Lorna Wing, MD

The sixth [blind man] no sooner had begun About the beast to grope, Then, seizing on the swinging tail That fell within his scope, 'I see,' quoth he, 'the Elephant Is very like a rope!' " 			--    From 

Autistic spectrum disorders (ASD), as defined here, include autistic disorder, Asperger disorder, childhood disintegrative disorder (CDD), and pervasive developmental disorder not otherwise specified. They are a group of early-onset neuropsychiatric disorders of unknown origin for which we lack truly effective therapies. (The terms "ASD" and "autism" are used interchangeably throughout this article.)

Current treatments for children with ASD include behavioral treatment programs and speech therapies,

 as well as a wide range of psychotropic medications. All are useful in some cases, but so far, have little enduring impact on core social and communicative deficits.

 It is understandable that many parents try nonconventional treatments that often border on, or are, quackery.

As in the fable of the elephant and the blind men who are unable to grasp the nature of the beast, researchers have argued at various points that ASD are environmentally induced, toxin induced, related to infections or autoimmunity, or of genetic origin. In fact, all these different claims may apply to as yet unidentified subgroups, since ASD are undoubtedly both etiologically and pathophysiologically heterogeneous.

In this article, we aim to highlight the intriguing features of ASD, the fact that there is considerable overlap between autistic and catatonic symptoms, and the observation that full catatonia seems to develop in a significant proportion of people with ASD. Maybe this observation is not unlike the sixth man's claim that the elephant is like a rope, but we believe that further study of catatonia in autism may provide a new window into the origins, pathophysiology, and, most importantly, treatment of autism.

Here we provide a brief review of the pertinent current literature showing that in the past 15 years, catatonia has been increasingly recognized in adolescents and young adults with ASD. We present and discuss a case vignette of an adolescent with ASD and catatonia and provide recommendations for the assessment and treatment of catatonia in ASD. A case vignette of lorazepam treatment for catatonia in ASD and a case vignette of electroconvulsive therapy (ECT) for catatonia in a child who also met the criteria for CDD, or late-onset autism, are presented, and practical and research implications are discussed.

Only 2 systematic studies of catatonia in autism have been reported.

 They suggest that catatonia-like features are present in about 1 of 7 (12% to 17%) adolescents and young adults with autism and constitute an important source of impairment in this population. In a recent study, 17% of a large referred sample of adolescents and young adults with autism satisfied modern criteria for catatonia.

 Thirty persons with autism aged 15 years or older met criteria for catatonia. Classic autistic disorder was diagnosed in 11 persons (37%), atypical autism in 5 (17%), and Asperger disorder in 14 (47%).

None of those under the age of 15 years had full catatonic syndrome, although isolated catatonic symptoms were often observed. In the majority of cases, catatonic symptoms started between the ages of 10 and 19 years. Five individuals had brief episodes of slowness and freezing during childhood, before 10 years of age. Schizophrenia was not diagnosed in any of the patients.

In a report based on a population study, 13 (11%) of 120 autistic persons aged 17 to 40 years (mean age 25.5 years) had clinically diagnosed catatonia with severe motor initiation problems.

 Another 4 had several catatonic symptoms but not the full syndrome. Autistic disorder was diagnosed in 8 of the 13 persons with catatonia; atypical autism was diagnosed in the remaining 5. The proportion of those with autistic disorder in whom catatonia was diagnosed was 11% (8 of 73); 14% of those with atypical autism (5 of 35) had catatonia.

An increasing number of case reports and case series on catatonia in autism that satisfy 

criteria for catatonia have been published in the past 15 years.

 There is considerable overlap of psychomotor symptoms between the 2 disorders (eg, muteness, echolalia, stereotypical movements, and other psychomotor peculiarities).

 The diagnosis of catatonia in publishedcases was based on significant worsening of these symptoms and emergence of other catatonic symptoms. Some of the authors report that patients responded to treatment with lorazepam (the benzodiazepine most often used) and/or ECT. It should be noted that none of these studies were controlled and the number of cases was very small.

Case 1: Catatonia in an autistic adolescent

had been diagnosed showed a decline in function at the age of 15 years when he stopped speaking except to family members. His posture deteriorated. He began to slump and would complain of back pain. At school, he refused to enter classrooms and would walk slowly through corridors with his head down. He was unable to retain food in his mouth. Eventually, he required support in order to stand. He showed poor fine and gross motor skills, with difficulty in holding and using implements (such as a knife to butter bread). His speech slowed excessively at home. He would not engage in activities other than those associated with his specific interest in transport, mostly aircraft. By the age of 17 years, it was very difficult to get him to leave the family home. He required assistance washing and dressing and stopped using the toilet. The psychiatrist considered a diagnosis of catatonia but demurred because of the seemingly selective occurrence of symptoms. During medical examinations, the patient was often uncooperative and unresponsive. He refused to take the prescribed diazepam and fluoxetine.

An intensive behavioral intervention was started using the framework set out by Shah and Wing

 for the treatment of catatonia in patients with autism. On evaluation 9 months later, he showed improvement, speaking more and walking almost everywhere independently. He was able to express a wider range of emotion verbally and nonverbally. Recent testing revealed an IQ of 89. Despite the progress made, he continues to show various motility problems across all circumstances, mainly slowness of movement, clumsiness, inability to control movement, poor coordination, freezing of movement, and awkward posture. The parents are pursuing other treatments for his condition.

A diagnosis of catatonia in this patient is likely. An algorithm for the assessment of catatonia in autism is shown in 

 From the age of 15, the patient showed various catatonic symptoms, including mutism, stupor, and posturing, that have remained present although his level of function has recently improved. His symptoms satisfy criteria for catatonia in autism shown in the 

The severity of catatonia should be determined by assessing the degree to which activities of daily living, occupational activities, and physiologic necessities (eating, drinking, and excretion) are affected. Definitions of mild, moderate, and severe catatonia can be found elsewhere.

 The level of impairment should guide the need for services and staffing levels, as well as the choice of available anticatatonic treatments. At his worst, the patient described above seemed to have severe catatonia--stupor, immobility for most of the day, and need of assistance with food intake--constituting a medical emergency. Patients with features of malignant catatonia (fever, altered consciousness, stupor, and autonomic instability as evidenced by lability of blood pressure, tachycardia, vasoconstriction, and diaphoresis) also fall in this category. Severe and malignant catatonia are indications for administration of lorazepam and ECT.

Does catatonia in autism respond to treatment?

An algorithm for the treatment of moderate catatonia in autism, as in the patient presented in the first case vignette, is shown in 

 The proposed schedule relies on the recommendations of some catatonia researchers

 and some published case reports and case series.

 The psychological approach to treatment outlined below should be available for use in combination with medication or alone if medication fails.

developed over the last year in a 35-year-old man who was living in a residential treatment facility. His diagnoses included atypical autism, moderate mental retardation, and seizure disorder. He stood motionless for hours, often in odd postures. His speech had decreased considerably, and he required assistance in daily living activities. The psychiatric examination did not reveal hallucinations or delusions. His medications included an antiepileptic agent for long-standing grand mal seizures and an atypical antipsychotic for a tentative diagnosis of psychosis not otherwise specified. Trials of antipsychotic medications have had no effect on his motor symptoms.

At this point, catatonia was diagnosed and a trial of lorazepam was started with the dosage titrated over the course of 2 weeks to 4 mg twice a day. The hospital administrators expressed concern that the patient might become dependent on benzodiazepines, but the psychiatrist assured them that lorazepam at the prescribed high dose is an accepted treatment for catatonia. The patient responded well to treatment, with decreased psychomotor slowness, fewer freezing episodes, and increased socialization. Three months after the start of lorazepam, catatonia had resolved. The patient functioned at baseline level again, and he had resumed work at a sheltered workshop. His medications include an antipsychotic, an antiepileptic, and lorazepam (4 mg twice a day). Lorazepam will be tapered and stopped if the patient continues to do well over the next 3 months.

A new addition to the anticatatonic armamentarium is the psychological method developed by Shah and Wing.

 In brief, the treatment involves keeping the person active, doing what he or she enjoys, using verbal or gentle physical prompts to overcome movement difficulties, and maintaining a predictable structure and routine for each day. The importance of educating caregivers to understand catatonic behavior and to realize that it is not under the control of the patient is paramount. Management techniques for specific issues such as incontinence, freezing in postures, eating problems, and episodes of excitement are specified. This approach can be used in conjunction with medical treatments or when medical treatments fail.

A 9-year-old boy with apparently normal development but high familial loading of psychosis was admitted for agitation, anxiety, perplexity, negativism, and psychomotor slowing. Stupor, mutism, fever, and facial flushing developed over the next 2 weeks. Results of extensive medical and psychiatric workups were negative. EEG recordings consistently showed overall slowing but no epileptic spikes. The symptoms satisfied criteria for CDD, which is characterized by massive regression after the age of 2 years but before the age 10 of years, followed by autistic symptoms. In the absence of recommended treatments for CDD, other medical and psychiatric diagnoses-- including encephalitis and malignant catatonia--were considered. After failed trials with anticonvulsants, antiviral medications, and high-dose benzodiazepines, expert opinions regarding an ECT trial were sought; ECT consent was obtained from the caregiver. Stupor, muteness, and refusal to eat and drink improved rapidly during the first course of 7 treatments, but agitation, stereotypies, repetitive speech, and poor level of function remained.

Despite the widely different developmental and recent history, the symptoms of this patient were similar to those of other children with autism and conformed tothe differential diagnosis of CDD. A second ECT course was given because the patient relapsed into stupor and immobility. Again, the most severe catatonic symptoms dissipated, but the patient remained impaired. The patient was discharged and continued with outpatient ECT (once every week or biweekly) for the next 5 months. During that time, the boy slowly returned to baseline function and now attends school and lives at home. There have been no relapses during the past 3 years.

Are autistic and catatonic regression related?

This case illustrates the importance of diagnosing catatonia amidst severe regression of unknown cause in prepubertal children in order to select effective therapies. CDD is considered rare, with a pooled estimate across 4 surveys of 1.7 per 100,000 subjects (95% confidence interval, 0.6-3.8 per 100,000).

 However, it is also likely that an unknown number of cases are not reported because neurologists and other pediatric specialists who are not familiar with the psychiatric classification of CDD label this condition differently (eg, as encephalitis). Textbooks describe CDD as sometimes being associated with known medical conditions, but usually no clear cause is found. Deterioration occurs over the course of weeks or months. Residual symptoms include impaired social interaction, restricted language output, and repetitive behaviors. Follow-up studies have suggested that older age at onset of autistic symptoms, as in CDD, may be associated with worse outcome.

Catatonia has also been reported infrequently in children,

 although no systematic studies in this age group have been done. The oldest description of catatonic symptoms (in a 3-year-old child) comes from de Sanctis (1908-1909).

 Lorazepam and ECT were reported to be effective in 2 more recently published reports.

CDD and childhood catatonia are both poorly studied and probably poorly recognized. Catatonia should be studied systematically in children, adolescents, and young adults with psychiatric, neurologic, and developmental disorders--especially autism and Prader-Willi syndrome.

 The symptom overlap between autistic regression in CDD and catatonia should be further assessed, and the presence of catatonic symptoms should be assessed during regression in children with autism.

 Most important, catatonia in children seems to respond to the same treatments as in adults.

Recent accounts provide empirical evidence that catatonia is diagnosable in 7% to 17% of acute psychiatric inpatients and is treatable.

 This contradicts earlier comments that catatonia may have disappeared in adult psychiatry.

 Given these conflicting views and the ambiguous nosologic status of catatonia in 

one could argue that catatonia has become like the elephant and the blind men. Many aspects of catatonia are described but the syndromal diagnosis is often not made, possibly leading to suboptimal therapy. The emerging evidence that a similar proportion of autistic adolescents and adults also meet criteria for autism has 2 major clinical implications.

First, catatonia should be considered in any autistic patient of any age when there is an obvious and marked deterioration in movement, pattern of activities, self-care, and practical skills. An outline for diagnostic evaluation in such cases is shown in 

. Researchers should seek further evidence that catatonia in autism responds to accepted anticatatonic treatments. Treatment responsiveness is key in clarifying the nature of the beast. Clinicians may find the proposed treatment algorithms for catatonia in autism, as in 

, helpful in the treatment of the disorder in these challenging patients.

Second, autism should be considered as the underlying condition in patients presenting with catatonia, especially in those with histories of developmental problems. Psychiatrists working with adults are usually much more familiar with schizophrenia and other psychotic disorders than autism. Therefore, catatonia may be misdiagnosed as a feature of schizophrenia, and any underlying diagnosis of autism may be missed, leading to possible suboptimal treatment of both catatonia and autism.

The lack of controlled studies and the very small number of published cases to date must be emphasized. It is not clear from the available evidence whether treatment with lorazepam and/ or ECT is helpful in all cases of autism in which catatonic features become exacerbated or only in those with very severe forms of catatonia. Another consideration is the likelihood that only examples of successful treatments will be published. These problems emphasize the necessity for more research in this field. Cooperation between centers would be particularly valuable because each clinician is likely to see only a few cases.

The authors thank the parents of the young man in the first case for allowing publication of details of the illness of their son, and Miss Kathryn M. Darke (BSc psychology, 1-to-1 support worker) for writing up the information they provided.

Dr Dhossche is a professor of psychiatry and medical director of the University Child Psychiatry Unit at the University of Mississippi Medical Center, Jackson. He serves on the board of directors of the American Academy of Clinical Psychiatrists. His recent work includes editing 2 books in the International Review of Neuro- biology series, 

Catatonia in Autism Spectrum Dis- orders.

He reports that he has no conflicts of interest concerning the subject matter of this article.

Dr Wing is a psychiatrist involved in researching developmental disorders, particularly autistic spectrum disorders. She joined with other parents of autistic children to found the National Autistic Society (NAS) in the United Kingdom in 1962. She currently works part-time as a consultant psychiatrist at the NAS Centre for Social and Communication Disorders at Elliot House. Dr Wing lives in England and is the author of many books and academic papers, including 

a 1981 academic paper that popularized the research of Hans Asperger and introduced the term Asperger syndrome. She reports that she has no conflicts of interest concerning the subject matter of this article.

 Scattone D, Knight KR. Current trends in behavioral interventions for children with autism. 

 Buitelaar JK. Why have drug treatments been so disappointing? 

 Wing L, Shah A. Catatonia in autistic spectrum disorders. 

 Billstedt E, Gillberg C, Gillberg C. Autism after adolescence: population-based 13-to 22-year follow-up study of 120 individuals with autism diagnosed in childhood. 

 Realmuto GM, August GJ. Catatonia in autistic disorder: a sign of comorbidity or variable expression? 

 Dhossche D. Brief report: catatonia in autistic disorders. 

 Zaw FK, Bates GD, Murali V, Bentham P. Catatonia, autism, and ECT. 

Articles

Current treatments for autistic spectrum disorders (ASD) are useful in some cases, but have little enduring impact. This had lead to many parents seeking nonconventional treatments that often border on quackery.

Lorna Wing, MD

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