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General Hospital Psychiatry 23 (2001) 285–293

Emergency Psychiatry in the General Hospital


The emergency room is the interface between community and health care institution. Whether through outreach or in-hospital service, the
psychiatrist in the general hospital must have specialized skill and knowledge to attend the increased numbers of mentally ill, substance
abusers, homeless individuals, and those with greater acuity and comorbidity than previously known. This Special Section will address those
overlapping aspects of psychiatry, medicine, neurology, psychopharmacology, and psychology of essential interest to the psychiatrist who
provides emergency consultation and treatment to the general hospital population.

Asperger’s disorder in the emergency psychiatric setting


Michele Raja, M.D.*, Antonella Azzoni, M.D.
Dipartimento di Salute Mentale, Ospedale Santo Spirito, Rome, Italy

Abstract
Asperger’s syndrome (AS) is a pervasive developmental disorder that may be unrecognized, especially if signs of other psychiatric
disorders coexist. The objectives of this paper are: 1) to ascertain the prevalence of AS in the emergency psychiatric setting; and 2) to
describe features of AS which may help to differentiate these patients from patients with psychotic disorders. Among 2500 patients admitted
to a psychiatric intensive care unit, 5 (0.2%) received a diagnosis of AS, for the first time. Besides impairment of social interaction, common
features were the following: male gender, left handedness, obsessive-compulsive symptoms, cognitive hyper-abilities, violent behavior,
sense of humor, low WAIS total score, high WAIS verbal/performance score ratio, unusual, restricted interest and clumsiness. Comorbid
schizophrenia is difficult to rule out in these patients. Psychotic symptoms should not be overvalued in making the diagnosis when specific
features of AS are present. © 2001 Elsevier Science Inc. All rights reserved.

Keywords: Asperger’s disorder; Autism; Psychotic disorders; Diagnosis; Psychiatric emergency

1. Introduction a pervasive developmental disorder (PDD), was introduced


as a new diagnostic category in the ICD-10 and the DSM-
Autism is a behaviorally defined syndrome characterized IV. It refers to people with autistic, odd and eccentric
by deficits in cognitive and social functioning and by ritu- behavior but well-developed language skills. However, the
alistic and compulsive behaviors. It begins in infancy or speech of Asperger individuals is often characterized as
early childhood and may be associated with rare diseases, tangential, pedantic, repetitive, or one-sided [1]. Probably,
whose etiologic role is unclear. The condition was described the syndrome Hans Asperger originally described may not
almost simultaneously by Kanner in Baltimore (in 1943) be captured by present diagnostic DSM-IV or ICD-10 cri-
and Asperger in Vienna (in 1944). Kanner reported the teria.
observation of exceptional children who appeared to be Within PDDs, DSM-IV identifies 5 subgroups. However,
asocial, lacking in communicative skills both verbal and the diagnosis of the different subgroups is difficult to estab-
nonverbal, and committed to repetitive ritualistic behaviors. lish, particularly between Autistic Disorder and AS. Ac-
He ascribed the condition to psychosocial factors. Asperger cording to ICD-10, AS is characterized by an impairment of
observed somewhat older children, less severely affected reciprocal social interaction, a lack of general delay in
and ascribed the condition to metabolic abnormalities. In language or cognitive development and restricted patterns
the decades since, opinion varied as to the relationship of behavior and interests. Although AS has been included in
between the severe Kanner syndrome and the less severe the ICD-10 as a distinct category, it is still unclear to what
Asperger syndrome. extent it differs from high functioning autism (HFA) [2–5].
In the last years, Asperger’s syndrome (AS), considered Furthermore, there is no consensus about the validity and
the diagnostic boundaries of other disorders characterized
by similar symptoms, including Nonverbal learning disabil-
* Corresponding author. Tel.: ⫹39-065898721; fax: ⫹39-065898721. ity, Developmental Gerstmann syndrome, right hemisphere
E-mail address: raja.russo@iol.it (M. Raja). syndrome, and right parietal lobe syndrome.

0163-8343/01/$ – see front matter © 2001 Elsevier Science Inc. All rights reserved.
PII: S 0 1 6 3 - 8 3 4 3 ( 0 1 ) 0 0 1 5 5 - 4
286 M. Raja, A. Azzoni / General Hospital Psychiatry 23 (2001) 285–293

Typically, patients with AS have characteristics such as disorders. His delivery at term was uneventful, except for a
motor clumsiness, pedantic speech, eccentricities, emo- prolonged labor. In early childhood, his parents noted
tional lability, anxiety, poor social functioning, impulsivity, speech delay, left-handedness and impairment in social in-
intrusive repetitive behavior and fixed habits that can mimic teraction, stereotypies, clumsy movements, enuresis and
symptoms of other illnesses, including schizophrenia, bipo- polydipsia. The patient had an extravagant interest in a little
lar disorder, anxiety disorder and obsessive-compulsive dis- puppet. Visual memory and mathematical intuitions were
order (OCD). Their disorganizing anxiety in response to prodigious. Attention and concentration were poor. In early
stress, which may be accompanied by increased oddness of teens, an unusual interest in horror films appeared, while
speech, can easily be misinterpreted as psychosis [6]. polydipsia, polyuria and nocturia continued to be present.
Only a small proportion of patients with childhood au- At age 15, his psychiatric symptoms were bizarre ideation
tism lead independent adult lives. Most subjects affected by on themes of horror, sexuality and persecution, obsessions
severe forms of autism live in institutions and are unlikely of violence and stereotyped behavior. Because of social
to attend services of general psychiatry. Other patients with withdrawal, abnormal ideas and hostility against his parents,
so-called HFA or AS improve enough to live independent the patient started neuroleptic treatment with moderate im-
adult life. These subjects may come in contact with general provement. Polydipsia worsened up to 15–20 L/day. A
psychiatry services in case of exacerbations of their illness magnetic resonance imaging (MRI) study demonstrated an
or other neuropsychiatric comorbid disorder. The first case empty sella and a normal brain; in the T1-weighted MRI
of AS we admitted to our ward raised a diagnostic contro- sagittal image, the hyperintense signal denoting a normal
versy among staff members that prompted us to focus at- neurohypophysis was absent. At age 31, the patient rejected
tention on the syndrome. Since then, we prospectively his parents claiming they were not his mother and his father.
searched for similar cases among patients admitted to our He menaced them with a knife and it was necessary to call
Psychiatric Intensive Care Unit (PICU). The objectives of police. Other episodes of aggression were directed against
this paper are: 1) to ascertain the prevalence of AS in a unknown people with bizarre explanations (“I think it my
PICU; and 2) to describe the features of AS which may help duty to assault people practicing martial arts”). In the last
to differentiate these patients from patients with psychotic years, a tendency to overturn normal rhythms of daily life,
disorders. in particular the sleep-wake cycle, had replaced the propen-
sity to violent behavior. Curiously, he was exceedingly able
to imitate famous actors. On admission, neuroleptics were
2. Case reports withdrawn. The patient had a high school diploma and
intermittently worked as a clerk in a public institution. The
As part of standard clinical care on the PICU, we ascer- neurological examination was normal, however, he was
tained basic characteristics for each admitted patient as clumsy, uncoordinated, had a poor gait, and presented trunk
follows: sex, age, years of education, employment, marital swinging. The Wechsler Adult Intelligence Scale (WAIS)
status and social class. Psychopathology was assessed using revealed an I.Q. of 79 (verbal: 96; performance: 59). Psy-
the Brief Psychiatric Rating Scale [7], the Scale for the chiatric examination revealed bizarre ideas (“I want to drink
Assessment of Positive Symptoms [8], the Scale for the the ocean”), motor stereotypies, fatuous attitude, unreason-
Assessment of the Negative Symptoms [9], the Mini Mental able insistence on particular subjects and a special interest
State Examination (MMSE) [10] and the Global Assess- in knives. The patient seemed over-concerned to the police-
ment of Functioning Scale (GAF) [11]. Neurological exam- women and asked bizarre questions on that theme (“Are
ination included the use of the Abnormal Involuntary Move- they armed with pistol?”, “Do they ride?”). Bizarre perse-
ment Scale (AIMS) [12], the Unified Parkinson’s Disease cutory delusions were also present. He claimed he had
Rating Scale (UPDRS) [13] and the Barnes Akathisia Scale declared war on the world in the past years and, for that
[14]. We made psychiatric diagnoses according to DSM-IV. reason, a policewoman had sent some birds to devour him.
At the end of each individual clinical evaluation, we met to
formulate consensus diagnoses. We found 5 patients af- 2.2. Case 2
fected by AS whose features we describe herein.
A 20-year-old boy with a diagnosis of “paranoid disorder
2.1. Case 1 with obsessive-compulsive ideation” was referred to our
ward because of violent behavior against his relatives. In the
A 33-year-old man, who had received a diagnosis of previous days, the patient had been taking risperidone 1
early onset psychosis, was referred for evaluation of his mg/day, thioridazine 50 mg/day, and chlor-demetil-diaze-
psychiatric disorder and of a severe polydipsia-polyuria pam 20 mg/day. There was no family history of psychiatric
syndrome. The case has already been published for the disorder. Two patient’s siblings were affected by adrenal
association of autistic-spectrum disorder, empty sella, neu- insufficiency and abnormal sexual development (inherited
rogenic diabetes insipidus and primary polydipsia [15]. deficiency of 21-␣-hydroxylase). The patient’s delivery at
There was no family history of endocrine or psychiatric term was uneventful and psychomotor development was
M. Raja, A. Azzoni / General Hospital Psychiatry 23 (2001) 285–293 287

normal, without any delay in language development. At 2.3. Case 3


school, outcomes had been poor. Since early childhood, he
appeared introverted and disinclined to relationships. He (“Agostino”) A 42-year-old man with a diagnosis of
had been exempted from military service with a diagnosis of “residual schizophrenia” was admitted because of re-emer-
OCD. In the past, several attempts to get a job had been gence of acute symptoms and violent behavior against his
useless and the patient was currently unemployed. From the mother. There was no family history of psychiatric disorders
age of 19 years, he had presented obsessive-compulsive but an episode of senile depression in his grandmother. The
symptoms with continuous demanding answers to his rela- patient’s delivery at term was precipitous and psychomotor
tives to be reassured. Thereafter, behavioral disturbances development was normal. The patient began to speak when
took on a more dramatic form, reminiscent of psychosis. His
he was 12–18 months old and since early childhood the
parents reported: 1) prodigious memory that enabled the
patient’s movements were clumsy. Encopresis was noted. In
patient to recall single words or punctuation marks through
the first years of elementary school, the patient was lively,
many printed pages; 2) difficult relationships with same age
extroverted and sociable. In the following years, he showed
children; 3) intolerance of any remark; 4) motor clumsiness
and motor stereotypies (trunk swinging); 5) resistance to impaired social interaction with his fellows, while he was
and distress over trivial changes in his environment; 6) capable of relationship with adult people. Paradoxically, in
unusual and restricted interest in the comic-strip magazine his relationship with adults, he appeared particularly nice,
“Dylan Dog,” with systematic collection of magazines, deep jocular and facetious. He depended on his brother and had
knowledge of technical data, biography of the authors and no friends or hobbies but only a strong interest in sport.
of the publishers; 7) obsessive-compulsive thoughts; and 8) When he had to make even trivial choices (e.g., to buy or
excellent sense of humor and unusual capacity to grasp the not to buy a magazine, to go shopping or not), he remained
double meaning of words. On admission, circumstantiality in doubt as to what to do, even for hours. From the age of
and obsessive rumination were evident. The patient was 14 years, he became mute and disinclined also to nonverbal
lucid and fully oriented. He showed resistance to complying communication. He maintained an absolute silence for two
with treatment. Thought was simple and poor of content. years, although there was evidence that he was able to
Persecutory and reference delusions were present, as well as understand. He was passive and used to shut himself in the
depression of mood with anxiety, tendency to bursts of bathroom and to stay there all day. Afterwards, he gradually
weeping and occasional suicidal thoughts. The patient re- began to talk again. He was treated with neuroleptic medi-
ported auditory hallucinations whose content he was not cation for 3 years and with electroconvulsive therapy, with-
able (or refused) to declare. Emotional withdrawal was out benefit. In the following years, other periods of tacitur-
severe and emotional range diminished. The patient had
nity were observed. He was admitted to several
neither friends nor job, interests or hobbies. He emphasized
neuropsychiatric hospitals. From the age of 17 years, he
his willingness to agree and live sociably with other people.
presented religious delusions. Then, for the first time, he
However, also in the hospital, he did not get along with
other patients, and reported to be lost in suicidal thoughts presented violent behavior. After the onset of these symp-
when people shunned him. MMSE score was 28. Neurolog- toms, behavioral disturbances took on a more severe form
ical examination revealed left-handedness, moderate akathi- with psychotic symptoms. During this period, he had been
sia, akinesia and tremors. He was clumsy and uncoordi- treated with haloperidol decanoate (100 mg/30 days) and
nated. Primary and secondary sexual features were normal. lorazepam (2.5 mg/day).
Mild gynecomastia was noted, possibly due to past neuro- On visit, the patient appeared lucid, oriented, quiet and
leptic treatment. Brain computed tomography (CT) with cooperative. His attitude was jovial, pleased, delighted and
contrast enhancement, electroencephalogram (EEG), echog- with a contented smile. He tended to repeat interlocutor’s
raphy of adrenal glands and routine laboratory tests were questions and to answer both in the affirmative and in the
normal. The WAIS revealed an I.Q. of 73 (verbal: 84; negative to the same question. Thought reading and with-
performance: 62). We planned to raise risperidone daily drawal and auditory hallucinations were noted. When we
dose, withdrew thioridazine and benzodiazepines, and asked him to write a sentence during the MMSE, he wrote
started valproate up to 1000 mg/day. Marked parkinsonian a bizarre phrase in Latin [“Ego sum Agostinianum” (his
tremor and hypersalivation became evident when risperi- Latinized first name)]. Likewise, his modalities of commu-
done daily dose grew up to 5 mg. Therefore, we withdrew nication were bizarre and awkward. No mood deviation was
this drug and started olanzapine, 10 mg/day. Three weeks present. While social functioning was endangered, affective
later, the patient was discharged. On discharge, depression, flattening was mild. He was repeatedly able to make every-
anxiety and persecutory ideation were improved, while hal- body laugh with his inventive remarks. In the ward, the
lucinations were no longer present. The patient was more patient’s behavior was never violent. On neurological ex-
capable of interaction, appeared aware of his disorder, and amination, the patient was left-handed, slow, clumsy, with
was able to criticize his behavior with humor (“I my self do stereotypies (trunk swinging, touching of genitals, echola-
not tolerate my querulousness”). lia), and a Parkinsonian tremor, more evident on right. Body
288 M. Raja, A. Azzoni / General Hospital Psychiatry 23 (2001) 285–293

schema disorder was also evident. MMSE score was 25, which were judged to be caused by misunderstanding of
with discrepancy between good results in arithmetic and bad people’s facial emotional expressions; 2) obsessions and
results in memory and picture tasks. The WAIS revealed an compulsions (e.g., cleaning his teeth for many hours); 3)
I.Q. of 46 (verbal: 55; performance: 40). Brain CT with memory talent, which contrasted with poor general intellec-
contrast enhancement revealed localized right parietal cor- tual functioning; 4) fine sense of humor; 5) unusual capa-
tical atrophy. We withdrew antipsychotic medication, bility to mimic; and 6) behavior different in disparate con-
treated the patient with lorazepam (2.5 mg/day), and dis- texts. That prompted us to change our original diagnosis
charged him slightly improved.
in AS.

2.4. Case 4 2.5. Case 5

An 18-year-old was admitted for behavioral disorder. In A 31-year-old was admitted for violent behavior against
the past, his father had been admitted to our ward several his unmarried mother, affected by epilepsy with abnormal
times for episodes of schizoaffective disorder, mixed type. personality. No information was available about his father.
The patient’s psychomotor development was normal. In The patient’s delivery at term was uneventful and his psy-
early childhood, the patient had difficult relationships with chomotor development was normal. Although his mother
his schoolmates. He tended to remain alone, to divert his reported “good school outcomes”, the patient had had to
mind, to excessive food intake and to stereotyped behavior. repeat a year at school. He had got a secondary school
Meanwhile, the patient’s relationship with his father was certificate of education. Since early childhood, he appeared
good, characterized by exchanges of cultural and philosoph- introverted and disinclined to relationships. He had no con-
ical views. At the age of 12–14 years, his school efficiency tact with anyone but with his grandfather (when this was
was good. In the same period, obsessive-compulsive traits alive) and his mother. On visit, the patient was mute and
and over-valued religious ideas first appeared. At the be- appeared sad. His face was dysmorphic. Speech was slow,
ginning of his junior high school, he left the school, shut monotonous, with repetitions of sentences, almost unintel-
himself up in his house, and fed poorly. He refused to take ligible, repetitive, and pedantic. His movements were
thioridazine prescribed by a psychiatrist who had made the clumsy. He looked at people out of the corner of his eye,
diagnosis of “catatonic schizophrenia” and “anorexia ner- with his head lowered, and stereotyped movements of his
vosa.” At the age of 16 years, there had been several arms and hands. Facial expression showed alternating of
episodes of coprophagy. Then, he was repeatedly admitted obsequious and submissive attitude with aggressiveness,
to a University Neuropsychiatric Institute for Children, re- related to specific themes and contexts. Notwithstanding his
ceived a diagnosis of Schizotypal Personality Disorder and social isolation and inability to function in almost all areas,
was treated with haloperidol, risperidone and fluoxetine. On the patient was abnormally interested in the study of eco-
admission, the patient was mute, inexpressive, unreactive nomics, spending a disproportionate amount of his time
reading books of economics. Also patient’s sexual interest
and with no eye contact. He spent his time engaged in
seemed out of proportion to his aloneness and emotional
mannerisms and stereotyped behaviors such as touching his
withdrawal. Furthermore, the demand of getting married
beard, twiddling his temples, walking backwards and for-
and the risk of remaining out of work because of his hos-
wards or swinging. Neurological examination was normal,
pitalization were perpetual obsessions for him. He denied
however, his movements were clumsy. We noted affective
hallucinations. Persecutory ideas were present and blunted
incongruity and flattening, emotional withdrawal, concep-
affect and emotional withdrawal were severe. Although the
tual disorganization and echolalia. The patient often re- patient sometimes felt guilty about injures to his mother, he
ferred to himself as a third person. The presence of perse- minimized the seriousness of his violent behavior. End-
cutory delusions and sporadic auditory hallucinations was lessly, he repeated the same demands or remarks, without
uncertain. No mood alteration was present. MMSE score considering other people’s attitudes and seemed unaware
was 24. The WAIS revealed an I.Q. of 73 (verbal: 77; that his stereotyped and repetitive behavior was excessive
performance: 71). Brain CT with contrast enhancement was and unreasonable. During hospitalization, he tended to re-
normal. The patient was treated with olanzapine— up to 15 main alone, and did not show interest in getting in touch
mg/day—for thirty days, with minimal improvement and with anybody. Brain CT with contrast enhancement re-
then switched to risperidone— up to 4 mg/day—with mod- vealed enlargement of cortical sulci and ventricles. An EEG
erate improvement. Forty days after admission, the patient showed unstable, diffuse theta activity (6 –7 Hz). We made
was discharged with a diagnosis of disorganized schizo- a diagnosis of schizoaffective disorder depressive-type and
phrenia and referred to a therapeutical community in order treated him with risperidone (up to 5 mg/day) and parox-
to provide a rehabilitative support. At present, the patient is etine (20 mg/day) with moderate improvement. Six, and 18
still in this center. In the last two years, the following months later, the patient, who had stopped taking drugs, was
features had been noted by the staff: 1) violent episodes, admitted for new episodes of violence against his mother.
M. Raja, A. Azzoni / General Hospital Psychiatry 23 (2001) 285–293 289

During his second hospitalization, he reported sporadic mild and had been treated by several professionals. However,
auditory and visual hallucinations. Otherwise, his clinical none of them had been recognized as affected by PDD.
condition was unchanged. We made a diagnosis of schizo- Sometimes it is difficult to differentiate between the
phrenia and restarted treatment with risperidone (up to 5 spectrum of autistic syndromes and psychotic disorders. A
mg/day). One year later, the patient was again admitted after longitudinal observation may be necessary to distinguish
an assault against his mother, blamed for having not found these different nosological entities [20]. Diagnosis of AS
a wife for him. As in previous occasions, the patient had may be delayed until adult age [21]. We misdiagnosed the
gone off treatment. His clinical symptoms were similar but fourth patient as affected by schizophrenia during his hos-
no hallucination was reported in this circumstance. The pitalization, because of his muteness and social withdrawal,
WAIS revealed an I.Q. of 88 (verbal: 94; performance: 81). and made the correct diagnosis several months later when
As a result of our increased experience with AS, we hy- we listened to a clinical report of the staff of the therapeutic
pothesized a diagnosis of AS since the patient satisfied the community about patient’s behavior. As regards the fifth
following DSM-IV criteria: 1) abnormal nonverbal behav- patient, we revalued our previous diagnosis because of our
iors regulating social interaction; 2) failure to develop peer increased experience with AS. Invariably, the diagnosis of
relationships appropriate to developmental level, since AS we made for the first time had a strong impact on the
childhood; 3) no interest in other people; 4) lack of social or patients and, even more, on their relatives. Most of them
emotional reciprocity; 5) abnormal interest in the study of seemed to calm down when they recognized the clinical
economics, unrelated to his real life; and 6) stereotyped and features of AS in their relatives and could conceptualize
repetitive motor mannerisms. Furthermore, some associated symptoms and behavior in term of a rather specific biolog-
features of AS were present: 1) motor clumsiness; 2) ob- ically based disorder instead of a heterogeneous protean
sessive symptoms; pedantic speech; low I.Q., with verbal disorder such as schizophrenia, still perceived by some of
I.Q. higher than performance I.Q. score. them as a mysterious and ominous madness. As Gillberg
[22] states, “it is usually very helpful from the psychological
point of view in helping families come to terms with the fact
that their child is handicapped. The association of autism
3. Discussion
with— even unspecific— brain dysfunction makes the hand-
icapping condition less mysterious.”
3.1. Diagnosis
3.2. Male sex
In these patients, a pattern of deficits in social interaction
and in the use of language, motor awkwardness, discrepan- All patients admitted to our ward, who received an AS
cies between verbal and performance cognitive abilities, diagnosis, were male. This could be due not only to the
restricted outstanding cognitive abilities (especially mem- prevalence of autism spectrum disorders higher in males
ory talent, good capability to mimic, sense of humor), clum- [23–26] but also to a referral bias. Patients with behavioral
siness, stereotyped movements and unusual, circumscribed disorder are more likely to be admitted to a PICU, especially
interest are consistent with a diagnosis of AS. Since there in case of violent behavior. Coeteris paribus, men’s violent
are different diagnostic criteria for AS, HFA and Pervasive behavior raises more concern, is less tolerated, and more
developmental disorder not otherwise specified, an intrinsic often leads patients to hospital admission.
diagnostic ambiguity exists in our patients that reflects the
standards of diagnosis in naturalistic setting and reflects the 3.3. Puberty
current controversy over the AS diagnosis. AS differs from
one investigator to another [1, 16 –19, ICD-10, and DSM- Most of our patients presented a worsening of their
IV], thereby making the interpretation of research findings illness near puberty. Pubertal deterioration may occur in
difficult. some autistic children [27]. Symptom aggravation is often
Among nearly 2500 patients admitted to our unit, 5 observed in adolescence. Most cases of autism taking a
received a diagnosis of AS. That points to a low prevalence deteriorating course in adolescence never return to preado-
(0.2%) in the population admitted to PICU. As in all non- lescent levels of language, communication and social func-
epidemiological studies, the sample may not be representa- tioning [22]. The onset or worsening of these conditions at
tive for AS. These patients presented symptoms and behav- or near puberty suggests a major role for sex hormones.
ioral disturbances reminiscent of psychotic disorder and
were referred to psychiatrists practiced in psychopathology 3.4. Physical anomalies
of adult people. Given the low prevalence in the population
of patients referred to general psychiatry services, and the Autistic patients do not have consistent physical features,
co-occurrence of psychotic or obsessive-compulsive symp- but often show physical anomalies, non specific abnormal
toms, it is not surprising that the diagnosis of AS may be EEGs, MRIs, CT scans and autopsy findings [28]. These
unrecognized. These patients had been ill for many years abnormalities result from genetic or environmental damages
290 M. Raja, A. Azzoni / General Hospital Psychiatry 23 (2001) 285–293

that occur in early pregnancy and suggest an abnormality in are not at increased risk for schizophrenia [36]. Neverthe-
fetal development [29]. The prevalence of medical condi- less, there possibly exists a subgroup of autistic children
tions with suspected etiologic relationship with autism var- who are at risk for the development of schizophrenia-like
ies between 10 and over 30% depending on the diagnostic symptoms. This group of patients may be more likely to
system employed [30]. Neuropathological and MRI studies seek treatment from psychiatric facilities and, therefore,
have demonstrated developmental disturbances of neuronal may not be sampled in studies whose subjects are drawn
migration in the cerebral cortex of autistic subjects. In from autism and developmental centers. The subjects sam-
patients with AS, neuro-radiologic findings [31] and neuro- pled from autism centers are more likely to comprise “un-
ophthalmologic disturbances [32] have been reported. complicated” cases of autism. In our patients, it is not easy
Empty sella and neurogenic diabetes insipidus (Case 1), to rule out the diagnosis of schizophrenia, since the criteria
localized cerebral atrophy (Case 3), and facial dysmorphia for the differential diagnosis between PDDs and schizophre-
(Case 5) were the abnormal physical features present in our nia are unreliable. DSM-IV states that, in the presence of a
sample. PDD, the additional diagnosis of schizophrenia “is made
only if prominent delusions or hallucinations are also
3.5. Psychiatric comorbidity present for at least a month (or less if successfully treated)”.
However, it is not clear what should be intended for “prom-
Comorbid psychiatric conditions are frequently observed inent”. A further problem is the difficulty to establish the
in patients with AS. In a series of 99 subjects with AS [33], presence of psychotic processes (delusions or thought dis-
psychiatric associated conditions included ADHD (28% of order) in children who have not yet developed relatively
cases), OCD (19%) and depression (15%). Obsessive-com- sophisticated expressive language skills, and ability to use
pulsive symptoms have been frequently described in autism adult rules of logic or notions of reality [50]. As in other
spectrum disorders, possibly due to serotoninergic dysregu- areas of psychiatric nosology, the longstanding presence of
lation [34]. The rate of Bipolar Disorder in family members more or less typical delusions or hallucinations without
of a group of autistic subjects was 4.2%, higher than in the concurrent mood alterations raises the issue of the diagnosis
general population. Furthermore, it was significantly higher of schizophrenia in patients with symptoms typical of other
in families of probands with AS, suggesting an etiological mental disorders. In our patients, many relatively specific
link between AS and manic depression [35]. In our sample, clinical features suggest a diagnosis of PDD (see Table 1).
2 patients presented depressive symptoms and 4 patients Some clinical symptoms are inconsistent with a diagnosis of
obsessive-compulsive symptoms. schizophrenia, including restricted outstanding cognitive
abilities, history of early language delay, sense of humor,
3.6. Pervasive Developmental Disorders and good capability to mimic and unusual and restricted inter-
Schizophrenia ests. Also, clumsy movements, atypical age of onset, poor
response to antipsychotics and absence of schizophrenia
A problematic topic is the possibility of comorbid diagnoses in relatives highlight the differences between
schizophrenia. The incidence of childhood schizophrenia is schizophrenia and these subjects. In childhood onset schizo-
slight. There is no evidence to justify differentiating a sub- phrenia, it has been reported a rate of familial schizophrenia
variety of schizophrenia according to childhood onset, and and spectrum disorders even higher than in more common
no reason to pool autism and schizophrenia. Although the later-onset cases [51].
two conditions can co-occur in the same individual, it ap-
pears that the frequency of schizophrenia among autistic 3.7. AS, right-hemisphere dysfunction, and nonverbal
patients (0.6%) is comparable to the frequency of schizo- learning disabilities
phrenia in the general population and that the two condi-
tions are not more commonly observed together than would It has been hypothesized that AS is a disorder of the right
be expected on a chance basis [36]. Differences from adult hemisphere [52]. The human right hemisphere is dominant
schizophrenia are more marked when onset is in childhood in certain types of spatial functions, particularly the analysis
than in adolescence but all are quantitative rather than of external space, and the orientation of the body within this
qualitative suggesting that the disorders are the same and space. It also plays a major role in emotion, both in the
that there should be no separate category for children or subjective experience and the external expression of emo-
adolescents [37]. In children, the diagnosis of schizophrenia tion as well as in the appreciation of emotion manifested by
often proves to be unfounded [38]. Despite evidence sup- others. It is also dominant in attention. Finally, there is
porting discontinuity between autism and schizophrenia evidence of right hemisphere involvement in the pathophys-
[36,39], patients with coexisting PDD and schizophrenia- iology of obsessive-compulsive symptoms. Some AS fea-
like psychosis have been described [40 – 46] and some in- tures suggest a possible right-hemisphere dysfunction. First,
vestigators have reported an association between past his- AS has clinical features in common with acquired right-
tory of autistic symptoms and present diagnosis of hemisphere dysfunction. Second, some features of so-called
schizophrenia [47– 49]. In general, individuals with autism Nonverbal Learning Disabilities (NVLD) including emo-
M. Raja, A. Azzoni / General Hospital Psychiatry 23 (2001) 285–293 291

Table 1
Clinical features of the 5 patients with a diagnosis of AS

Case 1 Case 2 Case 3 Case 4 Case 5

Sex Male Male Male Male Male


Handedness Left Left Left Right Right
Previous diagnosis Early-onset psychosis Paranoid disorder Residual schizophrenia Disorganized Undifferentiated
schizophrenia schizophrenia
Obsessive-compulsive symptoms Yes Yes No Yes Yes
Delusions Yes Yes Yes No Yes
Hallucinations No Yes Yes No Yes
Above average single cognitive Yes Yes No Yes No
abilities
Aggressive or violent behavior Yes Yes Yes Yes Yes
History of early language delay Yes No No No No
Sense of humor Yes Yes Yes Yes No
Good capability to mimic Yes No No Yes No
WAIS Total score 79 73 46 73 88
WAIS Verbal/Performance score 96/59 84/62 55/40 77/71 94/81
Periods of muteness No No Yes Yes Yes
Unusual and restricted interest Yes (horror films, Yes (dylan dog) No No Yes (books of
knives, policewomen) economics)
Worsening at puberty Yes Yes Yes
Neuroimaging Empty sella (CT and Normal (CT) Right parietal cortical Normal (CT) Cortical sulci and
MRI) atrophy (CT) ventricules
enlargement (CT)
Psychiatric family history Negative Negative Depression in Bipolar disorder Epilepsy with
grandmother (psychotic) in abnormal
father personality in
mother
Response to antipsychotic Poor Good Poor Moderate Moderate
medication
Clumsiness Yes Yes Yes Yes Yes

tional disturbances, abnormal language (poor prosody and way of thinking and an inability to identify and understand
pragmatics, good vocabulary), social difficulties are also human emotions and relationships. Possibly, this discrep-
observed in patients with AS and raise the question whether ancy between the inability to identify or understand human
there is a continuum between these disorders. A recent study emotions and the excellent sense of humor and capacity to
found patients with AS different from patients with HFA mimic reflects a more general unbalance of mental and
and similar to patients affected by NVLD [53]. In contrast cognitive symptoms in PDD (e.g., mental retardation and
with the more common language-based learning disabilities, excellent mathematical or mnemonic abilities, lack of ac-
which are associated with problems in reading and spelling, tivities or interests and over-involvement in confined inter-
NVLD are associated with problems in arithmetic and with ests) that could be due to defective development of some
a pattern of deficits in cognitive and adaptive functions brain areas associated with compensatory overgrowth of
attributed to the right hemisphere, including spatial cogni- other areas [54]. Finally, obsessive-compulsive symptoms,
tion, social-emotional functioning, visuo-perceptual and si- present in 4 patients out of 5, might also point out right
multaneous information processing. Third, also the reported hemisphere dysfunction. There is evidence of right hemi-
discrepancy between verbal and performance cognitive abil- sphere dysfunction in patients with OCD [55–56]. Mc-
ities of these patients is consistent with the possibility of Kelvey et al. [57] found abnormal right-hemisphere (and
right hemisphere dysfunction. A fourth clinical feature sug- cerebellar) function on single photon emission computed
gestive of right hemisphere failure is patients’ inability to tomographic imaging (SPECT) in three patients with AS,
make inferences about others’ mental states, as evidenced and enlargement of the right lateral ventricle, reflecting mild
by their difficult relationship with other people. Patients right hemispheric atrophy on MRI, in one of them. In a
with AS seem unable to recognize the meaning of human series of childhood epilepsy surgery candidates, 6 of 8
facial expressions. This deficit could be conceived as a children with AS had right brain lesions [58]. Interestingly,
selective form of prosopoagnosia, a deficit typical of right one of our patients had localized right parietal cortical
hemisphere lesions. Surprisingly, 4 of the 5 subjects pre- atrophy consistent with this hypothesis. However, neuroim-
sented unusual sense of humor and capacity to mimic. These aging assessment was inadequate to reveal possible hemi-
features are unexpected and seem even paradoxical in indi- spheric asymmetry in our patients. MRI imaging was not
viduals with a disorder characterized by a formal concrete available in 4 of them and no volumetric assessment of
292 M. Raja, A. Azzoni / General Hospital Psychiatry 23 (2001) 285–293

brain was performed by radiologists who were asked only to done with moderate or poor results, and 2 patients with
detect clinically significant structural abnormalities. olanzapine with moderate or poor results. Two patients,
both nonresponders to previous standard neuroleptic treat-
3.8. Handedness ment, were not treated with antipsychotics. Previously, 3
patients had been treated with SSRI without improvement.
Three patients out of 5 were left-handed. This points to a
right dominance and could seem in contrast with the hy-
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