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BEHAVIOR

Olson, Houlihan
MODIFICATION
/ TREATMENTS
/ April
USED 2000
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 with Lesch-Nyhan syndrome have been limited in number and
long-term success. This article reviews the behavioral treatments that 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

AUTHORS’NOTE: Please address editorial correspondence to Daniel Houlihan, Ph.D., Depart-


ment of Psychology, Box #35–Armstrong Hall, Mankato State University, Mankato, MN
56002-8400; phone: (507)389-6308; e-mail: HOULY@Vax1.mankato.msus.edu.
BEHAVIOR MODIFICATION, Vol. 24 No. 2, April 2000 202-222
© 2000 Sage Publications, Inc.

202
Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 203

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


from other disorders in which SIB is a common feature (e.g.,
Tourette’s 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
204 BEHAVIOR MODIFICATION / April 2000

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. The first evidence 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 infant may initially be 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). According to Nyhan, the choreoathetosis is more
prominent during stressful periods. It also often becomes worse with
age (Mizuno, 1986).
Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 205

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 from
his 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 patient’s 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-
formed below grade level. Parents reported that 2 of the children could
read and perform math 1 year above grade level. Anderson et al.
206 BEHAVIOR MODIFICATION / April 2000

(1992) offered several explanations for the children’s 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.
The occurrence of self-injury also appears to vary across conditions
and time. Parents in the study of 40 Lesch-Nyhan patients reported
Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 207

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 effectively controlled with 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).
208 BEHAVIOR MODIFICATION / April 2000

Restraint appears to be by far the most common method for 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 of
mouthguards 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 antidepressants
reported 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
Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 209

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. Duker’s subject was a 9-year-old
boy who had been biting his fingers since the age of 18 months. His
SIB 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 boy’s 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 from 40 bites per 2.5 hour session to 3 bites per 2.5 hour ses-
sion. Subsequently, the boy’s crying following a self-induced bite also
decreased from 18 seconds of crying following each bite to 0 seconds
of crying following each bite.
The Lesch-Nyhan patient in Duker’s (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 boy’s behavior.
Following the model established by Duker (1975), Bull and LaVec-
chio (1978) also applied extinction in an attempt to decrease SIB in a
10-year-old boy with a diagnosis of Lesch-Nyhan syndrome. Obser-
210 BEHAVIOR MODIFICATION / April 2000

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 subject’s 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,
which included relaxation exercises, a hierarchy of anxiety-provoking
situations, 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 play
therapy 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 forms of 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 response
prevention. Anderson et al. used an A-B-A-C-A-D design to eliminate
Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 211

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-
viding an 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
behavior noted in all three boys. Time-out was administered to three of
the five boys contingent on the SIB and decreased SIB in all three. One
boy 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
212 BEHAVIOR MODIFICATION / April 2000

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 0
occurrences 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 result may have been foreseeable. When rates of SIB
were compared across treatment agents, they were higher during the
mother’s 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. The incompatible responses included such
behaviors as drawing, playing games, eating candy, learning sign lan-
TABLE 1
Behavioral Interventions With Specified Lesch-Nyhan Subjects
Author Year Behavior Behavioral Intervention Generalization Follow-Up

Duker 1975 Self-biting Extinction Not across behaviors None


Head banging DRO (ineffective with head banging)
Bull & LaVecchio 1978 Self-biting Systematic Across settings 18 monthsa
Head banging densensitization
Other self-injury Extinction
Play therapy
Anderson, Dancis, 1978 Finger biting Punishment Across settings 22-24 monthsa
& 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 Yes
& Watts face, and nose DRO Not across behavior change agents
Scratching nose and face
Buzas, Ayllon, 1981 Picking and ripping DRI Not across settings 7 months
& Collins skin on mouth
Wurtele, King, 1984 Finger biting Extinction Across behavior change 6 and 10 weeks
& Drabman Self-instruction agents 6 monthsa
Relaxation Across settings
McGreevy 1987 Biting arms, hands, DRI Across settings 6 monthsa
& Arthur and fingers Punishment Not across all behaviors/
responses
Across behavior change agents
Grace, Cowart, 1988 Self-biting Self-instruction Across settings 19 weeksa
& Matson Positive reinforcement
Time-out

NOTE: DRO = extinction and verbal reinforcement for alternative behavior; DRI = differential reinforcement of incompatible behaviors.
213
214 BEHAVIOR MODIFICATION / April 2000

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 patient’s behavior
to 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 patient’s
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 potential
for damage. Therefore, Wurtele et al. decided to use a mouthguard typi-
cally used by athletes in conjunction with extinction. Other treatment
Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 215

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 construct an acrylic two-piece mouthguard to fit firmly over
the patient’s top and bottom teeth. In addition, staff members verbally
reinforced the patient for keeping the mouthguard correctly in 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 the
patient’s 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 gloves during 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, it
was 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 boy’s mouth following the SIB. Reinforcement for incompati-
ble behavior consisted of dispensing an equal amount of cola into the
boy’s mouth following toy touching. All of the boy’s restraints were
216 BEHAVIOR MODIFICATION / April 2000

removed except the fingerguards. To deter him from unnecessary


self-mutilation, beginning treatment sessions were conducted for only
5-minute sessions. According to McGreevy and Arthur (1987), results
gathered during the first treatment phase showed that in comparison to
baseline, SIB decreased by a factor of 20. When the baseline condition
was again initiated, the SIB increased by a factor of 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 phase
across 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 began biting the palms of his hands (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-
Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 217

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 incompatible
behavior (DRI) have been rated on the lowest level of intrusiveness in
interventions for decreasing behaviors. These methods have shown
success with this population (Anderson et 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.
218 BEHAVIOR MODIFICATION / April 2000

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 those procedures 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 et
al. (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 common ele-
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 of
the SIB.
Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 219

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 intervention is focusing on
one behavior only while ignoring other peripheral behaviors or 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-
220 BEHAVIOR MODIFICATION / April 2000

tion. These suggestions include finding common elements between


the training situation and 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 good with the cola might enhance the eventual
fading of the artificial reinforcers and punishers. A second suggestion
relates to generalization: Train family members, teachers, and others
involved 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 of
behavioral 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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Lynn Olson is a doctoral candidate in school psychology at the University of South Caro-
lina. She is currently completing her internship at the Children’s Hospital of Orange
County in southern California. Her clinical and research interests are in behavioral
pediatrics.

Daniel Houlihan is an associate professor of clinical psychology at Mankato State Uni-


versity. His clinical and research interests include behavioral interventions in the treat-
ment of childhood noncompliance, autism, and anxiety disorders.

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