Autism and Developmental Disorders
2019. Vol. 17, no. 1, 24–35
ISSN: 1994-1617 / 2413-4317 (online)
Сatatonia in Autism Spectrum Disorders: Diagnosis, Therapy and Clinical Science
Keywords: catatonia, adults, autism spectrum disorders, benzodiazepines, electroconvulsive therapy, vagal theory of catatonia.
Journal rubric: Research & Diagnosis of ASD
Article type: scientific article
For citation: Dhossche D.M. Сatatonia in Autism Spectrum Disorders: Diagnosis, Therapy and Clinical Science. Autizm i narušenie razvitiâ = Autism and Developmental Disorders, 2019. Vol. 17, no. 1, pp. 24–35. DOI: 10.17759/autdd.2019170104.
Catatonia has been increasingly recognized as a comorbid syndrome of autism at a rate of 12-17% in adolescents and young adults with autism spectrum disorders. Symptoms that should alert the clinician for catatonia are markedly increased psychomotor slowness which may alternate with excessive motor activity, apparently purposeless, and not influenced by external stimuli, extreme negativism or muteness, stereotypy, peculiarities of voluntary movement, increased stereotypies with or without self-injury, echolalia, or echopraxia. Upsetting life events, the loss of routine and structure, experiences of loss, conflicts with parents, caregivers, or peers, and discrepancies between the ability in the patient and parental expectations-especially, in higher functioning autistic youth, are known to precipitate catatonia as well as medical and neurological illnesses. Lacking controlled trials, successful use of benzodiazepines, sometimes at high dosages, and electroconvulsive therapy for catatonia in autism is supported by case-reports and case-series. Electroconvulsive therapy is indicated for the treatment of catatonia when lorazepam does not bring about rapid relief. Maintenance electroconvulsive therapy is important for sustained symptom remission in some cases. There is an urgent need for prospective studies of catatonia in autism spectrum disorders and for controlled treatment trials.
Keywords: catatonia, children, adolescents, adults, autism spectrum disorders, benzodiazepines, electroconvulsive therapy, vagal nerve.
DhosscheD.M. Catatonia in autism spectrum disorders: Diagnosis, therapy, and clinical sciensm // Autizm i narusheniya razvitiya = Autism & Developmental Disorders (Russia). 2019. Vol. 17. No 1 (62). Pp.. doi: 10.17759/autdd.2019170103
Dhossche Dirk M., MD., Ph.D., Professor of Psychiatry, Department of Psychiatry at the University of Mississippi Medical Center, Jackson, Mississippi, USA. E-mail: email@example.com
Catatonia is a severe and potentially life-threatening acute illness, especially in its malignant form when it is accompanied by autonomic dysfunction and high fevers but it remains treatable when recognized and treated promptly . It occurs in children and adolescents, as in adults, in a variety of forms including medical and autoimmune diseases such as lupus [1; 25; 27] or anti-N-methyl-d-aspartic acid (NMDA) receptor encephalitis [14; 22; 38], psychotic and affective disorder, toxic states such as Neuroleptic Malignant Syndrome (NMS), autism spectrum disorders (ASD), developmental disorders, including patients with Prader-Willi Syndrome  and Down Syndrome , tic disorders, posttraumatic conditions, and miscellaneous syndromes such as Kleine-Levin Syndrome and Pervasive Refusal Syndrome [12; 16].
There are no controlled studies on the use of benzodiazepines or electroconvulsive therapy (ECT) in children and adolescents. Clinical experience and case-reports support benzodiazepines and ECT, including maintenance ECT, as safe and effective treatments for pediatric catatonia that do not carry the risk for precipitating NMS [12; 16]. In general psychiatric adult patients, benzodiazepines are effective in more than half of cases up to 80%, the remaining cases uniformly respond to ECT . A remarkable finding in some catatonic patients is that high dosages of benzodiazepins, for example between 10-20 mg of lorazepam, are needed for relief and well tolerated without observable side effects.
Catatonia has been increasingly recognized as a comorbid syndrome of ASD at a rate of 12-17% in adolescents and young adults with ASD [3; 39] and with other intellectual disabilities [10; 34]. Two systematic clinician-based studies show that catatonia is found in 12% to 17% of adolescents and young adults with autism [3; 39].
Wing & Shah report that 17% of a large referred sample of adolescents and young adults with autism satisfied modern criteria for catatonia . Thirty individuals with autism aged 15 years or older met criteria for catatonia, with classic Autistic Disorder diagnosed in 11 (37%), atypical autism in 5 (17%), and Asperger Disorder in 14 (47%). Under age 15, no child demonstrated the full syndrome although isolated catatonic symptoms were often observed. In the majority of cases, catatonic symptoms started between 10 and 19 years of age. Five individuals had brief episodes of slowness and freezing during childhood before age 10. Obsessive-compulsive and aggressive behaviors preceded catatonia in some cases. Visual hallucinations or paranoid ideas were occasionally reported, but no diagnosis of schizophrenia could be made.
In the second study, 13 (12%) of 120 autistic individuals, between ages 17-40 years, had clinically diagnosed catatonia with severe motor initiation problems . Another four individuals had several catatonic symptoms but did not meet criteria for the full syndrome. Eight of the 13 individuals with catatonia suffered from Autistic Disorder; the remaining five were diagnosed with atypical autism. The proportion of those with Autistic Disorder that were diagnosed with catatonia was 11% (8/73). Fourteen percent (5/35) of those with atypical autism had catatonia.
A hospital-based study  of 101 child and adolescent psychiatric inpatients with "at risk" diagnoses including any pervasive developmental disorder, psychotic disorder not otherwise specified, intermittent explosive disorder, intellectual developmental disorder, neuroleptic malignant syndrome or previously diagnosed catatonia found that 18% of patients met criteria for catatonia, based upon three or more symptoms, including unexplained agitation or excitement, disturbed or unusual movements, reduction in movement, reduction or loss of speech and repetitive/stereotyped movements. The authors emphasized poor recognition of catatonia in these pediatric conditions, including, but not limited to, pervasive development disorders.
Breen & Hare  developed a 34-item third party report measure, the Attenuated Behaviour Questionnaire (ABQ), consisting of 15 motor symptoms, 5 affective alterations, and 14 behavioral alterations, commonly associated with catatonia in ASD. They tested the new measure in a British sample of convenience (N=99) of young people aged 12-25 years with existing diagnosis of ASD by interviewing the parent or long-term caregiver. Full data were available from 87 informants of whom 18 (21%) reported an existing diagnosis of catatonia. 42 (48%) of cases displayed three of more core catatonia-like attenuated behaviors. Scores on this new measure ABQ were higher in those with an existing catatonia diagnosis. Catatonia-like attenuated behaviors were associated with measures of depression and repetitive and restricted behaviors. The study did not report on how the existing clinical diagnoses of catatonia were obtained but supports the ABQ as clinical and research tool for catatonia symptoms in an ASD population.
Many patients with ASD cannot be diagnosed definitively with an affective or psychotic disorder due to the fact that these patients are nonverbal and have severe cognitive impairments yet the observable signs and symptoms are present and readily recognizable. Most cases of catatonia in children and adolescents with ASD are not associated with any underlying medical or psychiatric conditions. For example, in a sample of 58 children and adolescents with catatonia , 18 (31%) had a history of developmental disorder, i.e., ASD, intellectual disability or neurodevelopmental malformation. Only two of those had an identifiable underlying medical or genetic condition.
Clinical presentations of catatonia
Catatonia in ASD is currently diagnosed in DSM-5 based on the presence of 3 out of 12 symptoms : catalepsy, waxy flexibility, stupor, agitation, mutism, negativism, posturing, mannerisms, stereotypies, grimacing, echolalia or echopraxia. These symptoms may be present at baseline in patients with ASD. However, a sharp increase of these pre-existing symptoms or sudden appearance of new catatonic symptoms should alert the clinician to assess for a diagnosis of catatonia [11; 21; 39].
Several catatonia rating scales have been developed . The most commonly used scale for the assessment of catatonia is the Bush-Francis Catatonia Scale (BFCRS) ; a 23-item standardized instrument which is designed for diagnosing and for the assessment of severity. When using the BFCRS, catatonia may be diagnosed when two or more items on the first 17 items are present. Serial catatonia ratings are useful to detail changes over time and for measuring change during treatment  in individual cases and controlled studies. Although the Bush-Francis Catatonia Scale is useful in patients with autism, the scale was developed using a sample of general adult psychiatric patients. There are currently no catatonia rating scales that have been standardized in patients with autism. The KANNER scale, named after Leo Kanner (1894-1981) who described the neuromotor and neurodevelopmental features of autism , has been proposed as a unifying instrument for quantifying core features of catatonia, across a broad range of neuropsychiatric disorders, including autism and pervasive developmental disorders . The scale is comprehensive; however, it is untested and not validated in patients with autism.
Self-injurious behavior (SIB) occurs regularly in patients with ASD and includes behaviors such as self-hitting, punching, biting, scratching and kicking. SIB can lead to significant injury to soft tissue, bone, head trauma, retinal detachment, blindness or even death . Often SIB is under operant conditioning , however, sudden increases of SIB may sometimes be a part of the constellation of stereotypical behaviors seen in catatonia. Wing and Shah  reported the presences of catatonic stereotypies in 23-46% of autistic patients they assessed. Although they did not assess self-injury as a stereotypical behavior, however the repetitive nature of these behaviors suggests that there is a significant correlation between SIB, catatonia and ASD. Wachtel & Dhossche  hypothesized that some patients with ASD featuring extreme levels or increases of SIB could suffer from underlying catatonia and should be assessed for other symptoms of catatonia, and, as such, could be treated with ECT. The initial and promising experiences in this area and the profound effects of ECT in such cases have been described elsewhere .
Clinical treatments for catatonia
In 2014, DeJong et al.  have reviewed all pertinent papers from 1980 onwards on interventions used to treat catatonia in ASD, identifying 22 relevant papers on 28 cases both adult and pediatric, with the majority coming from the USA. They report some support for the use of ECT, high dose lorazepam and behavioral interventions in this patient group and lament the lack of strong evidence in this field, small number of cases, an evidence base consisting entirely of case studies, small case series, and clinical opinion, poor quality in terms of treatment protocols and objective measures of outcome. The study emphasizes the urgent need for prospective long-term studies and controlled trials.
P is a 14-year-old male who was born at term after a normal pregnancy. He was diagnosed with ASD and moderate Intellectual Disability at the age of 3 years of age due to social deficits, communicative delays, and cognitive deficits. He began to receive speech therapy and was enrolled in special education classes. Genetic testing was done and was found to be negative. His family history was negative. During elementary school, he was treated intermittently with stimulants for hyperactive and impulsive behaviors with good results. He also had mild tics that did not require treatment. He participated well in school, was fluent in conversation, and able to perform fairly independently.
In his first year of middle school, there was a sudden and sharp increase of abnormal movements including repeated turning of the head to the left, blinking, grimacing, stuttering, repetitive movements of the fingers, and rubbing of the eyes. He started to speak less and only in a high-pitched voice. He also had waxy flexibility when examined by a neurologist who found an otherwise neurologically intact adolescent. His food and fluid intake decreased and patient started to lose weight and sleep poorly. He had staring episodes, withdrawal, and episodes of compulsive hand washing and taking frequent showers. He became anxious and preoccupied with death and developed crying spells. His facial expression became tense and mask-like. His writing skills decreased and his grades dropped. A few weeks after onset of these symptoms, patient disclosed to an uncle that he was being bullied at school. Physical abuse and serious threats by peers were substantiated after an investigation by the school.
At the onset of these abnormal movements, magnetic resonance imaging of the brain and an electroencephalogram were done, showing negative results. Genetic testing, metabolic and auto-immune work-up, serum copper and ceruloplasmin were negative.
Over the next year, a long list of medications was prescribed by his psychiatrist targeting tics and anxiety, including several selective serotonin reuptake inhibitors (fluoxetine, sertraline), duloxetine, mirtazapine, atypical antipsychotics (risperidone, aripiprazole), fluphenazine, clonidine, guanfacine and (low doses of) benzodiazepines (4 mg of diazepam, 0.5 mg of lorazepam and clonazepam) to no avail or causing side effects that necessitated discontinuation of the medication. A single dose of zolpidem (10 mg) caused agitation and increased tics. P would not swallow pills consistently and took only liquid preparations or disintegrating tablets.
P continued to deteriorate needing assistance with feeding, getting dressed, brushing his teeth, and combing his hair. His speech consisted of high-pitched short sentences and remained greatly reduced. He shook his head and shattered his teeth constantly, startled in an exaggerated manner, remained withdrawn with episodes of agitation. His neurologist offered the diagnosis of catatonia and recommended ECT, more than one year after onset of symptoms.
A diagnosis of catatonia was confirmed with a systematic trial of lorazepam starting at 1 mg by mouth twice a day with rapid escalation up to 7 mg twice a day over ten days. He had minimal improvement and ECT was recommended. Increasing the dose of lorazepam to 16 mg resulted in sedation and increased agitation.
Bilateral ECT was started on an outpatient basis, while tapering lorazepam. After 12 bilateral ECT treatments, his catatonia resolved and patient returned to baseline. ECT was stopped and no maintenance ECT was required. He has not relapsed at 2-year follow-up. His maintenance treatment consists of olanzapine 25 mg PO and oral lorazepam 6 mg per day.
This patient had a delay in obtaining the appropriate diagnosis and treatment as symptoms started one year before receiving a formal diagnosis of catatonia by his neurologist. It is an unfortunate but not uncommon situation also nowadays. There is a persistent misconception that a patient with ASD could not also have symptoms of catatonia. This is probably a remnant of the long but erroneous perception that catatonia is indicative of a schizophrenia diagnosis. Patients with ASD often have difficulty expressing symptoms but a significant change in baseline level of functioning and behavior as seen in this case is cause for further evaluation and work-up.
It is possible to schedule ECT after the acute episode as maintenance treatment to avoid relapse and when there are incipient symptoms of catatonia. It is preferable to schedule ECT on a flexible basis with open communication between the outpatient provider and ECT service. Others have reported the importance of maintenance ECT for sustained symptom remission . Ongoing ECT treatments are often imperative to prevent relapse similar as in non-autistic populations [24; 30],
A significant stressor preceded the onset of catatonia. Traumatic events and stressors are not always easily identifiable as patient with ASD may find it difficult to express this stressor due to problems with communications and conveying subjective experiences. Shah & Wing  found that ongoing stressful experiences often precede the development of catatonia in autistic young adults. Life events, the loss of routine and structure, experiences of loss, conflicts with parents, caregivers, or peers, and discrepancies between the higher functioning autistic individual’s capabilities and the expectations of parents, can precipitate catatonia.
Observations that catatonia follows overwhelming anxiety due to trauma or perceived danger, the positive response of catatonia to anxiolytics such as benzodiazepines or barbiturates, and psychogenic theories of catatonia , are particularly applicable to people with autism due to their increased social, cognitive, and sensory vulnerabilities [13; 17]. A vagal theory of catatonia in ASD has been proposed  as an expansion of the general Polyvagal Theory on the biology of social engagement and attachment first formulated by Porges in 1995  as a unifying framework for unifying various pathophysiological and treatment aspects of catatonia in ASD.
Catatonia has been increasingly recognized in people with ASD. Assessment, diagnosis, and treatments are reviewed. The use of benzodiazepines, sometimes at higher doses than recommended for anxiety, and ECT are recommended in patients. There is an urgent need for prospective studies of catatonia in ASD and for controlled treatment trials.
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