Juvenile Amyotrophic Lateral Sclerosis With Dementia C1859806
A rare form of motor neuron disease that begins in youth, combining progressive muscle weakness with cognitive decline. Treatment focuses on symptom management and supportive care.
Key facts at a glance
- ICD-10
- G12.21
- 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
Progressive limb weakness
Cognitive decline
Swallowing difficulty
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Muscle fasciculations
(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
Riluzole with cognitive monitoring
Standard ALS disease-modifying therapy with close neuropsychological monitoring due to the added dementia component affecting treatment response and compliance.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Cognitive and behavioral interventions
Specialized psychological interventions addressing cognitive decline, behavioral changes, and executive function deficits common in ALS-FTD overlap syndrome.
Additional treatment options
Psychotropic medication management
Targeted pharmacotherapy for behavioral symptoms such as agitation, disinhibition, or psychosis that may accompany frontotemporal dementia features.
medicationMultidisciplinary neurological monitoring
Regular comprehensive assessments by neurologists, psychiatrists, and allied health professionals to track both motor and cognitive disease progression.
monitoringWhen 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 Juvenile Amyotrophic Lateral Sclerosis With Dementia C1859806?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy