TY - JOUR
T1 - Deep brain stimulation in the management of movement disorders in childhood
T2 - a UK-wide cross-sectional study
AU - Lumsden, Daniel E
AU - Ramiah, Ramalakshmi
AU - Smallbone, Todd
AU - Singleton, Will
AU - Amin, Sam
AU - Perides, Sarah
AU - Lumsdon, Gina
AU - Ashida, Reiko
AU - Ellenbogen, Jonathan
AU - Ashkan, Keymoumars
AU - Selway, Richard
AU - Kaminska, Margaret
AU - Hasegawa, Harutomo
AU - Lin, Jean-Pierre
N1 - Publisher Copyright:
© Author(s) (or their employer(s)) 2025. No commercial re-use. See rights and permissions. Published by BMJ Group.
PY - 2025/10/14
Y1 - 2025/10/14
N2 - Introduction We aimed to establish the clinical characteristics of children and young people (CAYP) currently receiving deep brain stimulation (DBS) therapy for the management of movement disorders in the UK to better inform planning of future service provision.
Methods Cross-sectional service evaluation of centres providing DBS for the management of movement disorders in childhood.
Results A total of 139 CAYP were identified across three centres. Median age at surgery was 9.8 years (range 2.0–18.9 years), and median duration of DBS was 4.4 years (range from 1 week to 15.75 years). Modal Gross Motor Function Classification System level was V (n=66). The most common causes of movement disorder were dyskinetic cerebral palsy (69/139, 49.6%), dystonia due to mutations in the lysine methyltransferase 2B gene, aka DYT-KMT2B, (13/139, 9.4%) and dystonia due to mutations in the Torsin-1A gene, aka DYT-TOR1A, (9/139, 6.5%). A monogenetic cause of dystonia without evidence of central nervous system pathology on MRI was identified in 30 CAYP (21.6%). Clinically significant dystonia was present in all CAYP, with significant chorea in 47/139 (33.8%) and significant spasticity in only 13/139 (9.4%). No tone-reducing medications were currently used by 43/139 (30.9%) of CAYP. The remaining 96/139 CAYP were currently receiving 1–6 tone-reducing medications, most commonly gabapentin (n=58), clonidine (n=50) and a form of benzodiazepine (n=43). Despite care being provided by paediatric services, 37/139 (26.6%) of CAYP were >18 years of age.
Conclusions CAYP currently receiving DBS therapy represent a heterogeneous population in terms of dystonia aetiology, functional level and additional pharmacological management. Only 102 CAYP<18 years of age are currently receiving DBS therapy in the UK, representing a small proportion of the population who could benefit from this intervention.
AB - Introduction We aimed to establish the clinical characteristics of children and young people (CAYP) currently receiving deep brain stimulation (DBS) therapy for the management of movement disorders in the UK to better inform planning of future service provision.
Methods Cross-sectional service evaluation of centres providing DBS for the management of movement disorders in childhood.
Results A total of 139 CAYP were identified across three centres. Median age at surgery was 9.8 years (range 2.0–18.9 years), and median duration of DBS was 4.4 years (range from 1 week to 15.75 years). Modal Gross Motor Function Classification System level was V (n=66). The most common causes of movement disorder were dyskinetic cerebral palsy (69/139, 49.6%), dystonia due to mutations in the lysine methyltransferase 2B gene, aka DYT-KMT2B, (13/139, 9.4%) and dystonia due to mutations in the Torsin-1A gene, aka DYT-TOR1A, (9/139, 6.5%). A monogenetic cause of dystonia without evidence of central nervous system pathology on MRI was identified in 30 CAYP (21.6%). Clinically significant dystonia was present in all CAYP, with significant chorea in 47/139 (33.8%) and significant spasticity in only 13/139 (9.4%). No tone-reducing medications were currently used by 43/139 (30.9%) of CAYP. The remaining 96/139 CAYP were currently receiving 1–6 tone-reducing medications, most commonly gabapentin (n=58), clonidine (n=50) and a form of benzodiazepine (n=43). Despite care being provided by paediatric services, 37/139 (26.6%) of CAYP were >18 years of age.
Conclusions CAYP currently receiving DBS therapy represent a heterogeneous population in terms of dystonia aetiology, functional level and additional pharmacological management. Only 102 CAYP<18 years of age are currently receiving DBS therapy in the UK, representing a small proportion of the population who could benefit from this intervention.
U2 - 10.1136/archdischild-2025-328889
DO - 10.1136/archdischild-2025-328889
M3 - Article (Academic Journal)
C2 - 41093365
SN - 0003-9888
JO - Archives of Disease in Childhood
JF - Archives of Disease in Childhood
M1 - archdischild-2025-328889
ER -