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Childhood-onset spasticity with hyperglycinemia — Clinical Guidelines

Overview

Nonketotic hyperglycinemia (NKH) is an autosomal recessive metabolic disorder characterized by elevated levels of glycine, leading to neurological impairments including spasticity in affected children 9. Childhood-onset spasticity associated with NKH requires comprehensive management to address motor symptoms and associated complications.

Diagnosis

Elevated plasma and cerebrospinal fluid glycine concentrations 9

Clinical presentation includes lethargy, seizures, and respiratory depression 9

Differential diagnosis should rule out other metabolic encephalopathies 9

Management

Physical and Occupational Therapy: Essential for improving motor function and preventing contractures 10

Botulinum Toxin A (BoNT-A) Injections: Effective for localized spasticity management; repeated cycles can reduce pain 1 10

Intrathecal Baclofen: Considered for severe spasticity, though complex to manage 8 11

Tizanidine: Used for spasticity and pain management; monitor for hypotension, especially with CYP1A2 inhibitors 7 12

Dantrolene: Alternative antispastic agent, though associated with side effects like acneiform eruptions 14

Special Populations

Pediatrics: Intramuscular midazolam can be safely used for anxiolysis during spasticity management procedures 4

Comorbidities: Careful consideration of drug interactions, particularly with CYP1A2 inhibitors when using tizanidine 7

Key Recommendations

Regularly assess and manage spasticity using a multidisciplinary approach including physical therapy and botulinum toxin injections (Evidence: Moderate 1 10)

Monitor for and manage potential drug interactions, especially when prescribing tizanidine, due to its metabolism by CYP1A2 (Evidence: Moderate 7)

Consider intrathecal baclofen for severe cases of spasticity, acknowledging the complexity of its management (Evidence: Expert opinion 8 11)

References

1 Turner-Stokes L, Buchwald K, Ashford SA, Fheodoroff K, Jacinto J, Narayanan A et al.. Pain Reduction with Repeated Injections of Botulinum Toxin A in Upper Limb Spasticity: A Longitudinal Analysis from the ULIS-III Study. Toxins 2025. link 2 Verduzco-Gutierrez M, Raghavan P, Pruente J, Moon D, List CM, Hornyak JE et al.. AAPM&R consensus guidance on spasticity assessment and management. PM & R : the journal of injury, function, and rehabilitation 2024. link 3 Zajac J, Chandawarkar A, Sherber N. #Botox 101: Subgroup Analysis of Twitter's Top 101 Neurotoxin Influencers. Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] 2021. link 4 Mian MN, Alwasiah N, Savitz A. The use and safety of intramuscular midazolam during in-office botulinum toxin injections in pediatric patients. Journal of pediatric rehabilitation medicine 2021. link 5 Lagnau P, Lo A, Sandarage R, Alter K, Picelli A, Wissel J et al.. Ergonomic Recommendations in Ultrasound-Guided Botulinum Neurotoxin Chemodenervation for Spasticity: An International Expert Group Opinion. Toxins 2021. link 6 Yelnik AP, Hentzen C, Cuvillon P, Allart E, Bonan IV, Boyer FC et al.. French clinical guidelines for peripheral motor nerve blocks in a PRM setting. Annals of physical and rehabilitation medicine 2019. link 7 Chaugai S, Dickson AL, Shuey MM, Feng Q, Barker KA, Wei WQ et al.. Co-Prescription of Strong CYP1A2 Inhibitors and the Risk of Tizanidine-Associated Hypotension: A Retrospective Cohort Study. Clinical pharmacology and therapeutics 2019. link 8 Carr BN, Sernas T, Mazzola CA. X-ray Imaging Analysis of Intrathecal Baclofen Pumps for Pediatric Emergency Medicine. Pediatric emergency care 2018. link 9 Mulligan JL. Neonatal nonketotic hyperglycinemia: a case study and review of management for the advanced practice nurse. Neonatal network : NN 2013. link 10 Ronan S, Gold JT. Nonoperative management of spasticity in children. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2007. link 11 Gatscher S, Becker R, Uhle E, Bertalanffy H. Combined intrathecal baclofen and morphine infusion for the treatment of spasticity related pain and central deafferentiation pain. Acta neurochirurgica. Supplement 2002. link 12 Smith HS, Barton AE. Tizanidine in the management of spasticity and musculoskeletal complaints in the palliative care population. The American journal of hospice & palliative care 2000. link 13 Burton BK, Pettenati MJ, Block SM, Bensen J, Roach ES. Nonketotic hyperglycinemia in a patient with the 9p- syndrome. American journal of medical genetics 1989. link 14 Pembroke AC, Saxena SR, Kataria M, Zilkha KD. Acne induced by dantrolene. The British journal of dermatology 1981. link 15 Peacock WJ, Eastman RW. The neurosurgical management of spasticity. South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 1981. link 16 Angel RW. The rebound phenomenon of Gordon Holmes. Archives of neurology 1977. link

Childhood-onset spasticity with hyperglycinemia