Diffuse leukoencephalopathy with spheroids presenting as primary progressive aphasia
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A 52-year-old right-handed man reported 2 years of progressive word-finding difficulties. He noticed hesitations when retrieving words and difficulty writing and calculating while working in his carpentry business. He was taking pravastatin for hyperlipidemia. Memantine was started prior to our clinical evaluation, and had no measurable effect. He had a great aunt with late-life amnestic dementia, his father died at age 59 years from lung cancer, and his mother died at age 75 of unknown causes. He had 18 years of education and no history of learning disabilities.
Footnotes
Disclosure: The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
Author contributions: Carly Oboudiyat and Marsel Mesulam conceptualized and wrote the original case report. Sandra Weintraub, Eileen Bigio, Borna Bonakdarpour, Matt Baker, and Rosa Rademakers contributed to the design of the article, as well as the editing process. Eileen Bigio also performed the autopsy and designed the pathology figure. Rosa Rademakers and Matt Baker also advised on the genetics and ran the genetic sequencing.
Study funding: Supported by research grants from NIA P30 AG13854 and R01DC008552.
- Received January 8, 2015.
- Accepted in final form April 24, 2015.
- © 2015 American Academy of Neurology
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Letters: Rapid online correspondence
- Re:Diffuse Leukoencephalopathy with Spheroids (DLS) with Early Apraxia of Speech
- Carly Oboudiyat, Behavioral Neurology Fellow, Northwestern Universitycarly.oboudiyat@northwestern.edu
Submitted January 19, 2016 - Diffuse Leukoencephalopathy with Spheroids (DLS) with Early Apraxia of Speech
- Samuel Lapalme-Remis, Neurology Fellow, Mayo Clinic, Rochester, MNlapalmeremis@gmail.com
- Jodi Warman Chardon, Ottawa, ON, Canada, Pierre Bourque, Ottawa, ON, Canada
Submitted October 27, 2015
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