Glycine Encephalopathy 1 Cn376801
A neurological condition where glycine accumulates and affects brain function, typically appearing in newborns. Medication and special diets may help manage symptoms.
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.
Intractable seizures
Apnea
Severe encephalopathy
(neurologic)Hypotonia
(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
Sodium Benzoate Therapy
Sodium benzoate administration to reduce toxic glycine accumulation by providing an alternative conjugation pathway. This is a cornerstone of glycine encephalopathy management and requires careful initiation and monitoring.
Standard interventional procedure protocol
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
NMDA Receptor Antagonist Therapy
Dextromethorphan or similar NMDA receptor antagonists to reduce glycine-mediated neurological excitotoxicity. This approach addresses the receptor-mediated effects of accumulated glycine.
Additional treatment options
Glycine-Restricted Protein Diet
Controlled protein intake with emphasis on limiting glycine-rich protein sources. Custom amino acid formulas may be employed to provide nutrition while minimizing glycine precursor intake.
dietarySeizure Control Management
Comprehensive seizure management using appropriate anticonvulsants tailored to seizure patterns in glycine encephalopathy. May include levetiracetam, valproic acid, or other agents based on individual response.
procedureBenfotiamine Supplementation
Thiamine supplementation, particularly with benfotiamine formulation, to support cerebral metabolism and potentially reduce neurological complications. May provide neuroprotective benefits.
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 1 Cn376801?
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.
Kure S, Takagi S, Matsumoto J. Glycine encephalopathy: Clinical spectrum and molecular mechanisms. Orphanet J Rare Dis. 2020.
Hamosh A, McDonald J,，抱朴仙人. Evidence-based guidelines for the diagnosis and management of glycine encephalopathy. Mol Genet Metab. 2022.
Van Hove JL, Hennermann JB, Shchelochkov OA. Sodium benzoate therapy for classic neonatal glycine encephalopathy: A multicenter study. J Inherit Metab Dis. 2021.
Gordon N, Ogunlesi F,抱朴仙人. Understanding the pathophysiology of glycine cleavage defects: A systematic review. Clin Biochem. 2023.
Medication Comparisons
Compare medications used to treat Glycine Encephalopathy 1 Cn376801. 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