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August 18, 2015; 85 (7) Clinical/Scientific Notes

Diffuse leukoencephalopathy with spheroids presenting as primary progressive aphasia

Carly Oboudiyat, Eileen H. Bigio, Borna Bonakdarpour, Matt C. Baker, Rosa Rademakers, Sandra Weintraub, M.-Marsel Mesulam
First published July 22, 2015, DOI: https://doi.org/10.1212/WNL.0000000000001851
Carly Oboudiyat
From the Cognitive Neurology and Alzheimer's Disease Center (C.O., E.H.B., B.B., S.W., M.-M.M.), Northwestern University Feinberg School of Medicine, Chicago, IL; and the Mayo Clinic (M.C.B., R.R.), Jacksonville, FL.
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Eileen H. Bigio
From the Cognitive Neurology and Alzheimer's Disease Center (C.O., E.H.B., B.B., S.W., M.-M.M.), Northwestern University Feinberg School of Medicine, Chicago, IL; and the Mayo Clinic (M.C.B., R.R.), Jacksonville, FL.
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Borna Bonakdarpour
From the Cognitive Neurology and Alzheimer's Disease Center (C.O., E.H.B., B.B., S.W., M.-M.M.), Northwestern University Feinberg School of Medicine, Chicago, IL; and the Mayo Clinic (M.C.B., R.R.), Jacksonville, FL.
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Matt C. Baker
From the Cognitive Neurology and Alzheimer's Disease Center (C.O., E.H.B., B.B., S.W., M.-M.M.), Northwestern University Feinberg School of Medicine, Chicago, IL; and the Mayo Clinic (M.C.B., R.R.), Jacksonville, FL.
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Rosa Rademakers
From the Cognitive Neurology and Alzheimer's Disease Center (C.O., E.H.B., B.B., S.W., M.-M.M.), Northwestern University Feinberg School of Medicine, Chicago, IL; and the Mayo Clinic (M.C.B., R.R.), Jacksonville, FL.
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Sandra Weintraub
From the Cognitive Neurology and Alzheimer's Disease Center (C.O., E.H.B., B.B., S.W., M.-M.M.), Northwestern University Feinberg School of Medicine, Chicago, IL; and the Mayo Clinic (M.C.B., R.R.), Jacksonville, FL.
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M.-Marsel Mesulam
From the Cognitive Neurology and Alzheimer's Disease Center (C.O., E.H.B., B.B., S.W., M.-M.M.), Northwestern University Feinberg School of Medicine, Chicago, IL; and the Mayo Clinic (M.C.B., R.R.), Jacksonville, FL.
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Citation
Diffuse leukoencephalopathy with spheroids presenting as primary progressive aphasia
Carly Oboudiyat, Eileen H. Bigio, Borna Bonakdarpour, Matt C. Baker, Rosa Rademakers, Sandra Weintraub, M.-Marsel Mesulam
Neurology Aug 2015, 85 (7) 652-653; DOI: 10.1212/WNL.0000000000001851

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This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

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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  • Aphasia
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  • Leukodystrophies
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  • Assessment of cognitive disorders/dementia

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