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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
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.
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
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.
907
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.
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.
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
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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ZSANCAK ET AL.
C. O
APPENDIX
Evaluation Scale of Orofacial Apraxia
Simple gestures:
1.
2.
3.
4.
5.
6.
1.
2.
3.
4.
Blow.
Send a kiss.
Click your tongue.
Whistle.
Sequential gestures: