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Familial psychopathology in delusional disorder
Familial Psychopathology in Delusional Disorder By George Winokur The family background of patients with simple delusional disorder was examined. Different groups of controls were used. Patients with simple delusional disorder were likely to have family members who were considered suspicious, secretive, jealous, or showed delusions or some kind of paranord disorder more frequently than the controls. Neither paranoid schizophrenics nor other patients with major psychiatric illnesses are likely to have family members that show the kind of characteristics that are usually seen in paranoid personalities.
I
N A PREVIOUS paper, we described 29 patients with delusional disorder (paranoia). Delusional disorder was found to be uncommon and quite likely to have been clinically diagnosed as paranoid schizophrenia. It was more frequently seen in men than women. The illness bore no relationship to I.Q. It did not incapacitate a person for working but it did create significant interpersonal difficulties. Familially it bore no relationship to affective disorder.’ Clinically the major symptoms were delusions of reference, jealousy, and persecution. Hypochondriasis and grandeur were seen but infrequently. Sexual problems were common. The patients were quite overtalkative and circumstantial when seen in the hospital. No patients met criteria for either depression or mania. There are some significant problems in terminology. The definition of the patients described above explicitly excluded any patients who manifested hallucinations. Kendler* has proposed that patients meeting the diagnosis for the nonhallucinatory form of delusional disorder be called “simple delusional disorder (SDDY and those others be called “hallucinatory delusional disorder (HDD).” Together these forms could be termed “delusional disorder (DD).” In conformance with these definitions, we will discuss familial psychopathology only in simple delusional disorder. Is it worth while to separate the hallucinatory from the nonhallucinatory form of delusional disorder? At this point it seems reasonable to consider them separately. Kendler et al’ has made the strongest case for looking at the two forms together. In a study of the relationship between paranoid psychosis (delusional disorder) and the schizophrenia spectrum disorders using the Danish adoption study sample, they have combined the hallucinatory and nonhallucinatory forms. When this is done, they find that familial delusional disorder is not related to schizophrenia in the adoptees. All of the cases of delusional disorder were seen in other control groups besides the biological relatives of the schizophrenic adoptee probands. The rationale for including both the hallucinatory and nonhallucinatory forms together is based on the statement that “three genetic studies have shown that the familial loading for schizophrenia is just as low in the paranoid psychotic patients with hallucinations as it is in the paranoid psychotic patients without hallucinations.” A perusal of the three studies that are cited show this to be a questionable conclusion. Only Debray separates the patients into hallucinatory and nonhallu-
From the Depariment of Psychiatry University of Iowa College of Medicine. Iowa City. Address reprint requests to George Winokur, MD. Department of Psychiatry, University of Iowa College of Medicine, Iowa City, IA 52242. (3 1985 by Grune & Stratton, Inc. 0010-440X/85/2603-0002$3.00/0 Comprehensive Psychiatry, Vol. 26, No. 3, (May/June)
1985
241
242
GEORGE WINOKUR
cinatory forms. In his study the hallucinatory forms are less likely to have a hereditary loading than are the nonhallucinatory forms. This could easily be interpreted as against the hypothesis that nonhallucinatory delusion disorder (SDD) is different from schizophrenia. However, the data are not definitive because in this paper Debray did not separate three diagnostic entities from each other in the families. Thus, he lumped at schizophrenia, chronic delusions, and personality disorders together (these combined constitute the familial loading). It is not possible to determine whether schizophrenia per se was more frequently seen in the nonhallucinatory form. Another study5 by Watt et al evaluated paranoid states of middle life. This study did not separate the groups with and without hallucinations. Further, Watt’s study contained only three patients (in a registry) who had a paranoid state. In one case, a patient with a paranoid state had a brother who had a paranoid illness with hallucinations. Whether the proband herself had hallucinations is not possible to tell. In any event, the numbers are small and it is not possible to determine whether hallucinations in paranoid patients breed together. However, there was a clear finding. Those patients who were considered as having paranoid states, i.e., those with delusional disorder with and without hallucinations, were less likely to have a family history of schizophrenia than those patients with schizophrenia. The third study that is relevant to a SDD-HDD separation is that of Kendler and Hays.6 They found 12 patients with delusional disorder and looked at the presence of schizophrenia in the families of these patients. They found only one person with schizophrenia in the families of delusional disorder patients. This was significantly less schizophrenia than was found in the families of schizophrenic patients. Seven of their 12 delusional disorder patients had hallucinations. If the one schizophrenic was seen in that particular group of patients, one out of seven would have a family history of schizophrenia. This would be about 15%. It is not possible from the paper to determine whether the one schizophrenic was in one of the families of delusional disorder patients with hallucinations or in one of the five remaining delusional disorder patients who had no hallucinations. If the former were true, however, there would be no significant difference in the proportion of families of the schizophrenics Y the hallucinating delusional disorder patients on the variable of the presence of familial schizophrenia. What one can say is that schizophrenia is less frequently seen in the family members of broadly defined delusional disorder patients and broadly defined delusional disorders are less frequently seen in the families of schizophrenics. Likewise, familial affective disorder does not seem to be related to delusional disorder. The value of separating HDD from SDD remains an open question. In order to determine whether there was a possible familial relationship of any sort in delusional disorder, we decided to look at more subtle kinds of findings, e.g., personality trait characteristics. These are the subject of this paper. MATERIAL
AND METHODS
Twenty-nine simple delusional disorder patients were selected according to the following criteria. All patients had to exhibit an unequivocal delusion that could have been present for any length of time. The delusions had to be possible, however implausible. As an example, a delusion of being controlled by radar that directed movements would not be acceptable. However, a delusion of infidelity would be quite in keeping with the definition.
DELUSIONAL
DISORDER-FAMILIAL
243
TRAITS
Exclusion criteria were the presence or suggestion of any hallucination at any time, bizarre or fantastic delusions at any time, evidence for an organic brain syndrome, illness beginning after age 60. meeting criteria for depression or mania, and inappropriate or markedly flattened affect. In a previous paper. the remainder of the methodology is given.’ ‘The 29 charts were reperused and specific attention was paid to the systematic family history that was recorded by the social workers. These systematic family histories were the result of interviews with multiple informants including family members and friends. They contained personality characteristics as well as evidence of major psychiatric disorders. Often the data contained information about the personalities of the paternal and maternal grandparents and the siblings of these grandparents. Likewise. the aunts and uncles of the proband as well as the cousins are noted. There was a formal outline for the presentation of the family history that the social worker followed. In addition to the social worker’\ material, the physician who saw the patient often added matenal on the family histories. Likewise. subsequent admissions contained new material added by social workers and physicians. ‘The family histories were similar regardless of the diagnosis. Thus, in one patient who had a diagnosla of general paresis, the following notes were made. Father-had temper tantrums and was deaf: mother--had “sick headaches”; sister-was hospitalized for hysteria; sister-had a near nervous breakdown; sister-may have had a nervous breakdown. Such a family history was obtained even though the patlenr had an illness that was known to be clearly organic and infectious. For comparison purposes 29 controls were drawn from the records of the University of Iowa P\ychiatric Hospitals. The controls were matched for age and sex and year of admission. If there were an! differences in the quality of workup over the years, the matching should control for that. As another control, patients who were admitted as part of a large study of schizophrenia. bipolar and unipolar illness (the Iowa 500) between the years 1934 and 1944 were investigated separately.. These patients were followed up after 20 to 30 years. The subjects were 29 patients who were originally diagnosed as having paranoid schizophrenia at the time of entry into the hospital and at follow-up continued to have that diagnosis using a prespecified set of criteria.” The criteria were used to separate paranoid versus nonparanoid schizophrenia. These criteria, specifically the ones for paranoid \chizophrenia, would include patients who met the prestated criteria for simple delusional disorder.
RESULTS Table 1 gives the frequency distribution of the clinical diagnoses between the control groups and the delusional disorder groups. As expected, most of the paranoid diagnoses occurred in the delusional disorder group. However, by chance the match turned up two control patients who overlapped the delusional disorder patients on the variable of diagnosis. One of these had an involutional paranoid state diagnosis, and the other had a paranoid state. These patients in fact would Table 1. Clinical Diagnosis in Delusional and Controls
Disorder
Patients
Delusional
N Diagnosis Affective disorder Schizophrenia Organic brain syndrome Drug abuse Hysteria Personality disorder Involutional paranoid state Paranoid state Paranoid psychosis Paranoia
Controls
Disorder
29 % 28 21 14
29 %
10 14 3 3 -
3 45 31 17
244
GEORGE WINOKUR
have met our criteria for simple delusional disorder. They were missed in the original evaluation because they did not contain the term “paranoia” in the hospital sign out diagnosis. All of the 29 delusional disorder patients had, at some point in their lives, been coded out as showing paranoia. An effort was made to evaluate the presence or absence of either remitting illness or chronic illness or both in the families of the two groups of patients, Presumably remitting illness would refer to some kind of affective illness and chronic illness would refer more likely to either paranoid symptomatology or schizophrenia. Table 2 presents those data. What is notable is that there is really very little difference between the controls and the patients with delusional disorders. Of course, chronic illness could refer either to chronic schizophrenia or chronic delusional disorder, so the resolving power of this dichotomy is not high. The major finding may be seen in Table 3 which-presents family history workups relevant to the following terms: suspiciousness, secretiveness, being delusional, or being paranoid. Clearly, more families of the delusional disorder patients (32%) showed these traits than control families. Of the nine families that had members that were called either suspicious, secretive, jealous, delusional, or paranoid, the following describes the quality of the material: (1) father feels cheated; maternal aunt is suspicious and chronically hospitalized; (2) mother is delusional about cancer; two sisters are suspicious; (3) father had a nervous breakdown and is considered a jealous person; (4) one brother is chronically delusional; one sister is paranoid schizophrenic; (5) sister is chronically paranoid, paternal grandmother is considered suspicious; (6) father lost money on business ventures and is considered secretive; paternal family is considered secretive; paternal great uncle is delusional; (7) brother is suspicious; (8) sister has paranoia; (9) half sister has involutional paranoid illness. Five of the nine families contained mutliple members who met these adjectival criteria. One of the controls had such a family member. In this case the index control had a hospital diagnosis of probable manic-depressive illness. His paternal uncle was diagnosed as having paranoid schizophrenia but probably had alcoholism with paranoid symptoms. As regards the two controls who fulfilled the criteria for delusional disorder, both had negative family histories for anything. Six patients had suffered from their delusional illness under a six-month period of time. Only one out of those 6 (17%) had a family history that contained suspiciousness, or secretiveness, or paranoid behavior, or delusions, or jealousy. This may be compared to the 8 out of the 23 (35%) that had a longer term illness. Of the 29 patients of the Iowa 500 control group, 10 met the criteria for delusional
Table 2. Major Psychiatric and/or
Disorders in First-Degree Extended Family
Relatives
Delusional N Remitting illness Chronic illness
Disorder
Controls
8 (2289%) 7 (24%)
29 8 (28%) 3 (10%)
DELUSIONAL
DISORDER-FAMILIAL
245
TRAITS
Table 3. Family History Traits of Suspiciousness, Jealousy, Secretiveness, Delusions, or Some Kind of Paranoid Disorder*
N (%) Positive for traits N (%) Negative for traits
Delusional Disorder
Controls
9 (32%) 20 (69%)
1 ( 3%) 28 (97%)
‘x2 = 7.73, d.f. 1, I’ < .005
disorder. The reason that they were not picked up in the original scanning of the delusional disorder patients is that at no point had they ever been coded out as having paranoia as a diagnosis. Of the 10 patients with delusional disorder, four had a hospital diagnosis of paranoid condition. In this group one person had family members with the traits that have been discussed above. Thus, a maternal grandmother in this patient was said to be sensitive, suspicious, and difficult to get along with, and a maternal cousin was “disturbed for 13 years in somewhat the same way as the patient and committed suicide.” Six of the 10 patients who fulfilled the criteria for delusional disorder had a sign out diagnosis of paranoid schizophrenia and one of these had a family member with a familial trait similar to the ones described above. The father in this case was considered “suspicious.” Thus, of the 10 people who were diagnosed as delusional disorder, two showed the traits of suspiciousness in their families. Of the 19 remaining paranoid schizophrenics who did not meet the delusional disorder criteria, none showed this kind of family background. DISCUSSION The major finding is that personality traits variously described as suspiciousness or jealousy or secretiveness and/or the presence of delusions in a family or the presence of a paranoid diagnosis in the family separate the patients with delusional disorder from the patients who are controls. This finding could be the result of a systematic error. Possibly the social workers or the families were sensitized to the symptoms in the delusional disorder patients and were more likely to report them in those cases. This seems unlikely because of the fact that so much care was taken by the interviewers in getting complete family histories and complete characterizations of family members but it cannot be ruled out. We used a second set of controls, namely, the patients who were part of the Iowa 500 group. The reason that we did this was that the family histories between 1934 and 1944 were uniformly good. In our original group of delusional disorder patients, some of the patients were admitted in the 1960s and 1970s. The family histories during this time were not as complete as the family histories earlier. As a consequence, we decided to take the period of time during which we had the most confidence in the quality of the family histories. There was a specific point in mind. We took patients who had a diagnosis of some kind of paranoid condition. either paranoid state or paranoid schizophrenia or involutional paranoid state. We assumed that if there was an observer bias to report the paranoid personality traits in patients with a paranoid diagnosis there would be a high degree of reporting in
246
GEORGE WINOKUR
this group. As it happened, only the patients who met the diagnosis of delusional disorder (2 of 10) had family members that showed paranoid personality type traits. The patients who had the paranoid schizophrenia type diagnoses or did not meet the rigid delusional disorder diagnostic criteria did not have such family members. Interestingly, we can arrive at a percentage of delusional disorder families that contain the paranoid personality type traits. We have 29 delusional disorder probands, 2 controls who met the criteria for simple delusional disorder, and 10 of the Iowa 500 group who met the criteria. Altogether this makes 41 patients who met criteria for delusional disorder. Eleven of them (27%) had family members who had paranoid personality traits. This should be considered an incomplete accounting of such traits in family members of delusional disorder in that some of the histories and family histories, particularly in the later years of the proband study, were not as complete as those in the early years. If one adds all of the controls (excluding the 2 delusional disorder patients) for the proband group (N = 27) to those of the Iowa 500 group who did not meet the criteria for delusional disorder (N = 19), one arrives at a group of 46 patients. Only one of these (2%) showed familial paranoid personality traits. As noted before, even in the case of chronic organic brain syndromes, an effort was made to evaluate the personality characteristics of the family. This occurred even in infectious illnesses, i.e., general paresis. However, a final answer must wait on a blind evaluation of the family members of delusional disorder patients versus controls using a validated instrument that could pick up the presence of paranoid personality traits. Such an instrument exists at the present time so that this research is possible in the future.9*10 Though it seems fairly clear from the available data that delusional disorder is a separate illness familially from schizophrenia, the problem of paranoid personality still poses some questions. Kendler and Gruenberg I1 blindly diagnosed paranoid personality disorder in relatives of schizophrenic spectrum adoptees from the Danish adoption study of schizophrenia. They found that paranoid personality was significantly more common in the interviewed biologic relatives of the schizophrenic spectrum adoptees than those relatives of control adoptees. The numbers are quite small. Six relatives were diagnosed as having either possible, probable, or definite paranoid personality disorder. Four of those six came from the group of biologic relatives of schizophrenic spectrum probands. One case came from the 138 relatives of the controls, and one case came from the adoptive relatives of a schizophrenic spectrum proband. Thus, the authors comment that the prevalence of paranoid personality disorder was greater in the biologic relatives of the schizophrenic spectrum probands (3.8%) than in the remaining nonbiologic relatives that were studied (.9%). Though this is significant, the data are quite small. Also, it is quite possible that some of the schizophrenic spectrum patients in the Danish study in fact had delusional disorder. So far as can be told, Kendler and his associates did not attempt to assess the presence of delusional disorder in the Danish schizophrenic adoptees, only in their relatives. If that were so, one of the questions that would arise is whether or not there was any relationship between the paranoid personality and the presence of delusional disorder amongst the index schizophrenic spectrum probands. This study is certainly an important one, and the authors rightly point out that “their study” does not support the view that paranoid personality disorder is genetically related to paranoid psychosis. Paranoid psychosis, having been shown
DELUSIONAL DISORDER-FAMILIAL
TRAITS
247
to have little genetic relationship to schizophrenia, and their finding that paranoid personality is genetically linked to schizophrenia would make a genetic link between paranoid psychosis and paranoid personality improbable. However, as noted above. the data are very small, and they cannot be considered a final answer anyway because patients in the schizophrenia spectrum group could have included delusional disorder probands. Kendler and Gruenberg12 have recently rediagnosed the schizophrenic spectrum probands in the Danish adoption study according to DSM-III criteria. One of these patients met criteria for delusional disorder (and also met DSM-III criteria for paranoid disorder). The findings as regards the relationship of paranoid personality disorder are equivocal in these data. Depending on which control group was used for the relatives of the schizophrenic spectrum patients, there was either a statistically significant or a marginally significant finding of an increase in paranoid personality disorder in the biological relatives of schizophrenic adoptees. There are three paranoid personality relatives out of 69 “schizophrenic spectrum” (as defined by Kendler and Gruenberg) relatives (4.3%), one out of the total 137 control relatives (0.7%), and one out of 37 (2.7%) relatives of schizophreniform, schizoaffective, other, delusional disorder, atypical psychosis, bipolar, other personality disorder. and other nonpsychotic mental disorder patients. Adding the 137 to the 37 of ill but non “schizophrenic spectrum” probands to make a composite control group. we find 2 of 174 (1.1%) relatives with paranoid personality disorder as opposed to .3 out of 69 (4.3%), not a significant difference (x2 with Yate’s correction = 1.17, df = 1, P < .28, two-tailed; Fisher’s exact = .257). One other study deals with the relationship of paranoid personality to schizophrenia.‘? Information was available on both interviewed and noninterviewed relatives, and there was a control group. More relatives of schizophrenics were diagnosed as having paranoid personality than relatives of controls. However, the definition of paranoid personality was weak because it “described those who were consistantly hostile not only to the interviewer but also to acquaintances, neighbors and hospital staff, but expressed no overt delusions.” Simply defining a personality disorder on the basis of hostility is unsatisfactory, particularly when a diagnosis of paranoid personality should include secretiveness, guardedness, overconcern with hidden motives and meanings, pathological jealousy, and an expectation of harm from other people. Further, the schizophrenics in this group could very well have included patients who had delusional disorder. One of the criteria for acceptance in this study was that of persistent delusions with a persecutory, grandiose, or somatic content. Other schizophrenic symptoms were needed, such as persistent or recurrent hallucinatory experiences, thought disorder of a schizophrenic kind, primary delusions, ideas of thought control or catatonic symptoms. The question of thought disorder and primary delusions is open to some subjective interpretation and consequently it is quite conceivable that delusional disorder was an appropriate diagnosis for some of the patients in this study. In any event the diagnosis of paranoid personality is not sufficient as a diagnosis with so few criteria as simple hostility, and certainly the possibility of contamination of the schizophrenic probands with delusional disorder patients must be taken into account. Once more, what is absolutely necessary is a family study of paranoid personality in the first-degree family members of delusional disorder patients versus family members of schizophrenic patients.
248
GEORGE WINOKUR
REFERENCES 1. Winokur G: Delusional disorder (paranoia). Compr Psychiat 18511-521, 1977 2. Kendler K: The nosologic validity of paranoia (simple delusional disorder): A review. Arch Psychiatry 37:699-706, 1980 3. Kendler K, Gruenberg A, Strauss J: An independent analysis of the Copenhagen sample of the Danish adoption study of schizophrenia. III. The relationship between paranoid psychosis (delusional disorder) and the schizophrenia spectrum disorders. Arch Gen Psychiatry 38:985-987, 1981 4. DeBray Q: A genetic study of chronic delusions. Neuropsychobiol 1:3 13-321, 1975 5. Watt J, Hall D, Olley P, et al: Paranoid states of middle life: Familial occurrence and relationship to schizophrenia. Acta Psychiat Stand 61:413426, 1980 6. Kendler K, Hays P: Paranoid psychosis (delusional disorder) and schizophrenia: A family history study. Arch Gen Psychiatry 38:547-551, 1981 7. Winokur G, Tsuang M: Paranoid vs non-paranoid schizophrenia: Definition and association, in Pert% C, Strowe G, Jansson B teds): Biological Psychiatry. Elsevier/North Holland, Biomedical Press, 198 1 8. Tsuang M, Winokur G: Criteria for subtyping schizophrenia, clinical differentiation of hebephrenic and paranoid schizophrenia. Arch Gen Psychiat 31:43-47, 1974 9. Pfohl B, Stangl D, Zimmerman M: The implications of DSM-III personality disorders for patients with major depression. J Aff Dis (in press) 10. Stangl D, Pfohl B, Zimmerman M, et al: A structured interview for the DSM-III personality disorders. (submitted for publication) 11. Kendler K, Gruenberg A: Genetic relationship between paranoid personality disorder and the “schizophrenic spectrum” disorders. Amer J Psychiat 139: 1185-l 186, 1982 12. Kendler K, Gruenberg A: An independent analysis of the Danish adoption study of schizophrenia VI. The relationship between psychiatric disorders as defined by DSM-III in the relatives and adoptees. Arch Gen Psychiatry 42:555-564, 1984 13. Stephens D, Atkinson M, Kay D, et al: Psychiatric morbidity in parents and sibs of schizophrenics and non-schizophrenics. Brit J Psychiat 127:97-108, 1975
I
N A PREVIOUS paper, we described 29 patients with delusional disorder (paranoia). Delusional disorder was found to be uncommon and quite likely to have been clinically diagnosed as paranoid schizophrenia. It was more frequently seen in men than women. The illness bore no relationship to I.Q. It did not incapacitate a person for working but it did create significant interpersonal difficulties. Familially it bore no relationship to affective disorder.’ Clinically the major symptoms were delusions of reference, jealousy, and persecution. Hypochondriasis and grandeur were seen but infrequently. Sexual problems were common. The patients were quite overtalkative and circumstantial when seen in the hospital. No patients met criteria for either depression or mania. There are some significant problems in terminology. The definition of the patients described above explicitly excluded any patients who manifested hallucinations. Kendler* has proposed that patients meeting the diagnosis for the nonhallucinatory form of delusional disorder be called “simple delusional disorder (SDDY and those others be called “hallucinatory delusional disorder (HDD).” Together these forms could be termed “delusional disorder (DD).” In conformance with these definitions, we will discuss familial psychopathology only in simple delusional disorder. Is it worth while to separate the hallucinatory from the nonhallucinatory form of delusional disorder? At this point it seems reasonable to consider them separately. Kendler et al’ has made the strongest case for looking at the two forms together. In a study of the relationship between paranoid psychosis (delusional disorder) and the schizophrenia spectrum disorders using the Danish adoption study sample, they have combined the hallucinatory and nonhallucinatory forms. When this is done, they find that familial delusional disorder is not related to schizophrenia in the adoptees. All of the cases of delusional disorder were seen in other control groups besides the biological relatives of the schizophrenic adoptee probands. The rationale for including both the hallucinatory and nonhallucinatory forms together is based on the statement that “three genetic studies have shown that the familial loading for schizophrenia is just as low in the paranoid psychotic patients with hallucinations as it is in the paranoid psychotic patients without hallucinations.” A perusal of the three studies that are cited show this to be a questionable conclusion. Only Debray separates the patients into hallucinatory and nonhallu-
From the Depariment of Psychiatry University of Iowa College of Medicine. Iowa City. Address reprint requests to George Winokur, MD. Department of Psychiatry, University of Iowa College of Medicine, Iowa City, IA 52242. (3 1985 by Grune & Stratton, Inc. 0010-440X/85/2603-0002$3.00/0 Comprehensive Psychiatry, Vol. 26, No. 3, (May/June)
1985
241
242
GEORGE WINOKUR
cinatory forms. In his study the hallucinatory forms are less likely to have a hereditary loading than are the nonhallucinatory forms. This could easily be interpreted as against the hypothesis that nonhallucinatory delusion disorder (SDD) is different from schizophrenia. However, the data are not definitive because in this paper Debray did not separate three diagnostic entities from each other in the families. Thus, he lumped at schizophrenia, chronic delusions, and personality disorders together (these combined constitute the familial loading). It is not possible to determine whether schizophrenia per se was more frequently seen in the nonhallucinatory form. Another study5 by Watt et al evaluated paranoid states of middle life. This study did not separate the groups with and without hallucinations. Further, Watt’s study contained only three patients (in a registry) who had a paranoid state. In one case, a patient with a paranoid state had a brother who had a paranoid illness with hallucinations. Whether the proband herself had hallucinations is not possible to tell. In any event, the numbers are small and it is not possible to determine whether hallucinations in paranoid patients breed together. However, there was a clear finding. Those patients who were considered as having paranoid states, i.e., those with delusional disorder with and without hallucinations, were less likely to have a family history of schizophrenia than those patients with schizophrenia. The third study that is relevant to a SDD-HDD separation is that of Kendler and Hays.6 They found 12 patients with delusional disorder and looked at the presence of schizophrenia in the families of these patients. They found only one person with schizophrenia in the families of delusional disorder patients. This was significantly less schizophrenia than was found in the families of schizophrenic patients. Seven of their 12 delusional disorder patients had hallucinations. If the one schizophrenic was seen in that particular group of patients, one out of seven would have a family history of schizophrenia. This would be about 15%. It is not possible from the paper to determine whether the one schizophrenic was in one of the families of delusional disorder patients with hallucinations or in one of the five remaining delusional disorder patients who had no hallucinations. If the former were true, however, there would be no significant difference in the proportion of families of the schizophrenics Y the hallucinating delusional disorder patients on the variable of the presence of familial schizophrenia. What one can say is that schizophrenia is less frequently seen in the family members of broadly defined delusional disorder patients and broadly defined delusional disorders are less frequently seen in the families of schizophrenics. Likewise, familial affective disorder does not seem to be related to delusional disorder. The value of separating HDD from SDD remains an open question. In order to determine whether there was a possible familial relationship of any sort in delusional disorder, we decided to look at more subtle kinds of findings, e.g., personality trait characteristics. These are the subject of this paper. MATERIAL
AND METHODS
Twenty-nine simple delusional disorder patients were selected according to the following criteria. All patients had to exhibit an unequivocal delusion that could have been present for any length of time. The delusions had to be possible, however implausible. As an example, a delusion of being controlled by radar that directed movements would not be acceptable. However, a delusion of infidelity would be quite in keeping with the definition.
DELUSIONAL
DISORDER-FAMILIAL
243
TRAITS
Exclusion criteria were the presence or suggestion of any hallucination at any time, bizarre or fantastic delusions at any time, evidence for an organic brain syndrome, illness beginning after age 60. meeting criteria for depression or mania, and inappropriate or markedly flattened affect. In a previous paper. the remainder of the methodology is given.’ ‘The 29 charts were reperused and specific attention was paid to the systematic family history that was recorded by the social workers. These systematic family histories were the result of interviews with multiple informants including family members and friends. They contained personality characteristics as well as evidence of major psychiatric disorders. Often the data contained information about the personalities of the paternal and maternal grandparents and the siblings of these grandparents. Likewise. the aunts and uncles of the proband as well as the cousins are noted. There was a formal outline for the presentation of the family history that the social worker followed. In addition to the social worker’\ material, the physician who saw the patient often added matenal on the family histories. Likewise. subsequent admissions contained new material added by social workers and physicians. ‘The family histories were similar regardless of the diagnosis. Thus, in one patient who had a diagnosla of general paresis, the following notes were made. Father-had temper tantrums and was deaf: mother--had “sick headaches”; sister-was hospitalized for hysteria; sister-had a near nervous breakdown; sister-may have had a nervous breakdown. Such a family history was obtained even though the patlenr had an illness that was known to be clearly organic and infectious. For comparison purposes 29 controls were drawn from the records of the University of Iowa P\ychiatric Hospitals. The controls were matched for age and sex and year of admission. If there were an! differences in the quality of workup over the years, the matching should control for that. As another control, patients who were admitted as part of a large study of schizophrenia. bipolar and unipolar illness (the Iowa 500) between the years 1934 and 1944 were investigated separately.. These patients were followed up after 20 to 30 years. The subjects were 29 patients who were originally diagnosed as having paranoid schizophrenia at the time of entry into the hospital and at follow-up continued to have that diagnosis using a prespecified set of criteria.” The criteria were used to separate paranoid versus nonparanoid schizophrenia. These criteria, specifically the ones for paranoid \chizophrenia, would include patients who met the prestated criteria for simple delusional disorder.
RESULTS Table 1 gives the frequency distribution of the clinical diagnoses between the control groups and the delusional disorder groups. As expected, most of the paranoid diagnoses occurred in the delusional disorder group. However, by chance the match turned up two control patients who overlapped the delusional disorder patients on the variable of diagnosis. One of these had an involutional paranoid state diagnosis, and the other had a paranoid state. These patients in fact would Table 1. Clinical Diagnosis in Delusional and Controls
Disorder
Patients
Delusional
N Diagnosis Affective disorder Schizophrenia Organic brain syndrome Drug abuse Hysteria Personality disorder Involutional paranoid state Paranoid state Paranoid psychosis Paranoia
Controls
Disorder
29 % 28 21 14
29 %
10 14 3 3 -
3 45 31 17
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GEORGE WINOKUR
have met our criteria for simple delusional disorder. They were missed in the original evaluation because they did not contain the term “paranoia” in the hospital sign out diagnosis. All of the 29 delusional disorder patients had, at some point in their lives, been coded out as showing paranoia. An effort was made to evaluate the presence or absence of either remitting illness or chronic illness or both in the families of the two groups of patients, Presumably remitting illness would refer to some kind of affective illness and chronic illness would refer more likely to either paranoid symptomatology or schizophrenia. Table 2 presents those data. What is notable is that there is really very little difference between the controls and the patients with delusional disorders. Of course, chronic illness could refer either to chronic schizophrenia or chronic delusional disorder, so the resolving power of this dichotomy is not high. The major finding may be seen in Table 3 which-presents family history workups relevant to the following terms: suspiciousness, secretiveness, being delusional, or being paranoid. Clearly, more families of the delusional disorder patients (32%) showed these traits than control families. Of the nine families that had members that were called either suspicious, secretive, jealous, delusional, or paranoid, the following describes the quality of the material: (1) father feels cheated; maternal aunt is suspicious and chronically hospitalized; (2) mother is delusional about cancer; two sisters are suspicious; (3) father had a nervous breakdown and is considered a jealous person; (4) one brother is chronically delusional; one sister is paranoid schizophrenic; (5) sister is chronically paranoid, paternal grandmother is considered suspicious; (6) father lost money on business ventures and is considered secretive; paternal family is considered secretive; paternal great uncle is delusional; (7) brother is suspicious; (8) sister has paranoia; (9) half sister has involutional paranoid illness. Five of the nine families contained mutliple members who met these adjectival criteria. One of the controls had such a family member. In this case the index control had a hospital diagnosis of probable manic-depressive illness. His paternal uncle was diagnosed as having paranoid schizophrenia but probably had alcoholism with paranoid symptoms. As regards the two controls who fulfilled the criteria for delusional disorder, both had negative family histories for anything. Six patients had suffered from their delusional illness under a six-month period of time. Only one out of those 6 (17%) had a family history that contained suspiciousness, or secretiveness, or paranoid behavior, or delusions, or jealousy. This may be compared to the 8 out of the 23 (35%) that had a longer term illness. Of the 29 patients of the Iowa 500 control group, 10 met the criteria for delusional
Table 2. Major Psychiatric and/or
Disorders in First-Degree Extended Family
Relatives
Delusional N Remitting illness Chronic illness
Disorder
Controls
8 (2289%) 7 (24%)
29 8 (28%) 3 (10%)
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DISORDER-FAMILIAL
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TRAITS
Table 3. Family History Traits of Suspiciousness, Jealousy, Secretiveness, Delusions, or Some Kind of Paranoid Disorder*
N (%) Positive for traits N (%) Negative for traits
Delusional Disorder
Controls
9 (32%) 20 (69%)
1 ( 3%) 28 (97%)
‘x2 = 7.73, d.f. 1, I’ < .005
disorder. The reason that they were not picked up in the original scanning of the delusional disorder patients is that at no point had they ever been coded out as having paranoia as a diagnosis. Of the 10 patients with delusional disorder, four had a hospital diagnosis of paranoid condition. In this group one person had family members with the traits that have been discussed above. Thus, a maternal grandmother in this patient was said to be sensitive, suspicious, and difficult to get along with, and a maternal cousin was “disturbed for 13 years in somewhat the same way as the patient and committed suicide.” Six of the 10 patients who fulfilled the criteria for delusional disorder had a sign out diagnosis of paranoid schizophrenia and one of these had a family member with a familial trait similar to the ones described above. The father in this case was considered “suspicious.” Thus, of the 10 people who were diagnosed as delusional disorder, two showed the traits of suspiciousness in their families. Of the 19 remaining paranoid schizophrenics who did not meet the delusional disorder criteria, none showed this kind of family background. DISCUSSION The major finding is that personality traits variously described as suspiciousness or jealousy or secretiveness and/or the presence of delusions in a family or the presence of a paranoid diagnosis in the family separate the patients with delusional disorder from the patients who are controls. This finding could be the result of a systematic error. Possibly the social workers or the families were sensitized to the symptoms in the delusional disorder patients and were more likely to report them in those cases. This seems unlikely because of the fact that so much care was taken by the interviewers in getting complete family histories and complete characterizations of family members but it cannot be ruled out. We used a second set of controls, namely, the patients who were part of the Iowa 500 group. The reason that we did this was that the family histories between 1934 and 1944 were uniformly good. In our original group of delusional disorder patients, some of the patients were admitted in the 1960s and 1970s. The family histories during this time were not as complete as the family histories earlier. As a consequence, we decided to take the period of time during which we had the most confidence in the quality of the family histories. There was a specific point in mind. We took patients who had a diagnosis of some kind of paranoid condition. either paranoid state or paranoid schizophrenia or involutional paranoid state. We assumed that if there was an observer bias to report the paranoid personality traits in patients with a paranoid diagnosis there would be a high degree of reporting in
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this group. As it happened, only the patients who met the diagnosis of delusional disorder (2 of 10) had family members that showed paranoid personality type traits. The patients who had the paranoid schizophrenia type diagnoses or did not meet the rigid delusional disorder diagnostic criteria did not have such family members. Interestingly, we can arrive at a percentage of delusional disorder families that contain the paranoid personality type traits. We have 29 delusional disorder probands, 2 controls who met the criteria for simple delusional disorder, and 10 of the Iowa 500 group who met the criteria. Altogether this makes 41 patients who met criteria for delusional disorder. Eleven of them (27%) had family members who had paranoid personality traits. This should be considered an incomplete accounting of such traits in family members of delusional disorder in that some of the histories and family histories, particularly in the later years of the proband study, were not as complete as those in the early years. If one adds all of the controls (excluding the 2 delusional disorder patients) for the proband group (N = 27) to those of the Iowa 500 group who did not meet the criteria for delusional disorder (N = 19), one arrives at a group of 46 patients. Only one of these (2%) showed familial paranoid personality traits. As noted before, even in the case of chronic organic brain syndromes, an effort was made to evaluate the personality characteristics of the family. This occurred even in infectious illnesses, i.e., general paresis. However, a final answer must wait on a blind evaluation of the family members of delusional disorder patients versus controls using a validated instrument that could pick up the presence of paranoid personality traits. Such an instrument exists at the present time so that this research is possible in the future.9*10 Though it seems fairly clear from the available data that delusional disorder is a separate illness familially from schizophrenia, the problem of paranoid personality still poses some questions. Kendler and Gruenberg I1 blindly diagnosed paranoid personality disorder in relatives of schizophrenic spectrum adoptees from the Danish adoption study of schizophrenia. They found that paranoid personality was significantly more common in the interviewed biologic relatives of the schizophrenic spectrum adoptees than those relatives of control adoptees. The numbers are quite small. Six relatives were diagnosed as having either possible, probable, or definite paranoid personality disorder. Four of those six came from the group of biologic relatives of schizophrenic spectrum probands. One case came from the 138 relatives of the controls, and one case came from the adoptive relatives of a schizophrenic spectrum proband. Thus, the authors comment that the prevalence of paranoid personality disorder was greater in the biologic relatives of the schizophrenic spectrum probands (3.8%) than in the remaining nonbiologic relatives that were studied (.9%). Though this is significant, the data are quite small. Also, it is quite possible that some of the schizophrenic spectrum patients in the Danish study in fact had delusional disorder. So far as can be told, Kendler and his associates did not attempt to assess the presence of delusional disorder in the Danish schizophrenic adoptees, only in their relatives. If that were so, one of the questions that would arise is whether or not there was any relationship between the paranoid personality and the presence of delusional disorder amongst the index schizophrenic spectrum probands. This study is certainly an important one, and the authors rightly point out that “their study” does not support the view that paranoid personality disorder is genetically related to paranoid psychosis. Paranoid psychosis, having been shown
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TRAITS
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to have little genetic relationship to schizophrenia, and their finding that paranoid personality is genetically linked to schizophrenia would make a genetic link between paranoid psychosis and paranoid personality improbable. However, as noted above. the data are very small, and they cannot be considered a final answer anyway because patients in the schizophrenia spectrum group could have included delusional disorder probands. Kendler and Gruenberg12 have recently rediagnosed the schizophrenic spectrum probands in the Danish adoption study according to DSM-III criteria. One of these patients met criteria for delusional disorder (and also met DSM-III criteria for paranoid disorder). The findings as regards the relationship of paranoid personality disorder are equivocal in these data. Depending on which control group was used for the relatives of the schizophrenic spectrum patients, there was either a statistically significant or a marginally significant finding of an increase in paranoid personality disorder in the biological relatives of schizophrenic adoptees. There are three paranoid personality relatives out of 69 “schizophrenic spectrum” (as defined by Kendler and Gruenberg) relatives (4.3%), one out of the total 137 control relatives (0.7%), and one out of 37 (2.7%) relatives of schizophreniform, schizoaffective, other, delusional disorder, atypical psychosis, bipolar, other personality disorder. and other nonpsychotic mental disorder patients. Adding the 137 to the 37 of ill but non “schizophrenic spectrum” probands to make a composite control group. we find 2 of 174 (1.1%) relatives with paranoid personality disorder as opposed to .3 out of 69 (4.3%), not a significant difference (x2 with Yate’s correction = 1.17, df = 1, P < .28, two-tailed; Fisher’s exact = .257). One other study deals with the relationship of paranoid personality to schizophrenia.‘? Information was available on both interviewed and noninterviewed relatives, and there was a control group. More relatives of schizophrenics were diagnosed as having paranoid personality than relatives of controls. However, the definition of paranoid personality was weak because it “described those who were consistantly hostile not only to the interviewer but also to acquaintances, neighbors and hospital staff, but expressed no overt delusions.” Simply defining a personality disorder on the basis of hostility is unsatisfactory, particularly when a diagnosis of paranoid personality should include secretiveness, guardedness, overconcern with hidden motives and meanings, pathological jealousy, and an expectation of harm from other people. Further, the schizophrenics in this group could very well have included patients who had delusional disorder. One of the criteria for acceptance in this study was that of persistent delusions with a persecutory, grandiose, or somatic content. Other schizophrenic symptoms were needed, such as persistent or recurrent hallucinatory experiences, thought disorder of a schizophrenic kind, primary delusions, ideas of thought control or catatonic symptoms. The question of thought disorder and primary delusions is open to some subjective interpretation and consequently it is quite conceivable that delusional disorder was an appropriate diagnosis for some of the patients in this study. In any event the diagnosis of paranoid personality is not sufficient as a diagnosis with so few criteria as simple hostility, and certainly the possibility of contamination of the schizophrenic probands with delusional disorder patients must be taken into account. Once more, what is absolutely necessary is a family study of paranoid personality in the first-degree family members of delusional disorder patients versus family members of schizophrenic patients.
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REFERENCES 1. Winokur G: Delusional disorder (paranoia). Compr Psychiat 18511-521, 1977 2. Kendler K: The nosologic validity of paranoia (simple delusional disorder): A review. Arch Psychiatry 37:699-706, 1980 3. Kendler K, Gruenberg A, Strauss J: An independent analysis of the Copenhagen sample of the Danish adoption study of schizophrenia. III. The relationship between paranoid psychosis (delusional disorder) and the schizophrenia spectrum disorders. Arch Gen Psychiatry 38:985-987, 1981 4. DeBray Q: A genetic study of chronic delusions. Neuropsychobiol 1:3 13-321, 1975 5. Watt J, Hall D, Olley P, et al: Paranoid states of middle life: Familial occurrence and relationship to schizophrenia. Acta Psychiat Stand 61:413426, 1980 6. Kendler K, Hays P: Paranoid psychosis (delusional disorder) and schizophrenia: A family history study. Arch Gen Psychiatry 38:547-551, 1981 7. Winokur G, Tsuang M: Paranoid vs non-paranoid schizophrenia: Definition and association, in Pert% C, Strowe G, Jansson B teds): Biological Psychiatry. Elsevier/North Holland, Biomedical Press, 198 1 8. Tsuang M, Winokur G: Criteria for subtyping schizophrenia, clinical differentiation of hebephrenic and paranoid schizophrenia. Arch Gen Psychiat 31:43-47, 1974 9. Pfohl B, Stangl D, Zimmerman M: The implications of DSM-III personality disorders for patients with major depression. J Aff Dis (in press) 10. Stangl D, Pfohl B, Zimmerman M, et al: A structured interview for the DSM-III personality disorders. (submitted for publication) 11. Kendler K, Gruenberg A: Genetic relationship between paranoid personality disorder and the “schizophrenic spectrum” disorders. Amer J Psychiat 139: 1185-l 186, 1982 12. Kendler K, Gruenberg A: An independent analysis of the Danish adoption study of schizophrenia VI. The relationship between psychiatric disorders as defined by DSM-III in the relatives and adoptees. Arch Gen Psychiatry 42:555-564, 1984 13. Stephens D, Atkinson M, Kay D, et al: Psychiatric morbidity in parents and sibs of schizophrenics and non-schizophrenics. Brit J Psychiat 127:97-108, 1975