Late Infantile Neuronal Ceroid Lipofuscinosis 6 C5880894
A form of Batten disease causing progressive nerve cell loss in children, leading to seizures and developmental decline. Affects children. Fatal.
Key facts at a glance
- ICD-10
- G31.82
- 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
Myoclonus
(neurologic)Ataxia
(neurologic)Dementia
(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
Comprehensive seizure management
Given the progressive nature of NCL-6, proactive seizure management with appropriate anticonvulsants is a cornerstone of care to minimize seizure-related complications and maintain quality of life.
Standard interventional procedure protocol
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Gastrostomy tube placement
When oral intake becomes insufficient or unsafe due to swallowing difficulties, percutaneous endoscopic gastrostomy provides reliable nutrition and hydration while reducing aspiration risk.
Additional treatment options
Motor function preservation therapy
Aggressive physical therapy intervention aims to preserve ambulation, maintain joint mobility, and prevent secondary complications from immobility for as long as possible.
physical_therapyVision support and environmental adaptations
Vision loss is a prominent feature; environmental modifications, tactile stimulation, and sensory integration activities help maintain quality of life and communication abilities.
complementaryCardiorespiratory monitoring
Regular cardiac and respiratory monitoring is recommended as autonomic dysfunction can develop, particularly during sleep.
monitoringWhen 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 Late Infantile Neuronal Ceroid Lipofuscinosis 6 C5880894?
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.
Mole SE, Schulz A, Badoe E, et al. Guidelines on the diagnosis, clinical management and therapeutic interventions for neuronal ceroid lipofuscinosis type 2 (CLN2) disease and other neuronal ceroid lipofuscinoses. Orphanet J Rare Dis. 2021.
Kohan R, Pesa E, Cismondi IA, Dodelson de Kremer R. Evidence of genotype-phenotype correlation in CLN6-related neuronal ceroid lipofuscinosis. Mol Genet Metab. 2020.
Sano T, Koyano F, Miyake N, et al. Long-term follow-up of patients with variant late infantile neuronal ceroid lipofuscinosis 6. J Child Neurol. 2019.
Johnson TB, Cain JT, White KA, et al. Therapeutic landscape for neuronal ceroid lipofuscinosis: molecular mechanisms and clinical trials. Neurobiol Dis. 2022.
Medication Comparisons
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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 25, 2026. · Read our editorial policy