A 67-year-old man was referred for 4 years of progressive quadriparesis and dysarthria. Symptoms began in 2015 as progressive right more than left lower limb weakness and difficulty walking. An evaluation in 2016 included magnetic resonance imaging of the brain and spinal cord with results interpreted as concerning for demyelinating disease. Cerebrospinal fluid analysis results showed 2 total nucleated cells, protein levels of 0.063 g/dL (to convert to grams per liter, multiply by 10), glucose levels of 110 mg/dL (to convert to millimoles per liter, multiply by 0.0555), and no oligoclonal bands. He was treated with prolonged courses of corticosteroids and several multiple sclerosis (MS) disease-modifying therapies but had ongoing symptom progression, including upper limb weakness and dysarthria. He was referred to the Mayo Clinic (Rochester, Minnesota) for an opinion.
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Simpson HD, Aksamit AJ, Zalewski NL. Longitudinally Extensive Spinal Cord Lesion in Erdheim-Chester Disease. JAMA Neurol. Published online July 27, 2020. doi:10.1001/jamaneurol.2020.2460
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