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A 42-year-old woman presented with a 2-month history of imbalance. She had longstanding bilateral hearing loss, retinitis pigmentosa, anosmia, and numbness in both feet. A recent self-imposed diet had led to ∼13 Kg weight loss over 7 months. She had a wide-based gait with bilateral foot drop. Both feet showed foreshortening of the fourth digit. Ankle dorsiflexion, plantarflexion, inversion and eversion (3–4/5), and knee flexion and extension (4+/5) were weak, but hip and upper limb power were normal. She was areflexic. Vibration and proprioception were impaired to the ankles, pain and temperature to midcalf. Coordination was normal. She had no spontaneous, gaze-evoked, head-shaking, or positional nystagmus.
Footnotes
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- Received August 20, 2017.
- Accepted in final form February 8, 2018.
- © 2018 American Academy of Neurology
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