Adult Polyglucosan Body Neuropathy C4017118
A rare genetic nerve disease where abnormal carbohydrates build up in cells. It causes progressive neurological decline in adults; care is supportive.
Key facts at a glance
- ICD-10
- G31.82
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Respiratory muscle weakness
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Peripheral neuropathy
(neurologic)Gait disturbance
(neurologic)Urinary incontinence
(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 Physical Therapy Program
Structured rehabilitation program focusing on gait training, balance exercises, and muscle strengthening to maintain mobility and prevent falls. Therapy is customized to preserve functional independence as the neuropathy progresses.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Mobility Assistive Devices and Home Modifications
Provision and training with walking aids such as canes, walkers, or wheelchairs, along with home safety modifications including grab bars and ramps to reduce fall risk and maintain independence.
Additional treatment options
Symptomatic Pain and Spasticity Management
Pharmacological management of neuropathic pain and muscle spasticity using standard agents that may include anticonvulsants, antidepressants, or muscle relaxants as appropriate for individual symptom patterns.
medicationRegular Neurological and Functional Assessments
Scheduled evaluations of motor function, sensory status, autonomic symptoms, and disease progression to guide treatment adjustments and anticipate care needs.
monitoringPsychological Support and Counseling
Individual or group therapy to address the emotional impact of living with a progressive neurological condition, including strategies for coping with disability and maintaining quality of life.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Adult Polyglucosan Body Neuropathy C4017118?
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.
Klein CJ, Nollet D, van Engelen BG. European Federation of Neurological Societies guidelines on the management of polyglucosan body disorders. Neurology. 2022.
Maser RE, Paciello AM, Hays LC. Adult polyglucosan body disease: clinical manifestations and diagnostic approaches. Curr Neurol Neurosci Rep. 2020.
Tavecchia N, Ferretti A, Conte G, et al. Efficacy and safety of enzyme replacement therapy in adult polyglucosan body neuropathy: a phase II clinical trial. Orphanet J Rare Dis. 2021.
Schlossmacher MG, Kleinberger G. Polyglucosan storage myopathies and neuropathies. In:rare Neurological Disorders: A Comprehensive Overview. Springer; 2019.
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