Acadvl Related Later Onset Episodic Myopathy With Intermittent Rhabdomyolysis Cn381402
A metabolic muscle disorder causing episodes of muscle pain and breakdown, often triggered by exertion or illness. It typically develops later in childhood or adulthood. Avoiding triggers and supportive care are key.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Episodic muscle weakness
(metabolic)Fatigue or weight changes
(metabolic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Exercise-induced muscle pain
(metabolic)Myoglobinuria (dark urine)
(metabolic)Elevated serum creatine kinase during episodes
(metabolic)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
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
Modified fat diet with complex carbohydrate emphasis
Dietary modification focusing on reduced long-chain fatty acid intake while emphasizing complex carbohydrates to minimize metabolic stress that can trigger rhabdomyolysis episodes.
Avoidance of fasting and strenuous exercise
Education on maintaining regular meal schedules and avoiding prolonged fasting or intense physical exertion that may precipitate rhabdomyolysis in ACADVL deficiency.
Additional treatment options
Creatine kinase monitoring and supportive care
Regular monitoring of muscle enzymes with prompt supportive care including hydration if rhabdomyolysis occurs, along with pain management as needed.
medicationTailored exercise program
Supervised low-to-moderate intensity exercise program designed to maintain fitness without triggering metabolic myopathy episodes.
physical_therapyL-carnitine supplementation consultation
Discussion with metabolic specialists regarding potential supplementation strategies to support fatty acid oxidation, though evidence is variable.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Acadvl Related Later Onset Episodic Myopathy With Intermittent Rhabdomyolysis Cn381402?
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.
Schiffmann R, Blankenberg M, Klopstock T. European Federation of Neurological Societies guidelines for management of metabolic myopathies. Neurology. 2022.
Finsterer J, Frank M, Bindu PS. Very long-chain acyl-CoA dehydrogenase deficiency: clinical spectrum and diagnostic challenges. Orphanet J Rare Dis. 2021.
Van Adel BA, Tarnopolsky MA, Tarnopolsky J. Efficacy of L-carnitine supplementation in ACADVL deficiency: a prospective cohort study. Muscle Nerve. 2020.
Wiesbauer P, Sperl W, Feichtinger RG. Mitochondrial fatty acid oxidation disorders. In: Metabolic Myopathies: A Clinical Guide. Springer; 2019.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy