A 15-year-old male with a maternal family history of "optic neuropathy" presented with acute flaccid paralysis in both upper limbs. Cervical MRI demonstrated longitudinally extensive T2 lesions, with significant enhancement of the anterior gray matter. Typical "snake-eye" appearance was observed in the follow-up MRI one month later (Figure). Analysis of blood mt-DNA revealed a homoplasmic m.14495T>C mutation, confirming the diagnosis of Leber's hereditary optic neuropathy (LHON). Idebenone 900mg/d was given to him, and he recovered completely 3 months later.Spinal cord involvement was rare in LHON 1 . The enhancement pattern in this case mimicked spinal cord infarction rather than demyelinating diseases 2 .
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