Hyperkinetic dysarthria

Dysarthria

1. Hyperkinetic
dysartHria
Dysfunction in the basal ganglia and
their major pathways

2. •Hyperkinetic dysarthria implies
pathologies in the basal ganglia, related
portion of the extrapyramidal system, or
sometimes the cerebellar control circuit.

3. •The diversity of lesion associated with
hyperkinetic dysarthria (and movement
disorder in general) reflects the diversity of
abnormal movements that may occur in
CNS disease and our limited understanding
of their anatomy and pathophysiology.

4. What causes hyperkinetic
movement
•An imbalance of either dopamine or
acetylcholine in the basal ganglia

5. •Chorea: As dancelike, because the movements
appear to be smooth and coordinated, but they
are actually unpredictable and purposeless

6. Some of the known disorders that cause
of chorea
a. Sydenham’s Chorea / chorea minor
b. Stroke
c. Tardive Dyskinesia
d. Anoxia
e. Carbon Monoxide

7. •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 hyperkinetic.
6. Usually disappears in 3-4 mo. w/o TX

8. •Chorea in stroke
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)

9. •Chorea in stroke
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

10. •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

11. c. Caused by taking antipsychotic
d. Women and elderly are more susceptible

12. Factors causing speech errors in chorea
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 dysarthria where one or two
processes are primarily affected)

13. 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

14. 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 

15. Speech characteristics in chorea
Prosody (may be primarily compensatory in 
nature) 
 a. Prolonged intervals between syllables 
(waiting for choreic movement to end) 
 b. variable rate of speech (hurrying before 
next choreic movement begins) 
 c.  others: monopitch, inappropriate 
silences and monoloudness 

16. Articulation errors 
•Imprecise consonants: and distorted vowels:  
result of involuntary choreic movements on 
voluntary movements of articulation 
•Prolongation of phonemes: choreic movements 
that force the holding of an articulatory 
position longer than normally required 

17. Phonation problems 
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) 
  

18. Respiratory difficulties 
•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) 

19. Resonance difficulties 
•Involuntary movements that alter the timing 
of velar elevation

20. 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

21. 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

22. 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

23. 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 (eye blinking and complex 
hand gestures and body movements: jumping, 
kicking) and vocal tics (throat clearing, 
shouting) 

24. 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

25. 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

26. 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

27. Essential tremor (organic tremor) sometimes
called familial tremor is
a progressive neurological disorder of which the
most recognizable feature is a tremor of
the arms or hands that is apparent during
voluntary movements such as eating and writing)

28. Essential 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

29. 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

30. Describe essential voice tremor
a. occurs in about 20% of the individuals with
essential tremor
b. characterized by a tremulous shaky vocal
quality caused by rhythmic, involuntary
contractions of the vf (6Hz), along with
vertical laryngeal movements

31. 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

32. 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

33. 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

34. Etiologies of Conditions 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

36. b. Drug induced dystonia (tardive dystonia):
chronic drug-induced dystonia (neuroleptic).
Withdrawal of the drug may stop dystonia
and Contractions appear near mouth and
face: grimacing, tongue protrusions;
sometimes generalizing to other body parts

37. c.  Meige’s syndrome:  Rare idiopathic 
disease; characterized by repetitive eye 
blinking and abnormal facial movement that 
are often dystonic in nature.
it 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 hyperkinetic.  

39. 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

40. •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]

41. 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 

42. How can we distinguish speech
characteristics of dystonia from chorea?
•More errors of 
articulation in 
dystonia than in 
chorea 
•Imprecise 
consonants, 
distorted vowels and 
irregular 
articulatory 
breakdown
•Chorea displayed 
more prosodic 
errors than those 
with dystonia
•Generally dystonia 
had more 
articulatory 
problems and chorea 
has more prosodic 
errors 

43. speech errors in 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 

44. b.  Prosody: monopitch, 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

45. Phonation:  harsh vocal quality; strained-
strangled quality, increased muscle tone in the 
larynx excessive loudness variations 
(unpredictable waxing an waning quality of 
dystonic contractions) 

46. •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 
•Resonance: may be present but if present is 
very mild

47. What is the TX for hyperkinetic
dysarthria
Mostly medication that suppress the 
involuntary movements 
Choreic and tic: Haloperidol 
Myoclonic jerks clonazepam or valproic 
SD and spasmodic torticollis: Botox (most 
effective) 

48. Behavioral treatment on trial period for those not 
too severely affected: 
 a.  Locate sensory tricks 
 b.  Relaxation therapy and related treatment 
  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