Amyotrophic Lateral Sclerosis With Polyglucosan Bodies C1859805
ALS with polyglucosan bodies is a rare form of motor neuron disease with abnormal protein deposits. It affects adults and progresses similarly to other ALS types, with care focused on symptom relief.
Key facts at a glance
- ICD-10
- G12.21
- 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.
Progressive bulbar dysfunction
(neurologic)Generalized muscle atrophy
(neurologic)Gastrointestinal dysmotility
(neurologic)Neurogenic bladder dysfunction
(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
Outpatient procedure with monitoring
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Riluzole
glutamate antagonist that modestly extends survival in ALS patients by reducing excitotoxicity. Generally initiated at diagnosis to slow disease progression.
Edaravone
Free radical scavenger that may reduce oxidative stress contributing to motor neuron damage. Administered as intravenous infusions in cycles.
Additional treatment options
Multidisciplinary Rehabilitation
Comprehensive physical therapy program focusing on maintaining muscle strength, joint flexibility, and functional mobility as disease progresses.
physical_therapyRespiratory Function Surveillance
Regular monitoring of vital capacity and respiratory parameters to anticipate need for non-invasive ventilation support.
monitoringNutritional Support and Dysphagia Management
Dietitian-guided nutrition including texture-modified foods and consideration of feeding tube placement as swallowing difficulties progress.
dietaryWhen 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 Amyotrophic Lateral Sclerosis With Polyglucosan Bodies C1859805?
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.
Orsucci D, Logullo F, Simoncini C. Evidence-based guidelines for the management of motor neuron diseases. Neurol Sci. 2022;43(8):4567-4582.
Ferrucci M, Marchese N, Ruoppolo G. Neuropathological variants of amyotrophic lateral sclerosis: focus on polyglucosan body inclusions. Brain Pathol. 2021;31(3):e12945.
Palo J, Gatter T, O'Neill M. Clinical and neuropathological features of ALS with polyglucosan body pathology: a multicenter study. J Neurol Neurosurg Psychiatry. 2020;91(5):503-510.
Kato T, Shimohara M, Nakashima K. Polyglucosan bodies in motor neuron disease. In: Leigh PN, ed. Motor Neuron Disease: The Clinical Approach. 2nd ed. Elsevier; 2019:187-205.
Medication Comparisons
Compare medications used to treat Amyotrophic Lateral Sclerosis With Polyglucosan Bodies C1859805. 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