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Movement Disorders

Vol. 15, No. 5, 2000, pp. 905910


2000 Movement Disorder Society

Dysarthria and Orofacial Apraxia in Corticobasal Degeneration


zsancak, MD, Pascal Auzou, MD, PhD, and Didier Hannequin, MD
Canan O
Federation des Sciences Neurologiques, Rouen, France

Summary: The authors evaluated dysarthria and orofacial


apraxia (OFA) in 10 patients with a clinical diagnosis of corticobasal degeneration (CBD). Nine patients were slightly dysarthric according to the French version of the Frenchay Dysarthria Assessment, which evaluates the motricity of the components of the vocal tract. The severity of dysarthria assessed
by an intelligibility score was correlated to the global severity
of the disease, but not to the duration of the disease. Voluntary
movements of the tongue and the lips were impaired in all
patients. OFA, evaluated with simple and sequential gestures,

was present in nine patients. Sequential gestures were more


frequently impaired. The score of OFA was not correlated to
the severity of dysarthria, suggesting independent underlying
mechanisms. Thus, when specifically assessed, dysarthria and
OFA are more frequent in CBD than usually reported. We
propose that the underlying pathophysiology is the result of a
deficit in programming and execution of repetitive movements.
Key Words: Corticobasal degenerationDysarthria
Orofacial apraxia.

Corticobasal degeneration (CBD) is a sporadic, progressive disorder characterized by the association of an


asymmetric, dopa-resistant, akinetic-rigid syndrome and
signs of cortical dysfunction such as apraxia, alien limb,
or sensory loss, often accompanied by other movement
disorders such as myoclonus or dystonia.1,2
Dysarthria results from disturbances in the muscular
control of speech mechanisms as a result of impairment
of any of the basic motor processes involved in the execution of speech. While often described incompletely as
imperfect articulation in speech, dysarthria can result
from abnormalities in articulation, but also from motor
disorders of respiration, phonation, resonance, and
prosody.3 It has been reported in 29% of patients in a
retrospective study of 147 cases of CBD4 and may be
present at the initial stage of the disease.1,5,6 Limb
apraxia is a prominent feature of CBD and is of great
importance in distinguishing CBD from other akineticrigid syndromes. On the contrary, orofacial apraxia
(OFA) has rarely been systematically assessed.7
We tested 10 patients with a clinical diagnosis of CBD
with particular reference to orofacial motricity. The aims

of this study were (1) to evaluate the frequency of dysarthria and orofacial apraxia and to search for relationships between these two signs, and (2) to describe the
types of impairment of the vocal tract.
METHOD
Ten patients were consecutively included in the study.
They fulfilled the following inclusion criteria: (1) asymmetric akinetic-rigid syndrome, (2) apraxia, (3) insidious
onset and gradual progression, and (4) absence of focal
lesions other than frontal/parietal atrophy on magnetic
resonance imaging. The analysis of speech and orofacial
apraxia was part of a global assessment, which included
neuroimaging and neuropsychologic assessment. All patients underwent neurologic examination by one of the
authors (C.O.).
There were six men and four women aged 72.3 years
(range, 67 to 78 yrs). The duration of the disease was 3
years (range, 0.5 to 5 yrs). Eight patients were righthanded, one was left-handed, and one was ambidextrous.
The global severity of the disease was evaluated by the
Schwab & England Capacity for Daily Living Scale.
Clinical features are summarized in Table 1.
Patients also underwent a comprehensive neuropsychologic examination that included the following tests:
the Purdue Pegboard Test8 (which tests unimanual and
bimanual dexterity), the Mini-Mental State Evaluation

Received August 23, 1999; revision received February 2, 2000. Accepted February 24, 2000.
zsancak,
Address correspondence and reprint requests to Canan O
MD, Departement de Neurologie, CHU de Rouen, 1 rue de Germont,
76031 Rouen Cedex, France; e-mail: c-ozsancak@yahoo.fr

905

ZSANCAK ET AL.
C. O

906

TABLE 1. Clinical features of 10 patients with corticobasal degeneration


Case no.

10

Total

Age (yrs)
Sex
Handedness
Disease duration (yrs)
Schwab & England (%)
Side of initial symptom
Asymmetric akinesia/rigidity
Postural instability
Myoclonus
Tremor (postural or action)
Dystonia
Limb apraxia
Sensory loss
Alien limb
Supranuclear gaze palsy
Pyramidal signs
Emotional lability
Dysarthria
Dysphagia

73
M
R
2
90
L
+

77
M
R
5
80
R
+
+

+
+

+
+

78
F
R
1.5
80
R
+

+
+

73
M
A
0.5
80
L
+
+

+
+
+

70
M
R
4
60
R
+
+

+
+

78
F
R
3.5
30
L
+
+
+
+
+
+
+

+
+

67
M
L
1.5
40
R
+
+

+
+
+

71
F
R
5
50
R
+
+

+
+
+

67
M
R
2
40
L
+
+

+
+
+

69
F
R
5
20
L
+
+
+
+
+
+
+
+

+
+
+
+

72.3
6 M, 4 F
8R, 1A, 1L
3

5 L, 5 R
10/10
8/10
3/10
6/10
6/10
10/10
3/10
3/10
3/10
6/10
3/10
9/10
2/10

A, ambidextrous.

(MMSE),9 the Mattis Dementia Rating Scale (MDRS),10


the Grober and Buschke Test11 (which tests memory
with controlled encoding and selective reminding), the
Boston Diagnostic Aphasia Examination (BDAE),12 the
Wisconsin Card Sorting Test,13 the Categorical Verbal
Fluency Test14 (which requires the subject to say as
many animal names as possible in 2 minutes), the Wech-

sler Adult Intelligence Scale (WAIS),15 the Block Design Subtest, and the Rey-Osterrieth Complex Figure
Test (Rey-O).16 The last two tests evaluate visuospatial
and visuoconstructive abilities sensitive to parietal lesions. Results are summarized in Table 2.
The control group for speech evaluation included 15
subjects (five men and 10 women, mean age 67 yrs, age

TABLE 2. Results of the neuropsychologic assessment


Case no.

10

Age (yrs)
Handedness
Side of initial symptom
Purdue pegboard test
Most affected hand
Least affected hand
Bimanual
MMSE (/30)
MDRS (/144)
Aphasia severity rating (BDAE)
Grober & Buschke
Immediate free recall
Long delay free recall
Total recall (free and cued)
WCST
No. of categories
Perseverative errors
Verbal fluency
WAIS-R block design
Rey-O copy

73
R
L

78
R
R

73
A
L

70
R
R

78
R
L

67
L
R

71
R
R

67
R
L

69
R
L

<1
<1
<1
24
130
5

<1
50
2
24
126
5

3
7*
<1
27
130
5

1
1
<1
24
100
4

Imp
45
Imp
26
131
5

<1
5*
<1
21
73
4

Imp
Imp
Imp
28
120
5

Imp
2
Imp
25
115
5

Imp
1
Imp
14
103
4

10
7
13

13
10
16

5
5
12

1
2
8

4
6*
16

NA
NA
NA

4
6
14

4
4
16

2
0
2

2*
20
20
7*
30*

2*
29
16*
5
10

2*
16*
19
10
32

3*
8
8
4
Imp

2*
13*
17*
6
31*

1
37
19
2
Imp

2*
6
19*
7*
Imp

5
3
19
3
9

1
11*
19*
3
10

Imp, motor handicap enabled the patient to perform the test; MMSE, Mini-Mental State Evaluation; MDRS, Mattis Dementia Rating Scale; BDAE,
Boston Diagnostic Aphasia Examination; WCST, Wisconsin Card Sorting Test; WAIS-R, Wechsler Adult Intelligence Scale; NA, not available.
* < mean 1 standard deviation.
< mean 2 standard deviations.
Dementia if MMSE <26.

Aphasia if severity rating <5.


Patient 2 refused the evaluation.

Movement Disorders, Vol. 15, No. 5, 2000

DYSARTHIA AND OROFACIAL APRAXIA IN CBD

907

pairments such as hypophonia, dysprosody, or rate


abnormalities.
Orofacial apraxia was assessed by asking patients to
perform 12 gestures on verbal command (see details provided in the Appendix). Six of the gestures were simple
gestures. Four were accompanied by the production of a
noise, and the last two were sequential gestures. If the
patient produced no movement or an incorrect one, the
request was repeated once and the score was established
on the second trial. For each gesture, a 5-point scale
(04, in which 4 corresponds to normal performance)
was used with a maximum score of 48. Evaluation of
OFA was made by the same person (C.O.). For each
group of gestures (simple, with noise, sequential), the
lowest score achieved by control subjects was used as the
cut-off score between normal praxis and apraxia.

range 5484 yrs) with no history of a central nervous


system disorder. The control group for OFA included
eight different subjects (four men and four women, mean
age 66 yrs, age range 4077 yrs).
Dysarthria was clinically evaluated with a French adaptation17 of the Frenchay Dysarthria Assessment developed by Enderby.18 This evaluation consisted of 28
items. The first 25 items assessed motricity of the systems implied in speech production during tasks with and
without speech. These different systems were classified
into seven categories (Reflex activities such as deglutition, Respiration, Larynx, Lips, Tongue, Jaw, Velum).
Each item was scored on a 9-point scale (08) in which
8 corresponds to a normal performance. The mean values
were then obtained for each of the seven categories and
added to obtain a Total Functional Score (TFS). The
maximum score was 56. The cut-off for normal performance for each category was taken as the mean minus 2
standard deviations of the scores obtained among the
control group.19
The last three items evaluated intelligibility while
reading single words and sentences and during conversational speech. These items were added to obtain a
maximum score of 24. A subject was considered dysarthric if his Intelligibility Score (IS) was below 24.19 This
might seem too high a standard for normal speech, but
all the control subjects obtained a score of 24. Furthermore, according to the grading established by Enderby,18
a score between 18 and 23 corresponds to slight dysarthria in which all the words and sentences are correctly
identified but speech presents with perceptual im-

Statistics
By using the Pearsons method, we searched for correlations between the IS and each of the following measures: the duration of the disease, the Schwab & England
score, and the OFA score.
RESULTS
Nine of 10 subjects were dysarthric because their IS
was below 24 (Table 3). The reduction of intelligibility
was slight with a mean IS of 20 out of 24 ( 2.9). The
TFS was altered in all patients with a mean TFS of 46.3
out of 56 ( 3.9).
The analysis of the seven categories revealed that
motricity of the lip and the tongue was always impaired.

TABLE 3. Results of the assessment of dysarthria and orofacial apraxia


Case no.
Dysarthria
IS (/24)
TFS (/56)
Categories
Lips (/8)
Tongue (/8)
Larynx (/8)
Reflex (/8)
Respiration (/8)
Jaws (/8)
Velum (/8)
Orofacial apraxia
Total (%)
Categories
Single (%)
Noise (%)
Sequential (%)

10

24
51.0*

23*
49.6*

23*
42.7*

22*
47.7*

20*
50.1*

20*
48.6*

19*
47.3*

19*
44.9*

17*
40.2*

15*
41.1*

7.0*
5.7*
7.3
8.0
7.7
8.0
7.3

7.4*
5.5*
7.7
6.0*
7.3
8.0
7.7

6.0*
6.3*
4.7*
8.0
4.7*
5.0*
8.0

6.4*
6.3*
5.7*
6.7*
6.7
8.0
8.0

7.4*
4.7*
7.3
7.7
7.0
8.0
8.0

7.4*
5.8*
6.3*
7.3
5.7*
8.0
8.0

6.4*
6.0*
6.3*
7.3
7.0
7.0*
7.3

7.2*
3.7*
6.3*
7.0*
5.7*
8.0
7.0

4.2*
5.5*
2.7*
7.0*
5.3*
7.5*
8.0

5.6*
5.2*
3.7*
6.0*
4.7*
8.0
8.0

85.4*

93.8*

89.6*

89.6*

70.8*

85*

93.8*

35.4*

58.3*

100

7.5
6.4
6.8
7.3
6.3
8.0
7.0
Cut-off
95.8

91.7
100
37.5*

100
100
62.5*

100
93.8*
50*

100
87.5*
62.5*

83.3*
87.5*
0*

87.5*
100
62.5*

100
100
62.5*

41.7*
37.5*
12.5*

70.8*
50*
37.5*

100
100
100

91.7
100
100

Cut-off
24
51.7

IS, intelligibility score; TFS, total functional score; M, mean; SD, standard deviations.
Cases were classed according to the severity of their dysarthria (1 for no impairment and 10 for the most severe handicap). The IS was the main
criteria. The TFS was used when two patients had the same IS. Patient values marked with an asterisk (*) are below the cut-off score.
Only two gestures with noise were assessed.

Movement Disorders, Vol. 15, No. 5, 2000

908

ZSANCAK ET AL.
C. O

Laryngeal function was impaired in seven patients. Reflex activities and respiration were impaired in five and
jaw motricity in three patients. Velar function was normal in all subjects.
The assessment of OFA revealed four patients below
the cut-off for single gestures and five for gestures with
noise production. The most striking result was the importance of the impairment in sequential gestures, which
was present in nine patients.
The severity of dysarthria assessed by the IS was
strongly correlated to the Schwab & England score (r
0.91, p <103), but not to the duration of the symptoms
(r 0.34) or to the OFA score (r 0.21).
DISCUSSION
Dysarthria is frequently present at the advanced stages
of CBD. It is rarely the initial symptom of CBD and is
then mostly associated with limb symptoms.6,2022 In a
review of 398 patients, 55% had dysarthria.23 Kompoliti
et al.4 reported dysarthria in 29% of 147 cases. However,
Wenning et al.6 show that dysarthria is more frequent in
CBD. In a group of 14 patients with neuropathologic
confirmation evaluated 3 years after onset of the disease,
speech was slow in five, slurred in four, dysphonic in
two, and unintelligible in one. Six years after onset,
speech was almost always abnormal (93%). Five patients
were mute and some had echolalia or palilalia.6 In another study of 14 CBD cases, dysarthria was present in
13 patients.24 Our data confirmed these results because
nine of 10 patients were dysarthric. Dysarthria occurred
early in the evolution of the disease because the mean
duration of the symptoms was 3 years. Like in other
atypical parkinsonian syndromes such as progressive supranuclear palsy or multiple system atrophy, dysarthria is
therefore an early sign in CBD. Its importance is related
to the global severity of the disease, not to the duration of
the symptoms.
The mean IS of 20 out of 24 corresponds to slight
dysarthria. Such scores are used for abnormal speech
without real loss of intelligibility. Even when their
speech is abnormal, such patients rarely need to repeat
themselves. The impairment in our patients was the result of slow, dysprosodic, or hypophonic speech. In fact,
dysarthria probably rarely handicaps patients with CBD,
explaining the low frequency reported in the literature.
The smaller incidence reported could also be the result of
the search for articulatory deficiency to define dysarthria.
Orofacial apraxia is the inability to perform movements on command with the muscles of the larynx, pharynx, tongue, lips, cheeks, and face, although automatic
movements of the same muscles are preserved.25 In the
largest retrospective clinical series of CBD, OFA was

Movement Disorders, Vol. 15, No. 5, 2000

reported in only 3% (five of 147 patients), suggesting its


rarity in CBD.4 In a systematic evaluation of apraxia,
Leiguarda et al.7 found no patient with OFA, whereas
seven had limb apraxia. OFA was also absent in a group
of 12 patients with CBD who underwent a comprehensive neuropsychologic evaluation.26 Of 13 patients,
Frattali and Sonies24 report six with OFA.
In the present study, simple gestures were impaired in
five patients and sequential ones in nine. Patients were
tested on verbal command and not on imitation. However, such abnormalities cannot be explained by an impairment in understanding. According to the BDAE
aphasia severity rating item, seven patients were not
aphasic. Three had a subnormal score of 4 out of 5,
corresponding to reduced verbal fluency. The most frequent abnormalities in orofacial praxis assessment were
hesitation, omission, and sequencing errors. Movements
were awkward, preceded by pauses during which abnormal movement patterns were sometimes carried out. The
difference between our data and those reported by others
may result, in part, from differences in the methods of
testing orofacial praxis, because other works considered
simple gestures almost exclusively. Leiguarda et al.7 did
not report their complete list of evaluation gestures but
the examples provided were all simple movements. In
our study, OFA seems more frequent, even for simple
gestures. However, in four patients, OFA was only revealed by sequential gestures. The search for OFA with
sequential gestures might therefore help diagnose CBD
in a patient with atypical parkinsonism.
Analyzing the clinical association between dysarthria
and OFA might help explore relationships between these
two signs. Schneider et al.5 report one patient with pathologic confirmation of CBD whose initial symptoms were
severe dysarthria and prominent orofacial apraxia. Our
study has shown that eight of 10 patients had both signs.
However, OFA was present without dysarthria in case
no. 1, whereas case no. 10 had the opposite pattern.
These dissociations call into question the possibility that
dysarthria and orofacial apraxia might result from a
single deficient mechanism. The absence of correlation
in our study between the IS and OFA score further argues
for the independence of these two signs.
Concerning the anatomic site of the lesions associated
with these disorders, OFA has been described in lesions
of the deep anterior 23 of the paraventricular white matter, the frontal and central operculum, the adjacent parts
of the first temporal convolution, the insula, the striatum,
and the thalamus.2731 Lesions of the lower parietal lobule have also been associated with OFA, although to a
lesser degree.27,32

DYSARTHIA AND OROFACIAL APRAXIA IN CBD


When dysarthria is clinically significant and of early
onset, the brunt of the cortical degeneration in CBD occurs predominantly in the anterior frontal and parasagittal cortex.5 Damage to frontostriatal circuits may also
account for dysarthria.33 A PET study in a case of CBD
with both signs showed hypometabolism predominant in
the left superior temporal gyrus, left inferior precentral
gyrus, and in the left supplementary motor area.34 Thus,
because regions responsible for OFA and dysarthria are
widespread and overlapping, it seems likely that these
signs coexist often in CBD.
Our second aim was to assess motricity of the systems
implied in speech in patients with CBD. Speech results
from the coordination between the respiratory system,
the larynx, and the supralaryngeal level corresponding to
the articulators. The clinical evaluation of motricity
therefore provides a simple tool which quantifies specific
impairments of each component of the vocal tract. Our
results revealed a constant pattern of lip and tongue impairment. The impairments of the different components
of the vocal tract that lead to dysarthria in CBD have not
been reported previously. Therefore, because pathologic
lesions in CBD mainly concern the extrapyramidal pathways, studies of parkinsonian dysarthria might provide
indirect pathogenic clues. In a study of lip motricity in
parkinsonian patients, Caligiuri35 observed increased rigidity with reduced maximal velocities and amplitudes.
Parkinsonism, present in all our patients with CBD,
might provide a similar explanation for labial and lingual
abnormalities. CBD also results in cortical cell loss in the
sensorimotor cortex. Cortical pyramidal cell loss might
have a greater influence on hypoglossal motor neurons
because the tongue is disproportionately represented in
the primate motor cortex.3638 Murray et al.39 reported
that reversible deactivation of the face motor cortex
caused severe impairments in tongue protrusion but only
minor effects on biting. A selective vulnerability of the
lips and the tongue could therefore be suggested.
However, another explanation could be an execution
deficit during sequential movements. The lips and the
tongue are mostly assessed by rating the velocity, amplitude, and regularity of sequential movements. For example, one of the items of lip motricity consists of asking
the patient to repeat the sound pa as rapidly, regularly,
and long as possible. Thus, the assessment of the tongue
and the lips could simply be more sensitive to reveal an
overall hypokinetic tendency in rapid, repetitive orofacial movements.
In conclusion, dysarthria and orofacial apraxia are frequent in CBD and mostly concern sequential rather than
simple gestures. We propose that the underlying pathophysiology is the result of both a deficit of programming

909

and execution of repetitive movements. Other parkinsonian syndromes should be thoroughly explored to determine the specificity of OFA in CBD.
Acknowledgment: The authors thank Prof. N. Quinn for his
help with the English text.

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APPENDIX
Evaluation Scale of Orofacial Apraxia
Simple gestures:
1.
2.
3.
4.
5.
6.

Stick out your tongue.


Try to touch the tip of your nose with your tongue.
Try to touch your chin with your tongue.
Direct your tongue to the left.
Direct your tongue to the right.
Show your teeth.
Gestures accompanied by the production of a noise:

1.
2.
3.
4.

Blow.
Send a kiss.
Click your tongue.
Whistle.
Sequential gestures:

1. Blow your cheeks then bite the inferior lip.


2. Show your tongue, click your tongue, then puff out
your cheeks.
For each gesture:
4
3
2
1
0

points: movement accurately performed


points: correct after hesitation
points: movement incomplete but can be identified
point: movement can barely be identified
points: oral movements either not carried out or completely irrelevant

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