Perceptual and Motor Skills, 1979,48, 1003-1007. @ Perceptual and Motor Skills 1979
MMPI CHARACTERISTICS ASSOCIATED W I T H CEREBRAL PALSY AND DYSTONIA MUSCULORUM DEFORMANS1 ANNE M. IMPERIO Fordham University at Lincoln C e ~ t e r THOMAS F. CULLINAN Child Development Center, Bronx
MANUEL RIKLAN St. Barnabas Horpital, Bronx
Summary.-The origins of dystonia musculorum deformans are now considered to be organic. However, misdiagnosis of dystonia as a functional psychiatric disorder-usually conversion reaction-has persisted. The present study describes personality traits as measured by the Minnesota Multiphasic Personality Invenrory in 30 persons with dystonia and in a control group of 37 persons with cerebral palsy. The data, examined by diagnosis, level of disabiliry, and sex, showed no differences for diagnostic groups or levels of disability. Males scored in the direction of greater psychopathology than did females. The male dystonics showed the highest elevations of MMPI scales of all the groups. Although only one person with dystonia musculorum deformans and none with cerebral palsy produced the profile usually associated with conversion reaction, 36% of all profiles showed two scales above a T score of 70. This finding suggested char young adults with a physically disabling disease may be at higher risk for developing maladaptive personality traits.
Dystonia musculorum deformans is a rare genetic neurological condition which is manifested by co-contraction of antagonist muscle systems. The onset typically is insidious, and symptoms generally appear between 7 and 12 yr. of age. Psychological studies of dystonics have been almost entirely limited to their cognitive functioning. Riklan, Cullinan, and Cooper (1976) reported that individuals of Jewish ancestry with no family history of the disease and onset occurring between 8 and 13 yr. of age scored significantly higher on the WISC or WAIS than did other dystonics not meering these criteria. An earlier report of Eldridge, Harlan, Cooper, and Riklan (1970) suggested that the gene which produced the condition may also enhance intelligence, as dystonics scored significantly higher on the WISC or WAIS than did a control group of siblings. A brief comment was made to the effect that examination of projective material in a group of dystonics did not indicate a pattern of either thought or behavioral disorder. The present study is the first formally to invescigate personality patterns in dystonia. The reasons for the study are threefold. First, we wished to expand on previous psychological investigations into the condition. Second, 'This research was supported in part by the United Cerebral Palsy Foundation. Requests for r e ~ r i n t sshould be sent to Anne M. I m ~ e r i o .Counselina Center. Fordham Universitv at ~ i n c o l nCenter, New York, New York i0023.
-
1004
A. M. IMPERIO, ET AL.
since a high percentage of dystonics are first misdiagnosed as suffering from conversion reaction (Cooper, Cullinan, & Riklan, 1976) we wished to find out whether the pactern common to those with conversion reaction (High. on Scales Hysteria and Hypochondriasis, Low on Depression) would be found in dystonics. Third, we explored the question that an illness such as dystonia, which is manifested after a period of normal functioning, would be more psychologically disabling as measured by the MMPI than would a condition, such as cerebral palsy, which was present from birch. W e expected that anxiety in the dystonics due to preoccupation with lost physical abilicies and worry over further progression of symptoms would result in greater psychological abnormality than would be seen in the cerebral palsied. Also, since many cases of dystonia first appear around puberty, we expected Scale 5 MasculinityFemininity to be aberrant in dystonics as compared to cerebral palsied. There have been previous MMPI studies of the cerebral palsied. Linde and Patterson (1958) reported chat MMPI scores obtained from a sample of cerebral palsied persons were significanrly higher on all except the Depression scale than normative data. Similarly, Muthard (1965) found thac cerebral palsied college students scored significantly higher on nearly all the MMPI scales than normative subjects. Sex differences were found, in thac cerebral palsied males scored significantly higher than females on. the Depression, Psychaesthenia, and Schizophrenia scales. Female cerebral palsied persons obtained a low Mf score which suggested that they tend to respond to their handicap with passivity. These were no significant differences on the MMPI scores according to level of disability. It was concluded that such college students were more in need of counseling than non-handicapped students.
The participants were 30 persons with dystonia musculorum deformans and 37 persons with cerebral palsy who were followed at the Institute of Neuroscience at St. Barnabas Hospital in the Bronx, New York. There were 16 males and 21 females in the latter group, and 12 males and 18 females in the former group. The mean ages for the palsied and dystonic groups were 27.7 and 27.1 yr., respectively (us = 8.49 and 7.73). The mean IQ, as determined by the WISC or WAIS, was 106.5 for the cerebral palsied persons and 111.5 for the dystonic subjects (US = 8.91 and 13.15). The mean years of education for the cerebral palsied subjects was 13.0 and the dystonic persons was 14.4, with both groups averaging one to two years of college (range 7 and 10 to MA). The major point of difference in the two groups, due to the nature of their illness, was in their past medical history. The groups were equated for level of disability on the familiar four-point rating scale with 1 = no impairment and 4 = marked impairment. The cerebral palsied group received a
mean rating of 2.36 and the dystonic group a rating of 2.41, with no significant difference between the means. Within the cerebral palsied group, 21 of the 37 persons had undergone chronic cerebellar stimulation (CCS), while 23 of the 30 dystonic persons had previous cryothalamectomies. Riklan, Cullinan, Shulrnan, and Cooper ( 1976) reported no adverse or differential effects in prepost studies of either cognition, emotional behavior, or perceptual function following either procedure in similar populations. All individuals in the study completed the MMPI. Those persons wich an inability to mark the answer sheet read the questions to themselves and indicated to the examiner either "yes" or "no" to each question.
RESULTSAND DISCUSSION The mean MMPI scores obtained for the validity and clinical scales for the two groups are presented in Table 1. Two-way analysis of variance for diagnosis and sex showed no significant differences between diagnostic groups on any of the scales. However, the dystonic group scored higher, or in the direction of greater pathology, than did the cerebral palsied group on each clinical scale. The males in both groups obtained consistently higher scores than did the females. The dystonic males scored significantly higher (tZti= 2.26, p < .05) than did the cerebral palsied males on the Masculinity-Femininity scale, and as a group obtained mean T scores above 70 on both the MasculinityFemininity and Schizophrenia scales. When compared with dystonic females, dystonic males scored significantly higher on both the Psychaesthenia ( t 2 s = 2.84, p < .01) and Schizophrenia (tZs = 2.91, p < .01) scales. One dystonic produced a profile usually associated wich conversion reaction. However, this person also had a strong family history of dystonia which tended to discount psychogenic origin of the disease. N o other profile in either group suggested this pattern. Using a criterion of two scales with scores above T score of 70 as an index of abnormality for an individual profile, 36% of all subjects showed abnormality. Thirty percent of the cerebral palsied and 43% of the dystonic groups met chis criterion. The 67 subjects were divided into three groups according to level of disability, mild (12 = 22), moderate ( n = 2 3 ) , and severe (qz = 22). A series of t tests for each disability group showed no significant differences on any scale. There was a trend, however, for the mildly impaired group to show greater abnormality relative to the severely impaired group. Nine of the 10 clinical scales had mean T scores in the direction of greater pathology in the mildly impaired group. The MMPI profiles of the two groups are similar and are considered to reflect general personality traits found in young, physically handicapped individuals of at least normal intelligence. Although dystonia musculorum defor-
looG
A. M. IMPERIO, ET AL. TABLE 1 MEANMMPI SCORES AS A FUNCTlON OF DIAGNOSIS AND SEX
Scale
Palsied
Dystonia Male
n Lie M c
Palsied Female
Dystonia Male Female
37 50.4 8.56
F, Validity M c
57.7 8.83
K, Test-taking Attitude M 53.6 u 8.80
Hypochondriasis M c
Depression M c
Hysteria M c
56.9 9.52 57.4 13.00 60.4 9.68
Psychopathic Deviate M
59.3
Masculinity-Femininity M 54.4 u
10.75
Paranoia M c
56.9 8.33
Psychaesthenia M u
Schizophrenia M u
57.16 11.91 64.20 13.22
Mania M c
61.5 12.15
Social Introversion M u
53.1 10.92
mans is often misdiagnosed as conversion reaction, the profiles of the dystonic persons individually and collectively (except for one case) did not exhibit the MMPI profile usually associated with conversion reaction. Thus the frequent misdiagnosis of dystonia as conversion reaction cannot be attributed to the fact that dystonics exhibit traits associated with either hysterical or somaticizing behaviors. Males and females in both groups deviated from the norm on the Mascu-
MMPI: CEREBRAL PALSY, DYSTONIA MUSCULORUM DEFORMANS 1007
linity-Femininity scale. However, our expectation that the dystonic persons would exhibit greater difficulty in identifying with sex-role behaviors and interests was supported for the dystonic males who scored significantly higher than cerebral palsied males on the Masculinity-Femininity scale. For dystonic males the later onset and progressive nature of their symptoms may increase somewhat their difficulties in sex-role identification. Past report of a depressed Masculinity-Femininity score for cerebral palsied females was supported and was also found for dystonic females. These results suggested that females may adapt to their physical handicap by passivity, compliance, and close adherence to a traditional female role. The dystonic males also were significantly more anxious and socially alienated than the dystonic females. The general treatment of handicapped persons in our society is less culturally congruent for males than for females and hence, may create particular difficulties for males. Present resdts suggest that, in general, both males and females who suffer from cerebral palsy, dystonia musculorurn deformans, or another similarly disabling condition might benefit from counseling or psychotherapy. Particular attention should be paid to sexual behavior and sex-role adaptation. '
REFERENCES COOPER,I. S., CULLWAN,T., & RIKLAN,M. The natural history of dystonia. In R. Eldridge & S. Fahn (Eds.), Advances in neurology. Vol. 14. New York: Raven Press, 1976. Pp. 157-170. ELDRIDGE,R., HARLAN, A., COOPER, I. S., & RIKLAN,M. Superior intelligence in recessively inherited torsion dystonia. Lancet, 1970, 1, 65-67. LINDE, T., & PATTERSON, C. H. The MMPI in cerebral palsy. Journal of ConruLing Psychology, 1958, 22, 210-212. MUTHARD,J. E. MMPI findings for cerebral palsied college smdents. Journal o f Consulting Psychology, 1965, 29, 599. RLKLAN,M., CULLINAN,T., & COOPER,I. S. Psychological studies in dystonia musculorum deformans. In R. Eldridge & S. Fahn (Eds.), Advances in neurology. Vol. 14. New York: Raven, 1976. Pp. 189-200. RIKLAN,M., CULLINAN,T., SHULMAN,M., & COOPER,I. S. A psychometric study of chronic cerebellar stimulation in man. Biological Psychiatry, 1976, 11, 543-574. Accepted April 13, 1979.
MMPI CHARACTERISTICS ASSOCIATED W I T H CEREBRAL PALSY AND DYSTONIA MUSCULORUM DEFORMANS1 ANNE M. IMPERIO Fordham University at Lincoln C e ~ t e r THOMAS F. CULLINAN Child Development Center, Bronx
MANUEL RIKLAN St. Barnabas Horpital, Bronx
Summary.-The origins of dystonia musculorum deformans are now considered to be organic. However, misdiagnosis of dystonia as a functional psychiatric disorder-usually conversion reaction-has persisted. The present study describes personality traits as measured by the Minnesota Multiphasic Personality Invenrory in 30 persons with dystonia and in a control group of 37 persons with cerebral palsy. The data, examined by diagnosis, level of disabiliry, and sex, showed no differences for diagnostic groups or levels of disability. Males scored in the direction of greater psychopathology than did females. The male dystonics showed the highest elevations of MMPI scales of all the groups. Although only one person with dystonia musculorum deformans and none with cerebral palsy produced the profile usually associated with conversion reaction, 36% of all profiles showed two scales above a T score of 70. This finding suggested char young adults with a physically disabling disease may be at higher risk for developing maladaptive personality traits.
Dystonia musculorum deformans is a rare genetic neurological condition which is manifested by co-contraction of antagonist muscle systems. The onset typically is insidious, and symptoms generally appear between 7 and 12 yr. of age. Psychological studies of dystonics have been almost entirely limited to their cognitive functioning. Riklan, Cullinan, and Cooper (1976) reported that individuals of Jewish ancestry with no family history of the disease and onset occurring between 8 and 13 yr. of age scored significantly higher on the WISC or WAIS than did other dystonics not meering these criteria. An earlier report of Eldridge, Harlan, Cooper, and Riklan (1970) suggested that the gene which produced the condition may also enhance intelligence, as dystonics scored significantly higher on the WISC or WAIS than did a control group of siblings. A brief comment was made to the effect that examination of projective material in a group of dystonics did not indicate a pattern of either thought or behavioral disorder. The present study is the first formally to invescigate personality patterns in dystonia. The reasons for the study are threefold. First, we wished to expand on previous psychological investigations into the condition. Second, 'This research was supported in part by the United Cerebral Palsy Foundation. Requests for r e ~ r i n t sshould be sent to Anne M. I m ~ e r i o .Counselina Center. Fordham Universitv at ~ i n c o l nCenter, New York, New York i0023.
-
1004
A. M. IMPERIO, ET AL.
since a high percentage of dystonics are first misdiagnosed as suffering from conversion reaction (Cooper, Cullinan, & Riklan, 1976) we wished to find out whether the pactern common to those with conversion reaction (High. on Scales Hysteria and Hypochondriasis, Low on Depression) would be found in dystonics. Third, we explored the question that an illness such as dystonia, which is manifested after a period of normal functioning, would be more psychologically disabling as measured by the MMPI than would a condition, such as cerebral palsy, which was present from birch. W e expected that anxiety in the dystonics due to preoccupation with lost physical abilicies and worry over further progression of symptoms would result in greater psychological abnormality than would be seen in the cerebral palsied. Also, since many cases of dystonia first appear around puberty, we expected Scale 5 MasculinityFemininity to be aberrant in dystonics as compared to cerebral palsied. There have been previous MMPI studies of the cerebral palsied. Linde and Patterson (1958) reported chat MMPI scores obtained from a sample of cerebral palsied persons were significanrly higher on all except the Depression scale than normative data. Similarly, Muthard (1965) found thac cerebral palsied college students scored significantly higher on nearly all the MMPI scales than normative subjects. Sex differences were found, in thac cerebral palsied males scored significantly higher than females on. the Depression, Psychaesthenia, and Schizophrenia scales. Female cerebral palsied persons obtained a low Mf score which suggested that they tend to respond to their handicap with passivity. These were no significant differences on the MMPI scores according to level of disability. It was concluded that such college students were more in need of counseling than non-handicapped students.
The participants were 30 persons with dystonia musculorum deformans and 37 persons with cerebral palsy who were followed at the Institute of Neuroscience at St. Barnabas Hospital in the Bronx, New York. There were 16 males and 21 females in the latter group, and 12 males and 18 females in the former group. The mean ages for the palsied and dystonic groups were 27.7 and 27.1 yr., respectively (us = 8.49 and 7.73). The mean IQ, as determined by the WISC or WAIS, was 106.5 for the cerebral palsied persons and 111.5 for the dystonic subjects (US = 8.91 and 13.15). The mean years of education for the cerebral palsied subjects was 13.0 and the dystonic persons was 14.4, with both groups averaging one to two years of college (range 7 and 10 to MA). The major point of difference in the two groups, due to the nature of their illness, was in their past medical history. The groups were equated for level of disability on the familiar four-point rating scale with 1 = no impairment and 4 = marked impairment. The cerebral palsied group received a
mean rating of 2.36 and the dystonic group a rating of 2.41, with no significant difference between the means. Within the cerebral palsied group, 21 of the 37 persons had undergone chronic cerebellar stimulation (CCS), while 23 of the 30 dystonic persons had previous cryothalamectomies. Riklan, Cullinan, Shulrnan, and Cooper ( 1976) reported no adverse or differential effects in prepost studies of either cognition, emotional behavior, or perceptual function following either procedure in similar populations. All individuals in the study completed the MMPI. Those persons wich an inability to mark the answer sheet read the questions to themselves and indicated to the examiner either "yes" or "no" to each question.
RESULTSAND DISCUSSION The mean MMPI scores obtained for the validity and clinical scales for the two groups are presented in Table 1. Two-way analysis of variance for diagnosis and sex showed no significant differences between diagnostic groups on any of the scales. However, the dystonic group scored higher, or in the direction of greater pathology, than did the cerebral palsied group on each clinical scale. The males in both groups obtained consistently higher scores than did the females. The dystonic males scored significantly higher (tZti= 2.26, p < .05) than did the cerebral palsied males on the Masculinity-Femininity scale, and as a group obtained mean T scores above 70 on both the MasculinityFemininity and Schizophrenia scales. When compared with dystonic females, dystonic males scored significantly higher on both the Psychaesthenia ( t 2 s = 2.84, p < .01) and Schizophrenia (tZs = 2.91, p < .01) scales. One dystonic produced a profile usually associated wich conversion reaction. However, this person also had a strong family history of dystonia which tended to discount psychogenic origin of the disease. N o other profile in either group suggested this pattern. Using a criterion of two scales with scores above T score of 70 as an index of abnormality for an individual profile, 36% of all subjects showed abnormality. Thirty percent of the cerebral palsied and 43% of the dystonic groups met chis criterion. The 67 subjects were divided into three groups according to level of disability, mild (12 = 22), moderate ( n = 2 3 ) , and severe (qz = 22). A series of t tests for each disability group showed no significant differences on any scale. There was a trend, however, for the mildly impaired group to show greater abnormality relative to the severely impaired group. Nine of the 10 clinical scales had mean T scores in the direction of greater pathology in the mildly impaired group. The MMPI profiles of the two groups are similar and are considered to reflect general personality traits found in young, physically handicapped individuals of at least normal intelligence. Although dystonia musculorum defor-
looG
A. M. IMPERIO, ET AL. TABLE 1 MEANMMPI SCORES AS A FUNCTlON OF DIAGNOSIS AND SEX
Scale
Palsied
Dystonia Male
n Lie M c
Palsied Female
Dystonia Male Female
37 50.4 8.56
F, Validity M c
57.7 8.83
K, Test-taking Attitude M 53.6 u 8.80
Hypochondriasis M c
Depression M c
Hysteria M c
56.9 9.52 57.4 13.00 60.4 9.68
Psychopathic Deviate M
59.3
Masculinity-Femininity M 54.4 u
10.75
Paranoia M c
56.9 8.33
Psychaesthenia M u
Schizophrenia M u
57.16 11.91 64.20 13.22
Mania M c
61.5 12.15
Social Introversion M u
53.1 10.92
mans is often misdiagnosed as conversion reaction, the profiles of the dystonic persons individually and collectively (except for one case) did not exhibit the MMPI profile usually associated with conversion reaction. Thus the frequent misdiagnosis of dystonia as conversion reaction cannot be attributed to the fact that dystonics exhibit traits associated with either hysterical or somaticizing behaviors. Males and females in both groups deviated from the norm on the Mascu-
MMPI: CEREBRAL PALSY, DYSTONIA MUSCULORUM DEFORMANS 1007
linity-Femininity scale. However, our expectation that the dystonic persons would exhibit greater difficulty in identifying with sex-role behaviors and interests was supported for the dystonic males who scored significantly higher than cerebral palsied males on the Masculinity-Femininity scale. For dystonic males the later onset and progressive nature of their symptoms may increase somewhat their difficulties in sex-role identification. Past report of a depressed Masculinity-Femininity score for cerebral palsied females was supported and was also found for dystonic females. These results suggested that females may adapt to their physical handicap by passivity, compliance, and close adherence to a traditional female role. The dystonic males also were significantly more anxious and socially alienated than the dystonic females. The general treatment of handicapped persons in our society is less culturally congruent for males than for females and hence, may create particular difficulties for males. Present resdts suggest that, in general, both males and females who suffer from cerebral palsy, dystonia musculorurn deformans, or another similarly disabling condition might benefit from counseling or psychotherapy. Particular attention should be paid to sexual behavior and sex-role adaptation. '
REFERENCES COOPER,I. S., CULLWAN,T., & RIKLAN,M. The natural history of dystonia. In R. Eldridge & S. Fahn (Eds.), Advances in neurology. Vol. 14. New York: Raven Press, 1976. Pp. 157-170. ELDRIDGE,R., HARLAN, A., COOPER, I. S., & RIKLAN,M. Superior intelligence in recessively inherited torsion dystonia. Lancet, 1970, 1, 65-67. LINDE, T., & PATTERSON, C. H. The MMPI in cerebral palsy. Journal of ConruLing Psychology, 1958, 22, 210-212. MUTHARD,J. E. MMPI findings for cerebral palsied college smdents. Journal o f Consulting Psychology, 1965, 29, 599. RLKLAN,M., CULLINAN,T., & COOPER,I. S. Psychological studies in dystonia musculorum deformans. In R. Eldridge & S. Fahn (Eds.), Advances in neurology. Vol. 14. New York: Raven, 1976. Pp. 189-200. RIKLAN,M., CULLINAN,T., SHULMAN,M., & COOPER,I. S. A psychometric study of chronic cerebellar stimulation in man. Biological Psychiatry, 1976, 11, 543-574. Accepted April 13, 1979.
