delusional disorders have confronted the psychiatric profession with ... tive designation "persistent delusional disorder". (ICD-10: ..... and supplement to, earlier treatments of the subject ...... statistisches Manual psychischer Störungen DSM-TV.
T. Fuchs
Delusional Diseases
1
Introduction
2
Nosology
3
Etiology
374
374
3.1
375 General Considerations
3.2 3.3
Contributions from Phenomenological Anthropology Cognitive Research on Delusional Disorders 377
4
Individual Forms
4.1
Morbid Jealousy
375
377 378
4.2
Erotomania
4.3 4.4
Body-Dysmorphic Disorder 379 Olfactory Delusional Syndrome 380
4.5
Delusional Parasitosis
4.6 4.7 4.8
378
380 Hypochondriacal Psychosis 381 Delusions Accompanying Physical Impairment Delusional Disorders in Old Age 381
5
Therapy
5.1
Somatotherapy
382
5.2
Psychotherapy
383
6
Course
7
References
Translator: E. Taub
F. Henn et al. (eds.), Contemporary Psychiatry © Springer-Verlag Berlin Heidelberg 2001
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383 383
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Delusional Diseases
1
lntroduction Ever since the publication of Esquirol's study on the "monomanias" (Esquirol 1838), monosymptomatic delusional disorders have confronted the psychiatric profession with special diagnostic and therapeutic problems. Paranoia, the "pure" delusional condition, indeed possesses special psychopathological significance, but has not always been able to retain its separate status in relation to the two large categories of the affective and schizophrenic illnesses. After a long history of controversy over the independence of paranoia as a diagnosis, the concept of the delusional disorders has been incorporated into the current diagnostic systems as a rather broad, etiologically nonspecific nosological category, which has, however, been empirically confirmed as valid by numerous family studies and studies of disease course. This review builds on the discussion of paranoid processes by Retterstol (1987) in the third (German) edition of this text and further presents the findings of more recent research on delusional disorders.
2 Nosology The following changes with respect to the delusional disorders are evident in DSM-IV (Saß et al. 1996; Kendler et al. 1989) and ICD-10 compared with the earlier diagnostic classification systems:
- Change of concept. Kraepelin's etiological conception of paranoia has been replaced by the descriptive designation "persistent delusional disorder" (ICD-10: F22.0). This step was taken not least because of the ambiguity of the term "paranoid," variously used in the past to designate the presence of delusions, a particular delusional content (e.g. persecution), a type of schizophrenia, or a personality disorder. The abandonment of this term also leads to the loss of the concept of paranoia as a specific delusional structure narrowly interwoven with individual development, and to the loss of the corresponding distinction between paranoid and paranoiac processes (Schmidt-Degenhard 1998). - Broadening of the category. The central feature of persistent delusional disorder is the presence of a delusion that is usually systematized and typically confined to a single subject and is not associated with an underlying Schizophrenie, affective, or organic illness or with a major impairment of personality. The disorder is thus a monothematic
and monosymptomatic delusional psychosis. In DSM-III, the corresponding category was restricted to persecution mania and morbid jealousy, but it has now been extended to include delusions of other, non-bizarre content (megalomania, erotomania, delusions related to the body). The major reason for this innovation was the finding in largescale, long-term studies in Scandinavia that the particular type of delusional content has no predictive specificity for the outcome of paranoia or schizophrenia (Opjordsmoen and Retterstol 1987; Retterstol1991a,b ). The possible types of delusional content thus now include the following: a) Delusion of being harmed, persecution mania b) Morbid jealousy (Othello syndrome) c) Erotomania (Clerambault syndrome) d) Megalomania (e.g. inventive mania) e) Litigation mania f) Hypochondriacal psychosis g) Delusional parasitosis h) Olfactory delusional disorder i) Body-dysmorphic delusional disorder (delusional dysmorphophobia) j) Induced delusional disorder (jolie deux) - Inclusion of hallucinations. The diagnostic criteria also now include clearly manifest hallucinations, as long as they are not visual or auditory; tactile, olfactory, and other vivid body-associated hallucinations are quite common in some forms of delusional disorder (e.g. delusional parasitosis, olfactory delusional syndrome). - Persistence. The delusional disorder is required to be present for 1 month according to DSM-IV, and 3 months according to ICD-10; when it is present for shorter times, it must be classified as an acute, predominantly delusional psychotic disorder (F23.3). The long-term findings by Opjordsmoen and Retterstol (1991) and Opjordsmoen (1993) indicate that there is a fairly large subgroup of acute delusional disorders, frequently of reactive origin, which remit within 6 months and do not recur, while patients with delusions lasting more than 6 months can expect a significantly worse course of illness. The authors therefore advocate a division of diagnostic categories according to a 6-month criterion, in analogy to the differentiation made between schizophreniform and schizophrenic illnesses. - Residual category. Finally, ICD-10 lists other or not otherwise specified persistent delusional disorders as a residual category (F22.8, F22.9), as well as induced delusional disorder, the so-called folie deux (F24).
a
a
As early as the initial studies by Winokur (1977) and Retterstol (1966, 1970), it was found that first-degree
3 Etiology
relatives of patients with delusional disorders had a frequency of paranoid illnesses only slightly higher than that of the general population. Since then, the nosological independence of the delusional disorders has been confirmed by further family studies (Kendler and Hays 1981; Kendler et al. 1985; Watt 1985), which revealed an elevated incidence of paranoid personality disorders in patients' relatives, but no increased occurrence of Schizophrenie or schizotypal disorders, as is found in the families of schizophrenics. For the paranoid psychoses of old age, too, family studies indicate that there is no genetic link to schizophrenia (Howard et al. 1997). The hypothesis of a continuous "paranoid spectrum," ranging from non-paranoid and paranoid schizophrenias to paranoia and paranoid personality disorder (Magaro 1981; Munro 1982), has thus not been confirmed: "Most delusional disorders probably have no link to schizophrenia" (Dilling et al. 1993, p. 103). Despite the frequent occurrence of secondary depressive mood alterations in the course of the delusional disorders, the studies referred to above also provide evidence agairrst their belanging to the dass of affective disorders.
3 Etiology 3.1 General Considerations
As already mentioned, the studies available to date fail to provide evidence for any significant role of hereditary factors in the causation of delusional disorders. Neuropathological causes are also usually not found, except in the case of paranoia of old age (see below). According to our present state of knowledge, most cases result from a combination of abnormal personality development, unfavorable environmental conditions, and a triggering situation of conflict; the delusion often bears an understandable relation to these preconditions. Thus a continuous growth of the biographically determined delusional theme is often found through the intermediate stages of an overvalued idea and of a delusion-like reaction to the final stage of an irreversible, chronic delusion. Two major themes continually recur in studies or descriptions of the primary personality of delusional patients: problems of self-confidence and of selfesteem. Thus most patients have personality disorders, usually of the paranoid or sensitive-narcissistic type (see, e.g. Enoch and Trethowan 1979; Opjordsmoen 1988b). According to Kretschmer (1966), a sensitive character is typified by high demands on oneself and corresponding susceptibility to impairment, with a
simultaneaus tendency toward introversion and affect retention. This structure is now usually subsumed under the further concept of the narcissistic disorder, which may thus also indude delusional patients with rather expansive features. Obsessiveness and rigidity are further features of many delusional patients; these are characteristics that impair the ability to gain distance from oneself and that may promote the development of an overvalued idea. Most patients can also be presumed to have a fundamental disturbance of self-esteem regulation and insecurity in their social relationships, whether because of genetic personality predispositions or because of developmental impairments in early childhood (Retterst0l 1987). Even in youth, these patients often manifest contact disturbances, inhibitions, affect retention, and tendencies toward distrust and withdrawal; supposed or actual slights are perceived in hypersensitive fashion, and deep-seated feelings of inadequacy are formed, along with resentment of others. Injurious or stigmatizing life experiences and conditions such as physical impairment, forced migration, loss of social dass, and minority status may further reinforce such tendencies, even in later periods of life (Tölle 1987; Fuchs 1994a,b, 1998a). Thus, in comparison to Schizophrenie or affective disorders, delusional disorders are particularly prone to appear in individuals of low social and educational status andin immigrants (Kendler 1982). In Winokur's study (1977) of 29 delusional patients, more than half had an intelligence quotient below 90. Further factors favoring the development of delusions indude social isolation (e.g. in incarceration psychoses), foreignlanguage environment, sensory deficits leading to an impairment of communication (Fuchs 1993b ), impairment of critical ability and affect processing of organic cerebral causes, and persistent substance abuse (Retterst01 1966; Munro 1988). When these conditions are present, the delusional theme may gradually come to the fore or, indeed, appear suddenly. The immediate provoking factor is usually the threatened or actual failure of a central personal concern, such as a sexual or marital conflict, a severe narcissistic insult, shame, or loss of social standing, which can no Ionger be compensated for. In this situation, the individual abjures personal responsibility by paranoid outward projection ("the others are agairrst me") or by inculpation of his or her own body, nature, or disposition ("my body is agairrst me"). Delusions thus often represent a recognizable, projective, and denying defense agairrst feelings of inferiority and inadequacy, agairrst the feared Iack of fulfillment of needs for intimacy and dependency, and even agairrst the individual's own aggressive tendencies, fueled by feelings of resentment and revenge. Projective identification can be seen as a reversal of
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shameful and degrading experiences: the supposed malevolence of others is used as a cover for one's own supposed inferiority (blame instead of shame; Morrison 1987). Kretschmer's conception of the sensitive relational delusional state is still of paradigmatic significance for the psychodynamic interpretation of the origin of delusions, even if this conception is primarily concerned with moral and sexual!ethical conflicts as decisive triggers of the disorder. This type of problern seems to have faded in importance in liberal, pluralistic societies; the sensitive relational delusional state is still very much in evidence in Japan, but has receded into the background in the West and is actually entirely unknown in the United States (Rasmussen 1978). Nonetheless, it may be presumed that the change of social values has redirected the central narcissistic problern of relational delusions onto the theme of physical appearance and attractiveness, so that they now typically manifest themselves as bodydysmorphic disorder or olfactory delusional syndrome. Now as before, the battle for self-esteem seems to be the real theme of most delusional patients, who, by displacing it onto the neighboring battlefield of the delusion, "acquire an unconscious pretext by means of which their potential or supposed defeat in life can be covered up, justified, or indefinitely postponed" (Adler 1927, p. 191). The functionality of the delusion, seen against this background, consists of a compensation for feelings of inadequacy or rejection (as in erotomania), a conviction of one's own greatness, or an enhancement of one's ability to wage battle against the imaginary opponent, with whom the delusional patient forms a "pseudo-community" (Cameron 1959). Tothis extent, chronic delusions also imply a self-stabilizing defense against a realistic confrontation with one's own life situation. Thus Roberts (1991) compared 17 patients with systematized delusions to a corresponding group of delusional patients in remission. The actively delusional patients attached a significantly higher degree of meaning to their lives and were less depressive, primarily because of the subjects of their delusions.
3.2 Contributions from Phenomenological Anthropology
Phenomenological anthropological research has also made a major contribution to our understanding of the paranoid disorders by attempting to explain delusions as an abnormality of the fundamental structures of the relationship of human beings to the world (Matussek 1963; Glatzel 1981; Blankenburg 1991, 1992). It is assumed that delusions generally consist not of false
ideas about neutral and objectifiable states of affairs, but rather of interpretations and assessments that patients attach to a situation in relation to themselves. Delusional content is thus primarily composed of "second-order realities" (Watzlawick 1988), which cannot be directly and objectively tested. Normal, correct judgments in this area can only be made if the individual is basically familiar with the world and capable of assessing it reliably; in Blankenburg's phrase, the experienced world must possess a natural self-evidence (natürliche Selbstverständlichkeit; Blankenburg 1971), with respect both to the individual's relationships with others and to his or her own corporeal nature. Delusional disorders develop precisely in the areas where reliable orientation in our environment depends not on rational knowledge and empirical evidence, but on this type of familiarity with the world and emotional soundness. This basic safeguarding mechanism is lacking in individuals predisposed to paranoia; distrust and control are used in its place. The paranoid individual is on the alert for possible injuries or malevolence and vigilantly scans his or her environment for signs of threat, deception, or betrayal. His or her social perception is thus directed toward hidden meanings, the possible use of pretenses by others, and the hidden "other side" of their behavior. In ambiguous or limited communication structures (e.g. neighborhood gossip, deafness, foreign-language environment), the uncertainty of social perception rises, and room is left for self-referential interpretations in which the greatest attention is paid to the least conspicuous matters - a fleeting gesture, undertones, or words left unsaid. If the paranoid individual further feels him- or herself to be threatened in a central personal concern, the tension of anxious distrust may become unbearable, until a new consistency is achieved by the construction of a delusional interpretation - the certainty of the contempt, betrayal, or malevolence of others, subjectively experienced as a seeing-through or unmasking of their real nature. This delusional "insight" reduces the previously overwhelming cognitive-emotional complexity of the situation (Luhmann 1973); the tormenting ambiguity of social perception gives way to a subjective emotional certainty, which, despite its negative meaning, is associated with relief and, not least, with the possibility of taking measures of caution or of opposition. Once constructed, the delusional paradigm progressively establishes itself, both by the self-confirming distorted selection of information and ignoring of contrary evidence and by the patient's self-fulfilling behavior, as when enmity toward the supposedly malevalent others eventually provokes an actual rejection by them. The essentially ambiguous nature of the secondorder realities described above implies that any
4
subjective assessment of them is necessarily aspect bound and provisional. Social relationships are, therefore, based on constant, mutual reassessment of behavior patterns and their interpretation; to be continued, they required one's own view of the shared situation always to be correctable by that of the other person. It is characteristic of delusional individuals to foreclose the possibility of correction and stand by their own interpretations with apodictic certainty, without any intersubjective modulation, as if they were statements about their own inner states (Spitzer 1989). In the anthropological literature, this phenomenon is known as the Iack of assumption of another perspective (Glatze! 1981; Blankenburg 1991; von Baeyer 1991). A delusional condition can thus be construed as an unsuccessful, surrogate-like encounter with others, which securely shields itself against the possibility of doubt: by refusing to switch perspectives, the delusional individual protects him- or herself from becoming burdened once again by the inner conflicts that have been projected onto others. At the same time, the Iack of assumption of another perspective brings the delusional individual into an egocentric position: the notion ofbeing observed or threatened by others arises tagether with the inability to take the point of view of an uninvolved, neutral third party and thereby eradicate the self-referential, delusional perspective (Fuchs 1994b). On the linguistic/semantic Ievel, these models are reflected in the theory of concretism, according to which a delusion is to be understood not as a symbolic statement about an intersubjectively constituted state of affairs, but as a non-declarative, as it were "exclamatory" expression by the patient of his or her mental state, in a manner analogaus to an interjection such as "Help!" or "Ouch!" (Holm-HaduUa 1982; Spitzer 1989; Mundt 1996). The matter troubling the patient is brought to expression, not as such, but masked, in concretistic fashion (e.g. Iack of self-esteem as bodily disfigurement in body-dysmorphic disorder, guilt as sexuaUy transmitted disease in hypochondriacal psychosis). Because the topic can no Ionger be reformulated in symbolic language, the expression of the delusion is also inaccessible to correction by reasoned argument.
3.3 Cognitive Research on Delusional Disorders
The findings of recent cognitive experimental research on the delusional disorders further add to our understanding of this subject. Patients with delusional disorders have been found to be characterized by abnormal attributive and cognitive styles, which may be demonstrated during the performance of specific
Individual Forms
tasks. Typically, premature conclusions are drawn from limited, ambiguous, or selectively perceived information; too much certainty is attached to one's own judgment in the assessment of probabilities; there is a tendency to ascribe a special significance to coincidental events; and, finally, unfortunate events tend to be blamed on others (Huq et al. 1988; BentaU and Kaney 1989; BentaUet al. 1991; Garety et al. 1991). This would imply that delusional patients Iack the ability to see themselves objectively or to assume an attitude of "healthy skepticism" toward their own judgments and thus cannot put events affecting themselves in an overarching, neutral context - or, as expressed elsewhere (Minkowski 1947; Berner 1978), that the thought processes of these patients are characterized by "the exclusion of coincidence." Although the tasks performed by the subjects in these studies were not related to the themes of their delusions, it should be borne in mind that these findings need not be interpreted as implying a preexisting vulnerability; they may, conceivably, indicate a feature of the course of the disorder. Nonetheless, these findings help to explain the cumulative origin of uncorrectable convictions: when the capacity for self-questioning and intersubjective correction is absent, social perception enters a vicious circle in which there is a progressively severe distortion of reality. The findings discussed above have now been incorporated into techniques of cognitive therapy in the delusional disorders (see below).
4
Individual Forms
The forms of delusional disorder listed in Sect. 2 may be broken down in terms of their basic areas of reference as foUows: 1. Delusions primarily with respect to relationships
with others: a) Injury, aggression (persecution mania, morbid jealousy, Iitigation mania) b) Self-aggrandizement, grandiosity (megalomania, erotomania) c) Shame and rejection (olfactory delusional disorder, body-dysmorphic disorder) 2. Delusions primarily with respect to one's own body: a) Delusional parasitosis b) Hypochondriacal psychosis The types of delusional disorder listed under item 1 above have in common that the central experience is often one of shame, inadequacy, or inferiority. This becomes explicit only in subitem (c), while it is made almost unrecognizable by defense mechanisms in the
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Delusional Diseases
other two groups - by aggressively torred projections (blame instead of shame) or by self-aggrandizement. The primarily intersubjective reference of these forms of delusional disorder is expressed in the patient's frequent self-referential perceptions and delusional ideas. Distinct from these forms of delusional disorder are the anxious-hypochondriacal forms, referring to the patient's own body, which are listed in item 2; in these forms, ideas of reference occur only rarely. The patient Iacks trust not in other people, but in his or her own body and its functions. Admittedly, the excessive stress placed on the patient's own body is generally secondary to a disturbance of the capacity for interpersonal relationships (Küchenhoff 1985). This dichotomy implies that not all forms of delusion regarding one's own body should be called hypochondriacal, as is usual in the Anglo-American literature. The hypochondriacal conviction of being ill is hardly the only form of disturbed and alienated corporeality; indeed, in the course of a person's life, very different themes may come to bodily expression, including shame, guilt, inferiority, and fear of death (Fuchs 1992). Body-dysmorphic delusional disorder, a pathological process of the social or "external body," thus has little in common with hypochondriacal psychosis per se. Furthermore, body-dysmorphic delusional disorder usually begins in adolescence, while the forms of delusional disorder primarily relating to one's own body usually begirr in middle age or old age (Musalek et al. 1989). The ensuing description of selected individual forms of delusional disorder is intended as an extension of, and supplement to, earlier treatments of the subject (see especially Retterst0l 1987).
gibly convinced of the partner's unfaithfulness and attempts to obtain evidence for it by constant questioning, checking, spying, and searching of the intimate sphere. This usually leads to severe marital conflicts. Typically, the supposed rival remains a rather obscure figure, and the possibility of "catching the partner in the act" tends to be avoided (Enoch and Trethowan 1979), which reflects the reluctance to confront reality that is inherent in the delusional disorders. lnstead, the patient's aggressive impulses are directed entirely agairrst the partner; morbid or delusional jealousy is therefore associated with a particularly high risk of violence, including homicide (Soyka 1992). Narcissistic, latently insecure, and compulsive-controlling personality traits are thought to be predisposing factors for the development of this type of delusional disorder (Enoch and Trethowan 1979). It is often triggered by persistent partnership conflicts or by failures that are painful for the patient, e.g. in the occupational sphere, which are displaced onto the relationship. Feelings of inferiority and inadequacy, fears of loss (possibly secondary to sexual impotence), and also, not uncommonly, the patient's own repressed tendency toward infidelity are projected onto the partner and typically progress from excessive to delusional jealousy. Morbid jealousy exhibits a typical circular structure of intrusive thoughts, distorted perception, and intense feelings of anxiety or rage; this structure has recently proved accessible to cognitive behavior therapy (Tarrier et al. 1990; Dolan and Bishay 1996). It can be inferred from the findings of severallongterm studies that morbid jealousy has a favorable prognosis in comparison to other delusional disorders and only rarely converts to another type of psychotic illness (Crowe et al. 1988).
4.1 Morbid Jealousy
Morbid jealousy is, along with persecution mania, one of the commoner delusional disorders (approximately 40o/o of all cases, according to Winokur 1977; Crowe et al. 1988). It predominantly affects men in late adulthood (Musalek et al. 1989). Only a minority of these patients are alcoholics; the supposed close relationship of this disorder to alcoholism is no Ionger postulated (Enoch and Trethowan 1979; Soyka et al. 1991). Alcoholic morbid jealousy is no Ionger counted among the delusional disorders in ICD-10, but rather among the disorders caused by psychotropic substarrees (F10.5). The differential diagnosis must exclude underlying schizophrenic or organic (e.g. dementing) illnesses. The delusional theme of jealousy is related exclusively to the patient's partner. The patient is incorri-
4.2 Erotomania
Erotomania (Clt~rambault syndrome) consists of the conviction that one is loved by another person, generally of higher social status, publicly known or held in high regard, who makes his or her passion known through secret signals or messages. The patients are predominantly women of modest social circumstances, in their fourth to sixth decades, who have not established Iasting partnerships. They often pursue the admired person (who is usually married) through innumerable letters, telephone calls, or even public scenes and are not discouraged in these activities even by nearly insuperable obstacles, in accordance with the avoidance of confronting reality that is typical of the delusional disorders (see
4 Individual Forms
Sect. 3.1). These patients succeed in reinterpreting their supposed admirers' acts of rejection, no matter how vigorous they may be, as expressions of Iove ("tests of devotion" etc.). Legal measures taken by the harassed victim of the delusion Iead, not uncommonly, to the patient's involuntary commitment to a psychiatric institution. The contrast between the generally not very successful, erotically unsatisfied, and lonely existence of the patients and the glamour of their supposed admirers suggests that the essential causative factor of erotomania is narcissistic wish-fulfillment (Segal 1989). In accordance with this hypothesis, these patients usually have an inhibited-sensitive or paranoid primary personality and are often notably physically unattractive (Hallender and Callahan 1975). It should be borne in mind, however, that similar manifestations may appear in the setting of schizophrenia or organic delusional syndromes (Signer and Cummings 1987; EI Gaddal 1989). The prognosis of pure erotomania is thought to be rather unfavorable; treatment with neuroleptic medications usually has only a palliative inftuence on the chronic course of the disorder (Opjordsmoen and Retterstol 1987). Directed reality confrontation or permanent separation from the loved person may have a beneficial effect (Segal 1989), but in many cases Ieads merely to a change of the "delusional object."
4.3 Body-Dysmorphic Disorder
Dysmorphophobia derrotes the subjective perception of one's own body as ugly or deformed, combined with the conviction that others perceive one's body in the same way. As a rule, the supposed defect is located in a specific, usually exposed part of the body, such as the face, ears, nose, jaw, teeth, hair, etc., but it may also be in the limbs or genitalia. These patients develop ideas of reference, imagine that they are being discriminated agairrst or ridiculed by others, and withdraw increasingly from social contact. They consult plastic surgeons, otorhinolaryngologists, or dentists and importune them for an operation that will change their appearance. The designation "phobia" indicates the close relationship of this syndrome to the social phobias; in view of these patients' environmental anxieties and disturbed contact behavior, the rejection of this previously used term by DSM-IV and ICD-10 is not necessarily appropriate. The new division into separate categories (F45.2: body-dysmorphic disorder; F22.0: somataform delusional disorder) is not in line with clinical experience, which suggests a continuum extending from excessive preoccupation with one's own
appearance, to a neurotically overvalued idea, to the delusional certainty of one's own deformity (Phillips 1991 ). The assignment of delusional disorders to the category of the schizophrenias, as was customary in the past (Zaidens 1950; Connolly and Gipson 1978), has no proponents today, even if the dysmorphophobic syndrome, like obsessive-compulsive manifestations, can be conceived of as a defense agairrst further psychotic disintegration. Bizarre delusional ideas about physical changes or experiences of being physically influenced from without are evidence for an underlying schizophrenic illness; moreover, dysmorphophobic convictions may also appear in the context of the affective disorders. The classification of the milder forms of dysmorphophobia, however, is still ambiguous in the current systems of classification. In psychodynamic terms, the connection to the themes of shame and self-esteem is evident; it is a matter of showing oneself and being seen by others. Excessive observation of oneself and an idealized conception of beauty, combined with feelings of inferiority and contact anxieties, characterize these patients (Küchenhoff 1984). As one would expect, sensitive-narcissistic, insecure, and compulsive personality disturbances are also often found in this patient group. The initial manifestation usually appears, not surprisingly, in adolescence, when the individual's body image, concept of self, and interpersonal relationships all come to a crisis. In this situation, the projection of deep-seated feelings of inadequacy, particularly sexual inhibitions, onto a biological bodily defect may perform a self-protective function (Fuchs 1993a): it frees the individual from the need for confirmation, e.g. in competition for partners, and allows negative aspects of the self to be concretized and externalized in a single part of the body, a parspro toto that becomes accessible to repair by cosmetic surgery. Body-dysmorphic disorder usually takes a chronic course Iasting several years or decades and is associated with an elevated risk of suicide (Phillips 1991). Surgical intervention can be expected to produce an improvement, if at all, only in milder forms of the disorder and should be firmly discouraged in more severe forms: when surgery is performed, patients often continue to complain of the supposed deformity because of unrealistic expectations from surgery, or eise the symptom migrates to another part of the body, because the underlying conftict concerning the patient's self-esteem remains unresolved (Hay I 970; Andreasen and Bardach 1977; Strian 1984). A marked affective or compulsive component, however, calls for the application of serotonirr reuptake-inhibiting antidepressants, which have been repeatedly reported to yield good results (Hollander et al. 1989; Phillips
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1991), while the outcome of neuroleptic therapy is usually unsatisfactory.
4.4 Olfactory Delusional Disorder
Olfactory delusional disorder is characterized by the conviction that the sufferer emits a repellent odor (e.g. of sweat, bad breath, or flatus). The primary experience is mainly in the area of perception, i.e. there are abnormal olfactory sensations which, however, are experienced in an "environmentally dependent" fashion, usually only in the presence of other people. There are corresponding ideas of reference, in which words or actions of others that are insignificant in themselves are interpreted as expressions of disgust and aversion. These patients combat the supposed odor by washing or applying perfume, without success; they usually resort to a more or less total social withdrawal. They not uncommonly develop hypochondriacal explanatory delusions of odor-producing diseases of the skin or gastrointestinal tract (Gattaz and Haas 1982). Olfactory phobia and olfactory delusional disorder have received the greatest amount of attention in psychopathology in Japan, where Morita (1947) grouped them together with dysmorphophobia, erythrophobia, and eye contact phobia as taijin-kyofu syndrome ("fear of mankind," or anthropophobia). The common features of these disorders are the underlying feeling of shame and inferiority, the manifestation of ideas of reference and contact phobia, and social withdrawal (Yamashita 1993; Kimura 1995). Morita believed that the basis of the syndrome lay in the so-called shinkeishitsu personality ("constitutional nervous temperament"), characterized primarily by introversion, hyperreflectivity, neurasthenia, and disturbance of self-esteem, and developed a specific form of treatment known as Morita therapy (Kora 1999). Because olfactory hallucinations also appear frequently in the settings of depression and schizophrenia, these differential diagnoses must be considered (Malasi et al. 1990). The presence of a pure delusional disorder is supported by the development of the delusion on the substrate of a sensitive-narcissistic personality and an existing relationship conflict, with experiences of inadequacy, insult, or exclusion. The perceived inferiority and self-contempt are concretistically projected by the delusion onto the physical sphere, aided not least by the special relationship of the sense of smell to emotional atmospheric experiences, sympathy, and antipathy. Such delusional conditions have a continuous transition into neurotic developments with delusion-like reactions, which are more accessible to psychotherapeutic intervention (Moesler 1992).
4.5 Delusional Parasitosis
Delusional parasitosis, first described by Ekbom (1938) under its German name Dermatozoenwahn ("dermatozoal delusion" or epidermozoophobia), consists of the unshakable certainty of being infested by intra- or subcutaneous parasites, usually accompanied by itch. These patients often subject themselves to extensive grooming rituals and bring small collections of skin particles to dermatological clinics or government health departments requesting that they be studied for the presence of parasites ("match-box sign"). The affected individuals, usually women in their fifth to seventh decade, feel severely tormented and socially stigmatized by their supposed condition. Tactile sensations (itching, paresthesia) are a major feature of this delusional disorder, for which the name "chronic tactile hallucinosis" has also been suggested (Bers and Conrad 1954), but they are not always present. A comparative study revealed that delusional patients with tactile phenomena, compared to non-delusional patients with tactile phenomena of other origin, were more often socially isolated and less frequently married, placed higher hygienic demands on themselves, and more commonly had a history of dermatological and mental illness (Musalek 1991). The nosology of this delusional syndrome has always been particularly difficult; it has been found in association with practically all types of underlying illness. In the diagnostic study carried out by Musalek et al. (1990), approximately 40o/o-50o/o of all cases were attributable to organic delusional disorders, usually secondary to arteriosclerosis; Marneros et al. (1988) found this figure to be as high as 70o/o. Rarer entities to be considered in the differential diagnosis include cocaine- or amphetamine-induced psychoses ("cocaine bugs"), vitamin B12 deficiency, diabetes mellitus, Iymphoma, and uremia accompanied by pruritus (Morris 1991). The supposed parasitic infestation may also be a delusional expression of feelings of guilt or punishment in the setting of melancholia, while a paranoid attribution of the infestation to another person (contamination delusion) is indicative of schizophrenia. Thus only a minority of cases can be considered pure delusional parasitosis. These cases are usually in the setting of a compulsive personality structure and social isolation. The partners of these patients relatively frequently develop an induced delusion (folie deux) (10o/o-20o/o; Musalek and Kutzer 1990). The prognosis of delusional parasitosis is relatively favorable: intensive application of pharmacological, psychotherapeutic, and sociotherapeutic measures Ieads to improvement, or even remission, in two thirds of these patients (Musalek 1991; Trabert 1993).
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4
4.6 Hypochondriacal Psychosis
The concept of monosymptomatic hypochondriacal psychosis (Munro 1988) has been used to subsume several disorders, such as olfactory delusional disorder, delusional parasitosis, and body-dysmorphic delusional disorder, that are treated as distinct in this review. As mentioned above, however, the term should be reserved for the hypochondriacal delusional disorders per se, corresponding to the earlier "paranoia hypochondriaca" (Serieux and Capgras 1909), in which patients imagine that they are suffering from an incurable or fatal illness. Hypochondriacal psychosis must also be distinguished from neurotic hypochondriases, which present as diffuse states of ill-being or neurasthenic exhaustion and in which the fear of suffering from a physical illness does not have the same uncorrectable character (Dilling et al. 1993). The so-called circumscribed hypochondriases may, however, assume a character similar to that of a delusion (Hallen 1970); they are characterized by persistent abnormal sensations and foreign-body sensations in particular areas of the body, e.g. in the mouth, which often Iead these patients to consult dentists or otorhinolaryngologists. The affected patients are distinguished by the persistence with which they demand radical diagnostic and operative procedures, usually from multiple physicians. There are often painful dysesthesias in several organ areas, in the skin, or in the sense organs; when these are especially bizarre, the differential diagnosis of coenesthetic schizophrenia should be considered. On the other hand, the fact that the delusion concerns the patient's own state ofhealth, rather than features of the external environment interpreted with reference to the self, suggests the possibility of a depressive disorder, and hypochondriacal delusions may indeed often persist at attenuated Ievels after the actual depressive phases have passed. It may also be necessary to exclude underlying disorders such as pernicious anemia, uremia, Iead poisoning, endocrine diseases, or cerebrovascular disease by specific diagnostic testing, even though this may reinforce the patient's fixation on a somatic cause. Hypochondriacal psychosis, like hypochondriacal syndromes in general, is typified by a loss of confidence in the corporeal basis of existence and an attitude toward the environment narrowly centered on bodily complaints. The patients' primary personality often exhibits anancastic features and a tendency toward somatization (Munro 1988). The overemphasis of the body is an expression of a loss of the capacity for interpersonal relationships; against this background, the demanding, hostile, and disempowering behavior
Individual
Forms
of these patients toward physicians is to be seen as a surrogate for interpersonal relationships (Fuchs 1992; Küchenhoff 1985). The content of the delusion is not uncommonly of a sexual nature (delusional syphilis or AIDS; Mahorney and Cavenar 1988); this may indicate a sensitive or erotic-conflictual experiential reaction as the origin of the illness. If the disorder takes a chronic course, as it usually does, the frequently occult depressive mood alterations should be watched for, as these are accompanied by an elevated risk of suicide (Bebbington 1976; Opjordsmoen 1988a).
4.7 Oelusions Accompanying Physical lmpairment
Delusions as consequences of actual physical impairment must be distinguished from hypochondriacal and body-dysmorphic delusional themes. Gaupp (1942) first described such delusions in the case of a teacher named Hager, who fett that he was no Ionger taken seriously by others, especially women, after an amputation in the war, developed a progressively severe persecution mania, and finally killed a young woman. According to Tölle (1987, 1993), it is primarily amputations and other invasive operations, deformities, or illnesses that Iead to social disadvantage and discrimination and are therefore perceived as shameful deficiencies. The resulting delusion of interpretation (delusion of being harmed), which not uncommonly remains hidden for very long periods, bears an important projective, ego-sparing function and is thus in keeping with the etiological concepts presented in Sect. 3.1 above. Clearly, a psycho-organic disturbance of information processing and critical ability plays an additional rote in many cases.
4.8 Delusional Disorders in Old Age
Paranoia beginning at age 60 or above occurs with a prevalence of approximately 1%-2% (Christenson and Blazer 1984). Typical delusional contents include those of injury, threat, or persecution by people near the patient's home, although fantastic or confabulatory delusional contents are not uncommon. The nosological classification of the disorders first described by Kay and Roth (1961) as late paraphrenias remains controversial; the existence of a continuous spectrum of transitional forms, from pure delusional disorders with or without hallucinations to unambiguously schizophrenic phenomena, makes it impossible to differentiate delusional disorders of old age with certainty from late-onset schizophrenia (i.e. arising between the ages of 40 and 60) or to establish a clear
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diagnostie distinction between schizophrenie and delusional disorders (Howard et al. 1994; Riecher-Rössler et al. 1995). In ICD-10, senile paranoia is assigned to the delusional disorders (F22.0); the presence of persistent auditory hallucinations constrains a classification as persistent delusional disorder of other type (F22.8), as lang as the criteria for schizophrenia arenot fulfilled (Dilling et al. 1993). Independently of these problems of classification, certain risk factors and provoking conditions for the onset of dementia in old age have been repeatedly described: - Fernale sex (female-to-male ratio typieally 7:1; Almeida et al. 1995). - Paranoid or schizoid premorbid personality traits (Kay et al. 1976; Fuchs 1998b). - Social isolation (Naguib and Levy 1987; Almeida et al. 1995), in accordance with Janzarik's (1973) concept of contact deficiency paranoia. - Sensory impairment, particularly deafness (in as many as 40% of cases), which may favor a paranoid misperception of social situations (Cooper et al. 1974; Fuchs 1993a). - Discriminating, injurious, or threatening life events such as forced migration from one's horneland (in as many as 50% of cases), birth out of wedlock, physical impairments, etc. (Fuchs 1994a, 1998a). - Mild cognitive disturbances that often can be detected only by neuropsychologieal assessment; patients with pure delusional disorder tend to have more severe cognitive deficits than those diagnosed as schizophrenic (Naguib and Levy 1987; Howard et al. 1994). Studies using computed tomography and magnetic resonance imaging have demonstrated organic brain disturbances in a majority of patients; patients of this type generally belang to the category of delusional disorders without schizophrenic manifestations of the firstrank (Förstl et al. 1991; Howard and Levy 1992). It is therefore assumed that at least a large fraction of senile paranoid illnesses are caused in part by neurodegenerative processes. This finding is of comparable significance to that of the mainly temporal and prefrontal cerebral abnormalities often found in younger schizophrenies, whieh probably reflect a disturbance of central nervaus system maturation (Häfner 1997). The course of the senile paraphrenias is usually chronie, although hospitalization is only rarely required. In the study by Howard and Levy (1992) of 64 patients, one third responded partially, and one quarter completely, to long-term neuroleptic treatment. The most effective form appeared to be a depot medication in a rather low dose (e.g. 14 mg fiupenthixol or 9 mg fluphenazine decanoate every
2 weeks). Nonetheless, even when neuroleptic therapy is effective, patients rarely gain insight into their illness. Most patients require permanent maintenance therapy (for a review, see Eastham and Jeste 1997).
5 Therapy Building a relationship of trust is the indispensable foundation of successful treatment, regardless of what further measures are taken, and this is especially true for the delusional disorders. The physician must thus avoid a direct confrontation with reality, at least at the beginning, but should also take care not to render additional support to the patient's delusional system by an excess of benevolent understanding or even assent.
5.1 Somatotherapy
There is a serious lack of empirical studies, particularly controlled studies, whieh is not surprising in view of the deficient insight and compliance of this group of patients. There are, however, a number of relatively frequently replicated observations that may be of heuristic value in individual cases. Neuroleptic therapy is often only moderately successful, but if a therapeutie relationship of trust is present, a treatment should be attempted in all cases, at adequate doses and over a sufficiently lang period of time. This may be justified to the patient by explaining that the purpose of medication is to heighten inner stability and to protect agairrst the stresses caused by the (supposed) threats or injuries. Despite such assurances, however, the medication itself or any side effects that may occur are not infrequently viewed by the patient in paranoid fashion. In a number of studies involving relatively few patients, pimozide at a dose of 2-6 mg was found to be an effective agent (Munro 1984, 1988; Pollock 1982; Kaschka et al. 1991); a combination of clomipramine and pimozide may also be useful in depressively colared delusional syndromes (Chiu et al. 1990). Nonetheless, it has not been demonstrated that these agents are fundamentally superior to other highly or intermediately potent neuroleptics. Pimozide may also have cardiotoxie effects in therapeutic doses, whieh may restriet its indications, partieularly for older patients. The frequent association of delusional disorders with latent or manifest depressive mood alterations (64% according to Munro 1988; 51% according to Marino et al. 1993) makes it seem reasonable to try
7 References
antidepressants in many cases. In particular, in both delusional and non-delusional dysmorphophobia, serotonin reuptake inhibitors have been found to be effective in as many as two thirds of all patients (Philips 1996).
cognitive approach at first seems contradictory to the principle of not arguing with the patient, it can be carried out using careful strategies of circumvention and has been reported to infl.uence the course of the illness favorably in as many as half of all patients (Chadwick and Lowe 1990; Kuipers et al. 1997).
5.2
Psychotherapy Psychotherapeutic approaches to the treatment of delusions have aroused interest only in recent years (Torch and Bishop 1981; Teusch et al. 1987; Nelki 1988; Mundt 1996). A durable, long-term relationship of trust is basic to any psychotherapeutic treatment of delusional patients. According to Mundt (1996), focusing directly on the delusion should therefore be avoided whenever possible, and explorations in this direction should not be too frequent. Instead, the delusional theme should be circumvented and thereby deactualized, in a manner similar to behavior-therapeutic extinction. If treatment is based on the conception that the delusion is a concretistic manifestation of a central life concern that has come turned badly for the patient (see Sect. 3.2), then the therapist's task is to direct his or her efforts to the concern underlying the delusion, without naming it as such. An interpretation of the delusion that is unacceptable to the patient should thus be avoided. Instead, the promotion of new experiences in emotionally neutral fields, and acceptance of the patient by the therapist, can implicitly and covertly help to satisfy the patient' s existential needs (e.g. for contact and esteem). It is important in both the pharmacological and the psychotherapeutic treatment of delusional disorders to recall their compensatory function, i.e. the safe removal of topics that cannot be otherwise processed by means of projection or grandiosity. Structural deficits often manifest themselves as depressive syndromes provoked by a sense of emptiness and loss of meaning when the delusion remits, particularly after successful treatment. The treatment should therefore be directed toward topics of practical importance for the patient's life, the mobilization of resources and preserved abilities, and the recognition of the patient's coping capacity, so that the stabilizing function of the delusion can be replaced by a gradual strengthening of the ego. Once this process is in place, reality confrontation can slowly begin. When this stage is reached in patients with delusions, or in milder cases with overvalued ideas, cognitive therapy strategies employing directed experiments in perception and behavior may be beneficial; such strategies are currently being developed and tested in connection with cognitive theories of the origin of delusions (see Sect. 3.2). Although the
6 Course Ever since Kraepelin's time, delusional disorders have been held to be chronic, nearly irreversible diseases: "No case of genuine paranoia ever comes to a eure" (Kraepelin 1899). Yet Retterst0l and Opjordsmoen, in their partly prospective, partly retrospective long-term studies of a total of 334 patients, were able to document a much more favorable prognosis of the paranoid psychoses, including the pure delusional disorders. In the study by Opjordsmoen (1988b) of 41 patients with delusional disorders (according to DSMIII-R) followed up after an average ofthree decades, 15 (37%) had gone into remission. Patients with delusional disorders also differed significantly from schizophrenics with respect to coping with life: they were more frequently married (76% vs. 47%), more frequently had children (73% vs. 37%), and were more frequently employed (46% vs. 26%). Even among 26 patients with classical paranoia in the narrow sense, Retterst0l found that more than one third had gone into remission (Retterst0l 1991a,b). This accords with the finding by Winokur (1977) of a social eure in one third of patients after several years of follow-up; 60% worked in their occupations, and 53% lived in stable marriages. It is to be hoped that the prognosis of the delusional disorders will improve still further as newer, more effective forms of treatment are developed.
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