Self-injurious behaviour in children: A treatable catatonic syndrome

There is an excellent reason for publishing a paper on an unusual paediatric syndrome – self-injurious behaviour (SIB) – in a general psychiatry journal: it is among the few paediatric conditions that are highly treatable. The repetitive stereotypic catatonic movements in children with autism and intellectual disabilities, which can have such injurious consequences, respond readily to such standard treatments of catatonia as benzodiazepines and electroconvulsive therapy (ECT). The authors of this paper have discovered this in their respective domains of inquiry: Lee Wachtel, leading a neurobehavioural service in a paediatric hospital, and Edward Shorter, who ascertained that in many historical accounts of paediatric SIB other symptoms of catatonia were present as well, reinforcing Wachtel’s hypothesis of SIB as a form of catatonia. This hypothesis thus has two important pillars of support: the readiness with which SIB responds to anti-catatonic remedies in the clinic today, and the historical evidence that SIB fits in with larger syndromes of catatonia.

Thanks to the efforts of Max Fink and the circle of scholars building upon his findings, catatonia has expanded steadily from a subtype of schizophrenia into an independent syndrome that may occur in a variety of psychiatric and non-psychiatric illnesses. The reinsertion of the catatonia diagnosis into psychiatry is being officially recognized in the newly-released DSM-5. We feel that this recognition represents one of the most important developments in psychiatric nosology today.

SIB is classically defined as any self-inflicted act resulting in bodily harm. It afflicts approximately 5–30% of individuals with intellectual disability (ID), and has negative effects on physical health as well as sharply impairing global psychosocial functioning.

It is remarkable to discover that the inclusion of self-injury in the catatonic spectrum is not new. In fact, strong evidence of this concomitance can be traced in the international psychiatric literature beginning in the late 19th century. The historical evidence of catatonia in self-injury patients offers fascinating parallels to modern self-injurious presentations, raising the central question as to how this rich historical knowledge escaped incorporation into classic models of SIB assessment and treatment, particularly when modern medicine has possessed a safe and efficacious treatment for catatonia, namely ECT, for nearly eight decades.

Self-injury in historical perspective

Paediatric medicine has always been preoccupied with self-injury in children because of the disorder’s tragic perversion of the healthy vitality we associate with childhood. In 1887, J Langdon Down, the virtual founder of the study of developmental disabilities, wrote of puberty, ‘It is important . . . to be on guard against any concealed suicidal impulse, and to note its slightest indication, as the tendency in such cases is to melancholia and in some instances temptation to self-inflicted injury’ (1887: 95). In 1926 Theodor Ziehen, a pioneer of paediatric psychiatry in Germany, noted ‘the remarkable self-mutilations without suicidal intent, which occasionally take place among abnormal children, for example among the schizophrenic’ (1926: 291). Neither author mentioned catatonia, a term coined in 1874 by German psychiatrist Karl Kahlbaum (1874).

Among the earliest descriptions of SIB in the context of catatonia comes from an Athenian physician, G. G. Caryophylis, who had interned in Paris and published a case study in a French journal in 1892. A 13-year-old boy from a prominent Athenian family, ‘was already attending school when, towards the month of November 1888, it was noted that he was refusing to eat’. The next development was that he stopped speaking. Then, ‘Whenever he was constrained, the patient simulated contractions, did contortions of every kind, tore out his hair, grabbed his larynx to strangle himself, struck his head against the wall, etc. He spent most of the time stretched out on a sofa mute and staring at the ceiling, insensible to all the caresses of his sisters and the injunctions of his parents.’ Dr Caryophylis, whose aversion to the lad is apparent in this account, restored him to health in a private clinic through a kind of ‘forced suggestion’ (1892). Although the term is not used, the negativism (refusal to eat), mutism, sterotypies, and stupor of catatonia shine through. There are many similar historical examples.

Yet as catatonia becomes one of the subtypes of schizophrenia in 1899, independent reports of paediatric catatonia start to become less common: When clinicians saw catatonia, they thought ‘schizophrenia’. Or, because schizophrenia seemed improbable in some of these children, observers thought very little of the stereotypies and alternation of stupor and agitation that are quintessentially catatonic, and found such symptoms unremarkable.

For much of the 20th century, child psychiatry lay in the hands of the psychoanalysts, who really had very little clinical interest in catatonia and conceived it as part of the child’s struggle to feel alive. Margaret Mahler and associates wrote in 1959, ‘Most autistic children have a relatively low cathexis of their body surface, which accounts for their grossly deficient pain sensitivity. . . . Instead of autoerotic activities, they show autoaggressive habits such as head knocking, self-biting, or other self-hurting, mutilating activities, and a mixing up of oral, anal, and phallic contents. In fact, these autoaggressive activities seem to serve the purpose of boundary cathexis of a distorted and deranged libido economy, constituting a pathological attempt to feel alive and whole.’ (Mahler accepted the concept of ‘deteriorated catatonia’ in the context of René Spitz’s ‘hospitalism’) (Mahler et al., 1959: 818, 826).

Self-injury: recent perspectives

After this wave had passed in paediatric nosology, catatonia became transformed into ‘stereotypic movement disorder’, which included self-injury. This was its status in DSM-IV-TR (American Psychiatric Association, 2000).

In the current DSM classification system, the study of self-injury syndrome outside of ‘stereotypical movement disorder’, a diagnosis presented as an independent disease entity, seems almost to have disappeared.

‘Almost’ is the operative term here, because interest in paediatric catatonia did begin to revive with Gregory Fricchione’s discovery in 1983 that adult catatonia responded readily to benzodiazepine treatment.

Shifting towards individuals with autism spectrum disorders, Wing and Shah’s (2000) large population-based study in the UK revealed the intriguing presence of catatonia in up to 17% of autistic adolescents and young adults, a figure that is subsequently reported at 12% in a 2006 Swedish study (Billstedt et al., 2005), and 18% in a mid-Western US study of autistic individuals (Ghaziuddin et al., 2012). Case reports of catatonia in autistic patients relieved with electroconvulsive therapy began to appear.

Multiple autistic patients with catatonia and concomitant extreme self-injury subsequently appear in the literature. Many of these patients presented with additional affective, psychotic and tic disorders, and demonstrated profound, life-altering symptom reduction with ECT (Consoli et al., 2012; Dhossche et al., 2010; Wachtel et al., 2010, 2011).

At this point, the spotlight fell again upon SIB as part of the larger syndrome of paediatric catatonia. A number of clinical observations led to the hypothesis that repetitive self-injury without operant function is a movement disorder, best characterized along the spectrum of agitated catatonic behaviour and with direct implications for utility of ECT (Wachtel and Dhossche, 2010; Wachtel et al., 2011). It was reported that multiple patients achieved relief from horrendous self-injury after enduring years of failed behavioural and medication trials. Many of these individuals had been confined to extensive protective equipment and already incurred irreparable tissue injury. Parents and care-givers have been astounded by the nearly miraculous abolition of self-injury, with one father remarking that recognition of repetitive self-injury along the catatonic spectrum, with subsequent implementation of ECT, ‘released (his son) from the shackles of hell’ (Consoli et al., 2012). Fink officially included repetitive self-injury in autism along the catatonic spectrum in the 2013 update to his authoritative 2003 catatonia textbook (Fink, 2013).

In sum, the presence of SIB in catatonia is unmistakeable in the historical literature, spanning several countries over a century. The recently renewed recognition of catatonic agitation inclusive of repetitive self-injurious behaviour, and subsequent implementation of anti-catatonic paradigms, has conferred profound benefit for several autistic youth with intractable and highly dangerous repetitive self-injury.

Despite the benefits to children with autism and other developmental disabilities suffering from intractable self-injury, the authors sadly fear that this recognition risks loss in the annals of history. Shakespeare’s Hamlet postulated that ‘desperate illnesses wrought by desperate appliance relieved, or sometimes not at all’, and unfortunately the pervasive stigma associated with ECT throughout its 75-year history makes many clinicians and patients’ families consider its appliance as too desperate to pursue (Shorter and Healy, 2007). Dhossche et al. (2010) has theorized that errors of omission may occur, where clinicians avoid the catatonia diagnosis in order to escape possible invocation of unpopular treatment options. Concerned by political backlash, by criticism from autism communities committed to neurodiversity, and by subsequent loss of funding, facilities may choose not to provide ECT to appropriate autistic paediatric patients diagnosed with repetitive self-injury in the form of agitated catatonia. Indeed, the successful usage of ECT in catatonia in intellectual disabilities is already stigmatized, wherein a wholly effective treatment is discarded based on appearance and misperception alone (Wachtel and Dhossche, 2012).

Nonetheless, those of us in the trenches of daily patient care who attend to the grisly results of self-injury remain hopeful that science will prevail, with ongoing characterization of all forms of paediatric catatonia, and specific phenotyping of all self-injury to allow rapid recognition of the catatonic form. Unhindered implementation of appropriate clinical paradigms should follow regardless of public perception. It is critical to note that national autism organizations including Autism Speaks place ‘self-injurious behaviour phenotyping’ high on their agendas, a clinical necessity that has also been recently emphasized by the US-based Autism and Developmental Disabilities Inpatient Research Collaborative (ADDIRC meeting summary, Sept. 28–29, 2012), as well as the nascent parent-driven EASI (Ending Aggression and Self-injury) Foundation (A. Lutz, 2012, personal communication).

We suggest that such work take root in the richly documented history of self-injury and catatonia. Given the global devastation wrought by self-injury, and the number of children who remain recalcitrant to classic interventions, this clinical connection is simply too promising to ignore.