TY - JOUR
T1 - Pallidal deep-brain stimulation associated with complete remission of self-injurious behaviors in a patient with lesch-nyhan syndrome
T2 - A case report
AU - Deon, Laura L.
AU - Kalichman, Miriam A.
AU - Booth, Cynthia L.
AU - Slavin, Konstantin V.
AU - Gaebler-Spira, Deborah J.
PY - 2012/1
Y1 - 2012/1
N2 - Thepurposeof this case report is to reviewthemanagement of a boy with Lesch-Nyhan syndromewith deep-brain stimulationwhohad remission of self-injurious behaviors as a result.This patientwas treatedwith intrathecal baclofen and, later, with deep-brain stimulation to reduce hypertonia. Goals were to improve wheelchair positioning for school attendance and to reduce the use of restraints for comfort. Intrathecal baclofen was implanted twice and decreased the hypertonia, but both were explanted because of infection. Deep-brain stimulation was initiated 2.5 years ago, and since that time, comfort and function have improved and caregiver burden has decreased. Improvements in dystonia with deep-brain stimulation have also occurred, and self-injurious behaviors have resolved.
AB - Thepurposeof this case report is to reviewthemanagement of a boy with Lesch-Nyhan syndromewith deep-brain stimulationwhohad remission of self-injurious behaviors as a result.This patientwas treatedwith intrathecal baclofen and, later, with deep-brain stimulation to reduce hypertonia. Goals were to improve wheelchair positioning for school attendance and to reduce the use of restraints for comfort. Intrathecal baclofen was implanted twice and decreased the hypertonia, but both were explanted because of infection. Deep-brain stimulation was initiated 2.5 years ago, and since that time, comfort and function have improved and caregiver burden has decreased. Improvements in dystonia with deep-brain stimulation have also occurred, and self-injurious behaviors have resolved.
KW - Care and comfort
KW - Deep-brain stimulation
KW - Dystonia
KW - Lesch-Nyhan
KW - Self-injurious behaviors
UR - http://www.scopus.com/inward/record.url?scp=84856851335&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84856851335&partnerID=8YFLogxK
U2 - 10.1177/0883073811415853
DO - 10.1177/0883073811415853
M3 - Review article
C2 - 21940691
AN - SCOPUS:84856851335
SN - 0883-0738
VL - 27
SP - 117
EP - 120
JO - Journal of Child Neurology
JF - Journal of Child Neurology
IS - 1
ER -
