYNGEAL: Monopitch, monoloudness,

harsh voiceRESONANCE: Normal to variable, including hyponasalityARTICULATION: Imprecision, irregular articulatory

breakdowns, distorted vowelsPROSODY: 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






Rigidity/decr ROM; probs scaling mvts


Invol. movements



Hypokinetic Dysarthria – Etiologies (N=167)

Degenerative78%

Vascular9%

Traumatic1%

Undet.4%

Toxic/metabolic2%

Infectious1% Other

1%Multiple

4%

Hypokinetic DysarthriaNeuromuscular Bases

Slow individual movementsFast or slow repetitive movementsReduced range of individual & repetitive movementsReduced forceExcessive tone (rigidity)

Confirmatory SignsRigidity False startsDecreased ROM Resting tremor (perioral tremulousness)

Bradykinesia Masked faciesSlow or fast repetitive movements

Meaning = Basal ganglia control circuit dysfunction

Hypokinetic DysarthriaRESPIRATORY-LARYNGEAL: Breathiness, short phrasesRESONANCE: hypernasalityARTICULATION: ImprecisePROSODY: Monopitch, monoloudness, short rushes,

short phrases, variable rate, reduced stress

DISTINCTIVE: Fast/accelerated rate, rapid/blurred AMRs, reduced loudness & stress, monopitch, monoloudness, breathiness, palilalia






Rigidity/decr ROM


Involuntary movements



Hyperkinetic Dysarthrias – Etiologies (N=141)

Toxic/metabolic12%

Infectious1%

Other6%

Multiple3%Unknown

67%

Vascular1% Traumatic

1%

Degenerative9%

Hyperkinetic Dysarthrias

Deviant characteristics are product of abnormal involuntary movements that occur on continuum of:

rhythmic irregular & unpredictablerapid 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)

Hyperkinetic Dysarthrias---Neuromuscular bases & confirmatory signs

Too Much Movement Chorea Dystonias Tremor Palatopharyngolaryngeal myoclonus

Chorea

Involuntary, rapid, nonstereotypic, unpatterned random, purposeless movements of a body part

at 1 or multiple levels at rest, during sustained postures, & during speech.

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 CsArticulation: distortions/irregular breakdowns; distorted

vowels; slow & irregular AMRs; smacking noisesProsody: prolonged intervals & phonemes; inappropriate

silences; variable rate; excess/insufficient/variable stress

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)

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

Dystonia--Speech CharacteristicsPhonation-Respiration: voice stoppages; strained-harsh

voice; audible inspiration; excess or alternating loudness; unsteady, tremor-like voice

Resonance: intermittent hypernasalityArticulation: distortions & irregular breakdowns;

distorted vowels; slow & irregular AMRs; noisesProsody: inappropriate silences; excess loudness

variation; excessive-inefficient-variable stress

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

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

Neurogenic SD -- Adductor

Primary ComplaintsPrimary Complaints: : ’d speaking effort & ’d speaking effort & fatigue; ETOH responsive; tight, strained, fatigue; ETOH responsive; tight, strained, voice & chestvoice & chest

Nonspeech Oral MechanismsNonspeech Oral Mechanisms: normal unless : normal unless tremor or dystonia elsewhere; abnormal tremor or dystonia elsewhere; abnormal laryngeal exam during phonation; jerky, laryngeal exam during phonation; jerky, arrhythmic thoracic/abdominal movements & arrhythmic thoracic/abdominal movements & facial grimacing secondary to laryngospasmfacial grimacing secondary to laryngospasm

Neurogenic SD -- Adductor (cont.)

Speech Characteristics Continuous or intermittent strained, jerky, squeezed,

effortful voice Voice arrests Tremor Occasional hypernasality Inappropriate silences, slow rate

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

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).

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

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

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

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)

Palatopharyngolaryngeal Myoclonus - Speech Characteristics

Phonation-Respiratory: myoclonic “beats” @ ~ 1-4 Hz; voice arrests when severe

Resonance: occasional intermittent hypernasalityArticulation-Prosody: Usually normal* but brief silent

intervals if myoclonus interrupts respiration, phonation, or articulation

*But usually assoc. with spastic, ataxic, or flaccid dysarthria






Rigidity/decr ROM


Invol. movements



Unilateral UMN – Etiologies (N=98)

Vascular90%

Traumatic4%

Tumor4%

Other1%

Multiple1%

Unilateral Upper Motor Neuron Dysarthria

Neuromuscular bases Weakness Incoordination Spasticity

Lesion foci Frontal lobe Corona radiata Internal capsule Brainstem

Unilateral Upper Motor Neuron Dysarthria (cont.)

Confirmatory signs

Hemiparesis/plegia Central face weakness Central tongue weakness

Unilateral UMN Dysarthria (cont.)

Primary speech characteristics Imprecise articulation Irregular articulatory breakdowns Slow (-1) & ? irregular AMRs Hoarseness Reduced loudness






Rigidity/decr ROM


Invol. movements



Mixed Dysarthrias – Etiologies (N=406)

Degenerative66%

Vascular11%

Traumatic5%

Demyelinating4%

Undet.3%

Multiple5%

Tumor4%

Inflamm.1%

Toxic/metabolic1%

Mixed Dysarthrias

Most Common MixesMost Common Mixes (300 Mayo patients) (300 Mayo patients) flaccid-spastic - 42%flaccid-spastic - 42% ataxic-spastic - 23%ataxic-spastic - 23% hypokinetic-spastic - 7%hypokinetic-spastic - 7% ataxic-flaccid-spastic - 6%ataxic-flaccid-spastic - 6% hypokinetic-hyperkinetic - 3%hypokinetic-hyperkinetic - 3% other - 19%other - 19%

Mixed DysarthriasDisease & 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

handouts

Documents

speech mechanism

journal of speech

motor speech disorders

speech consultants

speech pathologist

speech consultation

dysarthrias apraxia

accuracy of speech movements