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June 12, 2018; 90 (24) Clinical/Scientific Notes

IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease

Hugo Morales-Briceño, Belinda Cruse, Alessandro F. Fois, Ming-Wei Lin, Jocelyn Jiang, Dev Banerjee, Ron Grunstein, Winny Varikatt, Michael Rodriguez, Claire Shepherd, Victor S.C. Fung
First published May 16, 2018, DOI: https://doi.org/10.1212/WNL.0000000000005679
Hugo Morales-Briceño
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
MD
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Belinda Cruse
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
MBBS, FRACP
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Alessandro F. Fois
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
BMBCh, MRCP
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Ming-Wei Lin
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
FRACP, FRCPA
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Jocelyn Jiang
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
MBBS
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Dev Banerjee
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
MD
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Ron Grunstein
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
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Winny Varikatt
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
MBBS
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Michael Rodriguez
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
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Claire Shepherd
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
PhD
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Victor S.C. Fung
From the Movement Disorders Unit, Neurology Department (H.M.-B., B.C., A.F.F., V.F.), Department of Immunology (M.-W.L., J.J.), and Department of Tissue Pathology and Diagnostic Oncology, ICPMR (W.V.), Westmead Hospital; Woolcock Institute of Medical Research (D.B., R.G.), Sydney Medical School (H.M.-B., A.F.F., V.F.), the University of Sydney; Douglass Hanly Moir Pathology (M.R.), New South Wales; and Neuroscience Research Australia (C.S.), Sydney, Australia.
PhD, FRACP
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Full PDF
Citation
IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease
Hugo Morales-Briceño, Belinda Cruse, Alessandro F. Fois, Ming-Wei Lin, Jocelyn Jiang, Dev Banerjee, Ron Grunstein, Winny Varikatt, Michael Rodriguez, Claire Shepherd, Victor S.C. Fung
Neurology Jun 2018, 90 (24) 1113-1115; DOI: 10.1212/WNL.0000000000005679

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A 49-year-old man developed cold intolerance as an isolated symptom 2 years prior to presentation. At age 48, he developed jerks in the trunk and lower limbs during the day and tapping movements in the arms during sleep. After 6 months, sleep disturbance worsened with talking and gesticulations, culminating in sleeping 1–3 hours per night with associated daytime somnolence. He developed mild slurred speech, hypersalivation, and dysphagia. He reported poor memory but was able to continue driving and working. He had been diagnosed with obstructive sleep apnea 4 years prior.

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  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Received September 12, 2017.
  • Accepted in final form March 12, 2018.
  • © 2018 American Academy of Neurology
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Letters: Rapid online correspondence

  • Author response to Drs. Bally and Lang
    • Hugo Morales-Briceño, Neurologist, Westmead Hospital (NSW, Australia)
    • Victor Fung, Neurology Director, Westmead Hospital (NSW, Australia)
    Submitted August 14, 2018
  • Comment on: “IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease”
    • Julien F. Bally, Neurologist, University Hospitals of Geneva
    • Anthony E. Lang, Neurologist, Edmond J. Safra Program in Parkinson's Disease and Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital
    Submitted July 24, 2018
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