H49.4

Clinical Information

• A disorder characterized by slowly progressive paralysis of the external eye muscles

• A mitochondrial myopathy characterized by slowly progressive paralysis of the levator palpebrae, orbicularis oculi, and extraocular muscles. Ragged-red fibers and atrophy are found on muscle biopsy. Familial and sporadic forms may occur. Disease onset is usually in the first or second decade of life, and the illness slowly progresses until usually all ocular motility is lost. from Adams et al., Principles of Neurology, 6th ed, p1422)

Details

• H49.4 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.

• The 2023 edition of ICD-10-CM H49.4 became effective on October 1, 2022.

• This is the American ICD-10-CM version of H49.4 – other international versions of ICD-10 H49.4 may differ.