Hyperkinetic Dysarthria

1. What is the site of lesion for HyperK D.?
Dysfunction in the basal ganglia and their major pathways.

2. What does Hyperk D. mean and what does it produce and what impression does it make?
It means too much movement; it produces involuntary movements (excessive involuntary movements: chorea, myoclonus, tics, dystonia and essential tremor); the impression is that it interfere with normal speech production and that if involuntary movements could be eliminated, speech production might be normal.

3. What is unique about HyperK. D.?
The clinician can often make an accurate diagnosis just by observing the individual’s uncontrolled movements.

4. Hyperkinetic dysarthria is actually a group of various motor speech disorders with each being associated with one of the kyperkinetic movement disorders. Name them.
Hyperkinetic D. of myoclonus type, tics, essential tremor (no observable neurological pathology associated with ess. tremor), of dystonia type, chorea: Huntington’s, tardive dyskinesia

The neurological basis of hyperkinetic dysarthria is the same as for hypok. D. (basal ganglia:caudate nucleus, putamen and globus pallidus, the interconnections, the basal ganglia control circuit)

5. What causes hyperkinetic movement?
a. One theory suggests that there is an imbalance of either dopamine or acetylcholine in the basal ganglia.  Any condition that causes too much dopamine to be released into the basal ganglia has an excitatory effect on movement.
b. The disruptions of the many interactions of the neurochemicals

6. How is chorea described? As dancelike, because the movements appear to be smooth and coordinated, but they are actually unpredictable and purposeless—sometimes even jerky (writhing, highly complex, fleeting and irregular).

7. How might mild choreic movements be expressed? The person may appear only restless or jittery.  Sometimes if infrequent, they may try to hide them by turning them into purposeful movements.

8. How might severe choreic motions be expressed. They will interfere with voluntary movements: walking, swallowing speech

9. What are some of the known disorders that cause of chorea?
a.  Sydenham’s Chorea
b.  Stroke
c.  Tardive Dyskinesia
d.  anoxia
e.  Carbon Monoxide

10. What are the characteristics of Sydenham’s Chorea?
1.  rare disorder that affects children between 5 and 15.
2.  called St. Vitus Dance
3.  Associated with rheumatic fever
4.  may be caused by hypersensitive dopamine receptors or too much dopamine.
5.  40% of children exhibit hperki. D
6.  Usually disappears in 3-4 mo. w/o TX

11.What are the characteristics of Huntington’s Disease
1.  It is a progressive disorder caused by gradual degeneration of the neurons in the basal ganglia and cerebral cortex especially in the caudate nucleus and the putamen (striatum) in the basal ganglia.
2.  it is an inherited disorder with 50% of the children affected 6/100,000 (prevalence)
3.  Can appear in child hood or adolescence but is typical of middle age; average life span is about 15 years (some live 25-30) past onset
4.  Subtle intellectual deficits with subsequent dementia (personality changes, impaired problem solving and word finding difficulties; become inattentive, vague, withdrawn and depressed; angry outbursts and suicidal thoughts are common)
5.  Choreic movements interfere with voluntary actions: lurching walk, poor coordination; dysphagia and hyperkinetic dysarthria
6.  Eventually: bedridden, mute, and akinetic
7.  Cause of neuron degeneration: hypotheises: brain produces a toxin that kills the neurons; complex neurological changes are responsible for many of the symptoms.
8.  Known: there is a significant decrease in certain neurotransmitter receptors; reductions in important enzymes and other neuroactive chemicals.

12. What kind of chorea can a stroke cause?
a. Hemichorea because the involuntary movements occur only on the contralateral side of the body to the site of lesion (if damage is restricted to only one side of the brain usually affecting the basal ganglia or thalamus)
b. hemiballism (caused by damage to the subthalamic nucleus near the substantia nigra) characterized by wild and violent involuntary movements of the limbs contralateral to the  lesion; usually remit spontaneously after a period of days—months; can be treated successfully with meds.

13. What characterizes tardive dyskinesia?
a.  means late appearing involuntary movements
b.  can cause choreic movement to the face, mouth, and neck; lip smacking, tongue protrusions, chewing motions and grimacing
c.  caused by taking antipsychotic (neuroleptic) drugs after long-term use (not reversible); sometimes appear after the drug has been withdrawn (withdrawal-emergent dyskinesia); may make certain neurotransmitter receptors of the basal ganglia supersensitive to dopamine so that the BG will react as if it were receiving too much dopamine even if only normal amounts are present producing excessively excitatory effect on movement.
d. Women and elderly are more susceptible

14. The variety of speech errors in hyperk. D. are affected by two factors.  What are they?
a.  Chorea can affect many different muscle groups.  The voluntary movement of all the muscles are susceptible to interference from the involuntary movements so all of the processes of speech are affected.  (This is in contrast to other dysarthrias where one or two processes are primarily affected).
b.  The movements are unpredictable and variable.  For instance one minute the muscles of the lips and tongue may be affected, the next, the respiratory muscles may be affected or all could be affected at once or little interference from choreic movements.

15. What are the speech errors that are most evident in individuals with chorea?
Prolonged intervals between syllables and words
Variable rate of speech
Inappropriate silences
Excessive loudness variations
Prolonged phonemes
Rapid, brief inhalations or exhalations of air.
Voice stoppages
Intermittent breathy voice quality

16. Which process is affected most by chorea?
Prosody (may be primarily compensatory in nature)
 a. Prolonged intervals between syllables and (waiting for choreic movement to end)
 b. variable rate of speech (hurrying before next choreic movement begins)
 c.  others: monopitch, inappropriate silences and monoloudness

17. What are the most common articulation errors?
Imprecise consonants: and distorted vowels:  result of involuntary chroreic movements on voluntary movements of articulation
Prolongation of phonemes: choreic movements that force the holding of an articulatory position longer than normally required.

18. What are some phonation problems caused by choreic movements?
a.  harsh vocal quality
b.  breathy
c.  excess loudness variations
d. strained-strangled vocal quality
e.  voice stoppages
May be caused by intermittent, involuntary hyperadduction of the vf or intermittent vf abductions (variability of the movements)
 

19.What are some respiratory difficulties of choreic movements?
Unexpected inhalations and exhalations of air caused by involuntary movements of the chest or diaphragm.
They can cause: extraneous phonations, halting utterances, and short phrases and excessive loudness variations (caused by sudden increases in subglottic air pressure and involuntary exhalations during phonation)

20. What causes the resonance difficulties in choreic dysarthria?
Involuntary movements that alter the timing of velar elevation.

21. What are the defining characteristics of myoclonus?
a.  involuntary and brief contractions of a part of a muscle, a whole muscle or a group of muscles in the same area of the body.  The contractions may occur singly, or in a repeating irregular pattern or rhythmically.
b.  It can be found in cases of many medical conditions: kidney failure, epilepsy, anoxia, strokes, TBI, Alzheimer, Creutzfeldt-Jakob

22.  Describe some examples of focal myoclonus (specific muscles or body parts affected)
a.  Hemifacial spasm (spasms around the eye then spread to the entire face).  It is a common disorder and is painless but causes embarrassment.
b.  palatopharyngolaryngeal myoclonus: rare; the contractions are fairly rhythmic and occur about 1-3 times a second: 1-3Hz; typical causes : brainstem strokes, cerebellar lesions, encephalitis, and tumors; soft palate contractions are the most frequently noted movement: brief and rapid elevating contractions (may be visible); pharyngeal contractions(may be visible) can cause the Eustachian tube to open and close causing an annoying clicking sound (sometimes seen as visible neck twitches); Speech is only affected in the most severe cases: intermittent hypernasality, imprecise consonants and short interruptions of phonation. In mild cases the movement is so  rapid and of low intensity so speech is not affected.

23. Describe a tic disorder.
a. a tic is a rapid movement that can be controlled voluntarily for a certain period of time but is eventually performed because of compulsive desire to do so.
b.  there are motor (eyeblinking and complex hand gestures and body movements: jumping, kicking) and vocal tics (throat clearing, shouting coprolalia)
c.  stress increases frequency of tic
d.  etiology: mild brain damage, toxic reactions, but no identifiable CNS disorder in most cases.
e.  Idiopathic tics occur in about 10-12% of the children in the form of excessive eyeblinks for less than a month to about one year, after which most disappear.

24.  Describe the characteristics of Gilles de la Tourette syndrome
a.  the development of symptoms before age 14
b.  the slow appearance and disappearance of symptoms
c.  tic behaviors that change and evolve over time
e.  minor neurological abnormalities.
f.  Causes: supersensitive dopamine receptors in the striatum
g.  genetic link; all show symptoms by age 10; prevalence 3/100,000; boys more affected
h.  vocal tics include palilalia and echolalia

25.  Describe essential tremor (organic tremor) sometimes called familial tremor.
a.  Most common hyperkinetic movement disorder (300/100,000)
b.  benign movement disorder that begins as a tremulous movement; action tremor affected by stress and fatigue; progression is slow
c.  appears to be idiopathic; beginning at age 40-50; genetic
d.  mostly affects hands, arms, or head;
e.  has been associated with hemifacial spasm and focal dystonia
 

26.  What are the differences between parkinsonian tremor and essential tremor?
a.  Essential tremor is faster
b. Essential tremor is an action tremor that disappears at rest; parkinsonian tremor is a resting tremor that decreses during movement
c.  Essential tremor is not associated with any other neurological symptoms (bradykinesia, akinesia, dementia)

27.  Describe essential voice tremor.
a.  occurs in about 20% of the individuals with essential tremor.
b.  characterized by a tremulous quavering vocal quality caused by rhythmic, involuntary contractions of the vf (6Hz), along with vertical laryngeal movements.
c.  primarily evident with vowel prolongation.
d.  tremor of the lips, tongue or neck may accompany the tremor; may slow down speech in severe cases.

28. Characterize dystonia.
a.  abnormal muscle tone causing involuntary, prolonged muscle contractions that interfere with normal movement or posture; may affect a single group of muscles or multiple groups
b. movement is more sustained and slower than chorea
c.  dystonia is not necessarily constant and may appear/disappear during a movement (waxing and waning). Severe cases: contractions can be constant resulting in painful, fixed contractions of affected body part.
d.  types of dystonia (characterized according to number of affected body parts)
 a.  focal (only one body part: tongue, arm or hand)
 b.  segmental: affects two or more body parts (neck, larynx, soft palate, jaw and face
 c.  generalized dystonia: affects all four limbs and torso or neck
 d.  hemidystonia affects two or more body parts on the same side of the body

29. What are sensory tricks?
Simple movements or actions that an affected individual can perform to stop the involuntary contractions for a short period (they are idiosyncratic): a gentle touch to the affected body part; they tend to lose their effectiveness after long-term use.

30.  Describe some etiologies of where dystonia is the primary symptom
 a. Spasmodic torticollis: characterized by intermittent (sometimes no contraction is evident) dystonic contractions of the neck muscles which result in an involuntary turning of the head; the head also usually tilts upward as a result of the contractions; stress and anxiety affects frequency; speech is slow in rate, mildly reduced in intelligibility and lower in pitch for females.
 b.  drug induced dystonia (tardive dystonia): chronic drug-induced dystonia (neuroleptic).  Withdrawal of the drug may not stop dystonia and may occur only after the drug is withdrawn. Contractions appear near mouth and face: grimacing, tongue protrusions; sometimes generalizing to other body parts.

 c.  Meige’s syndrome:  rare idiopathic disease; characterized by repetitive eyeblinking and abnormal facial movement that are often dystonic in nature; appears in early middle age and gets progressively worse such that functional vision is impossible.
When the jaw, tongue, mouth and neck are sufficiently strong they cause hyperk. D

 d.  spasmodic dysphonia: Not always classified as a dystonia (sometime essential tremor); characterized by involuntary vocal fold movements during phonation. Unlike focal dystonia SD does not have a gradual waxing and waning  but are vigorous and active. Expressed as adductor (vocal folds either constantly adducted giving a strained quality or intermittently giving a jerky tight quality or a shaky quality) or abductor vocal folds are involuntarily abducted resulting in moments of breathiness or aphonia.
Etiology is unknown but it is thought to be related to BG disorder. Interesting feature is normal voice for nonlinguistic phonation [laughing, crying and unemotional (talking to children and pets)/emotionally charged vocalizations].

31.  How can we distinguish speech characteristics of dystonia from chorea?
a.  more errors of articulation in dystonia than in chorea. Imprecise consonants, distorted vowels and irregular articulatory breakdown. (only one of these imprecise consonants were in the top four prominent characteristics in chorea)
b.  Chorea displayed more prosodic errors than those with dystonia.  No prosodic errors were noted in the first four prominent characteristics of dystonia.
Generally dystonia had more articulatory problems and chorea has more prosodic errors.

32.  How are the processes of speech affected by dystonia?
a.  articulation: imprecise consonants, distorted vowels, irregular articulatory breakdowns and prolonged phonems due to sustained dystonic contractions o the oral muscles.  Irregularity of the breakdowns due to the intermittent nature of dystonia; when the contractions are absent articulation will appear normal
b.  prosody: moonopitch, monoloudness, inappropriate silences and short phrases; reduced stress in normally stressed words and syllables; due to dystonic muscular contractions of the vocal tract that reduce the range and speed of the laryngeal movements  to produce normal inflections
c.  Phonation:  harsh vocal quality; strained-strangled quality,(increased muscle tone in the larynx excessive loudness variations (unpredictable waxing an waning quality of dystonic contractions)
d.  Respiration: excessive loudness might be a result of affected muscles of respiration. May be caused by involuntary contractions or compensatory behaviors for the abnormal respiratory movements.
e.  Resonance: may be present but if present is very mild.

33. What are the key evaluation tasks for hyperK D.?
1.  Vowel prolongation
 a.  harsh or strained-strangled vocal quality,
 b. tremor,
 c. pitch and loudness variations caused by involuntary contractions of the oral,   laryngeal or respiratory muscles.
2.  AMR
 highlight irregular articulatory breakdowns and speech rate variations
3.  Conversational speech:
 a.  articulartoy errors (imprecise consonants, vowel distortions, and prolonged phonemes.
 b.  prosodic errors: (silences, monpitch, monoloudness, short phrases0
 c.  phonatory errors (harshness, excessive loudness variations)
 d.  respiratory errors (sudden inhalations or exhalations of air
4.  observations of associated involuntary movements
 see/know patterns (pg 252) of involuntary movements

34.  What is the TX for hyperk. D.
Mostly medication that suppress the involuntary movements
Choreic and tic: haloperidol
Myoclonic jerks clonazepam or valproic
SD and spasmodic torticollis: Botox (most effective)
Behavioral tx on trial period for those not too severely affected:
 a.  locate sensory tricks
 b.  relaxation therapy and related tx
  1. mental imagery
  2. habit reversal
 c. bite blocks (focal dystonic jaw movements to stabilize the jaw during speech (seems to suppress dystonic jaw movements)
 d.  easy onset of phonation for laryngeal involuntary movements.