Abstract
A case is presented of an 11-year-old girl with pervasive refusal syndrome (PRS) who ultimately recovered acutely and completely after an 18-month paediatric hospitalisation. There was an apparent absence of previously proposed important aetiological factors in PRS, such as family pathology and markedly traumatic or abusive experiences, and her recovery was sudden and complete. The authors consider the differential diagnoses of PRS paying particular attention to the possibility of a conversion disorder or catatonia, given the absence of PRS in the North American literature. Consideration of catatonia is important as it has a diagnostic test and responds rapidly to appropriate treatment, in contrast to conventional treatment for PRS and conversion disorder.
Introduction
Pervasive Refusal Syndrome was first described in the UK in 1991 by Lask and colleagues, when they described four girls aged 9–14 who presented with ‘a potentially life threatening condition manifested by a profound and pervasive refusal to eat, drink, walk, talk, or care for themselves in any way over a period of several months’. Since that time specific diagnostic criteria for PRS have been proposed, including presentation with clear food refusal and weight loss, social withdrawal and school refusal, and partial or complete refusal in two or more of the following domains: mobilisation, speaking and attention to self-care (Thompson & Nunn, 1997). In addition the child is perceived to show active and angry resistance to being helped or encouraged. This cluster of symptoms occurs in the absence of any organic or psychiatric condition that could better account for the symptoms presented. To date 24 cases of PRS have been described in the English literature (see Table 1).
Case reports of PRS in literature.
The case presented shares many of the features of previous case reports, but is different in terms of the resolution of symptoms and length of follow up. The authors discuss differential diagnosis from the perspective of different countries.
Case Report
An 11-year-old prepubertal girl (J) was referred by her general practitioner (GP) to a paediatric hospital for admission, with an eight-week history of gradual physical deterioration of undetermined cause. At the time of her admission she was refusing to eat any food, was reluctant to drink fluids, complained of lethargy and appeared uninterested in self-care. In the week prior to her admission, she had also complained of colicky abdominal pain.
The episode appeared to begin with a 1–2 week history of a persistent non-productive cough, which had failed to respond to over the counter medication and had prompted her parents to seek their GP’s advice. A clinical diagnosis of asthma was made, and salbutamol and becotide inhalers prescribed. Despite this treatment, her energy levels deteriorated and her oral intake decreased. She was referred to her local paediatric outpatients department for investigation. Chest x-ray demonstrated hyperinflated lungs. Her condition showed no response to a three-week course of oral steroids. Her fluid intake became inadequate and she started to complain of colicky abdominal pain. Constipation was treated with laxatives. Her sleep pattern became disturbed – she awoke frequently during the night and would come into the parental bed complaining of various aches and pains. J was no longer able to attend school and had lost a significant amount of weight (6kg). Her mother perceived that J had adopted a ‘sick role’, reporting that she seemed to spend most of her day lying on the couch at home, moaning about various physical complaints. There was a two-month history suggestive of low mood which co-occurred with her physical symptoms and predated any medical intervention, for which no specific trigger could be identified.
No organic cause for J’s symptoms was found. There was no significant past medical history. Physical examination was unremarkable. Blood tests (full blood count, urea and electrolytes, amylase, liver function tests, bone profile, thyroid function tests and venous blood gases), abdominal ultrasound and CT Brain were normal. Two EEGs (usual protocol and sleep-deprived protocol) demonstrated some generalised discharges which were not associated with clinical seizures – these findings were not felt to explain or contribute to her clinical presentation. She was referred to the Child Psychiatry liaison team for evaluation.
J was the youngest of three children. Her parents were married and aged in their mid forties. Her father was a self employed computer specialist and worked long hours. Her mother worked at home full-time. There was no history of parental adjustment problems or of marital discord. The parenting style was authoritative. Her siblings were well-adjusted and high functioning. The family had well developed social networks and a supportive extended family. Relationships within the family were harmonious. There was no history of abuse. A paternal aunt and a maternal uncle had suffered from depression. There was no other known family psychiatric history.
The girl’s developmental history was unremarkable. Pre-morbidly she was described as having been ‘plump’ but not overweight, and unconcerned about weight or body image. She had always been ‘a bit anxious’, especially in relation to her health. She would become ‘very panicky’ if hurt, especially at the sight of blood. She was ‘a sensitive child’ who was especially vulnerable to any perceived slight by her peers. She had two friends from her neighbourhood, though no close friends. She was happy to go on play dates or sleepovers but her preference generally was for her friends to come to her house. She enjoyed playing with her dolls, attending speech and drama, and sometimes swimming and basketball.
J reluctantly attended playschool from the age of four. At age five she commenced primary school, and her first year had gone well. Early in her second year, she became anxious about under achieving but this was resolved. At age seven, her teacher noted she appeared anxious and frequently complained of abdominal pain. There was no history of bullying.
Collateral history from her class teacher described her as a ‘quiet’, ‘reserved’ child who appeared ‘anxious’ and ‘nervous’ and complained of minor physical ailments at times. She was not perceived as unhappy or sad. Whilst enjoying the activities of the class, she was reluctant to put herself in a lead position. She worked willingly with groups, appeared to be socially engaged with a variety of children, but had no special friend. Academically she was described as an average student who did not appear to have any difficulty keeping up with her work.
On admission the girl had talked and engaged in conversation with staff and other children but to a limited degree. She was frequently preoccupied by her physical symptoms, i.e. colicky abdominal pain, a frequently picked scab on her head which was slow to heal, an intermittent non-productive cough, transient limp in her right leg and occasional headaches. With her family she engaged less. She spent a significant amount of her time with them moaning and crying, and had taken to covering herself with her bedclothes when they arrived for visits. She began adopting a similar response when she saw staff members – disengaging from the activity she was involved with, dropping her head, avoiding eye contact and sometimes covering her face with her hands or hair. When the girl did talk, she expressed an inability to explain her refusal to eat. In general it was noted that she found it difficult to express her feelings or worries. On one occasion however she requested an individual session during which she expressed a number of concerns that she wanted to have raised at the next family meeting. She was concerned that her parents weren’t letting her grow up and were ‘keeping (her) a baby’. She mentioned some incidents when she felt her mother had been unsupportive of her, e.g. when her mother promised to tell the doctor of her various complaints and then had failed to do so. She expressed some disappointment with her father, for being harsh with her (e.g. ignoring her or blaming her for something she didn’t feel she had control over) and for comparing her to her older siblings and thus depriving her of her own identity. She also stated during this session that she did not want to eat and get better for fear of her worries being forgotten.
Over the space of a number of months in hospital her initial limited engagement stopped completely. Initially, she refused solid food but managed an intake of 1000kcals/day by the use of a carbohydrate supplement dissolved in water. When after five weeks a decision was made to pass a naso-gastric feeding tube, J immediately refused any oral fluids.
Attempts were made to integrate J into ward life as much as possible – encouraging her to sit at the table with other children at mealtimes and attend play therapy in the mornings and school in the afternoons. In the early months of her admission she participated in school and ward life to a limited degree. However, when school re-opened after the summer holidays, she refused to re-attend. She was also encouraged to engage in activities off the ward with her family with limited success. After a few months she became less and less engaged – ultimately she would not talk, look at or interact with staff, her parents or peers.
She had bi-weekly individual sessions with a Clinical Psychologist (CM) but choose to stay silent for these, occasionally resisting being brought to the sessions but at other times walking unaided and appearing to listen to what was being said. However, these sessions became increasingly difficult as she would cover her face with her hair or avert her face so that no clue as to what she was thinking could be deduced. In addition, there were weekly family sessions which parents and siblings attended. Although the girl participated in these sessions in the early stages of her admission, she subsequently became increasingly reluctant to attend or join in. The girl invariably seemed very unhappy and angry during these sessions, often crying or screaming throughout. She frequently pulled and slapped her father, while insisting on sitting on his lap. The girl tried to give the appearance of not paying attention but clearly was alert to most of what was going on – if annoyed by what was said she would sometimes make growling or screaming noises. She became increasingly resistant to leaving the ward unless in a wheelchair, and ultimately the CM sessions were held on the ward. Since admission she refused all attempts made to encourage her to spend time outside the hospital, or to visit home. J also attended group work and while she seemed to listen to what was being said by peers and staff, she did not participate directly. Other interventions offered included Art Psychotherapy, Physiotherapy and Hydrotherapy.
Antidepressant medication was tried with no discernible effect. The girl received a sub therapeutic trial of Sertraline (150mg), and later of Paroxetine (20mg), both of which had to be discontinued after four weeks as a result of side effects. She did tolerate an eight-week trial of Venlafaxine (150mg), but with no response. Lorazepam was offered for extreme agitation and distress, at doses of .5mg as required up to 4mg total daily dose. Consultation with experts in the management of PRS shaped therapeutic revisions of her treatment programme. (BL, MF, MP) Narcokinesis (examination and suggestion while mildly sedated with IV amylobarbitone) did not have any beneficial effect. ECT was considered and an independent second opinion sought. This was rejected by her parents. Risperidone (2mg) was given for a total of four days. Child psychiatry inpatient placements were sought, but none were available within Ireland that would accept a child with NG feeding.
After a total of 18 months in hospital J had shown no response to any intervention provided. During the latter nine months of this admission she had refused to eat, drink, engage in self-care or communicate in any way with staff, other patients or family. It was felt that optimising the therapeutic milieu would best be achieved in a Child Psychiatry inpatient unit abroad which had expertise in managing PRS, and a transfer to a specialist unit in UK was arranged.
Two days before transfer, J wrote a note to the nursing staff – this was her first attempt to communicate with anyone in nine months. Uniquely, on the weekend in question, none of the girl’s regular nursing staff were on duty. Risperidone 2mg had been started two days previously. The note she wrote simply stated ‘I want to go home’. This was quickly followed by a further note consisting of rows of the words ‘I want to go home so ring my mammy’. It was decided that the girl should be allowed home immediately with the NG tube left in situ and reviewed daily. The following day she went clothes shopping with her family and had something to eat in a fast food restaurant. She began eating normally at home and her NG tube was removed three days later, and Risperidone discontinued. She started communicating with her family through prolific note writing. Two weeks later she was speaking fluently. Four weeks after her discharge from hospital, the girl was fully integrated into her new school class both academically and socially.
Her mother attended for follow up appointments with the liaison Psychiatry team, although the girl herself refused to ever re-attend. She continued to eat normally and do well in school. Her parents never pressed her on the reason for her symptoms, and the girl never indicated the reasons why she had stopped eating and communicating. She did, on occasion, make reference to some incidents that occurred during her time in hospital, showing she had been alert during that period, and retracted some statements about feeling unloved, infantilised or unfavourably compared to her siblings.
The girl continued to do well in school, achieved high grades in her state exams and, six years on, has gone on to University. She did not appear to experience any anxiety regarding study or school. Socially, she has a group of friends with whom she goes away for holidays or overnight stays; she maintained the same best friend that she made shortly after joining her new class upon discharge. She enjoys drama, attending discos, listening to music and shopping with her friends. Psychologically, her parents report that she has good coping skills – sharing some confidences with her mother, but more with her peers. Her mood is described as ‘very happy’. She gets mildly anxious regarding music exams but is able to deal with this appropriately. She has a good body image, takes pride in her appearance and has normal eating patterns. Physically, she has remained well, and missed only four school days over six years as a result of minor illness.
Discussion
This report describes the presentation of a young girl with features suggestive of pervasive refusal syndrome. Similarities to literature reports include the insidious onset of vague aches and pains progressing to more prominent somatic complaints, severe food reduction through to complete refusal of both food and fluid, and a gradual withdrawal from communication and self-care leading to complete and dramatic cessation and avoidance. This disorder has been more frequently been described in females and young children. Of the 24 cases described in the literature, 79% (n=19) are of females (mean age = 11.2 years; standard deviation
The original cases described by Lask and colleagues (1991) and Graham and Foreman (1995) proposed sexual abuse or domestic violence to be important aetiological factors in the development of PRS. Later case reports have disputed this association. Jaspers et al. (2009) suggest that it is trauma in general, rather than sexual abuse in particular, that may be important in the aetiology of PRS. In their review of published cases, they estimate that a history of recent trauma (e.g. witness to violence, marital/parental problems, bullying, refugee status etc.) was present in 79%. In the case presented, there was no history of abuse or family discord. Indeed the family was epitomised by the parents’ close, caring and steadfast support of both the girl and her siblings – throughout her 18-month admission they travelled long distances on a daily basis to visit their daughter, and on a weekly basis to attend family sessions. Neither was there a history of recent trauma or stressful life events. In their review of the literature, Von Folsach and Montgomery (2006) highlighted two other important aetiological factors in the development of PRS: parental psychiatric problems and previous child psychiatric difficulties. In the girl’s case, there was no history suggestive of parental psychiatric morbidity. Furthermore, although there was some suggestion of the girl having had anxious tendencies, they were not interpreted as having been clinically significant and there was not an overt display of anxious symptoms while an inpatient. An enmeshed mother-child relationship is also frequently cited as an associated feature of PRS but this was not apparent in the girl’s case. Prognostically, this case is also different in that despite the chronic nature of the girl’s symptoms, her recovery was sudden, rapid and complete within a four-week period. Many of the original cases described in the literature showed ‘slow but consistent progress’, with ‘the degree of improvement and time taken to recover being linked to the length of illness prior to presentation’ (Thompson & Nunn, 1997).
In considering aetiological factors in PRS, Lask and colleagues (1991) draw attention to similarities with the avoidance behaviour seen in post-traumatic stress disorder and suggest a two-stage process, the first being the experience of sexual abuse of a sadistic nature, and the second the fear of induced silence. In these cases, once the abuse was disclosed and the child protected, slow and gradual recovery took place. Nunn and Thompson (1996) view PRS as an extreme form of learned helplessness resulting from loss of personal and vicarious hopefulness. As with Seligman’s dogs (Seligman & Meyer, 1968), there is an experience (by the child) of uncontrollability of one’s future or lack of ability to avoid subsequent trauma (electric shock), with loss of hope leading to extreme passivity. Just as the dogs eventually showed refusal in the face of ‘forced escape/avoidance training’, so the child shows angry resistance to offers of help. Nunn and Thompson (1996) suggest that such uncontrollable events may include loss by death or separation, relocation, or severe illness in child or parent, often occurring with a background of maternal overprotectiveness or parental helplessness and hopelessness. This model provides valuable information about aetiology, treatment and clinical presentation.
The reason for this girl’s acute resolution continues to baffle the clinicians. It is unlikely that two days’ prescription of Risperidone would have had such an effect, and her improvement continued even when Risperidone was discontinued. It is possible that the threat of going abroad to a distant unfamiliar unit, coupled with the experience of the theft of her mobile phone in her presence, led to a sense of great insecurity and heightened anxiety that allowed her, for the first time since her admission, to consider going home to the safety of her own family. In the same way that a traumatic event can lead to a fear induced pervasive refusal, as suggested by Lask and colleagues, could such an event also reverse the process? It is also possible that the absence of her regular nursing staff, with whom she had developed an expected way of behaving, allowed her to react differently and ask to go home. Considering Nunn and Thompson’s model of learned hopelessness (1996), it is possible that the girl’s realisation that she could in fact determine whether or not she went to an in-patient unit in the UK may have mobilised her to become active and regain hope. In the absence of an explanation from the girl herself, all proposed explanations remain speculative.
Differential diagnoses
An underlying organic cause for the girl’s symptoms was ruled out following extensive medical investigation. Other diagnoses, including a major depressive disorder, anorexia nervosa, selective mutism, school refusal, a conversion disorder, somatisation disorder and chronic fatigue syndrome were considered but regarded as inadequate to explain the severity and pervasive nature of her symptoms. Catatonia, as a differential, was proposed by an American clinician following a review of the case and will be discussed separately. Similarly, Depressive Devitalisation, coined by Bodegard in 2004 to describe a variant of PRS, will also be discussed (Bodegard, 2004).
A Major Depressive Episode was the main differential diagnosis in this case. There was a history suggestive of low mood at the onset of the girl’s illness, and her symptoms included multiple psychosomatic complaints, fatigue, poor oral intake, loss of interest in her usual activities and failure to attend to her self-care. At times during her hospital admission she looked objectively depressed, sad, upset, tearful and mono-syllabic. This occurred in the context of a positive family history of depression and a pre-morbid temperamental style of anxiety. The oral steroids that the girl had received for treatment of her asthma early in the course of her presentation may have further contributed to her mood disturbance, but post-dated some of the depressive symptoms. Against this diagnosis, her mood was noted to vary considerably according to context, and at times she appeared quite content. Also, the girl’s resistance to eat or attend to her personal hygiene was the result of an active, wilful refusal rather than the passive refusal that might be expected with low mood. On balance it was felt that the girl’s presentation could not solely be accounted for by a depressive disorder. However, concern remained throughout her admission about the possibility of a depressive comorbidity and this was treated accordingly, albeit with little response.
Two of the essential features of Anorexia nervosa were absent in this case – an intense fear of weight gain and a distorted body image. Initial food intake was not linked to foods of high calorie content, and although her food refusal resulted in significant weight loss, it did not appear to be motivated by it. Furthermore, her refusal was not limited to eating and drinking. The essential feature of Selective Mutism is the persistent failure to speak in specific social situations (e.g. school, with playmates) where speaking is expected, despite talking in other situations. The girl’s mutism was not confined to a particular situation and she was initially more likely not to speak with family members, while speaking with nursing staff had continued. Unlike the typical selectively mute child, she was not functioning well in other areas of her life. Similarly, a diagnosis of School Refusal was discounted: while the girl had stopped attending her own school, she did initially attend the hospital school. Thereafter, her social avoidance was not restricted to school attendance; neither was anxiety a prominent feature of her presentation.
A Conversion or Dissociative Disorder was considered and, given the absence of PRS in the DSM or ICD manuals, this seems to be the most widely accepted label that most closely resembles her presentation. ICD-10 indicates that the ‘conversion’ is that of the unpleasant feelings engendered by an insoluble dilemma or disturbed relationships into physical symptoms. It suggests that typically these symptoms last weeks or months, but the prognosis is worse if the problems are chronic and ongoing. It also states that symptoms can vary from day to day or even hour to hour, which can suggest changes in conscious or selective control that can be hard to assess. In Garralda’s authoritative review of somatic presentations (Garralda, 1992a), she suggests that the initial trigger for conversion disorders in children is usually physical and that as a result they and their families are often hostile to subsequent psychological explanations and treatments. She also suggests that gross psychopathology in the child and family is not found in the majority of cases.
A Somatisation Disorder was considered on account of J’s frequent, multiple physical complaints, for which no organic cause could be found. However, these complaints were not the most prominent feature of her presentation, and unlike the help seeking behaviour usually associated with somatisation disorders, the girl actively refused to cooperate with examination. Chronic fatigue syndrome (CFS) was considered as the girl’s illness did appear to begin with a viral illness and she did initially complain of fatigue. However, although present, fatigue was not a prominent feature and other symptoms often associated with chronic fatigue syndrome in children (e.g. myalgia, lymph node tenderness, memory and attention difficulties (Bell et al., 2001)) were not present. While children with CFS often find the process of graded rehabilitation difficult, they do not usually actively refuse to cooperate to the same extent.
Our differential diagnoses also included a catatonic state accounting for, or contributing to, the girl’s presentation. This differential warranted particular consideration, especially as the girl’s condition continued to deteriorate, and as a diagnosis of catatonia would have had significant implications for treatment, the treatment approach to catatonia being markedly different to that of our working diagnosis. DSM-IV lists five clinical features of catatonic disorder: motor immobility, excessive motor activity (which is apparently purposeless and not influenced by external stimuli), extreme negativism or mutism, peculiarities of voluntary movement, and echolalia or echopraxia (American Psychiatric Association, 2000). These features are common to catatonic disorder due to a general medical disorder, the catatonic type of schizophrenia, and catatonic features as a specifier of mood disorder. Similarly, ICD-10 describes organic catatonic disorder, depressive stupor and catatonic schizophrenia (World Health Organisation, 1992). Such descriptions can be confusing as they include some seemingly contradictory signs – motor immobility is described along with excessive motor activity, extreme negativism along with automatic obedience, and mutism along with verbigeration (Penland et al., 2006). Also, more than 40 motor signs of catatonia have been described (Taylor & Fink, 2003). Of these, the most common signs reported are mutism, posturing, negativism, staring, rigidity and echophenomena. Taylor and Fink suggest that only two motor signs in a 24-hour period are required to make the diagnosis. Penland et al. argue that the reason for this lack of clarity is due to the extremely variable presentation of catatonia. The situation is further complicated in the case of children and adolescents, for whom there are no accepted, standardised diagnostic criteria (Takaoka & Takata, 2000). Penland and colleagues caution that ‘a high index of suspicion and broader delineation of catatonic signs and syndromes’ is required if potential cases of catatonia are not to be missed (Penland et al., 2006). Indeed, there is evidence that due consideration might not be given to this diagnosis in clinical practice (Van der Heijden et al., 2005) and that the presentation of catatonia in children is not as rare as previously thought, with estimates that catatonia occurs in 0.6% of the adolescent inpatient population (Cohen et al., 1999).
Discussions with a leading US clinician in the field of catatonia (Max Fink) drew attention to the similarities of presentation between the girl’s case and catatonia. Dr Fink argued that the girl’s symptoms should be reinterpreted: her refusal to speak as an indicator of mutism, refusal to eat/drink/attend to self-care as a demonstration of negativism, her repetitive moaning/grunting suggestive of tics, her refusal to engage as evidence of withdrawal. He also viewed as clinical relevant her periods of intense staring in family sessions and her rigidity upon physical examination. He was of the opinion that rather than pervasive refusal syndrome, her case should be conceptualised as catatonia, and consequently a different treatment approach should be adopted. This view was different to the view shared by other European consulting clinicians.
Established treatment for catatonia includes po/iv benzodiazapines, high dose barbiturates and ECT (Taylor & Fink, 2003). The treatment of catatonia with barbiturates dates back to the pioneering work of Bleckwenn in 1930, who wrote up and even filmed patients with catatonia showing dramatic recovery after the use of intravenous barbiturates (Bleckwenn, 1930). Over time, benzodiazepines (BZ), such as diazepam and lorazepam, have come to replace barbiturates. Both are believed to have their action through direct and indirect effects on the GABA – A receptor. A trial of oral BZ, albeit at lower than recommended doses (doses of up to 8mg/day of lorazepam were advocated by Dr Fink), was not associated with any notable effect. Barbiturates were used as part of the abreaction and narcokinesis and had if anything the opposite effect to that expected. Taylor and Fink (2003) suggest that 30–50% of cases of catatonia may not respond to lorazepam or amylobarbital challenge. Catatonia was considered in the differential diagnosis. Although a proper test was not done, the girl was given IV amylobarbitone on one occasion and was treated with lorazepam up to 4.0 mg daily without beneficial effect. However, it is accepted that following the catatonia treatment guidelines, this dose is not considered adequate for an effective treatment trial (Taylor & Fink, 2003).
There is a small but growing literature to support the use of ECT in young children (Fink & Carlson, 1995). ECT is considered to be an effective treatment for catatonia in this age group, although it is acknowledged that there is a paucity of systematic data to support this conclusion (Takaoka & Takata, 2000). Although young age is not a contraindication to ECT, concern over the possible impact of inducing a series of seizures in a still-developing brain means most clinicians are reluctant to advocate this line of treatment where alternatives exist (Cizadlo & Wheaton, 1995). In 1990, the report of the American Psychiatric Association (APA) Task Force on ECT cautioned that the use of ECT in children should be limited to instances ‘where other viable treatments have not been effective or cannot be safely administered’. The AACAP Practice Parameter for Use of Electroconvulsive Therapy With Adolescents (2004) did not address the use of ECT in preadolescent children because of ‘insufficient data and clinical experience’ in this age group.
A review of the literature in the area yielded two case reports of young girls which had some striking similarities with our case. The girls’ conditions had shown a good response to ECT. Cizadilo and Wheaton (1995) described the case of an 8½ year old girl who presented with a one-month history of pervasive low mood, social withdrawal and reduced appetite. There was no past history of psychiatric illness or of a precipitating stressor. A diagnosis of depressive disorder was made, and she was treated with an SSRI and individual psychotherapy. Her condition rapidly deteriorated to complete mutism, food refusal, enuresis and encopresis. The authors noted that while early on in her illness she had been overly compliant, later she was combative at times. A lack of response to an alternative antidepressant and lorezepam prompted the authors to consider ECT. The girl responded well to a course of 19 ECT treatments over a six-week period, and was discharged well three weeks later. Esmaili and Malek (2007) reported on a six-year-old girl referred with active food refusal, mutism, and marked weight loss (50% total body weight). There was a six-month history suggestive of low mood, in the absence of a previous history of psychiatric illness or identifiable trigger. Psychomotor retardation was prominent. Eight sessions of ECT over a four-week period resulted in resolution of her condition. However, other case reports for such severe somatic presentations when conceptualised as chronic fatigue syndrome have not been so favourable. (Garralda, 1992b).
Given J’s lack of response to the prescribed management strategy for PRS, the alternative conceptualisation of catatonia, together with its implications for treatment, was presented and discussed with her parents and the option of ECT proposed. However, ECT was not considered a viable option by her parents, in keeping with a general pervasive reluctance by child psychiatrists to consider ECT in children in Europe (Parmar, 1993).
Depressive devitalisation: A subgroup of refusal syndrome
Depressive Devitalisation, a term coined by Bodegard in 2004 in Sweden, describes a life threatening condition in asylum seeking children who presented with profound social withdrawal and pervasive loss of body functions (Bodegard, 2004). Many of these children had experienced multiple traumas. Similarities with PRS were identified in both presentation and putative aetiological causes. Notable differences existed in that the purposeful aspect of refusal, characteristic in PRS, was less pronounced in Depressive Devitalization, and these children presented with flaccid paralysis and general sensory loss not reported in PRS. Hopelessness and helplessness were seen as aetiologically important in both conditions. Bodegard (2004) proposes that ‘learned helplessness’ may have been generated by distortions in the parent (usually mother)-child interaction, also referred to as ‘lethal mothering’, whereby the mother’s attitude, mood and behaviour were consistent with a belief that the child would in fact die, leading to the child taking up the position of the ‘devitalized infant’. The number of reported cases in Sweden has been increasing rapidly, with estimates of more than 400 cases described, of varying severity (Ahmadi, 2005). It has been termed ‘the giving up syndrome’ or ‘the apathic children’ in media reviews or policy documents (Bodegard, 2004). The descriptions of this disorder to date do not support a reconceptualisation of our case from one of PRS to one of Depressive Devitalization, but do highlight the varied and life threatening responses children may present with when faced with perceived adverse life events.
Ethical considerations
Depressive Devitalization and PRS are considered to be subgroups of the same refusal syndrome, both with themes of learned helplessness, and both respond well to the management strategies proposed by Nunn et al. (Nunn et al., 1998
Management must proceed along a number of developmental domains – biological, psychological and social – with a focus on promoting personal hopefulness. The creation of an environment with clear communication of expectations, and incremental re-involvement in nutrition, self-care, mobilisation, socialisation and education, is necessary, all of which has to be pursued against a tide of resistance. Treatment must not become punitive. Failure to intervene in maladaptive behaviour may be unethical, even in the absence of child consent. This takes time and patience, and needs the co-operation and support of the parents. The majority of case studies have taken one year to recover after the institution of appropriate treatment (Lask, 2004). The consequences of behaviour associated with PRS (i.e. refusal to eat/drink other than via NG, to mobilise, to communicate or socialise or to progress academically, and the possibility of remaining an inpatient away from family, or housebound, for many months) are not easily measured. In the case described by Nunn and Thompson (1996), the 15-year-old girl had a history of symptoms spanning more than five years, two of which were spent housebound in a wheelchair. Adolescence is a time of significant developmental challenges, and deviations from expected developmental trajectories make the clinician very concerned about irretrievably losing skills through non-use, (use it or lose it’ principle). The clinician’s fear of ‘non-use’ and desire for a return to normal functioning is often linked with aggressive and innovative treatments.
J’s case also highlights the unique difficulties in treating children with medical and psychiatry needs in Ireland. At the time of her presentation in Ireland there was no child psychiatry unit able to accept a child who needed NG feeding. Despite the best efforts of all involved, treatment in a paediatric hospital without the support of a therapeutic milieu and psychotherapeutic day programmes added to the difficulties in her management.
These ethical dilemmas were well documented in a case report by Graham and Foreman (1995) describing an eight-year-old girl with PRS refusing to return home, and their need to disentangle the intrapsychic nature of her refusal from reality. They discussed the merits of searching for aetiological factors versus focusing on symptom relief. Fink and Klein (1995) held an opposing view, believing the ethical dilemma to be one of diagnostic confusion and considering the girl in question to meet criteria for catatonia and, therefore, a different treatment approach. Ethical issues might also be relevant when a diagnosis of PRS is not considered. In the case described by Cizadilo and Wheaton (1995), over time the clinical presentation had much in common with features of PRS, but there is no reference made to this as a possible diagnosis. It is recognised that PRS is a diagnosis rarely made in the United States. Whether the treatment outcome for this eight-year-old girl would have been any different had she been treated using the approach recommended in PRS rather than ECT is not clear. This also raises the reverse question of whether the outcome for children with PRS would be any different if conceptualised diagnostically as catatonia or an atypical form of depression, and treated as such.
Abreaction and narcokinesis
The treatment of mutism with benzodiazepines and barbiturates has a long history and has been used both by those following psychodynamic models of aetiology and those with neuropsychiatric conceptualisations. The term ‘abreaction’ has been used to describe a process in which difficult repressed emotions are brought to the surface within a therapeutic setting. Some advocates use hypnosis or medication to facilitate the disinhibition required (Houston, 1952). Abreaction has also been used in the treatment of hysterical paralysis and mutism, in both adults and children (Maurice-Williams & Marsh, 1985). In some descriptions recovery is immediate; in others recovery is transient and function deteriorates after the barbiturate or benzodiazepine has been metabolised. A recent survey of British psychiatrists showed that abreaction is rarely used in general psychiatric practice, though it is occasionally used in liaison work (Wilson, 2002). Narcokinesis has been recommended as an adjunct to the management of severe somatising disorders, as it might ‘bump start’ the process of recovery. In J’s case this was undertaken by a clinician from the UK with expertise in this field (MP). In this procedure, the subject is sedated with IV amylobarbitone and then asked to try very hard to move the affected part of the body themselves. They are given strong praise for whatever is achieved, together with suggestions of continued improvement. However, even in a deeply relaxed state, the girl was able to resist any suggestions made, and no response post treatment was noted.
Conclusion
Pervasive refusal syndrome is a rare disabling and potentially life threatening disorder not yet included in the major child psychiatry classification systems. True prevalence rates may be higher as there are other similar clinical reports in the literature which may have been conceptualised as other conditions, such as ‘severe chronic fatigue syndrome’, rather that PRS (Garralda, 1992b). It is essential to raise clinicians’ awareness of this condition in order to allow early recognition and appropriate treatment. Reports on PRS are notably absent from the American literature. Whilst PRS has many overlapping clinical features with other disorders, in this case the authors have highlighted the links with catatonia on account of the clinical insight offered by expert opinion. We have to come to understand that catatonia is a state with multiple aetiologies and is not confined to schizophrenia: for example, it presents in systemic illness, neurological insult, toxicity, bipolar illness and psychogenic disorders. Consideration of this diagnosis (i.e. catatonia) opens up a variety of therapeutic avenues.
Irrespective of her diagnosis, this girl presented with life threatening and disabling mental and physical symptoms that were, fortunately, followed by a full and lasting recovery. Whether her recovery may be attributed to her treatment regime, natural remission or certain environmental factors can not be determined. Had she received or tolerated alternative treatments – i.e. larger doses of medication, including benzodiazepines, or ECT – would her course or speed of recovery have been any different? Had treatment in a specialised child psychiatry unit in her country been available to her, how would this have affected her course of recovery? This case highlights the difference in diagnostic conceptualisations between different countries. This concept is not unique to PRS/catatonia/conversion disorder but also exists for other conditions, such as Bipolar/ADHD (Dubicka et al., 2008). These differences become important when different diagnostic concepts lead to very different treatment approaches. Diagnostic clarity and refinement continues to be an area of great concern and should be a focus of discussion in committees developing new diagnostic guidelines (DSM-V and ICD-11). There is also an urgent need to build awareness of differences in conceptualisation amongst clinicians and training bodies in different countries, and to continue to be open to learning from each other.
Footnotes
Acknowledgements
The girl and her parents have given permission for this case report to be published. Some of her personal details have been changed to protect identity. We would like to acknowledge the expert advice and support offered by Dr Carol Matthews, Dr Mike Prendergast, Dr Max Fink and Dr Bryan Lask in assisting us with the management of this case.
Funding
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflict of Interest
None declared.
Author biographies
She has a particular interest in increasing public awareness of child psychopathology and developing early intervention and screening at a primary care level. She contributes to the training of GPs. She is very involved in providing information for parents and families through a monthly lecture series held by Lucena Foundation, of which she is one of the founding members. These cover a very broad range of mental health difficulties, from ADHD to Autistic spectrum disorders, depression and other adolescent issues. She regularly works with teachers, providing informal sessions at school, contributing to service training days and running summer training programmes, and delivers talks to secondary school students and their parents. She has co-authored a guide for teachers regarding mental health in children and adolescents. She regularly contributes to postgraduate training courses in Educational Psychology, Special Education Needs and teacher training. She was a lead contributor to the development of a training package in Assessment of Mental Health needs of children and their families for Area Medical Officers and Health Visitors.