REVIEW PAPER


Paraphrenia Redefined

Arun V Ravindran, MD, PhD, FRCPC, MRCPsych1, Lakshmi N Yatham, MB, BS, FRCPC, MRCPsych2, Alistair Munro, MD, MPsyMed, FRCPC, FRCPE, FRCPsych3


Background: Paraphrenia is a disorder similar to paranoid schizophrenia but with better-preserved affect and rapport and much less personality deterioration. It is now diagnosed relatively infrequently and is not listed in the current Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) or International Classification of Diseases (ICD-10). However, it appears that some psychiatrists recognize the illness but label it “atypical psychosis,” “schizoaffective disorder,” or “delusional disorder” for lack of a better diagnostic category. Virtually no systematic research on paraphrenia has been conducted in the past 60 years.

Method: The authors distinguish paraphrenia from “late paraphrenia,” a diagnosis used mainly in the United Kingdom, and provide a neo-Kraepelinian description of paraphrenia that would be compatible with the formats of DSM-IV and ICD-10. Using a questionnaire adapted from this description, intake cases in 2 Canadian psychiatric centres (Ottawa [Ontario] and Dartmouth [Nova Scotia]) were surveyed. Cases of paraphrenia were distinguished from those of schizophrenia and delusional disorder and were examined at the time of intake and immediately prior to discharge.

Results: For logistical reasons, collecting a totally consecutive series was not possible. However, during an 18-month period, investigators in both centres identified 33 cases closely fitting paraphrenia. The outstanding features of these cases are enumerated, and an outline description of paraphrenia is derived.

Conclusion: It is possible to define and recognize paraphrenia; it is a viable diagnostic entity. Further research would benefit paraphrenia and schizophrenia patients. Cases in this study have been coded to permit follow-up investigations.

(Can J Psychiatry 1999;44:133–137)

Key Words: paraphrenia, paranoid schizophrenia, atypical psychosis, late paraphrenia, personality preservation, appropriateness of affect

Paraphrenia, a term introduced by Kraepelin (1) to describe “the uncertain group between paranoia and dementia praecox” is now excluded from the International Classification of Diseases (ICD) and Diagnostic and Statistical Manual of Mental Disorders (DSM) series, though the “uncertain group” continues to create problems in diagnosis. In modern terms, paraphrenia should lie on a spectrum between delusional disorder and paranoid schizophrenia, and one of the present authors consistently advocates its return to the diagnostic canon (2).

There has been no systematic research on paraphrenia in the past 60 years, except for on late-onset paraphrenia, a controversial diagnosis whose links with conventional paraphrenia are uncertain (3). Omitted from the DSM-III-R (4), an authority stated paraphrenia would remain excluded until further research was carried out and would meantime be subsumed under schizophrenia (5). Paraphrenia remains absent from the DSM-IV and ICD-10, which only serves to worsen the overinclusiveness of the schizophrenia category and to discourage original research on paraphrenia.

The present study attempts to redefine paraphrenia with modern criteria and to determine whether cases can be recognized according to these criteria.

Paraphrenia: The Concept

Kraepelin believed that paraphrenia was associated with paranoid schizophrenia and was marked by persistent delusions and hallucinations (1), but it did not show the characteristic deterioration of schizophrenia or the full characteristics of delusional disorder (6). Personality decay is minimal (7), and emotional rapport is well retained (8), but despite its relatively benign features (9), paraphrenia is as chronic as schizophrenia (10). Nowadays, a case like this is often diagnosed as “atypical psychosis,” “psychosis not otherwise specified,” or even “schizoaffective disorder” (11). These vague categories do not lend themselves well to research.

Paraphrenia’s decline began in 1921 when Mayer reported that, at follow-up, more than one-half of Kraepelin’s personal cases had deteriorated to schizophrenia (12). That the remaining one-half did retain their original features was downplayed in subsequent writings.

In the United Kingdom, the diagnosis of “late-onset paraphrenia” is used, but it is often difficult to distinguish this from late-onset schizophrenia, and there is an unwarranted implication that paraphrenia is exclusive to the elderly (13). Most authors suggest that paraphrenia is mostly an illness of middle and old age, but in fact, there is little reliable information about age of onset.

There is slight evidence to suggest that paraphrenia occurs only one-tenth as often in an inpatient population as does schizophrenia (14,15). This does not tell us if it is relatively uncommon or if its generally more benign characteristics enable more sufferers to remain outside hospital.

In clinical practice we find cases of “paranoid” disorder that do not present the well-encapsulated delusional system of delusional disorder yet do not appear as profoundly thought- and personality-disturbed as in paranoid schizophrenia. Kraepelinian paraphrenia (1) would be an acceptable diagnosis for these cases if we could show that they form a coherent group. As a first step, we have recast the description of paraphrenia in modern diagnostic terms as follows.

Diagnostic Criteria for Paraphrenia (2,16)

A delusional disorder of at least 6 months’ duration characterized by the following: 1) Preoccupation with 1 or more semisystematized delusions, often accompanied by auditory hallucinations. These delusions are not encapsulated from the rest of the personality as in delusional disorder. 2) Affect notably well-preserved and appropriate. Even in acute phases, there is an ability to maintain rapport with the interviewer. 3) None of the following: intellectual deterioration, visual hallucinations, incoherence, flat or grossly inappropriate affect, or grossly disorganized behaviour at times other than during the acute episode. 4) Disturbance of behaviour is understandable in relation to the content of the delusions and hallucinations. 5) Only partially meets Criterion A for schizophrenia. No significant organic brain disorder.

Associated Features: The illness is associated with distress and agitation, and irrational behaviour may appear as delusions become more vivid and judgement lessens. Patients may accuse others of persecution, complain to the authorities, or occasionally show aggression to imagined pursuers.

Age of Onset: Traditionally thought to be middle or old age, but this is unproven.

Course: A chronic illness, ameliorated but not cured by treatment.

Impairment: Intellectual functioning is unimpaired. Daily living, occupational activity, social functioning, and quality of marriage are likely to deteriorate during exacerbations.

Complications: Some paraphrenia cases appear to deteriorate to schizophrenia. In elderly patients, dementia may sometimes supervene.

Predisposing Factors: Deafness, social isolation, migrant status, and other severe stressors may play a part. It is possible, though evidence is uncertain, that premorbid paranoid and schizoid personality disorders occur more commonly with paraphrenia than by chance. Celibacy, lower-than-normal marital rates, and reduced fertility have been mentioned, possibly indicating abnormal personality traits.

Sex Ratio: Uncertain, but seems to become more common in females with advancing age.

Familial Pattern: There is a low frequency of schizophrenia in families of paraphrenia patients, suggesting that there is little or no genetic link between the 2 disorders.

Differential Diagnoses: Delusional disorder; schizophrenia, especially paranoid schizophrenia; major mood disorder with delusions; dementia; severe schizoid, schizotypal, or paranoid personality disorder; schizoaffective disorder; severe obsessive–compulsive disorder with near-bizarre features and rituals.

Treatment

It can be tentatively said that paraphrenia, like paranoid schizophrenia, responds to standard neuroleptic medications. Behavioural therapy may reduce the degree of delusional preoccupation, but psychotherapy is not of primary value (17).

Treatment Outcome

Clinical outcome is often satisfactory, with a surprisingly complete return to near-normal. However, treatment compliance is not always good, and relapse seems quite common. In older patients, age-related difficulties and adverse personality factors may make social rehabilitation more difficult than clinical improvement.

The Study

The study aimed to determine whether cases that matched our criteria for paraphrenia could be identified in clinical practice and distinguished from other schizophrenia-like illnesses. We estimated that a sample of 20 would be sufficient for our purposes, but in fact, 33 cases were identified during the investigation, and information on these will be presented.

We used 2 psychiatric centres: the adult inpatient services of The Nova Scotia Hospital (NSH), Dartmouth, Nova Scotia, and the Royal Ottawa Hospital (ROH), Ottawa, Ontario. In both hospitals, an intake of patients over 18 months was examined by means of a questionnaire based on our criteria. For logistical reasons, the patient series could not be totally consecutive.

Paraphrenia was defined as a schizophrenia-like disorder of at least 6 months’ duration characterized by the features already noted in the proposed outline diagnostic criteria.

Hypotheses

1) The disorder originally described by Kraepelin as paraphrenia and redefined (with minimal changes) by one of the authors is a recognizable diagnostic entity.

2) Paraphrenia has explicit characteristics that differentiate it from paranoia/delusional disorder and from paranoid schizophrenia (both as defined by the DSM-IV [18]).

Selection of Patients

Patients presenting to the assessment units of 2 major teaching hospitals (Ottawa and Dartmouth) between 8:00 AM and 5:00 PM, Monday to Friday, over 18 months, formed the parent population. Because of other demands on the researchers’ time, the total population could not be surveyed, so neither the parent series nor the paraphrenia cases are consecutive.

Method

Patients in the parent population were screened clinically by means of a standard comprehensive interview schedule administered by a psychiatric nurse. Patients with psychotic symptoms were interviewed by a psychiatrist and, if symptoms were atypical, were administered the Paraphrenia Project Questionnaire. “Atypical” meant that the patients did not meet DSM-III-R criteria for schizophrenia, schizophreniform disorder, delusional disorder, or brief psychotic disorder.

The nonblind investigation did not involve breach of confidentiality, physical intrusion, or any nonroutine psychiatric procedure other than the administration of the Paraphrenia Schedule. Informed patient consent was routinely obtained. The procedure was supervised by one author at the NSH and by another at the ROH, neither of whom had an ideological bias for or against the diagnosis of paraphrenia. The third author, who provided the theoretical background and devised the schedule, took no part in the identification or investigation of the patients.

Results

Patients were positively diagnosed with paraphrenia after all other patients with schizophrenia-like illnesses had been examined and discarded. In some cases, full information could not be obtained (reduced n is shown in brackets). Thirty-three cases matched the criteria for paraphrenia (ROH 19; NSH 14). Of these, 24 (72.7%) were female and 9 (27.3%) male.

Table 1. Sociodemographic factors (n = 33)

Factor
%
Marital Status
Single 30.3
Married 24.2
Separated or divorced 27.3
Widowed 18.2
Education (n = 17)
<Grade 12 23.5
Completed high school 52.9
Postsecondary 23.6
Living alone (n = 19) 55.0
Employed at time of study (n = 19) 19.0
Born outside Canada (n = 19) 42.9

Table 2. Age distribution

Age (years)
At illness onset (%)a
At time of study (%)b
< 30

34.4

12.1

30 to 39

21.9

24.2

40 to 49

25.0

18.2

50 to 59

6.3

21.2

³ 60

12.6

24.2

an = 32; mean age 38.7 years, SD 16.2; average duration of illness 8.9 years.
bn = 33; mean age 47.6 years, SD 16.7.

Table 3. Positive family history of psychiatric illness

Relation

%

Mother

9.7

Father

3.2

Siblings

25.8

Others

3.2

Total

35.5

Table 4. Background factors

Factor

%

Significant sensory deficit

3.0

Significant head injury

0.0

Severe psychological stress prior to current episode

31.3

Social isolation prior to admission (n = 19)

57.1

Prior history of treatment noncompliance

66.0

Admitted compulsorily in current episode

41.0

Previous positive response to neuroleptics

77.8

Table 5. Associated clinical features in active phase

Feature

%

Feelings of intimidation and persecution

96.9

Hallucinations (often vivid)

63.6

Agitation

90.6

Irrational behaviour due to delusions

81.3

Made threats or behaved aggressively

29.0

Complained to authorities prior to admission

40.6

Current substance abuse

12.1

Table 6. Features noted at discharge, following adequate
neuroleptic treatmenta

Feature

%

Return to near-normal thinking

65.4

Delusions no longer apparent

57.7

Appropriate affect

65.4

Good behaviour control

76.9

Improved reality testing

69.2

Apparent insight

50.0

Reasonably realistic planning for future

53.8

Improvement in rapport

58.3

Significant residual symptoms

26.9

aFor all items except “significant residual symptoms,”
the percentage represents cases with “marked” improvement.

Discussion

Although our case series is small (33 cases) our 2 hypotheses were readily confirmed. A filtering method with consecutive interview and questionnaire was used to identify cases that matched our criteria for paraphrenia and to distinguish these cases from delusional disorder and paranoid schizophrenia. All of these cases had been previously diagnosed as other than paraphrenia (usually delusional disorder, paranoid schizophrenia, or depressive disorder/dysthymia). Given adequate criteria such as provided here, any competent diagnostician can recognize a case of paraphrenia.

Of course, the small sample size makes generalization of our additional findings tentative, but the following results are of some definite interest.

1. Paraphrenia does not predominantly occur in elderly persons, despite traditional views to the contrary. About one-third of cases commence prior to age 30 years and more than 80% before age 49 years. (Note that a similar finding is true of delusional disorder) (19).

2. Until this study was carried out, these patients were diagnosed with various other things, especially delusional disorder, paranoid schizophrenia, and depression/dysthymia. The first 2 diagnoses are approximations; previous clinicians were at least placing the patients within the paranoid spectrum. Many paraphrenia patients are dysthymic, and it is not surprising that in the early phase of the illness this symptom might overshadow others, particularly if psychotic symptoms were not yet prominent.

3. The group is reasonably well-educated but markedly unsuccessful in employment terms.

4. Many of these patients show a degree of personality and interpersonal incompetence, since more than one-half of the group live alone and well over one-half (average age 47.6 years) are single, separated, or divorced; 57.1% reported themselves as socially isolated prior to the onset of the current episode.

5. Nova Scotia is a relatively economically depressed area and attracts few immigrants, but the capital region has a high immigration rate. Nevertheless, the percentage of Ottawa patients born outside Canada (42.9%) seems strikingly high.

6. Because of the nature of the illness, it is not surprising that a high proportion of patients in the acute phase of their illness are agitated, experience feelings of intimidation and persecution, have hallucinations, and act irrationally because of their delusions. However, despite the bad reputation of paranoid illnesses, less than one-third had made threats or behaved aggressively in the current episode. Conversely, 40% had acted out their delusions by complaining to the authorities, and a similar proportion required compulsory admission.

7. The present study did not investigate details, but 35.5% if patients reported a positive family history of psychiatric disorder. We cannot assert whether (as reported elsewhere) schizophrenia is uncommon in the background of paraphrenia patients. However, there was some indication that psychiatric illness in the family seems to be associated with an earlier onset of paraphrenia.

8. The frequency of substance abuse in paraphrenia subjects is not significantly higher than in the general population.

9. Abnormal sensory deficit was noted in only 1 patient, and 0 patients had ever experienced a head injury. The former is particularly interesting since deafness and, to a lesser extent, blindness are commonly cited as predisposing factors in delusional illnesses.

10. Almost one-third of the patients reported an episode of severe stress prior to their current illness onset, but specific details were not sought.

11. Outcome of treatment for the present episode is especially interesting. This group of patients was diagnosed with paraphrenia because of such features as well-preserved and appropriate affect and good rapport. Otherwise, many features were common to paranoid schizophrenia, but short-term treatment results in paraphrenia seem better than are usual in paranoid schizophrenia. Paraphrenia patients generally showed a return to good personality functioning, good behavioural control, better reality testing, more normal thinking, and well-preserved affect. In addition, 77.8% had made very considerable recoveries from previous episodes with the use of neuroleptic medication.

However, paraphrenia is chronic and is also a paranoid illness. Our data showed that 66% of patients had previously not complied with treatment, and at the time of discharge from hospital for the present episode, we observed that 50% had significant lack of insight, more than 40% still had recognizable delusions, and 25% retained marked residual symptoms of illness. Long-term follow-up is required to see whether such negative features tend to clear gradually if patients adhere to treatment. Our patients were identified and coded so that follow-up of this sort is feasible.

Conclusions

Apart from a small literature on late-onset paraphrenia (3), whose links with paraphrenia as a whole are unclear, there has been virtually no systematic work on paraphrenia in the past half-century or more. The present small study recapitulates Kraepelin’s early 20th century observations (1) and easily identifies cases of the disorder in 2 separate psychiatric centres. It is a first-stage investigation, and others should be encouraged to investigate the area further.

One of the authors for many years has advocated paranoia as a viable diagnosis and has seen it reconceptualized as delusional disorder in the DSM and ICD (20). For a very long time, cases of delusional disorder were diagnosed as schizophrenia, an error that is now unacceptable. There are other types of “paranoid disorder” that do not fit into the DSM-IV delusional disorder category and which are not schizophrenic, yet these often are labelled as paranoid schizophrenia or schizoaffective disorder for want of a specific diagnostic niche.

Our results propose that paraphrenia (“that uncertain group”) is a recognizable disorder and that practitioners bent on accurate diagnosis should be able to designate it as such. This would enhance research and clinical care in the field of paraphrenia. Removing a subgroup from the heterogeneous category we call schizophrenia would also make research on that disease more straightforward.

Our findings confirm previous impressions that paraphrenia, though potentially a “good outcome” psychosis when treated with standard neuroleptics, is nevertheless associated with considerable noncompliance and recidivism. A relative lack of personality disintegration may disguise a lack of insight or noncompliance, and prolonged and close follow-up of individuals with paraphrenia is warranted.

Clinical Implications

Limitations

References

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2. Munro A. A plea for paraphrenia. Can J Psychiatry 1991;36:667–72.

3. Almeida OP, Howard R, Förstl H, Levy R. Late paraphrenia: a review. Int J Geriatr Psychiatry 1992;7:543–8.

4. American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 3rd ed. Revised. (DSM-III-R). Washington (DC): American Psychiatric Press; 1987.

5. Williams JBW. Psychotic and mood disorders. Hospital and Community Psychiatry 1987;38:13–4.

6. Anderson EW, Trethowan WH. Psychiatry. 3rd ed. London: Baillière Tindall; 1973. p 165.

7. Arieti S. Interpretation of schizophrenia. New York: Brunner Mazel; 1955. p 11.

8. Curran D, Partridge M. Psychological medicine. 6th ed. Edinburgh: Livingstone; 1969. p 190.

9. Lewis A. Paranoia and paranoid: a historic perspective. Psychol Med 1970;1:2–12.

10. Bleuler E. Dementia praecox or the group of schizophrenias. Zinkin J, translator. New York: International Universities Press; 1950.

11. Dahl AA. Problems concerning the concept of reactive psychoses. Psychopathology 1987;20:79–86.

12. Mayer W. On paraphrenic psychoses. Zentralblatt für die Gesamte Neurologie und Psychiatrie 1921;71:187–206.

13. Grahame PS. Late paraphrenia or the paraphrenias. Br J Psychiatry 1987;151:268–9.

14. Fenton WS, McGlashan TH, Heinssen RK. A comparison of DSM III and DSM III R schizophrenia. Am J Psychiatry 1988;145:1446–9.

15. Munro A. Classification of patients not meeting DSM III R criteria for schizophrenia [letter]. Am J Psychiatry 1989;146:816–7.

16. Munro A. Paraphrenia. In: Bhugra D, Munro A, editors. Troublesome disguises: underdiagnosed psychiatric syndromes. Oxford: Blackwell Science; 1997. p 91–111.

17. Kingdon D, Turkington D, John C. Cognitive behaviour therapy of schizophrenia. Br J Psychiatry 1994;164:581–7.

18. American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 4th ed. (DSM-IV). Washington (DC): American Psychiatric Association; 1994.

19. Munro A. Delusional hypochondriasis. Clarke Institute of Psychiatry Monograph Series #5. Toronto: Clarke Institute of Psychiatry; 1982.

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Résumé

Historique : La paraphrénie est un trouble semblable à la schizophrénie paranoïde mais dans lequel l’affect et les rapports sont mieux préservés, et la personnalité, beaucoup moins détériorée. À l’heure actuelle, elle n’est pas souvent diagnostiquée et ne fait pas partie de la liste de la quatrième édition du Manuel diagnostique et statistique des troubles mentaux (DSM-IV), ou de la Classification internationale des maladies (CIM-10). Toutefois, il semble que certains psychiatres reconnaissent la maladie mais qu’ils l’étiquettent « psychose atypique », « trouble schizo-affectif » ou « trouble délirant », à défaut d’une meilleure catégorie diagnostique. Presque aucune recherche systématique sur la paraphrénie n’a été menée dans les 60 dernières années.

Méthode : Les auteurs distinguent la paraphrénie de la « paraphrénie avancée », diagnostic utilisé principalement au Royaume-Uni, et ils présentent une définition néo-kraepelinienne de la paraphrénie qui serait compatible avec les formats du DSM-IV ou de la CIM-10. À l’aide d’un questionnaire adapté de cette description, on a interrogé les patients hospitalisés dans deux centres psychiatriques du Canada [Ottawa (ON) et Dartmouth (N.-É.)]. Les cas de paraphrénie ont été distingués des cas de schizophrénie et de trouble délirant, et ont été examinés au moment de l’hospitalisation et immédiatement avant le congé.

Résultats : Pour des raisons logistiques, il a été impossible de recueillir une série complètement consécutive. Pendant 18 mois cependant, les chercheurs des deux centres ont identifié 33 cas qui correspondaient étroitement à la paraphrénie. Les caractéristiques particulières de ces cas sont énumérées, et une description préliminaire de la paraphrénie en découle.

Conclusion : Il est possible de définir et de reconnaître la paraphrénie ; c’est une entité diagnostique viable. D’autres recherches profiteraient aux patients souffrant de paraphrénie et de schizophrénie. Les cas de la présente étude ont été codés afin de permettre un suivi de la recherche.

Manuscript received January 1998, revised, and accepted June 1998.

1Director of Research, Royal Ottawa Hospital, Ottawa, Ontario.

2Staff Psychiatrist, University Hospital, University of British Columbia Site, Vancouver, British Columbia.

3Emeritus Professor of Psychiatry, Dalhousie University, Halifax, Nova Scotia.

Address for correspondence: Dr A Munro, 1759 Connaught Avenue, Halifax, NS  B3H 4C9

Can J Psychiatry, Vol 44, March 1999