A 35-year-old woman presented with a progressive 3-year history of personality changes and gait impairment. Neurologic examination revealed bilateral optic atrophy, spastic paraparesis, and impaired vibratory sensation in all limbs, and neuropsychological evaluation identified a frontotemporal cognitive impairment. In this article, we review the differential diagnosis for a young woman with chronic frontotemporal dysfunction, optic atrophy, and dorsolateral myelopathy in a stepwise multidisciplinary approach. Critical information obtained from the medical history led to a final diagnosis.
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