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Olson, Houlihan
MODIFICATION
/ TREATMENTS
/ April
USED 2000
FOR LESCH-NYHAN SYNDROME
LYNN OLSON
University of South Carolina
DANIEL HOULIHAN
Mankato State University
202
Olson, Houlihan / TREATMENTS USED FOR LESCH-NYHAN SYNDROME 203
ETIOLOGY
CLINICAL MANIFESTATIONS
GENERAL DEVELOPMENT
COGNITIVE ABILITY
SELF-INJURIOUS BEHAVIOR
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.
TREATMENT APPROACHES
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
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
CONCLUSION
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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.