E88.42

Applicable To

• Myoclonic epilepsy associated with ragged-red fibers

Approximate Synonyms

• Myoclonus epilepsy and ragged red fibers

• Myoclonus epilepsy and ragged red fibers syndrome

Clinical Information

• A mitochondrial encephalomyopathy characterized clinically by a mixed seizure disorder, myoclonus, progressive ataxia, spasticity, and a mild myopathy. Dysarthria, optic atrophy, growth retardation, deafness, and dementia may also occur. This condition tends to present in childhood and to be transmitted via maternal lineage. Muscle biopsies reveal ragged-red fibers and respiratory chain enzymatic defects. from Adams et al., Principles of Neurology, 6th ed, p986)

• A very rare mitochondrial abnormality characterized by myoclonic epilepsy and the microscopic finding of ragged-red fibers in muscle tissues.

Details

• E88.42 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM E88.42 became effective on October 1, 2022.

• This is the American ICD-10-CM version of E88.42 – other international versions of ICD-10 E88.42 may differ.