A 64-year-old woman was evaluated 6 years previously due to progressive gait difficulties rendering her reliant on a cane for ambulation. More recently, she had been confined to a wheelchair. She had a history of chronic diarrhea, bilateral cataract surgery in her early thirties, and “lumps” involving both Achilles tendons.

Our patient had a history of progressive ataxia and short-term memory impairment, and surgery for early-onset adult cataracts. On neurologic examination, she had inappropriate jocularity, mild dysarthria, muscle stretch hyperreflexia, bilateral Babinski signs, mild intention tremor, dysdiadochokinesis, a wide-base unstable gait requiring the assistance of a cane, and inability to perform tandem walking. Neuropsychological testing showed widespread neurocognitive deficits with poor attention and concentration, dyscalculia, and impaired memory.