XXXHYPERKINETICDYSARTHRIA
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| Hyperkinetic Dysarthria is difficult to define... why? | Hyperkinetic Dysarthria is difficult to define because it can be caused by many disorders.
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| All forms of Hyperkinetic Dysarthria are caused by what neuroanatomic basis? | Hyperkinetic Dysarthria is caused by dysfunction in the basal ganglia which produces involuntary movements that interfere with speech production.
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| Hyperkinetic means what? | Too much movement. Abnormal or excessive involuntary movement NOT excessive speed, just excessive and involuntary movements...
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| What are the speech characteristics of Hyperkinetic Dysarthria? | normal speech being executed is interfered with my regular or unpredictable involuntary movements that distort, slow, or interrupt it
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| Hyperkinetic Dysarthria is characterized by: | Variable articulatory precision
Vocal harshness
Prosodic abnormalities
Perceptually distinguishable from all other dysarthrias thus clinician can make an accurate diagnosis just by observing the patient's movements only 6% of dysarthrias...
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| What are the components of the Basal Ganglia Control Circuit: | Association Cortex: makes a rough plan of a movement
The Basal Ganglia smooths and refines the movement
The Thalamus makes further refinements
The Primary Motor Cortex sends the refined movement to the muscles
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| What is the neurological basis of hyperkinetic dysarthria? | Primarily, hyperkinetic dysarthria is associated with a disease of the basal ganglia control circuit...
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| What is it that has an EXCITATORY affect on the CORTEX? | The VentroLateral VL Nucleus of the Thalamus is exciting to the Cortex...
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| Basal Ganglia output goes to the cortex through what? | Complex interconnections in the VL nucleus...
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| The impulses from the BG have what kind of effect on the thalamus? | The impulses from the BG have an inhibitory effect on the thalamus which then inhibits Cortical Neuronal Firing
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| Many hyperkinesias result from a failure of the VL pathways to do what? | Many hyperkinesias result from a failure of the VL pathways to properly inhibit cortical motor discharges.
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| What happens if there is destruction to the subthalamic nucleus? | increased thalamic and cortical firing due to reduced inhibitory output from the basal ganglia.
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| What kind of an imbalance can result in a hyperkinesia? | An imbalance between the excitatory ACh and inhibitory Dopamine neurotransmitters
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| A relative increase in Dopamine or a relative decrease (proportionally) in ACh within the basal ganglia circuit can result in | hyperkinesia may result from a relative increase in dopamine or a
relative decrease in Ach within the basal ganglia circuit
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| Etiologies of Hyperkinetic Dysarthria may include: | Chorea, Sydenham's Chorea, Huntington's Disease / Chorea, Huntington's Disease, Stroke/Vascular Disorders (less likely), Toxic-metabolic conditions/Tardive Dyskinesia, and
Cerebral anoxia
Carbon monoxide poisoning
as they can damage the BG
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| Chorea: | a degenerative disease
rapid, involuntary, random, purposeless movements of a body part
jittery can range to wild range that interferes with walking, swallowing, speech etc.
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| Sydenham's Chorea: | affects children 5 - 15 years of age
related to rheumatic fever, cause unknown
Generally clears in 3 - 4 months without treatment
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| Huntington's Disease/Chorea | Inherited Autosomal Dominant Degenerative CNS Disorder
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| Huntington's Disease/Chorea is caused by: | gradual degeneration of neurons in the BG and cerebral cortex severe loss of neurons in the caudate nucleus and putamen
Onset in 40s - 50s
Death 15 - 30 years post onset
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| What are the clinical features and disease progression for Huntington's Disease? | Subtle intellectual deficits early on
Significant dementia later
Personality changes
Impaired problem solvig and word finding
Generalized chorea develops
Final stages: bedridden, mute and akinetic
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| Describe what generalized chorea is like: | lurching walk, poorly coordinated fine motor movements, dysphagia, and hyperkinetic dysarthria
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| A unilateral lesion as relates to chorea? | hemichorea - random movements on only one side
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| Lesions to the subthalamic nucleus can cause | hemiballism
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| What is hemiballism / hemiballismus? | Characterized by wild and violent involuntary
(throwing) movements of the limbs contralateral to
the lesion
– Considered extreme versions of choreic movements
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| Toxic-metabolic conditions can cause: | tardive dyskinesia
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| What is Tardive Dyskinesia? | Tardive Dyskinesia is caused by toxic-metabolic conditions. Choreic movements of the face, mouth, and neck and usually the limbs as well.
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| What does Tardive mean? | Late appearing
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| What does Dyskinesia mean? | disorder of voluntary movement
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| Tardive Dyskinesia can occur as a result of certain drugs: | antipsychotic drugs taken over a period of months or years; often seen in patients with schizophrenia - develops in 20% of patients on long-term neuroleptics CAN ALSO DEVELOP IN DRUGS THAT BLOCK DOPAMINE!
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| What is withdrawal-emergent dyskinesia | Withdrawal-emergent dyskinesia can happen after the neuroleptic drugs are withdrawn. It may also take 3 - 5 years for drug withdrawl to result in remission of the dyskinesias.
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| Hyperkinetic Dyskinesias movements of the face: | Movements of the face include repetitive:
– Lip smacking, pursing, puffing
– Tongue retractions and protrusions
– Chewing motions
– Grimacing
This interferes with speech and eating
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| Severity range of choreic movement due to: | Different muscle groups affected (e.g., neck, head, torso)
– Unpredictable movements of any muscle or combinations of muscles at any given time, that all muscles of speech affected simultaneously or islands of error-free speech
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| What component of speech is most greatly impacted with Hyperkinetic Dysarthria? | Prosody
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| How is Prosody impacted in Hyperkinetic Dysarthria? | Two of the most prominent errors:
– Prolonged interval between syllables and words
– Variable rate of speech
This could be due to the individual’s attempt to compensate for the movements
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| Additional speech errors in hyperkinetic dysarthria? | Other errors:
– Inappropriate silences
– Monopitch
– Monoloudness
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| How is Articulation impacted in Hyperkinetic Dysarthria? | Imprecise Consonants, Distorted Vowels due to holding an articulator position for too long or involuntary contractions in the vocal tract.
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| How is Phonation impacted with Hyperkinetic Dysarthria? | Harsh vocal quality
Excess loudness variations
Strained-strangled vocal quality
Voice stoppage (phonation intermittently
ceases during speech)
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| What causes phonation abnormalities in hyperkinetic dysarthria? | Phonation abnormalities due to intermittent,
involuntary hyperadduction of the vocal folds
OR
Intermittent involuntary hypoadduction of the
vocal folds causing breathy voice
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| How is respiration affected in hyperkinetic dysarthria? | Respiration
– Rapid unexpected inhalations and exhalations
noted in some patients
– Due to involuntary movements of the chest and or
diaphragm
– Can cause extraneous phonations, halting
utterances, and short phrases
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| How is resonance impacted with hyperkinetic dysarthria? | Resonance
– Intermittent hypernasality in some patients
– Due to involuntary movements that affect the
normal timing of velar elevation
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| What are the distinctive speech errors in chorea? | Prolonged intervals b/t syllables and words
Variable rate of speech
Inappropriate silences
Excess loudness variations
Prolonged phonemes
Rapid, brief inhalations or exhalations of air
Voice stoppage
Transient breathiness
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| Oral Mech Exam for Hyperkinetic Dysarthria: | Jaw, face, tongue, and palate are usually
normal in size, strength, and symmetry
Gag reflex is often normal
Drooling occasional
Chewing and swallowing difficulties can occur
Motor unsteadiness in
choreiform movements
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| Dystonia | Dys: disordered
Tonia: muscle tone
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| Dystonia causes what? | Dystonia causes involuntary, prolonged muscle contractions that interfere with normal movement or posture; slow as sustained quality with waxing and waning can appear in one, few or many muscles.
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| Etiologies of Dystonia: | Causes can be secondary symptoms to:
– Focal CVAs of the basal ganglia
– TBI
– Carbon monoxide poisoning
– Cerebral anoxia
– Tumors
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| Primary Dystonias: | Primary dystonias include:
– Spasmodic Torticollis
– Drug-induced dystonia
– Meige’s Syndrome
– Spasmodic dysphonia
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| Spasmodic Torticollis | cervical dystonia
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| Spasmodic Torticollis/cervical dystonia | Intermittent dystonic contractions of the
neck, resulting in involuntary turning of the
head, which is tilted upward
Contractions are intermittent
Sensory trick -- gently touch the turned
away side of the face to stop the neck
muscle contraction
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| Spasmodic Torticollis/cervical dystonia speech? | Speech tends to be:
– Slow in rate
– Mildly reduced intelligibility (still functional)
– Lower in pitch (in females)
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| Spasmodic Dysphonia | involuntary vocal fold movements during phonation, voice will sound 'jerky' and 'tight' involuntary contractions are vigorous and active and usually affects the adductors in the larynx but may affect abductors BG disorder?
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| Which has more articulation errors, dystonia or chorea? | More articulation errors in dystonia.
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| Which has more prosodic errors, dystonia or chorea? | More prosodic errors in chorea.
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| Myoclonus | myo = muscle
clonus = alternating contraction and relaxation
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| Myoclonus is distinguished by: | involuntary and brief contractions of parts of a muscle, a whole muscle, or a group of muscles from the same area in the body.
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| Myoclonus can appear as parat of many medical conditions such as: | epilepsy and TBI
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| PM is a type of Myoclonus: | Palatopharyngolarygeal myoclonus
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| PM is a rare disorder characterized by: | Abrupt rhythmic or semi-rhythmic unilateral or
bilateral movement of the soft palate,
pharyngeal walls, and laryngeal muscles
– Caused by brainstem strokes, cerebellar lesions,
encephalitis, tumors, or idiopathic
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| What are the speech characteristics of PM? | Speech characteristics
– Affected in most severe cases; when involved:
occasional intermittent hypernasality
Imprecise consonants
Short interruptions of phonation or myoclonic beats
during vowel prolongation
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| Tic Disorders: | rapid movement that can be controlled
momentarily but is performed due to compulsive
desire
Motor tics
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| Motor Tics | Motor tics
– Common: repetitive eye blinks, facial twitches, grimaces
– Head gestures, squatting, kicking, hopping, or shoulder
shrugging, which are more obvious
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| Vocal Tics | Vocal tics
– Throat clearing, grunts, or barking
– Occasionally, the person also might shout compulsive
utterances or obscene words (coprolalia)
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| Tics | Etiology unknown
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| Gilles de la Tourette Syndrome | multiple motor and voca tics
development of symptoms before the age of 14
slow appearance and disappearance of symptoms
tic behaviors that change and evolve over time
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| Gilles de la Tourette Syndrome | Familial link in 35%, 3 per 100,000 more often boys than girls, motor and vocal tics
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| Palilalia | Pal? repetition of one's own speech
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| Echolalia | Echo? repetition of someone else's speech
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| Vocal and Motor Tics with Tourettes are often accompanied by | obsessive-compulsive behaviors
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| Essential Organic Tremor is a | benign hyperkinetic movement disorder
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| Essential Organic Tremor causes what? | Tremulous movements in affected body parts
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| What is the most common of the hyperkinetic movement disorders? | ESSENTIAL ORGANIC TREMOR - most common of the hyperkinetic movement disorders!
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| Essential Organic Tremor prevalence? | 300 per 100,000
first appear in 40's or 50s
50% are genetic
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| Essential Organic Tremor most commonly affects what body parts? | Hands, arms, or head - evident when performing a movement. Onset is generally gradual and progression is slow.
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| Essential VOICE Tremor | Co-occurs in 20% of those who have Essential Organic Tremor. = Tremulous voice quality caused by rhythmic and involuntary contractions of the vocal folds along with vertical laryngeal movements
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| Tremor Frequency of Essential Voice Tremor? | 5 - 6 Hz which tends to get slower with increasing age - has a wavering quality during vowel prolongations.
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| Key Evaluative Tasks for Dystonia: | Vowel prolongatio
AMRS
Conversational Speech
Observation of involuntary movements
– Chorea, myoclonus, tics, essential tremor,
dystonia
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| Treatments for Dystonia: | Pharmaceutical - designed to suppress the involuntary movements.
The treatments are NOT CURES. Symptoms come back and adverse side effects occur.
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| Botox is a successful medication for what types of dystonias? | spasmodic torticollis, spasmodic dysphonia, and several other dystonias.
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| Behavior Based Treatment for Dystonia: | Sensory tricks (but they don't last)
Relaxation therapy - mixed results
Supportive psychotherapy - mixed results
Mental imagery - mixed results
Bite blocks (for dystonic jaw movements - increases articulatory stability)
Easy Onset of Phonation
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