Motor Speech Disorders Quiz 2 (PART OF EXAM 1)

rapid, small steps done in an attempt to keep the center of gravity between the feet while the trunk leans forward involuntarily and shifts the COG forward

- usually seen in Parkinson's in hyperkinetic dysarthria

• Damage to basal ganglia circuit

• Basal ganglia

• Caudate, putamen, & globus pallidus • Subthalamic nuclei • Substantia nigra • Connections to cortex through thalamus • Connections to reticular formation through red nucleus

Regulates tone

Integrates & controls component parts comprising complex

movement patterns

Inhibits fortuitous movements

Depends on proper balance of neurotransmitters:

• Dopamine – deficiency causes hypokinesias

• ACH – deficiency causes hyperkinesias

Parkinson’s disease – related to dopamine deficiency in striatum & depigmentation of substantia nigra

• Rigidity – involuntary contraction of muscle being moved passively • Limited ROM• Bradykinesia = slow movements• Hesitations and false starts• Repetitive movements• Resting tremor• Masked facies• Festinating gait

• Reduced ROM• Hesitations and false starts• Fast or slow repetitive movements• Generally no resting tremor in speech muscles

• Monopitch & monoloudness

• Reduced loudness & stress• Accelerated rate/short rushes

• Inappropriate silences• Imprecise articulation• Breathiness• Occasional harshness• Rapid/blurred AMRs

• Dysfluencies (rapid sound, syllable, or word repetitions with reduced ROM)

• Palilalia – pathological reiterative utterances • Increased rate and decreased loudness with successive

repetitions • Most common toward end of utterances • Can be inhibited temporarily, but with effort

• Fast rate *

• Reduced stress• Monopitch & monoloudness • Inappropriate silences• Breathiness

Basic A & P same as for hypokineticMost often associated with diseases of basal ganglia control circuit

Deviant characteristics are caused by abnormal, involuntary movements

◦ May be rhythmic or irregular, fast or slow◦ Often affect prosody, but can be manifest in any aspect of speech

◦ Abnormal movements may only occur during speech

Involuntary movements vary considerably, so there are many subtypes

Many etiologies

◦ Most frequent:

◦ Toxic-metabolic

◦ Idiopathic

Bizarreness often leads to dx as psychogenic disorder

“hyper” refers to excessive (involuntary) movements, NOT to rate of speech; rate is usually slow

◦ Chorea

◦ Rapid, unsustained, unpredictable movements

◦ Worse during movement than at rest

◦ Can interrupt course of intended movement

◦ Primary orofacial findings

Motor unsteadiness: quick, unpatterned movements at one or more levels of

speech mechanism at rest or during sustained postures

Range, strength, & symmetry usually normal

Occasional drooling

Dysphagia not uncommon

◦ Respiration-phonation:

◦ Sudden forced inspiration or expiration

◦ Excess loudness variations

◦ Strained-strangled voice

◦ Voice stoppages

◦ Transient breathiness

◦ Resonance:

◦ Intermittent hypernasality & weak pressure consonants

◦ Articulation:

◦ Irregular consonant & vowel distortions

◦ Slow & irregular AMRs

◦ Prosody:

◦ Prolonged intervals and phonemes◦ Inappropriate silences

◦ Variable rate

◦ Excessive, inefficient, or variable stress patterns

◦ Dystonia

◦ Slower than chorea

◦ Primary orofacial findings

Slow, waxing/waning movements at 1 or more levels

Usually present at rest & during sustained postures, sometimes only during speech

May improve with sensory “tricks”

◦ Light touch to jaw, cheek, back of neck

◦ Hold pipe, hard candy, etc. between teeth

Dysphagia

Blepharospasm – forceful, spasmodic eye closure

Facial grimacing

◦Primary speech characteristics:◦Respiration-phonation:◦Sudden forced inspiration or expiration◦Excess loudness variations◦Strained-strangled voice◦Voice stoppages◦Transient breathiness

◦Resonance:◦Intermittent hypernasality& weak pressure consonants

◦Articulation:◦Irregular consonant & vowel distortions◦Slow & irregular AMRs

◦Prosody:◦Prolonged intervals and phonemes◦Inappropriate silences◦Variable rate◦Excessive, inefficient, or variable stress patterns

◦Slower than chorea

◦Primary orofacial findings:◦Slow, waxing/waning movements at 1 or more levels◦Usually present at rest & during sustained postures, sometimes only during speech

◦May improve with sensory “tricks”:◦Light touch to jaw, cheek, back of neck◦Hold pipe, hard candy, etc. between teeth

Dysphagia:◦Blepharospasm –forceful, spasmodic eye closure◦Facial grimacing

◦Respiration-phonation:◦Voice stoppages◦Strained-harsh voice◦Audible inspiration◦Excess loudness◦Unsteady/tremor-like voice

◦Resonance:◦Intermittent hypernasality

◦Spasmodic dysphonia: group of relatively isolated voice disorders characterized by strained or breathy voice quality resulting from adductor or abductor laryngospasm◦Neurologic, psychogenic, and idiopathic types

◦Neurogenic variety considered hyperkinetic dysarthria because seems related to dystonia or tremor:◦Often occurs with dystonia or tremor elsewhere in body

◦Average age at onset = 45-50, but may be anytime between 30s and 80s◦Usually begins insidiously◦Remission is rare with neurogenic type◦Onset often associated with flu-like symptoms and/or psychological stress◦Exacerbated by stress, anxiety, fatigue, depression◦Probably related to basal ganglia or cerebellar control circuit dysfunction

◦Adductor type◦Primary complaints:◦Increased effort and fatigue associated with speaking◦Tight, strained voice

◦Primary physical findings:◦Spasms of true cords & arytenoids, sometimes also false cords and pharyngeal constrictors, during speech◦Jerky/arrhythmic abdominal or thoracic mvts., usu. 2°laryngospasm◦Facial grimacing

◦Respiration –phonation: -strained, jerky, squeezed, effortful voice-voice arrests when severe

◦Resonance:-usually normal, sometimes hypernasal

Articulation & Prosody:-inappropriate silences, silent artic mvts.

◦Abductor type:

◦Primary complaints:◦Same as adductor, but no strained voice◦“run out of air”

◦Primary physical findings:◦Spasms are abductor◦May not see thoracic/abdominal mvts. or facial grimacing

◦Primary speech findings:◦Respiration –phonation: brief breathy or aphonic segments at beginning of utterances or in voiceless environmentsArticulation –prosody: phrases may be short 2°air wastage

◦Abnormal, involuntary movements 2°prolonged use of drugs,: ◦Usually neuroleptic/antipsychotic drugs:◦Developed for schizophrenia & bipolar disorder; now also given for mood disorders (depression), anxiety, and sleep disorders◦Sometimes other drugs given for gastrointestinal disorders also cause tardive dyskinesia

◦Tardive –late in appearing; symptoms often don’t appear until months/years after drug is started

◦Orofacial findings:◦Involuntary movements of mouth, face, tongue, & jaw similar to dystonia & chorea

◦Similar to dystonia & chorea

◦If tardive dyskinesia is diagnosed early in its course and if the medication can be stopped/changed, dyskinesia may resolve:◦However, some cases worsen when medication is stopped and others retain symptoms for months/years after stopping medication

◦Hyperkinetic dysarthria occurs in about half of people with tardive dyskinesia

◦Heterogeneous disorder◦Includes multiple motor tics◦Onset before age 21◦4 males: 1 female

◦May be accompanied by:◦Obsessive-compulsive disorder◦ADHD◦Stuttering◦Dyslexia◦Panic attacks◦Multiple phobias◦Depression◦Mania

◦Orofacial findings:◦Multiple tics

◦Speech characteristics:◦Respiration/phonation:◦Coughing, grunting, throat clearing, screaming◦Resonance:Sniffing◦Articulation/prosody:◦Humming, whistling, lip smacking, echolalia, palilalia, coprolalia

◦Focal hyperkinetic dysarthria◦Occurs in 20% of people with essential tremor:◦50% familial, remainder idiopathic

◦Benign, but occasionally spreads to other body parts◦Onset usually gradual◦Worsens with fatigue and stress, may improve with alcohol◦Often accompanied by head or extremity tremor◦Test with vowel prolongation

◦Orofacial findings:◦Jaw, lip, tongue, & palate/pharynx tremor may be present

◦Instrumental findings:◦Rhythmic, vertical laryngeal mvts.◦Adductor & abductor oscillations of cords synchronous with voice tremor

◦Speech findings:◦Respiration/phonation:◦Quavering, rhythmic, waxing/waning tremor, most obvious on vowel prolongation◦Voice arrests possible

◦Other aspects usually WNL

◦Rare disorders assoc. with lesion in dentate nucleus, red nucleus, & inferior olive◦Etiology usu. brainstem or cerebellar stroke◦Delayed onset common when caused by acute lesion◦Can be idiopathic◦Pt. complains of earclicks, often unaware of myoclonus, usu. don’t complain about speech

◦Orofacial findings:◦Abrupt rhythmic or semi-rhythmic movements of palate, pharynx, larynx, lips, nares, tongue, and respiratory muscles

◦Speech findings:◦Respiration/phonation:◦ Often not apparent during connected speech except when severe◦ Usually evident during vowel prolongation

◦Resonance: occasionally intermittent hypernasality◦Artic/prosody: usu. WNL◦Often accompanied by other dysarthria types

◦Common but not well-studied because speech symptoms are usu. transient◦Lesion often in internal capsule, corona radiata, or frontal lobe, sometimes in brainstem◦80% have hemiparesis/plegia◦Often occurs with aphasia &/or AOS, & can be masked by them

◦Orofacial findings:◦Unilat. central facial weakness◦Unilat. lingual weakness

◦ Rarely more than mildly diminished intelligibility beyond acute period◦ May sound spastic early on

◦ Respiration/phonation:◦ Sometimes harshness/hoarseness◦ Occasionally reduced loudness◦ Resonance: usu. WNL

◦ Artic/prosody:◦ Imprecise artic:◦ Some have irregular artic breakdowns-could be dmgto cerebellocorticalpathways◦ Slow rate, slow AMRs

◦Any combo of two or more

◦Common associated disorders:◦ALS (amyotrophic lateral sclerosis):◦ Progressive degenerative disease of UMN & LMN◦ Spastic-flaccid dysarthria◦ Atrophy & fasciculations (LMN) plus strained/strangled voice quality and slow rate (UMN)

◦MS (multiple sclerosis):◦ Demyelinating disease◦ Dysarthria not always present, but spastic-ataxic most common if present

◦Genetic metabolic disorder caused by inadequate dietary processing of copper◦Hypokinetic-spastic-ataxic dysarthria

◦aka Steele-Richardson-Olsewskisyndrome◦Nonfamilialdegenerative disease often mistaken for PD◦Onset usu. during 6thdecade, death within 10 years◦Paralysis of vertical gaze is distinguishing feature◦Tremor is usu. absent◦Assoc. with neuronal atrophy, esp. in BS & cerebellum, ventricular dilation, & neurofibrillary tangles◦Hypokinetic-spastic-ataxic dysarthria

MSD resulting from disturbed planning or programming of volitional speech actions/patterns in the absence of paralysis, paresis, or incoordination

Correct phonemes or phoneme sequences are selected, but ability to translate them into neural commands that generate movements leading to accurate artic. and prosodicallynormal speech are impaired◦Trouble setting act into motion◦Impaired transition from one gesture to next

◦Slow rate of speech◦Articulatory groping◦Errors of phoneme distortion and substitution ◦Syllable segmentation◦Disturbed prosody –misassigned/abnormal stress◦Initiation of speech and artic. transitions are particularly difficult◦Prolonged cons., vowels, and inter sound, syllable, and word durations

typically CVA or TBILesion usually in:◦Broca’s area◦Insula◦Supplementary motor area◦BG

Frequent associated symptoms:◦Broca’s aphasia◦Some argue that AOS is part of Broca’saphasia, but there are pts. with one & not the other

◦Hemiplegia/paresis

◦Limb apraxia:◦Sympathetic apraxia◦R hemiparesis and L limb apraxia◦Usually seen with lesions involving internal capsule and corpus callosum

◦Nonverbal oral apraxia◦Dysarthria◦One can mask presence of other◦Dysarthria 2°paralysis, paresis, incoordination

Sometimes confused with phonemic paraphasias◦Especially during recovery phase from conduction aphasia, when pt. becomes aware of incorrect phoneme productions and attempts to correct them: conduite d’approche

AOS◦Errors mostly initial◦Errors more related to phonetic complexity◦Sequencing errors rare◦Additions rare◦Abnormal fluency◦Syllable segmentation

Paraphasia◦Errors can be anywhere◦Errors less related to phonetic complexity◦Sequencing errors common◦Additions common◦Normal fluency◦No syllable segmentation

Speech characteristics depart from less severe form

◦Reduced variability of articulatory characteristics:

◦Limited speech sound repertoire◦Speech may be limited to a few meaningful or unintelligible utterances◦Imitation of isolated sounds may be in error, and errors may be limited in variety◦Errors may be highly predictable◦Automatic speech may not be better than volitional◦Muteness may be present, but rarely for longer than 1 or 2 weeks if 2°AOS◦Usu. accompanied by severe aphasia and nonverbal oral apraxia

• AOS of insidious onset, gradual progression & prolonged course in which AOS is the first, only, or most salient feature & in which criteria are not met for diagnosis of another neurodegenerative disease• e.g., progressive supranuclear palsy, corticobasal syndrome• Probably occurs in a majority-to-substantial majority of those with the agrammatic or nonfluent variant of primary progressive aphasia (nfvPPA)

• Probably a variant of asymmetric cortical degeneration syndromes, like Primary Progressive Aphasia• Therefore, AOS can be the presenting and prominent manifestation of a degenerative neurologic disease

• Slow overall speech rate• Lengthened segments between words• Sound distortions• Increased sound distortions or distorted sound substitutions as utterance length or complexity increases• Syllable segmentation within multisyllabic words

• Type 1: predominantly articulatory abnormalities• Distortions & distorted substitutions, repeated sounds, attempted self-correction• More evident when aphasia is present & > AOS• Tends to be association with widespread involvement in premotor, prefrontal, temporal-parietal lobes, caudate, & insula

• Type 2: predominantly prosodic abnormalities• Segmentation of words & syllables• More evident in PPAOS without aphasia• Tends to be associated with involvement in premotor cortex & midbrain atrophy

• Type 3: no clear difference in prominence of articulatory vs. prosodic abnormalities

• Mutism = absence of speech

• Etiologies:• Neurogenic

• Organic, non-neurogenic:• (e.g., profound congenital hearing loss)• Psychogenic• Elective

• Anarthria = speechlessness:• 2° severe loss of neuromuscular control over the speech musculature• Sometimes 2° AOS, but since apraxic mutism normally resolves in a few days, usu. refers to very severe dysarthria• May be cognitively intact but may also have impairment; difficult to test reliably• Drive/desire to communicate may be normal

• Etiologies (usu. bilat dmg.):• CVA• TBI• Degen. disease• Brainstem tumors• Anoxic encephalopathy

• Anarthria plus total immobility of body except for vertical eye movements & blinking• Sufficient cognitive ability to communicate through eye movements• Typically involves spastic quadriplegia• Respiratory impairment & severe dysphagia common• Poor prognosis for long-term survival:• Very rarely people recover; speech is then dysarthric

• Basilar artery occlusion:• affects: • Pons• Descending motor pathway to SC & lower CNs

• spares:• Supranuclear oculomotor pathways• Reticular formation• Cortical connections of pons & midbrain

• Rare• Characteristics:• Hypotonicity of facial & speech musculature• Preserved reflexive cough & yawn• Preserved facial emotions• Mutism• Dysphagia• May have preserved limb movement• Etiology: bilat dmg to rolandic operculum

• Occurs in children (6-9 yrs. old) after surgery in posterior fossa• Often onset after surgery is delayed• Almost always transient• See text for further description

• Disorders of arousal: All voluntary behavior is diminished or absent:• Caused by damage to reticular activating system

• Kinds:• Coma: state of unresponsiveness:• Absence of sleep/wake cycles• Etiologies: TBI & CVA

• Like coma, but wake-sleep cycle preserved• When awake, don’t respond meaningfully to external stimuli• Preservation of brainstem (vegetative) functions but not cortical functions• Etiologies: TBI, anoxia, drug toxicity, Wernicke’s encephalopathy• Most pts. improve within 3-6 mos., but some don’t: persistent vegetative state

• More common than vegetative state• Degree of awareness and responsiveness• Respond to some stimuli, but not consistently• Typically bedbound, incontinent, and require tube feeding• May occasionally produce words

• Mutism & general unresponsiveness: • abulia – diminished motivation:• lack of initiation of even simple motor activities in spite of preserved ability to do perform the activities• when severe, manifests as mutism and is labeled akinetic mutism

• Alertness & basic cognitive functions intact• Appear apathetic

• Responses to ext. stim. may be absent or very delayed, sometimes by minutes:• May only respond to powerful and persistent stimuli

• Speech responses: • delayed, brief, and may be aphonic or whispered• artic usually WNL• prosody – monotonic• content – concrete, literal, unelaborated• May appear to be stubborn and uncooperative• Lesions in frontal lobe, or lesion that disconnects thalamic nuclei from ascending RAS impulses• Not uncommon: see after CVA, TBI, tumor, etc.

• Neurogenic dysfluency (neurogenic stuttering, cortical stuttering):• Disrupted speech fluency, with onset coinciding with brain insult• May occur in context of dysarthria, AOS, or aphasia, but can be only symptom

• Stuttering on both content and function words• Can occur on final consonants• No adaptation effect; may get progressively worse• Usually no significant secondary behaviors• Aware of stuttering, but not usually anxious/emotionally invested (world does not revolve around the dysfluency)

• Palilalia:• Compulsive repetition of one’s own words/phrases• Already discussed under Parkinson’s disease

• Echolalia:• Unsolicited repetition of another’s utterance• Associated with dementias, stroke (transcortical aphasias, esp. mixed transcortical), developmental disability, autism• Diffuse cortical pathology

• Rare• Occurs when neurologic disease causes artic & prosodic changes that are perceived as a foreign accent, but are not actually entirely consistent with native speaker of the language in question• Not language specific• Etiologies: CVA, TBI (usu. L premotor or frontotemporal cortex)

• Sometimes called psychogenic disorders• Usually associated with conversion disorders or life stresses

• Stress – state of physical and/or mental tension that alters equilibrium:• Reactions to stress vary from one person to another:• Intrinsic personality traits• Ability to find appropriate outlet for stress• Physiologic predisposition to have excess reaction to stress• Psychosomatic disorders – effects of psychological and sociocultural stress on physiology and biochemistry, resulting in organic disease:• Stress triggers biologically predisposed organic disease

conversion of psychological stress into physical symptoms for which there are no demonstrable organic basis:• Unconscious simulation of illness• Onset frequently associated with traumatic event• Often have other unexplained physical symptoms, or a hx of them

• Often there is some “gain” from speech disorder:• Avoiding confrontation• LOA from stressful job

• Likely to deny possibility that symptoms are psychogenic• Used to be called “hysteria” (“hysterical aphonia”)

- deliberate “faking” of physical or psychological symptoms for consciously motivated external purposes• Seek to avoid work, evade life responsibilities, gain money

• May be hard to diagnose; look for:• Evaluation results that are not consistent with history and complaints• Ill-defined, vague, or overdramatized complaints• Uncooperative responses during evaluation

• Carefully document findings and the degree to which they correspond with known patterns of disease

• Does disorder fit “lawful” patterns of a MSD?• Is oral exam consistent with speech characteristics?• Is speech disorder: • consistent?• suggestible?:• If you suggest that something unlikely to change speech will make the disorder better or worse, and it does, that suggests that the disorder may be functional• susceptible to distractibility?:• Organic disorders won’t improve if you distract the patient, but functional disorders or malingering may

• Does speech fatigue in lawful manner?:• i.e., does the person become breathier, use shorter phrases, become hypernasal?• If they say that they are becoming weak, but what you are observing is increased tension, then it is probably not organic

• Is it reversible?:• If it responds rapidly to symptomatic treatment during the evaluation, it is probably not organic

• Malingering can be very difficult to “prove”, but you can note the following:• Clinical findings that are incompatible with the patient’s complaints• Ill-defined or vague symptoms, or complaints that change as you proceed through the evaluation• Over-dramatized complaints• Poor cooperation during the evaluation, not because of physical problems, but because the patient realizes they don’t know how to perform parts of the evaluation in a way that will be consistent with their claims

• Basic demographics:• Age at onset: late 60s – early 70s• Probably more frequent in women

• Can be accompanied by dysarthria and nonverbal oral apraxia as it progresses• May be the first clinical signs and symptoms of more specific neurologic diseases, such as progressive supranuclear palsy, but probably not Alzheimer’s disease in most cases• Patients are appropriate candidates for management in many cases

no problem; state it• See earlier class notes for differentiating factors: • Among dysarthrias• Among dysarthria, AOS, phonemic paraphasias, and psychogenic disorders• See text for additional disorders

describe the disorder, explain why dx not possible• Patient can’t/won’t/didn’t cooperate• Speech symptoms atypical• Symptoms not the ones you expect to see together

•Speech diagnosis should be related to known/suspected neurologic diagnosis or lesion location•Diagnose all speech, language, and cognitive disorders that are present

•Sometimes diagnosis is that, “Speech is WNL”:• Early in degenerative diseases• Pre-surgical recording• Functional etiology: pt. perceives symptoms no one else does

◦ Cerebellum: coordinates posture, locomotion, & coordinated activities◦ Receives input from motor cortex & periphery◦ Sends output through thalamus to cortex, then to periphery◦ Major function: error control

◦ Cerebellar lesions: errors in force, speed, timing, range, & direction of movement = incoordination◦ Lesions that cause severe, chronic speech problems are usually bilateral◦ With unilateral lesions, speech often improves rapidly

◦ Degenerative diseases:◦ Friedreich’s ataxia:- Hereditary disease, onset in childhood◦ Olivopontocerebellar atrophy:- Hereditary disease with onset between 20 & 40

◦ Vascular disorders:◦ Strokes, aneurysms, or AVMs in vertebrobasilar system

◦ Tumors◦ TBI

◦ Paraneoplastic cerebellar degeneration:◦ Rare condition assoc. with lung, ovarian, breast, Hodgkin’s lymphoma, & other cancers, typically in middle-aged women◦ Autoimmune reaction targeted against Purkinje cells

◦Hypotonia◦ Slow voluntary movements◦ Jerkiness of movement◦ Wide-based gait◦ Intention (terminal) tremor◦ Dysmetricjaw, face, & tongue AMRs

◦ Irregular & transient articulatory breakdowns◦ Irregular AMRs◦ Vowel distortions◦ Excess & equal stress (scanning speech)◦ Excess loudness variations◦ Dysprosody