This document provides information on various hyperkinetic movement disorders affecting the basal ganglia including chorea, dystonia, ballismus, tics, and myoclonus. It discusses two specific conditions - Sydenham's chorea, which is associated with rheumatic fever, and Huntington's disease, an inherited neurodegenerative disorder. Speech symptoms are described for both quick and slow involuntary movements, with chorea most prominently affecting prosody, articulation, and phonation. Prognosis is generally good for Sydenham's chorea but progressive cognitive decline and death occur in Huntington's disease over 10-15 years.

1. CEREBRAL PALSY
(Disorders of Movement)
-HIMANI BANSAL
IInd year BASLP

2. CONTENTS
• Hyperkinesias and dyskinesias
• Ballismus
• Tremor
• Tic disorder
• Myoclons
• Athetosis
• Chorea
• Dystonia
• Hypokinesias

3. BASAL GANGLIA

4. Signs of Basal ganglia lesion
• Fluctuating tone
• Hyper and primitive reflexes (similar to
spastics)
• Dys/hyperkinesia – (Involuntary
extraneous motor activity/invlountary
movements - quick/slow)
• Athetosis (BG)
Slow, worm-like writhing ,irregular
movements especially in the hands, fingers &
wrist progressing from proximal to distal
parts in a wave like fashion
• Tremor
Rhythmic oscillation (back & forth, pendular
movement) of an extremity, head or trunk

5. Signs of Basal ganglia lesion
• Dystonia
Similar to athetosis, but abnormal movements
may involve the trunk muscles than the
extremities
• Ballismus (Subthalamic nuclei)
Rapid, violent, flailing involuntary movements
of the extremities
• Myoclonous
Involuntary movements due to rapid, abrupt,
twitching unsustained contractions in large or
small muscle groups

6. Signs of Basal ganglia lesion
• Chorea
Quick, jerky, quasi purposive (no purpose)
involuntary movements of the extremities,
head or trunk (fragments of purposive
movements following one another in
disorderly fashion)

7. Signs of Basal ganglia lesion
• Hypokinesia/ bradykinesia/akinesia
Delay in initiation of movement, slow
movements
Resting tremor
Postural abnormalities

8. HYPERKINESIAS

9. • associated with basal ganglia control circuit.
• It may be manifest in any or all levels of speech, and it often
has prominent effects on prosody.
• Normal movements are over ridden by the involuntary,
unpredictable movements

10. ETIOLOGY
• Unknown (59%)
• Focal seizure disorder (1%)
• Other (8%)
• Toxic/Metabolic (17%)
• Degenerative (9%)
• Familial ataxia + alcoholism (1%)
• Infectious (2%)
• Trauma (1%)
• Post Cerebellar tumor removal
(ataxia and palatal myoclonus)
• Vascular (1%)

11. Classification
• Predominantly quick
Chorea (Sydenham’s, Huntington’s)
Myoclonic jerks
Tics (Gilles de la Tourette’s syndrome)
Infection
Ballism
• Predominantly slow
Athetosis
Infection
Stroke
Tumor
Dystonia
Drug induced dyskinesia (Tardive dyskinesia)

12. Neuromuscular condition (neurological
symptoms)-
• Predominantly quick
Quick involuntary movements of limbs, trunk,
head & neck which are unpredictable (called
dance like becoz movts are smooth &
coordinated), but are purposeless (sometimes
jerky & abrupt)
Variable muscle tone
Hyper and primitive reflexes (similar to
spastics)
• Predominantly slow
Twisting & writhing movements as in athetosis
Slow involuntary movements as in dystonia
Hypertonia

14. Oral speech mechanism (structure &
function)
• All components of speech production are equally
affected
• Predominantly quick
Brief interruptions of any basic motor process of
speech, e.g. chorea
Repetitive rhythmic jerking of the parts of the
speech musculature as in myoclonus, e.g. of focal
myoclonus is palatopharyngeal myoclonus

15. • Predominantly slow
Involuntary movements which are slower and
more gradual onset. They can be prolonged for
variable moments of time, waxing & waning
results.

16. PATIENT PERCEPTION OF SPEECH AND
COMPLAINTS:
• those with non-rhythmic hyperkinesias [chorea, dystonia]
affecting jaw, face, tongue, and larynx tend to describe their
speech as slurred, slow, halting or 'hard to get out'.
• patients with prominent laryngeal hyperkinesias [tremor or
dystonia] often complain that their voices are shaky, tight, close off
or don't want to come out.

17. Speech symptoms
• Characteristics of chorea: (Predominantly
quick):
• Movements of chorea affect different muscle
groups including face, neck, head & torso. As a
result all components of speech production are
more or less equally affected.
• Prosodic breakdown: Prolonged intervals
between syllables & words, variable rate,
reduced or equal stress placement, monopitch,
monoloudness, inappropriate silences

18. • Articulatory inaccuracy: Imprecise consonant
production, vowel distortions, prolonged
phonemes
• Phonatory interruptions: Excessive loudness
variations, voice stoppages or voice breaks,
variable pitch, harsh/strained-strangled/breathy
voice quality

19. • Respiratory interruptions: Sudden forced
unexpected inspiration or expiration
• Resonatory insufficiency: Hypernasality
(intermittent)

20. • Characteristics of palatopharyngeal myoclonus:
(Predominantly quick):
• Muscular contractions of SP, phx, lx
• Myoclonus movts are rhythmic & occur at a rate
of 1-3 beats/sec even while sleeping (not
detected during speech but audible in vowel
phonation)
• Speech affected only in severe cases becoz the
contractions are quick & low intensity

21. • Respiratory insufficiency: Myoclonic movements
of diaphragm-irregular interruptions of
respiration & outflow of air
• Phonatory insufficiency: Short interruption of
phonation, organic voice tremors (in isolation or
along with tremor of other parts of the body)

22. • Resonatory insufficiency: Intermittent
hypernasality
• Articulatory insufficiency: Imprecise consonants

23. • Characteristics of dystonia (Predominantly slow):
• Articulatory inaccuracy: Imprecise consonant
production, distorted vowels, irregular articulatory
breakdown, prolonged phonemes
• Prosodic breakdown: Monopitch, monoloudness,
inapproprite silences, short phrases
• Phonatory insufficiency: Harsh or strained-
strangled voice quality, excessive loudness variation
• Respiratory & Resonatory insufficiency: not
common

24. SYDENHAM’S CHOREA
• Synonym: Saint vitus
dance/disease
• Definition: SC is a disorder of the NS occurring
due to the toxic effect of acute rheumatism
characterized chiefly by involuntary movements.

25. • Etiology:
 Rheumatism (rheumatic fever)
 May occur after scarlet fever & diphtheria
 Unknown- hypersensitive dopamine receptors in
BG or too much dopamine in BG
• Incidence:
 5-20 yrs (childhood & adolescence)
 Females affected more than males (3:1)

26. • Pathology:
• Macroscopic: Odema & congestion
• Microscopic: Changes in the corpus striatum,
substantia nigra & subthalamic nucleus. Cortical
abnormalities (degeneration of ganglion cells)
seen

27. • Symptoms:
• Mode of onset: Insidious. First c/o child clumsy
& drops things. Described as restless, fidgety,
unable to keep still. Sometimes symptoms are
abrupt & may be described as fright.
• Involuntary movts: most prominent & quasi
purposive
Upper limb movts occur at all joints are always
more conspicuous than lower limbs
Movts are intensified on voluntary effort &
excitement, disappear during sleep.

29. • Associated movts: when voluntary acts are
performed, it is associated with other movts.,
e.g. making a fist may be associated with tongue,
lip movts.
• Voluntary movts.- In initial stages, not affected
but later stages, power of muscles affected.
• Hypotonia
• Respiration-jerky & irregular
• Severe cases- swallowing & mastication
problems (hence they have to be artificially fed)

30. • Posture: Choreic posture of the hand, thumb &
fingers are hyperextended at the
metacarpopharyngeal joints and wrist is flexed
(due to hypotonia)
• Mental state: might exhibit emotional instability,
above avg. IQ. Severe cases- persistent state of
excitement associated with insomnia (maniachal
chorea)
• Cardiac abnormalities seen (due to rheumatism)

31. • Speech symptoms:
• Mild cases –speech not affected, severe cases-
Hyperkinetic dysarthria
• Dysarthria could be temporary & variable in
terms of severity.
• If there is recurrence of the condition, dysarthria
also may recur.

32. • Tx:
• Medical: Sedatives
• Supportive therapy- PT, OT, ST required when
choreic symptoms are active
• Should be kept in bed till the symptoms
disappear
• Should avoid excitement
• Should take care of skin, body joints, which may
be abraded due to constant rubbing of the
bedclothes

33. • Prognosis: Death very rare (only in 2%)
• Most recover within 2-3 mths
• In some, recurrences as many as 3-4 attacks may
appear. Interval between attacks could be 1 or 2
yrs.

34. HUNTINGTON’S CHOREA
• Defn: Hereditary disorder characterized
pathologically by degeneration of the ganglion
cells of the forebrain (cortex) and striatum (BG),
and clinically by choreiform movements and
progressive dementia, which usually begins
during early middle life.

35. • Etiology: Hereditary (autosomal dominant
inheritance)
Cause of neuron degeneration unknown, but this
results in decrease in the neurotransmitter
receptors in the BG & reduction in important
enzymes & neuroactive chemicals in the brain.

36. • Pathology: Atrophy of the ganglion cells of the
forebrain leading to reduced amount of white
matter of the cerebral hemisphere.
Ganglion cells of the caudate & putamen also are
affected
• Incidence: Both genders are equally affected
Age of onset: 30-45 yrs (middle age)
• Prevalence: 6 per 100,000

37. • Symptoms:
1) Motor symptoms:
 Insidious (sudden) development of involuntary
choreic movements which are progressive,
 More conspicuous in the face & upper limbs
initially and are usually more jerky and rapid
then Sydenham’s chorea. Hence dysarthria
could be the early symptom.
 As it progresses, choreic movements may be
seen in the lower limbs also.
 Due to involvement of the cerebellum, ataxia of
upper limbs & of gait are noticed.

39. 2) Dementia: Becoz of the degeneration of cortical
motor neurons in the frontal cortex, mental
changes develop after a few years of onset
leading to progressive dementia, intellectual &
cognitive deficits, delusions.

40. • Speech characteristics:
Hyperkinetic dysarthria seen in the initial
stages.
Later stages may be associated with language
impairment in receptive & expressive modes.
This is superimposed by dementic component
(loss of memory, attention, vigilance, cognitive-
linguistic deficiency)

41. • Prognosis: Progresses very fast, terminal
stages- patient will be bedridden with physical
disabilities & mental disturbances and
terminates fatally, usually in 10-15 yrs.
Spontaneous recovery never occurs.
• Tx:
• Surgery is not recommended because of
intellectual impairment
• Due to mental deterioration, institutional care is
required.
• PT, OT & ST may be helpful in initial stages only
• In ST- AAC (non-verbal approach)

42. Myoclonus
• Involuntary single or repetitive brief jerks of a
body part, if repetitive, jerks can be rhythmic or
nonrhythmic.
• Focal or multifocal
• Occur spontaneously or may be induced by
visual, tactile, or auditory stimuli or sometimes
by voluntary movements. When brought on by a
movement, it is known as action myoclonus.

43. • Associated with lesions from the cortex to the
spinal cord
• Can occur in epilepsy, where it is considered as a
component of seizure.

44. Tic disorder (Gilles de la Tourette
syndrome) (Fast)
• Tic: Rapid, stereotyped, coordinated or
patterend movt. that can be controlled
voluntarily for a certain period, but is performed
frequently becoz of a compulsive desire to do so
(under partial voluntary control)
• Etiology:
Mild BD,
Toxic reactions to medications,
Supersensitive dopamine receptors in the
striatum

45. • Incidence: Occur in 10-12% of all children
• Prevalence: 3 per 100,000
• Gender: Boys more affected
• Onset: Before 14 yrs

46. • Symptoms:
Tics (two types):
1) Motor tics: repetitive eye blinks, brief facial
twitches, or grimaces. Or more obvious like hand
gestures, squatting, hopping, shoulder shrugging
etc.
2) Vocal tics: Throat clearing, grunts, barking
noises, or more obvious like copralalia.
• Other symptoms:
Palilalia, echolalia
Obsessive-compulsive behaviors

47. • Course:
 Slow appearance & disappearance of symptoms,
 Tic behaviors may change over time,
 Occur for less than a month or upto a year and
then disappear.
 Some may persist & will be joined by other
associated behaviors.

48. Ballism (fast)
• Gross, abrupt contractions of axial and proximal
muscles of the extremities that can produce
flailing
• When unilateral, it is called hemiballismus
• Gives appearance of severe from of chorea
• Lesion within or near the subthalamic nuclei

49. Essential (organic) tremor (slow)
• Essential/organic: no apparent external cause
(idiopathic), 50% cause - heredity hence also
called familial tremor
• Prevalence: 300 per 100,000
• Onset: Gradual, Between 40-50, can occur at
younger or older ages

50. • Course: slow progression
• Symptoms:
 Rhythmic oscillating/tremulous movt. of hand,
arms or head,
 Action tremor (evident when a movt is
performed), increases with stress & fatigue

51. • Essential voice tremor- tremulous phonation,
quavering voice quality (becoz of involuntary
contraction of VF - 6/sec)
• Tremor of the lips, tongue, neck may be seen
• In severe cases, slow rate of speech

52. Essential tremor Parkinson tremor
Fast Slow
Action tremor Resting tremor
Do not have other
neurologic symptoms
like bradykinesia,
akinesia , dementia etc,
Exhibit these symptoms

53. Dystonia (slow)
• Causes prolonged muscle contraction that
interfere with normal movement
• This effect not constant- contractions appear &
disappear during an ongoing movement (waxing
& waning movement)
• In severe cases contractions could be constant
• The abnormal posture may cause torsion of a
body part

54. • Types of dystonia
Focal dystonia: one part of the body
Segmental dystonia: two or more parts of the
body
Generalized dystonia: all 4 limbs, torso & neck
Hemidystonia: two or more parts on the same
side of the body

57. • Sometimes dystonia can be alleviated
temporarily by sensory tricks, e.g. gentle touch,
mint in mouth etc.

58. Spasmodic torticollis
• is a segmental dystonia characterized by
intermittent dystonic contractions of neck
muscles which result in an involuntary turning
of the head (usually upward tilting)
• Speech- slow rate, mild reduction in
intelligibility, low pitch for females

60. Dyskinesia (fast or slow)
• Abnormal, hyperkinetic, involuntary movements
which may be restricted to certain body parts
• Orofacial dyskinesia (mouth, face, tongue and
jaw)
• A type of dyskinesia due to intake of
antipsychotic drugs is tardive dyskinesia

61. Tardive dyskinesia
• Tardive (late-appearing)
• Movt. disorder that causes invol. movts of the
face, mouth & neck. Sometimes limb also
affected

62. • Etiology: Long-term (mths/yrs) use of
antipsychotic drugs (neuroleptic) drugs. These
make certain neurotransmitter receptors in
neurons of the BG supersensitive to dopamine.
As a result BG will react as if it were receiving
too much dopamine even if only normal
amounts are present.

63. • Gender: Women likely to be more affected
• Age: Elder individuals more susceptible
• Symptoms: Lip smacking, tongue protrusions,
chewing motions, grimacing
• Speech characteristics: Hyperkinetic dysarthria
• Recovery: Stopping medications will not reverse
the condition. Sometimes tardive dyskinesia will
occur after the medication is withdrawn
(withdrawal-emergent dyskinesia)

65. Meige’s syndrome
• Idiopathic
• Symptoms: Repeated eye blinking, abnormal
facial movements (jaw, tongue, mouth & neck).
Eye blink becomes so frequent that functional
vision is imposible
• Hyperkinetic dysarthria
• Onset: Middle age
• Course: Progressively worsens

66. HYPOKINESIAS

67. associated with basal ganglia control circuit pathology.
 It may be manifest in any or all levels of speech,
its characteristics are most evident in voice, articulation, and
prosody.

68. Aberrations in the maintenance of proper background
tone and supportive neuromuscular activity on which the
quick discrete, phasic movements of speech are
superimposed.

69. Impressions that speech sound are “all there” but
have been attenuated in their range or amplitude
as well as their ability to vary with normal
flexibility and speed.

70. Definition
• Hypokinesias are perceptually distinguishable
MSD associated with basal ganglia control
circuit pathology. They may manifest in any or
all of the respiratory, phonatory, resonatory &
articulatory level of sp, but its characteristics are
most evident in voice, articulation & prosody. Its
deviant speech characteristics reflect the effects
of rigidity, reduced force & range of movement &
slow but sometimes fast repetitive movements
on speech. (Duffy,1995)
• Hypokinetic means “decreased movement”

71. ETIOLOGIES:
Degenerative
Parkinson’s disease (31%)
Parkinsonism (26%)
Vascular (10%)
Undetermined (6%)
Toxic/metabolic (3%)
Traumatic (2%)
Other (1%)

72. Neuromuscular condition (neurological
symptoms)-
• Delay in initiation of movement - akinesia
• Limited range & speed of movements-
bradykinesia
• Paucity of movements/reduced automatic
movement
• Rigidity (becoz of increased tone)
• Tremor at rest (resting tremors) which
disappears on voluntary act

73. Oral speech mechanism (structure &
function)
• Repetitive tongue & lip movements produced
rapidly
• Placement fairly accurate, but reduction in the
range & speed of articulators

74. Speech symptoms
• Predominantly articulatory and prosodic
disorder
• Articulatory inaccuracy: Imprecise consonant
production becoz of bradykinesia leading to
slurred speech
• Fluency breakdown: Repeated phonemes at the
beginning of an utterance, palilalia , increased
rate of speech within a phrase or overall variable
rate of speech, inappropriate silences in speech
becoz of akinesia
• Prosodic breakdown: Monopitch,
monoloudness, reduced stress, short rushes of
speech

75. • Phonatory insufficiency: Harsh or breathy voice
quality, reduced loudness, tremulous voice,
high/low pitch
• Resonatory insufficiency: Hypernasality
(uncommon), if present it is mild
• Respiratory insufficiency: Faster breathing rate,
shallow breath support, short breathing cycles,
incoordinated movts of respiratory muscles

76. • Akinesia (delay in initiation of movements lasting
for a few secs-stuck in one posture, a brief touch from
another person is only required to continue or initiate a
movement)
• Trouble stopping a movement
• All movements (esp. fine) lack power, slowly performed
and lack range of movement (bradykinesia).
Hence the following are affected
Blinking eyes
Movements of articulators, mastication & deglutition
Speech (slurred & monotonous)
Writing (micrographia)
Emotional movements (mask like face)
Arm swinging during walking (initially one, later both)

78. • Gait - Festinating gait (slow, shuffling, small
steps
• Difficulty in starting to walk or stopping when
pushed forward or backward (propulsion or
retropulsion).
• So great difficulty in walking but can run easily.
• If the person is given a good shove backwards,
instead of normally catching their balance,
he/she tends to fall back like a tree.

79. Body in a forward bent attitude (that causes
retropulsion- stooped posture)- walk as if a there is
a loss of gravity). This could be a compensation for
the postural imbalance.

80. • Postural deficits: difficulty adjusting to postural
change. When seated at the edge of a table and if
the table is tilted, instead of leaning uphill and
preserving balance, he topples over.

81. • Frequent falling while walking- becoz of the
combination of rigid/bradykinetic shuffling gait and
postural adjustment deficit. Unable to make
appropriate kinetic-postural adjustment necessary to
prevent themselves from falling.
• A bizarre but typical parkinsonian fall occurs when
the patient is unable to initiate stepping movement
with their feet although s/he has already initiated
forward movement of the trunk. To avoid falling on
their face, s/he usually drops to the knees.

82. Reflexes: Not affected but amplitude may be
reduced
Other symptoms (autonomic):
Pain in limbs & spine during later stages
Extreme restlessness, excessive sweating,
flushing of skin, excessive greasiness on the face
Increased salivation
Better tolerance for cold than heat

83. • In II type- Encephalitic symptoms such as
torticollis (twisting of head or neck to one side
caused by contraction of the muscles) and
dystonic attitudes if the trunk & limbs may
occur.

84. PATIENT’S PERCEPTION OF SPEECH
AND COMPLAINTS:
c/o voice being quiter or weak or cannot be heard
in noise are common.
c/o rate being too fast and that words are
indistinct, and their speech lacks emotional tone.
c/o dysfluencies with no fear of situations, anxiety
or anticipation.

85. •Speech symptoms: Hypokinetic
dysarthria (reduced range of movement of
speech structures)
• Bradykinesia (slow speech movements)
• Palilalia (motor speech perseveration, multiple
repetitions of words, phrases or sentences in a
context of decreasing loudness & increasing rate.
Also seen in other nueropathologies like
psuedobulbar palsy, alzheimer’s disease,
multiple infarct dementia & ideopathic cerebral
calcinosis)

86. • Festinating speech (Initiation of speech clear,
but later becomes unclear, speaks faster & faster
towards the end of the sentence, so speech
becomes slurred & fades)
• Monotonous speech
• Prosodic deviations-abnormal stress, lack of
intonation & fluctuation
• Soft voice
• Breathy voice
• Dysfluency

88. DIRECT, ACOUSTIC &
PHYSIOLOGIC FINDINGS

89. LEVEL DIRECT, ACOUSTIC & PHYSILOGIC OBSERVATIONS
Respiratory Reduced
Vital capacity
Amplitude of chest wall movts.
Air flow volume during vowel prolongation.
Intraoral pressure during AMRs.
Syllables per breath group.
Maximum vowel duration .
Increased
Respiratory rate.
Latency to being exhalation.
Latency to initiate phonation after exhalation initiated.
Irregular breathing patterns.
Paradoxic rib cage & abdominal movts.
Difficulty altering breathing patterns for speech.

90. Laryngeal Bowed vocal cords.
Tremulousness of arytenoids cartilages.
Asymmetry of laryngeal structures & movts during phonation.
Ventricular fold movt. During phonation.
Decreased
Intensity
Pitch & loudness variability.
Speed to initiate phonation.
Increased Fo.
Increased glottal resistance.
Increased shimmer.
Poor pitch control.
Velopharyngeal Increased nasal air flow during non nasal target productions..
Reduced velocity & degree of velar movt during speech.
Abnormal spread of nasalization across syllables.

91. Articulatory/rate prosody Reduced
Amplitude & velocity of lip movt.
Amplitude & duration of lip muscle action potentials
Jaw stability during vowel prolongation
Tongue endurance & strength.
Speech rate.
First & second formant transition rates.
Variation in syllable duration.
Increased
Connected speech & AMR rates.
Rate variability.
Frequency & duration of pauses during connected speech.
Articulatory undershoot of lip & velum.
Lip rigidity/stiffness.
Abnormal lip & jaw tremor at rest, during sustained
Postures & active & passive movt.
Spirantization of stops & affricates.
Continuous voicing.
Spread of nasalization across vowels.

92. • Treatment:
Medical:
• Levadopa increases dopamine content, but side
effects are dryness of mouth, blurred vision,
mental manifestation like confusions,
hallucinations etc.
• Anticholinergic drugs decrease the amount of
Ach activity in the striatum.
• Or combined
These drugs are not permanent cures. They only
reduce many of the symptoms and slow the
progression.

93. Surgical: Destructive stereotactic surgery to the
thalamus
Supportive therapies:
• PT & OT: Massaging & passive movements to
reduce tremor & rigidity. Reeducational walking
exercises to improve gait.
• Speech therapy

94. Speech therapy: Goals
1. To provide compensatory mechanisms to
increase the speech intelligibility
2. To control rate of speech (DAF, pacing
techniques, alphabet boards, metronome)
3. To improve articulatory precision (articulatory
drills)
4. To improve range of movement of oral
structures (oro motor exercises)
5. To improve voice characteristics viz. quality &
voice range, increase intensity (pushing &
pulling techniques, hard glottal attack, voice
amplifiers, instrumental biofeedback)

95. 6. To improve the respiration (breath support)
7. To improve prosodic features – contrastive
stress exercises, inflection therapy to improve
intonation, inhalation at natural syntactic
pauses
• Behavioral mod. based speech therapy
• Prosthetic (assistive speech devices)- computer
based softwares, assistive amplifiers, portable
DAF devices