Movement Disorders Vol. 6, No. 3, 1991, pp. 248-252,
0 1991 Movement Disorder Society
Dystonic Tics in Patients with Tourette’s Syndrome Joseph Jankovic and Lael Stone Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas, U.S.A.
Summary: Dystonic tics, in contrast to clonic tics, are relatively slow and temporarily sustained twisting, pulling, or squeezing movements producing briefly maintained abnormal postures. Because dystonic tics often present diagnostic difficulties, we studied these motor tics in 156 patients with Tourette’s syndrome (TS). In addition to clonic motor and vocallphonic tics, seen in all patients, 89 (57%) of them exhibited one or more dystonic tics. The most common dystonic tics were oculogyric deviations (43 patients), blepharospasm (23), and dystonic neck movements (1 1). Blinking (1 10) and facial twitching (86) were the most common clonic tics. Except for possibly a greater familial occurrence, a higher frequency of previous head trauma, and associated attention deficit disorder, the group of patients with dystonic tics did not significantly differ in any of the major clinical variables from those TS patients without dystonic tics. We conclude that dystonic tics are typical motor manifestations of TS and that patients with these motor tics are not different from those with more typical clonic tics. Key Words: Dystonic tics-Clonic ticsTourette’s syndrome-Head trauma-Attention deficit disorder.
tonic tics. To determine whether patients with dystonic tics represent a distinct “disease entity” (2), and are hence pathogenically different from those with clonic tics, we studied the clinical correlates of the two types of tics in 156 TS patients.
Because of the variable expression of Tourette’s syndrome (TS), patients with this movement and behavioral disorder are often misdiagnosed. In order to improve the diagnostic criteria for TS, an attempt has been made to better characterize the different motor manifestations (1). Motor and vocal/ phonic tics have been categorized as either simple or complex. Simple motor tics are further subdivided into clonic or dystonic. While clonic tics, particularly when associated with involuntary noises, are usually appropriately diagnosed, dystonic tics are often not recognized as part of the spectrum of TS. When the expression of TS is dominated by dystonic tics, the disorder is sometimes wrongly diagnosed as dystonia (2). Dystonia, in contrast to dystonic tics, is a chronically repetitive, patterned, and sustained movement, often producing a persistently abnormal posture (3). Furthermore, movements and postures associated with idiopathic or secondary dystonias are less suppressible than dys-
PATIENTS AND METHODS All 185 medical records of patients with tics evaluated at the Baylor College of Medicine Movement Disorders Clinic between 1982 and 1989 were reviewed. During the initial visit, the patients, relatives, and other informants were interviewed about the distribution and character of tics present currently and in the past. For the purposes of this retrospective survey, dystonic tics were defined as temporarily sustained abnormal movements or postures occurring out of a background of normal motor activity. Because tics are often suppressed during examination (and videotaping), we relied on descriptions of the various tics provided by patients, parents, and other observers. To elicit an accurate history of tics during the course of TS, the patients were asked whether they experienced jerk-like (clonic) or sustained (dystonic) contractions of mus-
Address correspondence and reprint requests to Dr. J. Jankovic at Department of Neurology, Baylor College of Medicine, 6550 Fannin #1801, Houston, TX 77030, U.S.A.
248
DYSTONIC TICS IN TOURETTES SYNDROME cles. We were particularly interested in obtaining detailed descriptions of dystonic tics; examples, such as blepharospasm, oculogyric deviations, and bruxism, were described for each anatomic region. The dystonic tics were witnessed in most patients at the time of examination, and in some they were videotaped and recorded by surface EMG (Figs. 1, 2). In addition to obtaining data about the motor and vocaVphonic tics, we collected information about prior head and neck trauma, behavioral problems, such as obsessive-compulsive traits, attention deficit, and other psychologic or psychiatric manifestations. We excluded seven patients who had the combination of tics (clonic and dystonic) and coexistent persistent dystonia (torsion dystonia). These patients, in addition to exhibiting clonic and dystonic tics as defined above, had a persistent, chronic, patterned dystonic movement and abnormal posture, always present in the same distribution and involving the same group of muscles (e.g., torticollis). The DSM-IIIR criteria for TS were met by 156 patients, who constitute the study population (4). The patients were divided into two groups: TS patients with clonic and dystonic tics (dystonic group) and TS patients with clonic tics but without dystonic tics (clonic group). The clinical and demographic data were entered into a relational data base, and the two groups were analyzed with respect to major clinical variables. Pearson's chisquare method was used to test for differences between the groups; p < 0.05 was considered suggestive of differences, whereas p < 0.005 was considered statistically significant when corrected for multiple comparisons.
249
RESULTS There were 89 patients who had the combination of clonic and dystonic tics (dystonic group) and 67 who had only clonic tics (clonic group). There was no difference between the two groups in any of the demographic variables (Table 1). By definition, dystonic tics, such as blepharospasm, ocular deviations, and bruxism were noted only in the dystonic group. There was no difference in the anatomic distribution of tics between dystonic and clonic groups (Tables 2-4). Patients with dystonic tics had a higher prevalence of attention deficit disorder (ADD) and a greater frequency of headheck trauma (Table 5). This difference remained significant even when adjusted for multiple comparisons. When compared with patients with only clonic tics, TS patients with dystonic tics seemed more likely to have parents and/or paternal relatives with tics; however, this difference was not statistically significant when adjusted for multiple comparisons (Table 6). DISCUSSION This study examines the possibility that TS patients with dystonic tics differ in some way from other TS patients. Dystonic tics were defined as temporarily sustained abnormal movements or postures, often with abrupt onset, occurring out of a background of normal motor activity. We recognize the limitations of this retrospective study, particularly the difficulties in ascertaining symptoms. We feel, however, that the clinical data was collected accurately and without bias regarding any possible differences between clinical correlates of clonic versus dystonic tics.
FIG. 1. Surface EMG recording from pectoralis in a 50-year-old man with clonic and dystonic tics of the right shoulder. The short ( I s) bursts are associated with prolonged dystonic movements of the right shoulder.
500 ms
Movement Disorders, Vol. 6, N o . 3, 1991
J . JANKOVIC AND L . STONE
250
111
I
FIG. 2. Surface EMG recording from left coracobrachialis (top tracing) and left deltoid (bottom tracing) in an 18-year-old woman with TS manifested chiefly by involuntary loud vocalizations, abducting (dystonic) movements, and jerk-like (clonic) rotatory movements of the left shoulder.
500 ms
All our patients with dystonic tics also had clonic motor and vocal/phonic tics. We found that the 89 patients with dystonic tics were virtually indistinguishable from the 67 TS patients who had only clonic tics. However, patients with dystonic tics had suffered more headheck trauma and had a higher frequency of associated ADD than the other TS patients. Although statistically significant, the differences are minor and probably do not justify a separation of patients with dystonic tics into a distinct "disease entity" (2). Although our population is selected and therefore cannot be considered representative of all TS patients, their demographic and clinical characteristics are similar to those reported in other large clinic- (5-8) and population-based series (9). We exTABLE 1. Demographic features of TS patients TS Patients
Total
With dystonic tics" (n = 89)
With clonic tics" (n = 67)
Variable
n
%
n
%
n
%
Males Right-handed Left-handed Ambidextrous Mean age at initial visit (yr4 Mean age at onset (yrs)
127 137 16 3
81 88 10 2
73 75 10 2
82 84 11 2
54 62 6 1
80 93 7 1
20
20
6.7
6.3
" No significant difference between the groups.
Movement Disorders, Vol. 6 , No. 3, 1991
20 7.7
cluded all patients with atypical features and seven patients who had the coexistence of tics and persistent dystonia. An association between tics and dystonia has been noted previously (10-12). While dystonic tics are often misdiagnosed as dystonia, they should be easily differentiated if they are accompanied by other features of TS (clonic motor and voTABLE 2. Distribution of tics in TS patients TS patients With dystonic tics"
Total
With clonic tics"
Distribution
n
%
n
%
n
%
Blinking Blepharospasm Ocular deviations Forehead Nose Lips Mouth Tongue Face Bruxisrn Jaw opening Head jerk Neck Shoulder Arm Hand Leg Foot Trunk Abdomen Pelvic thrust
110 23
70 15
68 23
76 26
42 -
63 -
43 22 22 23 18 22 101 16 29 74 35 87 39 21 27 14 27 13 14
28 14 14 15 11 14 65 10 18 47 22 56 26 13 17 9 17 8 9
43 15 12 11
48 17 13 12
7 10 22 10 10 49 9 28 12 35 18 6 13 12 16 8 8
10 15 33
8
9
12 52 16 20 46 23 53 21 15 14 12 15 5 6
13 58 18 22 51 26 59 24 17 15 13 17 6 7
" No significant differences between the groups.
I5 I5 73 13 42 18 52 27 9 19 18 24 12 12
251
DYSTONIC TICS IN TOURETTE'S SYNDROME TABLE 5. History and associated disorders in TS patients
TABLE 3. Complex motor tics in TS patients TS Patients With dystonic tics"
Total
TS Patients
With clonic tics"
Tics
n
%
n
%
n
%
Kicking Biting Jumping Stamping Joint-popping Tapping Scratching Grabbing own genitalia Hitting self Pain with motor tics
10 20 5 4 8 11 6
6 13 3 3 5 7 4
7 13 4 2 2 7 4
8 15 5 1 1 8 5
3 7 1 2 6 3 2
4 10 1 3 9 4 3
17 8
11 5
11 4
12 5
6 4
9 6
16
10
12
13
4
6
~~
~~
" No significant differences between groups. cavphonic tics and typical behavioral symptoms), do not produce persistently maintained abnormal posture, and are at least temporarily suppressible. All these features were present in our patients with dystonic tics. Some of the abnormal movements or postures, such as blepharospasm, bruxism, and torticollis, seen in our patients with dystonic tics are also typically seen in patients with idiopathic torsion dysTABLE 4. Vocailphonic tics in TS patients ~
TS Patients With dystonic tics"
Total Tics Throat clearing Grunting Hummigg Sniffing Sucking noise Squeaking Barking ClicWclacking Screaming Palilalia Echolalia Spitting Whistling Coprolalia Copropraxia Echopraxia Spasmodic dysphonia
With clonic tics"
n
%
n
%
n
%
93 78 23 78 7 22 18 15 8 4 42 12 8 57 24
51 48 15 41 3 15 11 11 6 3 25 9 6 29 12
57 54 17 46 3 17 12 12 7 3 28
11
7 33 13 12
42 30 7 37 4 7 7 4 2 1 17 3 2 28 12 5
63 45 10 55 6 10 10 6 3
16
60 50 15 50 5 14 12 10 5 3 27 8 5 37 I5 10
2
1
2
3
With dystonic tics
Total
With clonic tics
Variable
n
%
n
%
n
%
Birth complications Congenital defects Headneck trauma"
18 19
11 12
8 9
9 10
8 8
12 12
16 19 55 13
10 12 35 8
13 17 37 8
14 19 41 9
3 2 18 5
4 3 27 7
77
49
45
50
32
48
ADD^
OC traits Depression Sleep abnormalities
ADD, attention deficit disorder; OC, obsessive-compulsive. " p < 0.05. p < 0.005.
tonia, while others, such as oculogyric deviations, are more commonly associated with secondary dystonias. Electrophysiologic recordings in some of our patients showed that dystonic tics had a duration >300 ms, usually 1-2 s, whereas clonic tics were more jerk-like, lasting
0 1991 Movement Disorder Society
Dystonic Tics in Patients with Tourette’s Syndrome Joseph Jankovic and Lael Stone Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas, U.S.A.
Summary: Dystonic tics, in contrast to clonic tics, are relatively slow and temporarily sustained twisting, pulling, or squeezing movements producing briefly maintained abnormal postures. Because dystonic tics often present diagnostic difficulties, we studied these motor tics in 156 patients with Tourette’s syndrome (TS). In addition to clonic motor and vocallphonic tics, seen in all patients, 89 (57%) of them exhibited one or more dystonic tics. The most common dystonic tics were oculogyric deviations (43 patients), blepharospasm (23), and dystonic neck movements (1 1). Blinking (1 10) and facial twitching (86) were the most common clonic tics. Except for possibly a greater familial occurrence, a higher frequency of previous head trauma, and associated attention deficit disorder, the group of patients with dystonic tics did not significantly differ in any of the major clinical variables from those TS patients without dystonic tics. We conclude that dystonic tics are typical motor manifestations of TS and that patients with these motor tics are not different from those with more typical clonic tics. Key Words: Dystonic tics-Clonic ticsTourette’s syndrome-Head trauma-Attention deficit disorder.
tonic tics. To determine whether patients with dystonic tics represent a distinct “disease entity” (2), and are hence pathogenically different from those with clonic tics, we studied the clinical correlates of the two types of tics in 156 TS patients.
Because of the variable expression of Tourette’s syndrome (TS), patients with this movement and behavioral disorder are often misdiagnosed. In order to improve the diagnostic criteria for TS, an attempt has been made to better characterize the different motor manifestations (1). Motor and vocal/ phonic tics have been categorized as either simple or complex. Simple motor tics are further subdivided into clonic or dystonic. While clonic tics, particularly when associated with involuntary noises, are usually appropriately diagnosed, dystonic tics are often not recognized as part of the spectrum of TS. When the expression of TS is dominated by dystonic tics, the disorder is sometimes wrongly diagnosed as dystonia (2). Dystonia, in contrast to dystonic tics, is a chronically repetitive, patterned, and sustained movement, often producing a persistently abnormal posture (3). Furthermore, movements and postures associated with idiopathic or secondary dystonias are less suppressible than dys-
PATIENTS AND METHODS All 185 medical records of patients with tics evaluated at the Baylor College of Medicine Movement Disorders Clinic between 1982 and 1989 were reviewed. During the initial visit, the patients, relatives, and other informants were interviewed about the distribution and character of tics present currently and in the past. For the purposes of this retrospective survey, dystonic tics were defined as temporarily sustained abnormal movements or postures occurring out of a background of normal motor activity. Because tics are often suppressed during examination (and videotaping), we relied on descriptions of the various tics provided by patients, parents, and other observers. To elicit an accurate history of tics during the course of TS, the patients were asked whether they experienced jerk-like (clonic) or sustained (dystonic) contractions of mus-
Address correspondence and reprint requests to Dr. J. Jankovic at Department of Neurology, Baylor College of Medicine, 6550 Fannin #1801, Houston, TX 77030, U.S.A.
248
DYSTONIC TICS IN TOURETTES SYNDROME cles. We were particularly interested in obtaining detailed descriptions of dystonic tics; examples, such as blepharospasm, oculogyric deviations, and bruxism, were described for each anatomic region. The dystonic tics were witnessed in most patients at the time of examination, and in some they were videotaped and recorded by surface EMG (Figs. 1, 2). In addition to obtaining data about the motor and vocaVphonic tics, we collected information about prior head and neck trauma, behavioral problems, such as obsessive-compulsive traits, attention deficit, and other psychologic or psychiatric manifestations. We excluded seven patients who had the combination of tics (clonic and dystonic) and coexistent persistent dystonia (torsion dystonia). These patients, in addition to exhibiting clonic and dystonic tics as defined above, had a persistent, chronic, patterned dystonic movement and abnormal posture, always present in the same distribution and involving the same group of muscles (e.g., torticollis). The DSM-IIIR criteria for TS were met by 156 patients, who constitute the study population (4). The patients were divided into two groups: TS patients with clonic and dystonic tics (dystonic group) and TS patients with clonic tics but without dystonic tics (clonic group). The clinical and demographic data were entered into a relational data base, and the two groups were analyzed with respect to major clinical variables. Pearson's chisquare method was used to test for differences between the groups; p < 0.05 was considered suggestive of differences, whereas p < 0.005 was considered statistically significant when corrected for multiple comparisons.
249
RESULTS There were 89 patients who had the combination of clonic and dystonic tics (dystonic group) and 67 who had only clonic tics (clonic group). There was no difference between the two groups in any of the demographic variables (Table 1). By definition, dystonic tics, such as blepharospasm, ocular deviations, and bruxism were noted only in the dystonic group. There was no difference in the anatomic distribution of tics between dystonic and clonic groups (Tables 2-4). Patients with dystonic tics had a higher prevalence of attention deficit disorder (ADD) and a greater frequency of headheck trauma (Table 5). This difference remained significant even when adjusted for multiple comparisons. When compared with patients with only clonic tics, TS patients with dystonic tics seemed more likely to have parents and/or paternal relatives with tics; however, this difference was not statistically significant when adjusted for multiple comparisons (Table 6). DISCUSSION This study examines the possibility that TS patients with dystonic tics differ in some way from other TS patients. Dystonic tics were defined as temporarily sustained abnormal movements or postures, often with abrupt onset, occurring out of a background of normal motor activity. We recognize the limitations of this retrospective study, particularly the difficulties in ascertaining symptoms. We feel, however, that the clinical data was collected accurately and without bias regarding any possible differences between clinical correlates of clonic versus dystonic tics.
FIG. 1. Surface EMG recording from pectoralis in a 50-year-old man with clonic and dystonic tics of the right shoulder. The short ( I s) bursts are associated with prolonged dystonic movements of the right shoulder.
500 ms
Movement Disorders, Vol. 6, N o . 3, 1991
J . JANKOVIC AND L . STONE
250
111
I
FIG. 2. Surface EMG recording from left coracobrachialis (top tracing) and left deltoid (bottom tracing) in an 18-year-old woman with TS manifested chiefly by involuntary loud vocalizations, abducting (dystonic) movements, and jerk-like (clonic) rotatory movements of the left shoulder.
500 ms
All our patients with dystonic tics also had clonic motor and vocal/phonic tics. We found that the 89 patients with dystonic tics were virtually indistinguishable from the 67 TS patients who had only clonic tics. However, patients with dystonic tics had suffered more headheck trauma and had a higher frequency of associated ADD than the other TS patients. Although statistically significant, the differences are minor and probably do not justify a separation of patients with dystonic tics into a distinct "disease entity" (2). Although our population is selected and therefore cannot be considered representative of all TS patients, their demographic and clinical characteristics are similar to those reported in other large clinic- (5-8) and population-based series (9). We exTABLE 1. Demographic features of TS patients TS Patients
Total
With dystonic tics" (n = 89)
With clonic tics" (n = 67)
Variable
n
%
n
%
n
%
Males Right-handed Left-handed Ambidextrous Mean age at initial visit (yr4 Mean age at onset (yrs)
127 137 16 3
81 88 10 2
73 75 10 2
82 84 11 2
54 62 6 1
80 93 7 1
20
20
6.7
6.3
" No significant difference between the groups.
Movement Disorders, Vol. 6 , No. 3, 1991
20 7.7
cluded all patients with atypical features and seven patients who had the coexistence of tics and persistent dystonia. An association between tics and dystonia has been noted previously (10-12). While dystonic tics are often misdiagnosed as dystonia, they should be easily differentiated if they are accompanied by other features of TS (clonic motor and voTABLE 2. Distribution of tics in TS patients TS patients With dystonic tics"
Total
With clonic tics"
Distribution
n
%
n
%
n
%
Blinking Blepharospasm Ocular deviations Forehead Nose Lips Mouth Tongue Face Bruxisrn Jaw opening Head jerk Neck Shoulder Arm Hand Leg Foot Trunk Abdomen Pelvic thrust
110 23
70 15
68 23
76 26
42 -
63 -
43 22 22 23 18 22 101 16 29 74 35 87 39 21 27 14 27 13 14
28 14 14 15 11 14 65 10 18 47 22 56 26 13 17 9 17 8 9
43 15 12 11
48 17 13 12
7 10 22 10 10 49 9 28 12 35 18 6 13 12 16 8 8
10 15 33
8
9
12 52 16 20 46 23 53 21 15 14 12 15 5 6
13 58 18 22 51 26 59 24 17 15 13 17 6 7
" No significant differences between the groups.
I5 I5 73 13 42 18 52 27 9 19 18 24 12 12
251
DYSTONIC TICS IN TOURETTE'S SYNDROME TABLE 5. History and associated disorders in TS patients
TABLE 3. Complex motor tics in TS patients TS Patients With dystonic tics"
Total
TS Patients
With clonic tics"
Tics
n
%
n
%
n
%
Kicking Biting Jumping Stamping Joint-popping Tapping Scratching Grabbing own genitalia Hitting self Pain with motor tics
10 20 5 4 8 11 6
6 13 3 3 5 7 4
7 13 4 2 2 7 4
8 15 5 1 1 8 5
3 7 1 2 6 3 2
4 10 1 3 9 4 3
17 8
11 5
11 4
12 5
6 4
9 6
16
10
12
13
4
6
~~
~~
" No significant differences between groups. cavphonic tics and typical behavioral symptoms), do not produce persistently maintained abnormal posture, and are at least temporarily suppressible. All these features were present in our patients with dystonic tics. Some of the abnormal movements or postures, such as blepharospasm, bruxism, and torticollis, seen in our patients with dystonic tics are also typically seen in patients with idiopathic torsion dysTABLE 4. Vocailphonic tics in TS patients ~
TS Patients With dystonic tics"
Total Tics Throat clearing Grunting Hummigg Sniffing Sucking noise Squeaking Barking ClicWclacking Screaming Palilalia Echolalia Spitting Whistling Coprolalia Copropraxia Echopraxia Spasmodic dysphonia
With clonic tics"
n
%
n
%
n
%
93 78 23 78 7 22 18 15 8 4 42 12 8 57 24
51 48 15 41 3 15 11 11 6 3 25 9 6 29 12
57 54 17 46 3 17 12 12 7 3 28
11
7 33 13 12
42 30 7 37 4 7 7 4 2 1 17 3 2 28 12 5
63 45 10 55 6 10 10 6 3
16
60 50 15 50 5 14 12 10 5 3 27 8 5 37 I5 10
2
1
2
3
With dystonic tics
Total
With clonic tics
Variable
n
%
n
%
n
%
Birth complications Congenital defects Headneck trauma"
18 19
11 12
8 9
9 10
8 8
12 12
16 19 55 13
10 12 35 8
13 17 37 8
14 19 41 9
3 2 18 5
4 3 27 7
77
49
45
50
32
48
ADD^
OC traits Depression Sleep abnormalities
ADD, attention deficit disorder; OC, obsessive-compulsive. " p < 0.05. p < 0.005.
tonia, while others, such as oculogyric deviations, are more commonly associated with secondary dystonias. Electrophysiologic recordings in some of our patients showed that dystonic tics had a duration >300 ms, usually 1-2 s, whereas clonic tics were more jerk-like, lasting
