A Review of Behavioral Treatments

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BEHAVIOR MODIFICATION / April 2000Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME

Lesch-Nyhan syndrome is a genetic disorder resulting in hyperuricemia, choreoathetosis, men-

tal retardation, and self-mutilation. The most salient feature of this disorder is the self-injurious

behavior (SIB). Although the utility of behavioral interventions with SIB has been well docu-

mented, behavioral interventions withLesch-Nyhan syndrome have beenlimited in number and

long-termsuccess. This article reviews the behavioral treatmentsthat have been used in treating

individuals with Lesch-Nyhan syndrome and discusses the strengths and weaknesses of these

methods. Suggestions for future directions in the use of behavioral interventions for controlling

SIB in Lesch-Nyhan syndrome are provided.

A Review of Behavioral Treatments

Used for Lesch-Nyhan Syndrome

LYNN OLSON

University of South Carolina

DANIEL HOULIHAN

Mankato State University

Lesch-Nyhan syndrome was first described as a distinct disorder in

1964 (Lesch & Nyhan, 1964). It is a rare recessive genetic disorder

passed on the X chromosome (Fernald, 1976). Although a few iso-

lated cases in females have been reported (Yukawa et al., 1992),

Lesch-Nyhan syndrome occurs almost exclusively in males (Nyhan,

1973, 1976).

The syndrome is characterized by a consistent set of behavioral and

neurological symptoms, including spasticity, choreoathetosis, moder-

ate mental retardation, and self-injurious behavior (SIB). The most

salient characteristics have been the behavioral aspects of the disorder

(Nyhan, 1976), most specifically self-mutilation, which generally

leads to the correct diagnosis of Lesch-Nyhan syndrome (Christie

et al., 1982; Mizuno, 1986). According to Baumeister and Frye

202

AUTHORSNOTE:Please address editorialcorrespondence to Daniel Houlihan, Ph.D., Depart-

ment of Psychology, Box #35Armstrong Hall, Mankato State University, Mankato, MN

56002-8400; phone: (507)389-6308; e-mail: [email protected].

BEHAVIOR MODIFICATION, Vol. 24 No. 2, April 2000 202-222

2000 Sage Publications, Inc.


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(1985), the incidence and severity of SIB in this disorder separate it

from other disorders in which SIB is a common feature (e.g.,

Tourettes syndrome, schizophrenia, mental retardation, etc.). Spe-

cific features of the SIB of Lesch-Nyhan patients distinguish their

behavior from other forms of SIB. First, a prominent feature includes

the loss of tissue created by the SIB in the patients and the swiftness

with which these patients can perform the behaviors despite their

motor difficulties (Nyhan, 1976). In a study of 40 Lesch-Nyhan

patients, Anderson and Ernst (1994) found that 90% had permanent

physical damage due to their SIB. Second, the compulsive nature of

the behavior, as opposed to the stereotypic nature found in other disor-

ders, also differentiates the self-destructive behavior of the Lesch-Nyhan patient (Fernald, 1976). Third, the patients with Lesch-Nyhan

appear to have no deficiency in their sensation of pain (Lesch &

Nyhan, 1964; Nyhan, 1973, 1976). Finally, the patients are aware of

their inclination for SIB and become agitated and fearful when

restraints are removed (Lesch & Nyhan, 1964; Nyhan, 1973, 1976).

Given these features of SIB, it seems obvious that a successful

treatment is imperative for these patients. Further, as ethics and legal

requirements have evolved over the past two decades, both physicians

and mental health workers have become increasingly obligated to the

Least Restrictive Treatment Model for these clients. According to

Foxx (1982), the right of persons with retardation to receive the least

restrictive treatment includes living arrangements, as well as interven-tions to increase or decrease behaviors. Therefore, it is necessary to

evaluate all methods used, as well as the successful methods, with the

Lesch-Nyhan patient to facilitate the use of the Least Restrictive

Treatment Model with these patients.

ETIOLOGY

The etiology and biochemical abnormalities associated with the

disorder are well understood (Baumeister & Frye, 1985). The respon-

sible mutation is found on the X chromosome (Nyhan, 1976). Seeg-

miller, Rosenbloom, and Kelly (1967) were the first to identify the ori-gin of the disorder. The authors discovered the complete absence of

Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 203


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the hypoxanthine guanaine phosporibosyl transferase (HGPRT)

enzyme, an enzyme responsible for the metabolism of pruines. The

consequence of this defect is hyperuricemia (Nyhan, 1976), in which

uric acid is distributed in excess amounts throughout the bodily fluids.

Lesch and Nyhan (1964) and Nyhan (1973) reported uric acid in the

urine four to five times that of typical persons. Thefirstevidence of the

hyperuricemia usually occurs early in infancy with orange crystals

appearing in the diapers (Christie et al., 1982).

CLINICAL MANIFESTATIONS

Despite a clear understanding of the etiology of this disorder, it is

less clear how these abnormalities result in the remaining clinical

manifestations of the disorder (Baumeister & Frye, 1985). These fea-

tures of the disorder include spastic cerebral palsy, SIB in the form of

biting the lips and fingers, and mental retardation (Lesch & Nyhan,

1964).

GENERAL DEVELOPMENT

According to Nyhan (1973), those who develop Lesch-Nyhan syn-

drome appear to mature normally to the age of about 6 to 8 months.

Around that time, however, the initial cerebral symptom of the disor-der, athetosis (continual, slow movements especially in the extremi-

ties) develops. The infantmay initiallybe hypertonic or hypotonic, but

all will eventually become hypertonic. This defect in motor develop-

ment remains significant according to Nyhan (1973, 1976), and in

time, all persons with Lesch-Nyhan syndrome will require assistance

standing and sitting, with walking being a near impossibility. Bull and

LaVecchio (1978), however, reported that a child with whom they

worked learned to walk with the aid of crutches.

Another fundamental feature of the disorder is choreoathetosis

(spastic movements of the limbs and facila muscles) (Christie et al.,

1982; Nyhan, 1973). Accordingto Nyhan, the choreoathetosisis more

prominent during stressful periods. It also often becomes worse withage (Mizuno, 1986).

204 BEHAVIOR MODIFICATION / April 2000


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Additional complications arise due to the choreoathetosis. First,

although all learn to speak to some degree, their speech is usually

slurred, a condition referred to as dysarthria (Christie et al., 1982;

Dizmang & Cheatham, 1970; Libby, Polloway, & Smith, 1983; Nyhan,

1973, 1976; Scherzer & Ilson, 1969). Second, patients have difficulty

swallowing and often vomit, sometimes leading to choking and mal-

nutrition (Nyhan, 1973, 1976).

COGNITIVE ABILITY

Mental retardation has been described as a central feature of the

disorder (Lesch & Nyhan, 1964; Nyhan, 1973). Nyhan (1976)reported that the IQs of Lesch-Nyhan patients are normally below 50.

Results of various intelligence tests, however, have been equivocal

and have not firmly backed this assertion. Physical limitations of

Lesch-Nyhan patients often preclude accurate intelligence testing,

thus rendering an accurate assessment of intellect nearly impossible

(Lesch & Nyhan, 1964; Nyhan, 1973, 1976). Christie et al. (1982)

reported IQs among nine children to range from 25 to 101, with the

mean being 58. Scherzer and Ilson (1969) administered the Full

Range Picture Vocabulary Test (FRPV) and selected vocabulary sub-

tests of the Stanford-Binet Intelligence Scale to a 6-year-old patient

with Lesch-Nyhan and found normal language comprehension.

Results within the normal range were substantiated by reports fromhis preschool development class.

In creating a survey in which cognitive ability could be inferred

based on parents responses, Anderson, Ernst, and Davis (1992)

responded novelly to the Lesch-Nyhan patients inability to receive an

accurate measure of intelligence using contemporary measures of

intelligence. A sample size of 42 out of 60 responded to the survey.

The results of the survey showed that all patients older than the age of

5 years (N = 36) were oriented to person, place, and time. Parents

responses were also used to estimate grade level. Thirty-eight subjects

were older than the age of 4. Of those 38, 5 could read at grade level,

and 6 could perform math at grade level; 85% of the children per-

formedbelow grade level. Parents reported that 2 of the children couldread and perform math 1 year above grade level. Anderson et al.



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(1992) offered several explanations for the childrens inability to per-

form at grade level. First, a specific learning disability may be present.

Second, stress often leads to self-injury and other negative responses.

It is possible that these negative behaviors impact concentration and

interest in the tasks, thus indirectly hindering performance. Finally,

Anderson et al. also believe the language difficulties of Lesch- Nyhan

children could preclude the communication of learned or problem

topics with the result being that a proper continuum of education can-

not be created. Hence, it would appear that the degree of mental retar-

dation may not be consistent among all Lesch-Nyhan patients.

SELF-INJURIOUS BEHAVIOR

One of the most intensive aspects of Lesch-Nyhan syndrome is the

SIB elicited (Lesch & Nyhan, 1964). Common forms of SIB in these

patients include biting of the fingers and lips (Baumeister & Frye,

1985; Christie et al., 1982; Lesch & Nyhan, 1964). According to

Anderson and Ernst (1994), in a sample size of 40, 45.0% had perma-

nent damage done to the lip, 45.0% to the inside of the cheek, and

32.5% had produced damage from rubbing. Other forms of SIB

reported have included throwing the head, arm, or leg while being

wheeled through a doorway, arching the spine, head snapping, and

head banging (Anderson & Ernst, 1994). Christie et al. (1982) addi-

tionally reported poking eyes and nose.According to Lesch and Nyhan (1964), self-mutilative behavior

usually begins with the emergence of the first teeth. Clinical experi-

ence with a number of these patients shows that the average age for

SIB to begin is just after 2 years (Christie et al., 1982; Hoefnagel,

Andrew, Mireault, & Berndt, 1965; Mizuno, 1986). Dizmang and

Cheatham (1970) further believe that there is a critical period during

which new forms of SIB erupt. Based on their observations, the

authors stated that the forms of SIB emerge between the ages of 1 and

5, and after the age of 5, new forms of SIB rarely develop. Based on

parental reports, Dizmang and Cheatham (1970) noted that the SIB

also followed an injury of the targeted area in four of five patients.

Theoccurrence of self-injury also appears to vary acrossconditionsand time. Parents in the study of 40 Lesch-Nyhan patients reported



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that SIB occurred less frequently during low stress activities such as

riding in the car and interacting with friends (Anderson & Ernst,

1994). On the other hand, presentation of new people, illness, and the

removal of restraints were cited as high stress events that tended to

increase SIB. Anderson and Ernst reported that 42% of their patients

occasionally used their SIB to obtain some goal. A follow-up study

performed by Mizuno (1986) over a period of 10 years showed that in

some patients, SIB tended to slightly decline after the age of 10. Chris-

tie et al. (1982) also observed this decline in SIB with age and

hypothesized that the decrease is due to an increased level of self-

control with age.

OTHER AGGRESSIVE BEHAVIOR

Patients with Lesch-Nyhan syndrome do not always direct their

aggression toward themselves. Common forms of other-directed aggres-

sion include spitting, biting, pinching, and hitting (Christie et al., 1982;

Hoefnagel et al., 1965; Nyhan, 1973, 1976). Anderson and Ernst

(1994) reported that 35 of 40 patients were aggressive toward others.

They reported abusive behaviors including biting (47.5%), hitting

(47.5%), hair-pulling (62.5%), kicking (62.5%), throwing their head

at others (60.0%), and other behaviors including spitting, swearing,

insulting, and knocking objects away (55.0%). Verbal aggression in

the form of swearing commonly emerges with age (Hoefnagel et al.,1965; Nyhan, 1973).

TREATMENT APPROACHES

Several treatment approaches have been employed to manage the

biochemical and aggressive abnormalities of the syndrome. The

hyperuricemia can be effectivelycontrolledwith the use of allopurinal

(Balis, Krakoff, Berman, & Dancis, 1967; Sweetman & Nyhan,

1967). Allopurinal, however, has no effect on the behavioral, cerebral,

and neurological characteristics of the disorder (Christie et al., 1982;

Nyhan, 1976).



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Restraintappears to be by far themost common methodfor control-

ling SIB. Anderson and Ernst (1994) reported that 18 of 37 patients

were restrained 100% of the time while only 5 of 37 were never

restrained. During the night, 74.4% of the patients were always

restrained and 21.6% were never restrained. Furthermore, there have

been several articles in the literature dedicated to describing effective

methods of restraint for the Lesch-Nyhan patient (Ball, Datta, Rios, &

Constantine, 1985; Nyhan, 1976).

There have also been several forms of dental management used to

prevent SIB. First, extraction of teeth has been used to decrease self-

mutilative behavior. Anderson and Ernst (1994) reported that 24 of 40

patients had teeth extracted to prevent further injury. The use ofmouthguards have been reported by Anderson and Ernst as well as

Sugahara, Mishima, and Mori (1994).

Medications have also been used to control SIB. According to

Buitelaar (1993), neuroleptic medications are most frequently used

for the behavioral control of SIB. Anderson and Ernst (1994) reported

the use of benzodiazepines, neuroleptics, antidepressants, chloralhy-

drates, and anticonvulsives. According to parental reports, benzodi-

azepines and anticonvulsives appeared to provide the most benefits.

Six of 12 parents reporting the long-term use of benzodiazepines

reported that the drug helped a lot, and all 5 parents whose children

used anticonvulsive drugs on a long-term basis reported that the drug

helped a lot. Three of 4 parents whose child had taken antidepressantsreported that the drugs were ineffectual. Further, Buitelaar (1993)

advises caution when using medications for the treatment of SIB for

several reasons. First, the efficacy of medications for SIB usually

depend on sedating properties, which impede both cognitive and

physical abilities. Second, considerations of the long-term side effects

also would discourage the use of medications such as neuroleptics.

REVIEW OF BEHAVIORAL INTERVENTIONS

Although restraint is the most commonly used form of behavior

management, there are many problems associated with its use. Picker,Poling, and Parker (1979) cite three specific problems with the use of



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restraint. First, SIB is not eliminated through the use of restraints, it is

only precluded. Second, restraint limits the activities in which the

restrained individual can participate. Finally, for ethical and legal rea-

sons, restraint is not a preferential procedure in managing SIB. Ide-

ally, clinicians and physicians working with patients are guided by the

Least Restrictive Treatment Model in which patients are granted the

opportunity to live within the least restrictive environment (Foxx,

1982). Restraint lies within the most restrictive level of methods of

controlling behavior.

Duker (1975) was the first to report employing behavioral interven-

tions with a Lesch-Nyhan patient. Dukers subject was a 9-year-old

boy who had been biting his fingers since the age of 18 months. HisSIB had been controlled by wearing dish-washing gloves. The boy

also engaged in frequent head banging. A behavior analysis in two set-

tings, the car and a new ward, indicated that the boys behaviors were

maintained by social reinforcement and stimulus change. Conse-

quently, Duker determined that extinction, as well as a stimulus

change technique in which the controlling stimuli would be removed

from the situation, would be used to treat the self-mutilative behavior.

Hospital personnel were trained to ignore the SIB and reward the

adaptive behavior. During the treatment phase, finger-biting behavior

decreased from40 bites per 2.5 hour session to 3 bites per 2.5 hour ses-

sion. Subsequently, the boys crying following a self-induced bite also

decreased from 18 seconds of crying following each bite to 0 secondsof crying following each bite.

The Lesch-Nyhan patient in Dukers (1975) study also displayed a

second response class of SIB, head banging. Duker attempted to ame-

liorate this behavior through extinction and reinforcement of appro-

priate behavior. However, because the behavior increased and the boy

injured his nose, the treatment procedure was discontinued for the

head-banging behavior. Duker explained that given the spasmatic

movements of the Lesch-Nyhan patient, head banging may be a fea-

ture of the illness rather than a learned response. No follow-up was

reported on the boys behavior.

Following the model established by Duker (1975), Bull and LaVec-

chio (1978) also applied extinction in an attempt to decrease SIB in a10-year-old boy with a diagnosis of Lesch-Nyhan syndrome. Obser-



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vations of both positive and negative reinforcement for SIB provided

the impetus for the choice of extinction as a primary treatment compo-

nent. Additional observations, however, revealed that anxiety-

producing events frequently preceded the occurrence of SIB. Bull and

LaVecchio (1978) responded to the subjects anxiety and fear of non-

restraint by adding a component of systematic desensitization, which

has been shown to decrease anxiety through the concurrent associa-

tion of anxiety-producing events and relaxation. Target behaviors for

this subject included biting, biting attempts, spitting, neck snapping,

vomiting, injury to others, and damage to other parts of the body.

Treatment of the individual began with systematic desensitization,

whichincluded relaxation exercises, a hierarchy of anxiety-provokingsituations, and the removal of restraints during the session (Bull &

LaVecchio, 1978). Extinction occurred by having the therapist leave

the room when SIB began and return when the SIB discontinued.

Observation through a one-way mirror ensured the consistent and

contingent application of the procedures. Following the third session,

the therapist only needed to turn away from the child for SIB to cease.

During the session, Bull and LaVecchio observed that the child

engaged in no SIB while he was alone but significantly increased SIB

when the injuries received attention. As a final phase of the treatment,

Bull and LaVecchio used play therapy as a modeling opportunity to

encourage the subject to verbalize his thoughts and feelings.

The results of the extinction, systematic desensitization, and playtherapy treatment package showed that the combination was success-

ful in eliminating the SIB. Through the course of 10 sessions, actual

biting, head banging, biting attempts, other formsof self-injury, injury

to others, neck snapping, spitting, and vomiting were all reduced to

zero occurrences per hour and were maintained at that level for the

final five sessions. At follow-up at 18 months following treatment, the

boy required no restraints, no longer engaged in verbal insults and

coprolalia, and was learning to walk with crutches.

Anderson,Dancis, and Alpert (1978) compared the effectiveness of

punishment (electric shock), positive reinforcement of the SIB by

contingent attention, positive reinforcement of the non-SIB by non-

contingent attention, and time-out to a baseline consisting of responseprevention. Anderson et al. used an A-B-A-C-A-D design to eliminate



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potential carryover effects. Their subjects consisted of five males ages

3, 5, 11, 12, and 13 years; four of them self-abused by finger biting,

and one self-abused by head banging.

Punishment was the first condition in all cases and consisted of pro-

vidingan electric shock contingent on the SIB (Anderson et al., 1978).

In all five cases, the electric shock increased the occurrence of SIB.

The second condition, positive reinforcement contingent on non-

SIB, consisted of social reinforcement in the form of smiles or atten-

tion when the child was not engaged in SIB. The procedure was

administered to three of the five boys with a reduction in self-mutilative

behaviornoted in allthree boys. Time-outwas administered to three of

the fiveboys contingenton the SIB and decreased SIB inall three. Oneboy received time-out in combination with reinforcement of non-SIB,

and this procedure proved effective in eliminating SIB.

Anderson et al. (1978) also extended their treatment to include gen-

eralization training. The training included three progressive steps:

training to other therapists, hospital personnel, and family.

Follow-up was also performed by Anderson et al. (1978). Each of

the five boys was followed up between 22 and 24 months after the end

of treatment. A therapist made home visits and asked the parents to

record SIB attempts, amount of time spent in restraints, and the condi-

tions surrounding the use of restraints. The percentage of time spent in

restraints following treatment was as follows: 0% (two boys), 5%,

12%, and 39% of the day.Gilbert, Spellacy, and Watts (1979) observed a 4.5-year-old male

patient whose SIB increased when confronted with a change in the

current environment. The boy commonly engaged in abusive behav-

iors such as banging his legs, arms, head, face, and nose, as well as

scratching his nose and face with his hands. Prior to treatment, his SIB

was managed through restraint; his arms were held by elbow splints

and his legs were tied to his wheelchair. Like many Lesch-Nyhan

patients, he became anxious and upset when his restraints were

removed (Gilbert et al., 1979).

Baseline data were collected over four 20-minute sessions at which

time the elbow splints were removed (Gilbert et al., 1979). Baseline

data revealed that he engaged in nose-hitting behaviors under two dis-tinct conditions: when attention was removed and when presented



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with novel stimuli. This behavior occurred at a rate of two times per

minute. Based on the baseline observations, Gilbert et al. (1979)

employed extinction and verbal reinforcement for alternative behav-

ior (DRO). During the first sessions, a therapist employed the proce-

dures while the mother watched through a one-way screen; at the end

of sessions, the mother would participate in the procedure while being

videotaped for feedback purpose. The father also participated in ses-

sions when possible. Treatment occurred for eighteen 20- to 30-minute

sessions. The arm splints were removed progressively with the first

being removed for all 18 sessions, and the second during the final 9.

Gilbert et al. (1979) reported that during the first nine sessions (one

splint removed), SIB decreased from 60 occurrences per session to 0occurrences per session. On the 10th session (both splints removed),

the SIB increased to 40 occurrences per session. By the 18th session,

SIB had decreased to 10 occurrences per session.

To determine if generalization across responses had occurred, the

boy was put near a table (Gilbert et al., 1979). The treatment had not

generalized; the boy continuously kicked the table. However, because

the boy had to leave the hospital, treatment was not administered to

that behavior.

At follow-up, the authors found that generalization did not occur

across settings or across behavior change agents; the parents put the

splints on within days of returning home from the hospital (Gilbert

et al., 1979). This resultmay have been foreseeable. When rates of SIBwere compared across treatment agents, they were higher during the

mothers presence than when either the therapist or the helper was

present. When the boy was with the mother, his SIB never dropped

below an average of 80% of the initial level per 5-minute interval (Gil-

bert et al., 1979). Hence, the problem appears to be generalized across

behavior change agents, not the treatment itself.

Buzas, Ayllon, and Collins (1981) employed a differential rein-

forcement of incompatible behaviors (DRI) procedure to reduce the

injurious behavior of picking and ripping the skin on his mouth.

Behaviors recorded by Buzas et al. included time unrestrained, SIB of

damaging his lip, finger to mouth responses, crying, and vocalizations

of wanting to be restrained. Theincompatible responses included suchbehaviors as drawing, playing games, eating candy, learning sign lan-



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213

TABLE 1

Behavioral Interventions With Specified Lesch-Nyhan Subjects

Author Year Behavior Behavioral Intervention Generalization

Duker 1975 Self-biting Extinction Not across behaviors

Head banging DRO (ineffective with head banging)

Bull & LaVecchio 1978 Self-biting Systematic Across settings

Head banging densensitization

Other self-injury Ext incti on

Play therapy

Anderson, Dancis, 1978 Finger biting Punishment Across settings

& Alpert (4 subjects) Time-Out, DRO Behavior change agents

Head banging (1 subject) Positive reinforcement

Gilbert, Spellacy, 1979 Banging legs, arms, Extinction Not across settings

& Watts face, and nose DRO Not across behavior change agents

Scratching nose and faceBuzas, Ayllon, 1981 Picking and ripping DRI Not across settings

& Collins skin on mouth

Wurtele, King, 1984 Finger biting Extinction Across behavior change

& Drabman Self-instruction agents

Relaxation Across settings

McGreevy 1987 Biting arms, hands, DRI Across settings

& Arthur and fingers Punishment Not across all behaviors/

responses

Across behavior change agents

Grace, Cowart, 1988 Self-biting Self-instruction Across settings

& Matson Positive reinforcement

Time-out

NOTE: DRO = extinction and verbal reinforcement for alternative behavior; DRI = differential reinforcement of incompatible behavi


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guage, and so on. Attention served as the reinforcer and occurred dur-

ing periods of nonrestraint. Surprisingly, during the first five sessions,

which lasted 2 hours and 2 minutes, the boy made no attempts at abu-

sive behavior and only raised his hands to his mouth twice, at which

time the physical therapist removed his hand from his mouth (Buzas

et al., 1981). Following the sixth session of no SIB, the case manager

decided to teach the boy to feed himself. Within 15 minutes, there

were two occurrences of SIB. The authors observed, however, that

attention to the patient promptly followed the behavior. Therefore,

Buzas and colleagues began training staff to follow through with the

extinction procedure. Education consisted of teaching the attendants

to reinforce appropriate behavior and to divert the patients behaviorto more appropriate alternatives when SIB did occur.

Echoing the status of the respondent in the Gilbert et al. (1979)

study, on return for follow-up at 7 months, Buzas and colleagues

(1981) found their patient in restraints 100% of the day and night. The

therapist took the subject to another room, removed his restraints, and

found that no SIB occurred. Again, the problem appears to be one of

generalization across behavior change agents, rather than one of treat-

ment effectiveness.

Wurtele, King, and Drabman (1984) also reported the use of behav-

ioral techniques to reduce the SIB of a Lesch-Nyhan patient. The

patient was a 13-year-old male who engaged in finger biting. His SIB

had been managed by wrapping his thumbs in towels and Ace ban-dages. When he was unrestrained, the boy would often hold his hands

behind his wheelchair in an attempt to prevent the SIB. Furthermore,

the boy reported that the antecedents of his SIB included muscle ten-

sion and a voice telling him to bite. Direct observation of the patients

interactions revealed that attention was one of the maintaining vari-

ables, whereas anxiety tended to exacerbate the behavior. Baseline

revealed that the patient attempted SIB at a rate of approximately one

occurrence every 5 minutes during the hourlong sessions (Wurtele

et al.). Based on the previous failure of time-out/punishment to sup-

press the SIB, the authors chose extinction as the treatment for the cur-

rent study. The staff, however, were reluctant to use extinction alone

due to the possibility of an extinction burst and the consequent potentialfor damage. Therefore, Wurtele et al. decided to use a mouthguard typi-

cally used by athletes in conjunction with extinction. Other treatment



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modalities included relaxation to reduce the anxiety that occurred

when restraints were removed, self-instruction as a reminder not to

engage in the behavior, and social support. Following the sixth ses-

sion, however, the patient removed the mouthguard and bit himself,

and subsequently learned to manipulate the mouthguard to facilitate

biting. In response to this problem, Wurtele et al. (1984) had a pediat-

ric dentist constructan acrylic two-piece mouthguard to fit firmly over

the patients top and bottom teeth. In addition, staff members verbally

reinforced the patient for keeping themouthguard correctlyin place.

During some activities, such as meals, the mouthguard had to be

removed (Wurtele et al., 1984). For these activities, fingerless biking

gloves, rather than the cumbersome wraps, were used to protect thepatients thumbs.

Training facilitated generalization across both behavior change

agents and settings. The researchers trained the parents to apply the

procedures in three phases (Wurtele et al., 1984). First, the patients

learned how to care for and use the mouthguard as well as the gloves.

Second, the parents received instruction on prompting their son to use

relaxation techniques. Finally, the parents received an explanation for

the procedure and necessity for fading the use of the mouthguard.

Follow-up occurred at 6 weeks, 10 weeks, and 6 months. At 6

weeks, he continued to wear the mouthguard and the glovesduring the

day, and the mother reported that attempts at SIB occurred only 10 to

15 minutes per day (Wurtele et al., 1984). At the 10-week follow-up, itwas reported that the subject was continuing to verbally cue himself to

obtain from SIB. At the 6-month follow-up, it was determined that the

subject still occasionally used the mouthguard as a deterrent against

SIB but still frequently wore the gloves (Wurtele et al., 1984).

McGreevy and Arthur (1987) attempted to reduce the self-biting of

the arms, hands, and fingers of a 2-year-old boy using an A-BC-A-BC

design. The treatment phase consisted of DRI and punishment. Dur-

ing the baseline phase, data were collected for both SIB and toy touch-

ing, the latter to be defined as the incompatible behavior. The punish-

ment procedure consisted of dispensing a small amount of vinegar

into the boys mouth following the SIB. Reinforcement for incompati-

ble behavior consisted of dispensing an equal amount of cola into theboys mouth following toy touching. All of the boys restraints were



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removed except the fingerguards. To deter him from unnecessary

self-mutilation, beginning treatment sessions were conducted for only

5-minute sessions. According to McGreevyand Arthur (1987), results

gathered duringthe first treatment phase showed that in comparison to

baseline, SIB decreased by a factor of 20. When the baseline condition

wasagaininitiated, theSIB increased by a factorof 7 over the previous

phase. The final treatment phase showed that DRI and punishment

were indeed the controlling variables associated with the decrease in

SIB. During this phase, treatment sessions were gradually extended to

10- and then 30-minute sessions, and the fixed-ratio schedule (FR = 1)

was faded to an FR = 2 and then to an FR = 15.

McGreevy and Arthur (1987) also initiated a generalization phaseacross both settings and behavior change agents. During this final

phase, settings included the center and the home, and change agents

included staff and the parents. The boy was reinforced on a fixed-ratio

schedule of 20 responses (FR = 20). During this phase, SIB increased

from no SIB during treatment sessions to three SIBs during a 3-hour

session and three SIBs during a 2-hour session at the center and the

home, respectively. At the treatment center, fading of the fingerguards

was initiated; two fingerguards were removed. Subsequently, SIB of

finger biting increased while incompatible behaviors decreased. Bit-

ing of the arms, forearms, and back of his hands remained zero. Fur-

thermore, the problem of symptom substitution emerged when the

boy beganbiting the palms of hishands(McGreevy & Arthur, 1987).Follow-up with the boy occurred 6 months after treatment. Teach-

ers and parents reported that the boy maintained low levels of SIB fol-

lowing treatment. He continued to bite the palms of his hands and fin-

gers, while SIB to his arms, forearms, and back of his hands remained

at a zero level (McGreevy & Arthur, 1987).

Grace, Cowart, and Matson (1988) employed a self-assessment

procedure with time-out and positive reinforcement to reduce com-

pulsive self-biting in a 14-year-old male with Lesch-Nyhan syn-

drome. A multiple baseline design across settings (hospital room and

bedroom) was used to evaluate the results of the self-assessment pro-

cedure. Self-assessment consisted of pointing to a happy face for

non-SIB and a sad face for SIB. A trainer modeled both the SIB (bit-ing) and non-SIB (hands on the lap). Correct evaluations were posi-



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tively reinforced with hugs, whereas incorrect evaluations were pun-

ished through a 30-second time-out. Self-assessment sessions began

at 3- and 7-minute intervals and were gradually lengthened to 1-hour

periods.

Results of the self-assessment training showed marked reductions

in SIB (Grace et al., 1988). The subject originally showed variable

rates of SIB in both settings with frequency of biting behaviors rang-

ing from 1 to 60 bites per 30-minute session and 1 to 37 bites per 30-

minute session in the hospital and bedroom setting, respectively.

Within 3 days of intervention in both settings, the subject was display-

ing no SIB. Follow-up at 19 weeks showed no recurrence of SIB

(Grace et al., 1988).

CONCLUSION

Based on the studies performed by these researchers (see Table 1),

it would appear that clinicians and physicians could follow the Least

Restrictive Model in decreasing the SIBs of the Lesch-Nyhan patient.

The most common methods used in these studies included DRI, DRO,

and extinction. Foxx(1982) has defined behavioral procedures used in

decreasing behaviors on three levels based on the aversiveness and the

intrusiveness of the procedures. Differential reinforcement of other

behaviors (DRO) and differential reinforcement of incompatiblebehavior (DRI) have been rated on the lowest level of intrusiveness in

interventions for decreasing behaviors. These methods have shown

success with this population (Andersonet al., 1978; Buzas et al., 1981;

Duker, 1975; Gilbert et al., 1979; McGreevy & Arthur, 1987).

On the other hand, Foxx (1982) has rated extinction on the second

level of the three levels of intervention, which means that the interven-

tion is moderately intrusive and aversive. Several cautions would be

necessary in employing extinction with this population. Given the

degree of tissue loss and the swiftness with which these patients self-

injure, it would be dangerous to rely on an intervention in which

improvement may be slow and an extinction burst would be quite

probable.



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Punishment procedures constitute the most intrusive methods of

decreasing behaviors (Foxx, 1982). Foxx has defined two categories

of punishment procedures: Type I and Type II. Type I punishment pro-

cedures refer to thoseprocedures using an aversive stimulus following

the occurrence of an undesirable behavior. Type II punishment proce-

dures refer to those procedures in which reinforcement is withdrawn

following the occurrence of a behavior. Examples include time-out,

response cost, and withdrawal or social attention (Foxx, 1982).

Few studies have mentioned the use of either Type I or Type II pun-

ishment procedures with the Lesch-Nyhan patient. The results of

these studies have been equivocal. A Type I punishment procedure in

the form of electric shock contingent on SIB was used by Anderson etal. (1978), with the result being an increase in SIB. Contrasting this

result, McGreevy and Arthur (1987) provided a vinegar solution con-

tingent on SIB. This intervention produced a reduction in SIB; how-

ever, one must note that McGreevy and Arthur counterbalanced the

punishment procedure with a reinforcement procedure. Anderson

et al. (1978) and Grace et al. (1988) successfully used a Type II pun-

ishment procedure, time-out, to decrease SIB. Once again, however,

these procedures were paired with reinforcement procedures.

The equivocal results of these studies have two primary implica-

tions for the use of punishment procedures with the Lesch-Nyhan

patient. First, in following with the principle of the least restrictive

environment, less intrusive measures should be eliminated first; pun-ishment alone will not lead to the desired results. Second, punishment

techniques, when believed necessary, should be paired with reinforce-

ment techniques.

Overcorrection falls within the realm of Type I punishment proce-

dures. To our knowledge, there have been no attempts at using over-

correction in changing the SIB of a Lesch-Nyhan patient documented

in the literature. According to Foxx (1982), there are two commonele-

ments of overcorrection that may be applied together or alone: (a) cor-

rection of the environment, and (b) practicing germane forms of the

behavior. A possible reason for the failure to use overcorrection with

the Lesch-Nyhan patient could be that the severe motor difficulties

encountered may preclude correction of the environmental effects ofthe SIB.



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Symptom substitution is another consideration when working with

the Lesch-Nyhan patient. According to Willems (1974), one of the

greatest dangers of employing a behavioral interventionis focusing on

one behavior onlywhile ignoring otherperipheralbehaviorsor associ-

ated features of a disorder. In the case described by Duker (1975),

self-biting was decreased while head banging increased concurrently.

The authors rationalized the increase in head banging as a secondary

result of spasms found in the Lesch-Nyhan patient. One could argue,

however, that spasms would be unaffected by behavior therapy, and

therefore, the authors overlooked a more plausible explanation. Alter-

natively, it could be argued that the head-banging behavior was a case

of symptom substitution. McGreevy and Arthur (1987) also encoun-tered a similar problem when their subject began biting the palms of

his hands simultaneously with a decrease in biting of the arms, fore-

arms, and backs of the hands. The authors provided no explanation for

this behavior, except to note that its occurrence was apparently unre-

lated to the punishment techniques used. The possibility of symptom

substitution was ignored. Neither Duker (1975) nor McGreevy (1987)

and Arthur provided a plausible explanation or solution for the behav-

ior. What these studies do show, however, is that symptom substitution

is a possible by-product of behavioral treatment with the Lesch-

Nyhan patient that must be anticipated and, at some point, addressed.

Likewise, many of the published studies involving a behavioral

intervention with a Lesch-Nyhan patient suggest significance in gen-eralization and maintenance of treatment gains. Stokes and Baer

(1977) highlighted the importance of making generalization an active

procedure within a treatment regime. In three of the four studies

reviewed in which long-term effects were maintained across settings,

specific measures and training for generalization were activated as

part of the treatment protocol (Anderson et al., 1978; Bull & LaVec-

chio, 1978; Wurtele et al., 1984). In the fourth study by Grace et al.

(1988), measures of generalization were not specified. Further, Gil-

bert et al. (1979) attempted to facilitate generalization across behavior

change agents by involving parents in training. It must be noted, how-

ever, that additional training was suggested, as SIB never decreased

below baseline while the mother was present. Specific training sug-gestions have been provided by Foxx (1982) to enhance generaliza-



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tion. These suggestions include finding common elements between

the training situationand the natural situation, using common aversive

stimuli rather than artificial aversives, and varying the conditions in

which treatment occurs.

In addition to training generalization, Foxx (1982) has also pro-

vided some guidelines for enhancing maintenance of progress. First,

pair natural reinforcers with artificial ones so that the artificial rein-

forcers can be faded. For instance, in the study by McGreevy and

Arthur (1987), Foxx (1982) would suggest that pairing the word no

with the vinegar and goodwith the cola might enhance the eventual

fading of the artificial reinforcers and punishers. A second suggestion

relates to generalization: Train family members, teachers, and othersinvolved with the child as behavior change agents. Third, Foxx sug-

gests that a gradual delay period between the behavior and the conse-

quences would enhance maintenance. Finally, Foxx suggests that by

varying treatment conditions, one can avoid the possibility of creating

discriminative stimuli in which negative consequences will be

avoided.

In conclusion, the SIB of Lesch-Nyhan patients is severe and

requires intervention. The most common intervention, restraint, is

also the most restrictive. Despite the differences in the characteristics

of the self-mutilative behavior evidenced by the Lesch-Nyhan patient

as compared with other patients who display SIB, Lesch-Nyhan

patients have responded just as favorably to less restrictive models ofbehavioral interventions. Furthermore, Foxx (1990) has shown that

behavioral interventions for SIB can be successful, and long-term

effects of these interventions can be maintained. Therefore, with indi-

vidualized interventions and some adjustments in previously used

treatment packages, the benefits of behavioral interventions with SIB

will be realized for the Lesch-Nyhan patient as well.

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A Review of Behavioral Treatments