Movement Disorders

Vol. 15, No. 5, 2000, pp. 905–910

© 2000 Movement Disorder Society

Dysarthria and Orofacial Apraxia in Corticobasal Degeneration

Canan Özsancak, MD, Pascal Auzou, MD, PhD, and Didier Hannequin, MD

Fédération des Sciences Neurologiques, Rouen, France

Summary: The authors evaluated dysarthria and orofacial
apraxia (OFA) in 10 patients with a clinical diagnosis of cor-
ticobasal degeneration (CBD). Nine patients were slightly dys-
arthric according to the French version of the Frenchay Dys-
arthria Assessment, which evaluates the motricity of the com-
ponents 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 degeneration—Dysarthria—
Orofacial apraxia.

Corticobasal degeneration (CBD) is a sporadic, pro- of this study were (1) to evaluate the frequency of dys-
gressive disorder characterized by the association of an arthria and orofacial apraxia and to search for relation-
asymmetric, dopa-resistant, akinetic-rigid syndrome and ships between these two signs, and (2) to describe the
signs of cortical dysfunction such as apraxia, alien limb, types of impairment of the vocal tract.
or sensory loss, often accompanied by other movement
disorders such as myoclonus or dystonia.1,2 METHOD
Dysarthria results from disturbances in the muscular
Ten patients were consecutively included in the study.
control of speech mechanisms as a result of impairment
They fulfilled the following inclusion criteria: (1) asym-
of any of the basic motor processes involved in the ex-
metric akinetic-rigid syndrome, (2) apraxia, (3) insidious
ecution of speech. While often described incompletely as
onset and gradual progression, and (4) absence of focal
imperfect articulation in speech, dysarthria can result
lesions other than frontal/parietal atrophy on magnetic
from abnormalities in articulation, but also from motor
resonance imaging. The analysis of speech and orofacial
disorders of respiration, phonation, resonance, and
apraxia was part of a global assessment, which included
prosody.3 It has been reported in 29% of patients in a
neuroimaging and neuropsychologic assessment. All pa-
retrospective study of 147 cases of CBD4 and may be
tients underwent neurologic examination by one of the
present at the initial stage of the disease.1,5,6 Limb
authors (C.O.).
apraxia is a prominent feature of CBD and is of great
There were six men and four women aged 72.3 years
importance in distinguishing CBD from other akinetic-
(range, 67 to 78 yrs). The duration of the disease was 3
rigid syndromes. On the contrary, orofacial apraxia
years (range, 0.5 to 5 yrs). Eight patients were right-
(OFA) has rarely been systematically assessed.7
handed, one was left-handed, and one was ambidextrous.
We tested 10 patients with a clinical diagnosis of CBD
The global severity of the disease was evaluated by the
with particular reference to orofacial motricity. The aims
Schwab & England Capacity for Daily Living Scale.
Clinical features are summarized in Table 1.
Received August 23, 1999; revision received February 2, 2000. Ac- Patients also underwent a comprehensive neuropsy-
cepted February 24, 2000. chologic examination that included the following tests:
Address correspondence and reprint requests to Canan Özsancak,
MD, Département de Neurologie, CHU de Rouen, 1 rue de Germont, the Purdue Pegboard Test8 (which tests unimanual and
76031 Rouen Cedex, France; e-mail: c-ozsancak@yahoo.fr bimanual dexterity), the Mini-Mental State Evaluation

905
906 C. ÖZSANCAK ET AL.

TABLE 1. Clinical features of 10 patients with corticobasal degeneration

Case no. 1 2 3 4 5 6 7 8 9 10 Total
Age (yrs) 73 77 78 73 70 78 67 71 67 69 72.3
Sex M M F M M F M F M F 6 M, 4 F
Handedness R R R A R R L R R R 8R, 1A, 1L
Disease duration (yrs) 2 5 1.5 0.5 4 3.5 1.5 5 2 5 3
Schwab & England (%) 90 80 80 80 60 30 40 50 40 20 −
Side of initial symptom L R R L R L R R L L 5 L, 5 R
Asymmetric akinesia/rigidity + + + + + + + + + + 10/10
Postural instability − + − + + + + + + + 8/10
Myoclonus − − + − − + − − − + 3/10
Tremor (postural or action) + − + + − + − − + + 6/10
Dystonia − + − + + + − + − + 6/10
Limb apraxia + + + + + + + + + + 10/10
Sensory loss − − − − − + − + − + 3/10
Alien limb − − − − + − − − + + 3/10
Supranuclear gaze palsy − − − + − + − − + − 3/10
Pyramidal signs − − − − + + + + + + 6/10
Emotional lability + − − − − − + − − + 3/10
Dysarthria − + + + + + + + + + 9/10
Dysphagia − + − − − − − − − + 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 De-
sign Subtest, and the Rey-Osterrieth Complex Figure
Test (Rey-O).16 The last two tests evaluate visuospatial
and visuoconstructive abilities sensitive to parietal le-
sions. 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. 1 3 4 5 6 7 8 9 10
Age (yrs) 73 78 73 70 78 67 71 67 69
Handedness R R A R R L R R R
Side of initial symptom L R L R L R R L L
Purdue pegboard test
Most affected hand <1† <1† 3† 1† Imp <1† Imp Imp Imp
Least affected hand <1† 50 7* 1† 45 5* Imp 2† 1†
Bimanual <1† 2† <1† <1† Imp <1† Imp Imp Imp
MMSE‡ (/30) 24 24 27 24 26 21 28 25 14
MDRS (/144) 130† 126† 130† 100† 131† 73† 120† 115† 103†
Aphasia severity rating (BDAE)㛳 5 5 5 4 5 4 5 5 4
Grober & Buschke
Immediate free recall 10† 13 5† 1† 4† NA 4† 4† 2†
Long delay free recall 7† 10 5† 2† 6* NA 6† 4† 0†
Total recall (free and cued) 13† 16 12† 8† 16 NA 14† 16 2†
WCST
No. of categories 2* 2* 2* 3* 2* 1† 2* 5 1†
Perseverative errors 20† 29† 16* 8† 13* 37† 6 3 11*
Verbal fluency 20 16* 19 8† 17* 19 19* 19 19*
WAIS-R block design 7* 5† 10 4† 6† 2† 7* 3† 3†
Rey-O copy 30* 10† 32 Imp 31* Imp Imp 9† 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.
㛳
Aphasia if severity rating <5.
Patient 2 refused the evaluation.

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

 DYSARTHIA AND OROFACIAL APRAXIA IN CBD 907

range 54–84 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 40–77 yrs).
Dysarthria was clinically evaluated with a French ad-
aptation17 of the Frenchay Dysarthria Assessment devel-
oped by Enderby.18 This evaluation consisted of 28
items. The first 25 items assessed motricity of the sys-
tems implied in speech production during tasks with and
without speech. These different systems were classified
into seven categories (Reflex activities such as degluti-
tion, Respiration, Larynx, Lips, Tongue, Jaw, Velum).
Each item was scored on a 9-point scale (0–8) 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 perfor-
mance 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 conver-
sational speech. These items were added to obtain a
maximum score of 24. A subject was considered dysar-
thric 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. Further-
more, according to the grading established by Enderby,18
a score between 18 and 23 corresponds to slight dys-
arthria in which all the words and sentences are correctly
identified but speech presents with perceptual im-
pairments such as hypophonia, dysprosody, or rate
abnormalities.
Orofacial apraxia was assessed by asking patients to
perform 12 gestures on verbal command (see details pro-
vided 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
(0–4, 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.
Statistics
By using the Pearson’s method, we searched for cor-
relations between the IS and each of the following mea-
sures: 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. 1 2 3 4 5 6 7 8 9 10
Dysarthria Cut-off
IS (/24) 24 23* 23* 22* 20* 20* 19* 19* 17* 15* 24
TFS (/56) 51.0* 49.6* 42.7* 47.7* 50.1* 48.6* 47.3* 44.9* 40.2* 41.1* 51.7
Categories
Lips (/8) 7.0* 7.4* 6.0* 6.4* 7.4* 7.4* 6.4* 7.2* 4.2* 5.6* 7.5
Tongue (/8) 5.7* 5.5* 6.3* 6.3* 4.7* 5.8* 6.0* 3.7* 5.5* 5.2* 6.4
Larynx (/8) 7.3 7.7 4.7* 5.7* 7.3 6.3* 6.3* 6.3* 2.7* 3.7* 6.8
Reflex (/8) 8.0 6.0* 8.0 6.7* 7.7 7.3 7.3 7.0* 7.0* 6.0* 7.3
Respiration (/8) 7.7 7.3 4.7* 6.7 7.0 5.7* 7.0 5.7* 5.3* 4.7* 6.3
Jaws (/8) 8.0 8.0 5.0* 8.0 8.0 8.0 7.0* 8.0 7.5* 8.0 8.0
Velum (/8) 7.3 7.7 8.0 8.0 8.0 8.0 7.3 7.0 8.0 8.0 7.0
Orofacial apraxia Cut-off
Total (%) 85.4* 93.8* 89.6* 89.6* 70.8* 85* 93.8* 35.4* 58.3* 100 95.8
Categories
Single (%) 91.7 100 100 100 83.3* 87.5* 100 41.7* 70.8* 100 91.7
Noise (%) 100 100 93.8* 87.5* 87.5* 100† 100 37.5* 50* 100 100
Sequential (%) 37.5* 62.5* 50* 62.5* 0* 62.5* 62.5* 12.5* 37.5* 100 100

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 C. ÖZSANCAK ET AL.
Laryngeal function was impaired in seven patients. Re-
flex activities and respiration were impaired in five and
jaw motricity in three patients. Velar function was nor-
mal 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 im-
portance 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 <10−3), 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.6,20–22 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 an-
other 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 su-
pranuclear 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 re-
sult 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 move-
ments on command with the muscles of the larynx, phar-
ynx, 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 compre-
hensive 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. How-
ever, such abnormalities cannot be explained by an im-
pairment 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 fre-
quent abnormalities in orofacial praxis assessment were
hesitation, omission, and sequencing errors. Movements
were awkward, preceded by pauses during which abnor-
mal 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 re-
vealed 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 patho-
logic 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 2⁄3 of the paraventricular white mat-
ter, the frontal and central operculum, the adjacent parts
of the first temporal convolution, the insula, the striatum,
and the thalamus.27–31 Lesions of the lower parietal lob-
ule 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 oc-
curs predominantly in the anterior frontal and parasagit-
tal 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 im-
pairment. 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 path-
ways, studies of parkinsonian dysarthria might provide
indirect pathogenic clues. In a study of lip motricity in
parkinsonian patients, Caligiuri35 observed increased ri-
gidity 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.36–38 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, am-
plitude, and regularity of sequential movements. For ex-
ample, 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 orofa-
cial movements.
In conclusion, dysarthria and orofacial apraxia are fre-
quent in CBD and mostly concern sequential rather than
simple gestures. We propose that the underlying patho-
physiology is the result of both a deficit of programming
909
and execution of repetitive movements. Other parkinso-
nian syndromes should be thoroughly explored to deter-
mine the specificity of OFA in CBD.
Acknowledgment: The authors thank Prof. N. Quinn for his
help with the English text.
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774. pletely irrelevant

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