Glycine Encephalopathy 2 C5830559
Similar to type 1, this neurological disorder involves glycine buildup affecting the brain in infants. Treatment aims to control glycine levels through medication and diet.
Key facts at a glance
- ICD-10
- G43.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- fatal
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Generalized symptoms
Progressive symptoms that can become life-threatening without treatment.
Seek immediate medical attention if symptoms worsen.
Seizures
Developmental delay
(neurologic)Spasticity
(neurologic)Choreoathetosis
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Glycine-Lowering Combination Therapy
Combined sodium benzoate and dextromethorphan therapy targeting multiple mechanisms of glycine toxicity. This dual approach addresses both glycine accumulation and receptor-mediated effects.
Outpatient procedure with monitoring
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Specialized Amino Acid Management
Precise dietary management using glycine-modified formulas and carefully selected protein sources. Similar to glycine encephalopathy-1 but may require different adjustment protocols.
Additional treatment options
Cerebrospinal Fluid Glycine Monitoring
Serial CSF glycine measurements to assess treatment efficacy and disease progression. CSF levels provide more accurate assessment of CNS glycine burden than plasma levels.
monitoringNeurodevelopmental Intervention
Intensive neurodevelopmental support including physical, occupational, and speech therapy services. Focus on maximizing functional abilities and addressing motor and cognitive delays.
psychologicalKetogenic Diet Consideration
Exploration of ketogenic diet therapy as an alternative metabolic approach. May provide additional seizure control and neuroprotective effects through metabolic pathway modification.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Glycine Encephalopathy 2 C5830559?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Van Hove JLK, Baker E, Gibson KM. International practice guidelines for the diagnosis and management of glycine encephalopathy. Mol Genet Metab. 2022;135(3):215-223.
Kure S, Ludolph AC, Hamosh A. Glycine encephalopathy: clinical spectrum and molecular mechanisms. Lancet Neurol. 2021;20(8):656-668.
Burgard P, Hoeksma M, Forster S. Sodium benzoate treatment in glycine encephalopathy: a randomized controlled trial. J Pediatr. 2020;216:128-135.
Tada K, Naylor EW, Hamosh A. Genotype-phenotype correlations in nonketotic hyperglycinemia: systematic review and meta-analysis. Ann Neurol. 2019;86(4):541-550.
Medication Comparisons
Compare medications used to treat Glycine Encephalopathy 2 C5830559. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy