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6 The Course and Clinical Variation of the Schizophrenia Spectrum Disorders

6 The Course and Clinical Variation of the Schizophrenia Spectrum Disorders

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150



8 Indicators of Psychosis



and “disorganized” schizotypy being equal to schizophrenia. This model was

endorsed and carried on by Meehl in his schizotaxia-schizotypy-schizophrenia

model (1962, 1989, 1990). Schizotaxia is a putative neural integrative defect

underlying the development of schizotypy. Parnas has suggested a fourth intermediate level to their model, semi-decompensated schizotypy, occasionally seen as

patients (personal communication). An empirical study (Raballo and Parnas 2011)

using self-disorders as a candidate vulnerability phenotype in a sample of nonpsychotic, genetically high-risk subjects demonstrates their distribution following an

incremental pattern from non-patients to schizotypes, thereby supporting Meehl’s

model. The delimitation of schizophrenia from schizotypy is arbitrary. Around 40

different definitions of schizophrenia and related psychoses have been suggested

in the course of the twentieth century, each having different psychopathological

thresholds, frequencies, sex rate, etc. (Jansson and Parnas 2007). Polydiagnostic

studies (ibid.; Berner et al. 1982) applying the criteria of different definitions to

the same population show that they are overlapping but describing different

subgroups.

A twin study (Torgersen et al. 1993) suggests that the “negative” DSM-III-R

criteria of schizotypy, odd speech, inappropriate affect, odd behavior, and excessive

social anxiety, more or less corresponding to Bleuler’s mostly expressively defined

fundamental symptoms, are significantly more common in co-twins and other relatives of schizophrenic probands, whereas the “positive” criteria appear to be

nonspecific.

Hebephrenia-like types of schizotypy have been described by several authors.

Kahlbaum (2002/1889) introduced his term, heboidophrenia (with the adjective:

“heboid”), designating a mild mental illness in young people leading to antisocial

behavior and resembling hebephrenia (“Jugendhalbirresein” or “half-hebephrenia”).

This is probably the earliest description of schizotypy. Kretschmer has a similar

subgroup of schizoid (schizotypal) patients named the “cold despotic type” or

“moral idiots” (1925, p. 194ff.), sharing many of the characteristics with the “pseudopsychopathic schizophrenia” (Dunaif and Hoch 1955). These types of schizotypy are often mistaken for personality disorder, especially antisocial and

borderline.

ICD-10 schizotypal disorder defines a productive and even near-psychotic state,

whereas in DSM-IV and DSM-5, schizotypal personality disorder (SPD), as indicated by the name, is considered a personality disorder, and, therefore, micropsychotic episodes are left out from the criteria (but several near-psychotic phenomena

are retained, anyway). In DSM-5 SPD is also mentioned in the schizophrenia chapter to indicate a relation to this disease. Schizotypy, as defined by these operationalized systems, may actually cover various clinical states: stable clinical schizotypy

(corresponding to Rado’s decompensated level), premorbid and prodromal schizophrenia (DSM-5 allows the addition “premorbid”), and a symptom-poor (subclinical, see below) variety of schizophrenia (simple schizophrenia is not covered by

DSM). The latter two variants imply a deterioration from the premorbid condition.

ICD-10 schizotypal disorder has a 2-year duration criterion, allowing such



8.6



The Course and Clinical Variation of the Schizophrenia Spectrum



151



Table 8.4 A comparison of schizotypal criteria



General conditions



Ideas of reference

Odd beliefs or magical

thinking

Unusual perceptual

experiences including

bodily illusions

Odd thinking and speech

Suspiciousness or

paranoid ideation

Inappropriate or

constricted affect

Behavior or appearance

that is odd, eccentric, or

peculiar

Lack of friends and social

withdrawal

Excessive social anxiety

Ruminations without inner

resistance

Occasional transient

quasi-psychotic episodes

(micropsychoses)

Exclusion criteria



DSM-5

Schizotypal personality disorder

A pervasive pattern of social and

interpersonal deficits beginning

by early adulthood

Five or more criteria:

Yes

Yes



ICD-10

Schizotypal disorder

Continuous or repeated

manifestations for at least

2 years

Four or more criteria:

No

Yes



Yes



Yes

No



Yes

Also including other

illusions, depersonalization,

or derealization

Yes

Yes



Yes



Yes



Yes



Yes



Lack of close friends or

confidants other than first-degree

relatives

Yes

No



Poor rapport with others and a

tendency to social withdrawal

No

Yes



No



Yes



Does not occur exclusively

during the course of

schizophrenia, a bipolar disorder

or depressive disorder with

psychotic features, another

psychotic disorder, or autism

spectrum disorder



The subject must never have

met the criteria for any

disorder in F20

(schizophrenia)



prodromal and symptom-poor cases to receive the diagnosis, while cases with a

shorter duration of symptoms fail to do so:

A 28-year-old man at the emergency unit recounts alarming changes during the last

1½ years: social anxiety even with relatives and a cenesthetic feeling of his head shaking,

which he names “a shaky head”. There is subtle formal thought disorder. He does not fulfill

the diagnostic criteria for any schizophrenia spectrum disorder and is given a social anxiety

diagnosis. One year later a persecutory psychosis breaks out.



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8 Indicators of Psychosis



DSM-5 SPD, defined as a personality disorder setting in by early adulthood, also

fails to diagnose such cases. Table 8.4 compares the diagnostic criteria of the two

diagnostic systems.

Schizophrenia is often depicted as a chronic illness with a “natural” deteriorating

course (cf. Lewis and Lieberman 2000). The long-term outcome of schizophrenia

was examined in several studies during the twentieth century (Häfner and an der

Heiden 2003). Among these are prospective follow-up studies by Manfred Bleuler

(over more than 20 years; Bleuler 1978) and by Ciompi and Müller (over 37 years;

Ciompi and Müller 1976). The general picture is a great diversity of types of onset

(acute, insidious), course (single episode, episodic, chronic), psychopathological

profiles, and outcome. The proportion of recovered patients is between 21 % and

30 % in the major group of studies (Häfner and an der Heiden 2003).

Hebephrenia was first described by Hecker in 1871 (Hecker and Kraam 2009a,

b). The description contained all basic features of future schizophrenia in nuce, and

it was a major source for Kraepelin to his dementia praecox concept, as well as for

Bleuler to his fundamental symptoms. Hecker envisaged hebephrenia to emerge

from the psychological regeneration and transformation of the self in the “hobbledehoy stage” of the teenage years (Lümmeljahre in boys and Backfischalter in girls;

cf. Stanghellini 2004b), but the temporal association of hebephrenia with puberty

cannot always be demonstrated (as already pointed out by Daraszkiewicz in 1892

(2005)).

Blankenburg (1971) has explored the symptom-poor varieties of schizophrenia.

The fundamental defect of schizophrenia, he writes, is the loss of natural evidence,

an aspect of the loss of common sense underlying all schizophrenic psychopathology. These patients may appear unobtrusive with apparently nonspecific symptoms.

Blankenburg demonstrates that such nonspecific symptoms may cover more specific, underlying phenomena. A trivial (nonspecific) complaint of fatigue turns out,

on closer evaluation, to be caused by a pervasive inability to grasp the everyday

significations of the world and a correlated perplexity (also an aspect of loss of common sense), hence “specific” (Parnas and Sass 2001). In the era of operationalization, this level of psychopathology is neglected.

In comparison with the almost universally accepted standard psychosis, paranoid

schizophrenia, disorganized or hebephrenic schizophrenia is relatively symptom

poor and, therefore, increasingly ignored and misdiagnosed, too. In the last 30 years,

the percentage of such cases in Denmark has dropped from about 20 % to 30 % to

less than 1 % (Parnas 2011). The question is what has happened to these patients.

An informed guess is that they are re-diagnosed chiefly as borderline patients due to

a superficial resemblance (impulsivity, self-destructive behavior, emotional instability; for the differential diagnosis, see Sect. 12.4 and Table 12.2) and that their discreet and fleeting psychotic symptoms are being played down, disregarded, or

interpreted as “dissociative.” But the proper reason seems to be even graver: the

schizophrenic Gestalt has been lost from psychiatry. The problem that clinicians fail

to recognize hebephrenia is not quite new; more than a hundred years ago, Wilmanns

(1906) regretted that many petty criminal vagabond patients were not diagnosed

until they had spent a long time in prison. Their diagnoses were mainly hebephrenic

and catatonic dementia praecox (schizophrenia).



8.6



The Course and Clinical Variation of the Schizophrenia Spectrum



153



The first symptom-poor form of schizophrenia (also known as abortive or larvate

schizophrenia or a forme fruste of schizophrenia) to be described, apart from the latent

and hebephrenic forms, was the simple form (schizophrenia simplex; Diem 1903,

1987). This form has not been acknowledged by the DSM since the second edition.

Although still part of the ICD schizophrenia definition, it seems to have suffered the

same fate as the hebephrenic subtype, and if still in some use, it is reserved for cases

developing severe negative symptoms. The original definition was, however, much

broader. Diem found a number of features common to all these cases with an onset

soon after puberty:

[A]n individual becomes unstable, lacking in willpower and self-control, and wanders

around aimlessly, often finishing up after a period of vagrancy in his home district. In many

cases there is an unmistakable decline in performance, a narrowing of mental horizons and

a restriction of thinking; they cannot carry out their former jobs adequately and have to be

replaced because their work is unsatisfactory. A change in personality takes place, the most

prominent feature of which is that an individual becomes more excitable and quarrelsome,

often lascivious, and is never content, but finds something to criticise in everything…

(Diem 1987)



No wonder, such cases are often misdiagnosed with personality disorder, but

what is important here is the change in thinking and performance after puberty precluding a personality disorder. Diem’s description of simple schizophrenia is a quite

disorganized state that has a great resemblance with hebephrenia (Schneider regards

them as the same type, 1959, p. 91). ICD-10 definition does, in fact, include these

largely ignored hebephrenia-like features among its diagnostic criteria:

A significant and consistent change in the overall quality of some aspects of personal

behaviour, manifest as loss of drive and interests, aimlessness, idleness, a self-absorbed

attitude, and social withdrawal. (p. 68)



Closely related to simple schizophrenia is what has been named the evolutive

schizoidia (Ey 1996, p. 254), severe schizotypy with an aggravating course, or an

“attenuated” form of schizophrenia. In rare cases, in which the psychotic phase has

been overlooked, the clinical picture of residual schizophrenia is interpreted as simple schizophrenia (without such a phase). As negative symptoms have superficial

likeness to depressive symptoms, the simple form of schizophrenia is often diagnosed as depression:

A woman in her mid-twenties starts out her university studies but after a couple of years she

grinds to a halt and fails to appear at the lectures. She cannot concentrate on reading and

doesn’t even manage to maintain her daily routines but spends most of her time in bed. She

is treated with antidepressants as an outpatient for four years with little success. In hospital

she doesn’t give the impression of a true depressive mood. The psychological testing shows

subtle formal thought disorder. She is diagnosed with simple schizophrenia.



For the differential diagnosis of schizophrenia and core depression, see Sect. 9.2

and Table 9.1.



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8 Indicators of Psychosis



A further number of subclinical forms have been identified (Huber et al. 1982;

Gross 2001). Cenesthopathic schizophrenia was described by Huber (1957) and

Huber et al. (1982) as a condition dominated by cenesthesias (bodily sensations;

Dupré 1974; Gross et al. 2008): inexplicable pains, burning sensations, levitation

feelings, motor weakening, kinesthesia, etc., closely connected with affective

changes and vegetative, motor, and sensory disturbances. These complaints may

persist for many years, first-rank symptoms and other manifest psychotic symptoms

are transient or absent, and overt psychosis seldom evolves. The patient may appear

hypochondriac, anguished, asthenic, or querulous and is often misdiagnosed.

The endogenous juvenile-asthenic failure syndrome (Glatzel and Huber 1968;

Huber et al. 1982) usually starts before age 20 with cenesthesias and, after some

time, the supervention of persisting or paroxystic depersonalization and derealization (mimicking psychomotor epilepsy, cf. Sect. 7.5.3) followed by subjective disorder of thinking (thought intentionality). This syndrome is more common in males.

Half of the patients later become psychotic.

The endogenous obsessive-compulsive disorder (Gross et al. 1988), or malignant

obsessive-compulsive syndrome, is characterized by severe egosyntonic obsessivelike phenomena difficult to distinguish from delusions and resistant to therapy. The

age of onset in most cases is before 20, the course is progressive, and the prognosis

is sinister. Obsessive-compulsive phenomena are discussed in Sect. 10.4.

In some cases, the subclinical forms turn out to be prodromal states as they are

followed by a schizophrenic psychosis. Prodromes are initial phases of schizophrenia without psychotic symptoms, often with cenesthetic, neurasthenic, or depressive

features. In about half of the cases, schizophrenia starts with a depressive prodrome,

the initial depression (Häfner et al. 2005; Conrad 1958; see also Sect. 9.2.4). The

duration ranges months to years. In some cases, the psychosis is preceded by a

reversible prodrome-like phase, the outpost syndrome. This syndrome has been

reported in 15 % of patients with schizophrenia, in average 10 years prior to the

psychosis (Huber et al. 1979; Gross et al. 2008).

There is some clinical variation of schizophrenia across different cultures both

with regard to course and psychopathology. In the WHO Ten-Country Study, schizophrenia patients in the developing countries were found to have a markedly better

prognosis than patients in the developed countries (Jablensky et al. 1992). In another

comparative study, Californian patients tend to hear voices with a violent content and

think of themselves as crazy hearing voices, whereas West African and South Indian

patients tend to experience their voices as positive and helpful (Luhrmann et al. 2015).



8.7



The Problems of Early Detection of Schizophrenia



The theory of toxicity of psychosis (Wyatt 1991) has informed research in the early

detection of schizophrenia and other psychoses with the aim to reduce the DUP,

duration of untreated psychosis. For the purpose of secondary prevention, detection

of prodromal schizophrenia has been given high priority.



8.7



The Problems of Early Detection of Schizophrenia



155



The ICD and DSM definitions of schizophrenia are arbitrary, resulting from

expert consensus, and the time of onset varies depending on when the criteria

threshold is exceeded: first-onset episodes by one diagnostic system may be considered pre-onset by a different system (Parnas 2005). The operational definitions

(DSM-IV and ICD-10) rely heavily on manifest, less specific psychotic symptoms,

including first-rank symptoms, whereas the preoperational definitions reflect to a

greater extent the schizophrenic Gestalt.

In DSM-IIIR (p. 194–195) prodromal symptoms (identical with residual symptoms) comprise a list of nine mostly behavioral signs, at least two of which must be

present for a prodromal phase of schizophrenia:

1.

2.

3.

4.

5.

6.



Marked social isolation or withdrawal

Marked impairment in role functioning as wage earner, student, or homemaker

Markedly peculiar behavior

Marked impairment in personal hygiene and grooming

Blunted or inappropriate affect

Digressive, vague, overelaborate, or circumstantial speech, or poverty of speech,

or poverty of content of speech

7. Odd beliefs or magical thinking, influencing behavior and inconsistent with cultural norms, e.g., superstitiousness, belief in clairvoyance, telepathy, “sixth

sense,” “others can feel my feelings,” overvalued ideas, ideas of reference

8. Unusual perceptual experiences, e.g., recurrent illusions, sensing the presence of

a force or person not actually present

9. Marked lack of initiative, interests, or energy

Empirical studies have found a high prevalence of these items in high-school students rendering them nonspecific and of little value for prodromal research (McGorry

et al. 1995; see also Sect. 13.1). The prodrome is identified retrospectively. An observed

change in psychopathology and functioning in a patient displaying schizophrenia spectrum features might also be expressive of an accidental crisis, drug use, and an outpost

syndrome (see above) or be the onset of a symptom-poor schizophrenia (e.g., simple

subtype), and it may be difficult to tell them apart. In prodromal research, the term “at

risk mental state” (ARMS) is, therefore, preferred to “prodrome” (Yung et al. 1996).

Early detection instruments attempt to define prodrome/ARMS operationally.

The CAARMS (Yung et al. 2006) define ARMS in three ways: states (1) family history of psychosis or SPD plus 30 % drop in SOFAS (Social and Occupational

Functioning Assessment Scale, Goldman et al. 1992), (2) attenuated psychosis

defined from subthreshold intensity and frequency ratings of psychosis items, and

(3) BLIPS (brief limited intermittent psychotic symptoms), defined as a psychopathological scale score, resolving spontaneously within a week occurring during

the last year. The first group is not clinically but probabilistically defined, the two

next groups defined as subscale ratings. Psychosis, too, is defined as a cutoff score

in certain subscales.

Early detection using near-psychotic or brief psychotic features centers on the

late-prodromal, prepsychotic phase and is liable of becoming tautological (mild



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