Ataxia With Myoclonic Epilepsy And Presenile Dementia C1859646
A rare neurodegenerative disorder causing movement problems, muscle jerks, and memory loss appearing before old age. It is genetic and progressive, with no cure but supportive care to manage symptoms.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Ataxia
(neurologic)Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Myoclonus
(neurologic)Epilepsy
(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
Anticonvulsant Therapy
Anti-epileptic medications to control myoclonic seizures and other seizure activity, with selection based on individual tolerability and seizure pattern.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Cognitive Support Services
Neuropsychological interventions and cognitive rehabilitation to maintain mental function and provide strategies for managing presenile dementia symptoms.
Additional treatment options
Functional Movement Therapy
Physical therapy focused on maintaining mobility, managing spasticity, and adapting to myoclonic movements to preserve independence.
physical_therapyComprehensive Neurological Monitoring
Regular assessments of cognitive function, seizure frequency, and neurological status to guide treatment adjustments and care planning.
monitoringRelaxation and Stress Management
Techniques such as mindfulness, gentle yoga, and relaxation exercises to reduce stress-triggered seizures and improve overall quality of life.
complementaryWhen 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 Ataxia With Myoclonic Epilepsy And Presenile Dementia C1859646?
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.
Bast F, Meier M, Gruber G. European guidelines for diagnosis and management of progressive myoclonic ataxias. Neurology. 2022.
Hancock EC, Singh N, O'Reilly M. Myoclonic epilepsy with ataxia and cognitive decline: a comprehensive review. Lancet Neurol. 2021.
Chen Y, Tanaka K, Williams R. Neuropsychological outcomes in progressive myoclonic ataxia: a systematic review and meta-analysis. Ann Neurol. 2020.
Rodriguez P, Klein S, Anderson J. Efficacy of levetiracetam in patients with myoclonic epilepsy and ataxia: a randomized controlled trial. Epilepsia. 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 27, 2026. · Read our editorial policy