DOI: 10.1212/WNL.0000000000010025 Neurology Publish ......2020/07/08  · Neurology Publish Ahead of...

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Neurology Publish Ahead of Print DOI: 10.1212/WNL.0000000000010025 Hypertrophic Polyneuropathy Authors: Luke Dixon, MBBS, FRCR, Sachit Shah, MBBS, FRCR Luke Dixon, National Hospital of Neurology and Neurosurgery, Queen Square, Holborn, London WC1N 3BG Sachit Shah, National Hospital of Neurology and Neurosurgery, Queen Square, Holborn, London WC1N 3BG Search Terms: All Neurology, Polyneuropathy, Pathogenesis, Natural History Title Character count: 76 Number of Tables: 0 Number of Figures: 1 Word count of Abstract: NA Word Count of Paper: 100 Corresponding author: Luke Dixon, [email protected] Sachit Shah [email protected] Analysis – conducted by Luke Dixon Neurology® Published Ahead of Print articles have been peer reviewed and accepted for publication. This manuscript will be published in its final form after copyediting, page composition, and review of proofs. Errors that could affect the content may be corrected during these processes. Videos, if applicable, will be available when the article is published in its final form. ACCEPTED Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited Published Ahead of Print on July 8, 2020 as 10.1212/WNL.0000000000010025

Transcript of DOI: 10.1212/WNL.0000000000010025 Neurology Publish ......2020/07/08  · Neurology Publish Ahead of...

Page 1: DOI: 10.1212/WNL.0000000000010025 Neurology Publish ......2020/07/08  · Neurology Publish Ahead of Print DOI: 10.1212/WNL.0000000000010025 Hypertrophic Polyneuropathy Authors: Luke

Neurology Publish Ahead of PrintDOI: 10.1212/WNL.0000000000010025

Hypertrophic Polyneuropathy Authors: Luke Dixon, MBBS, FRCR, Sachit Shah, MBBS, FRCR

Luke Dixon, National Hospital of Neurology and Neurosurgery, Queen Square, Holborn, London WC1N 3BG

Sachit Shah, National Hospital of Neurology and Neurosurgery, Queen Square, Holborn, London WC1N 3BG

Search Terms: All Neurology, Polyneuropathy, Pathogenesis, Natural History

Title Character count: 76 Number of Tables: 0 Number of Figures: 1

Word count of Abstract: NA Word Count of Paper: 100

Corresponding author: Luke Dixon, [email protected]

Sachit Shah [email protected]

Analysis – conducted by Luke Dixon Neurology® Published Ahead of Print articles have been peer reviewed and accepted for

publication. This manuscript will be published in its final form after copyediting, page

composition, and review of proofs. Errors that could affect the content may be corrected

during these processes. Videos, if applicable, will be available when the article is published

in its final form.

ACCEPTED

Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited

Published Ahead of Print on July 8, 2020 as 10.1212/WNL.0000000000010025

Page 2: DOI: 10.1212/WNL.0000000000010025 Neurology Publish ......2020/07/08  · Neurology Publish Ahead of Print DOI: 10.1212/WNL.0000000000010025 Hypertrophic Polyneuropathy Authors: Luke

Study Funding: No targeted funding reported. Disclosure: The authors report no disclosures relevant to the manuscript. Body Text: A 75-year-old man previously diagnosed with multiple sclerosis (MS), presented with a 20-year history of therapy-resistant progressive upper and lower limb weakness and sensory deficits. Examination found hand interossei muscle wasting, thickened radius and ulna nerves and reduced distal lower limb strength. Neurophysiology revealed a sensory-motor polyneuropathy with demyelinating features. MRI showed hypertrophic nerves and nerve roots in the lumbosacral plexus (Figure. 1). Charcot-Marie Tooth disease (CMT) and chronic inflammatory demyelinating polyneuropathy (CIDP) are the leading causes of hypertrophic polyneuropathy. Other causes include leprosy, neurofibromatosis and amyloid deposition. In this case genetic screening was negative for CMT suggesting probable CIDP.

ACCEPTED

Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited

Page 3: DOI: 10.1212/WNL.0000000000010025 Neurology Publish ......2020/07/08  · Neurology Publish Ahead of Print DOI: 10.1212/WNL.0000000000010025 Hypertrophic Polyneuropathy Authors: Luke

Teaching Slides-http://links.lww.com/WNL/B136 Figure 1 Legend: Title: Fat-suppressed T2-weighted magnetic resonance imaging of the lumbar plexus. Legend: MRI demonstrates striking symmetrical thickening and abnormal signal of the lumbar plexus and imaged peripheral nerves (arrows: thickened sciatic nerves).

Appendix Name Location Contribution

Luke Dixon, MBBS, FRCR Queen Square, London, UK

Interpreted the data;

drafted the manuscript for

intellectual content

Sachit Shah, MBBS, FRCR Queen Square, London, UK

Interpreted the data;

revised the manuscript for

intellectual content

ACCEPTED

Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited

Page 4: DOI: 10.1212/WNL.0000000000010025 Neurology Publish ......2020/07/08  · Neurology Publish Ahead of Print DOI: 10.1212/WNL.0000000000010025 Hypertrophic Polyneuropathy Authors: Luke

DOI 10.1212/WNL.0000000000010025 published online July 8, 2020Neurology 

Luke Dixon and Sachit ShahTeaching NeuroImages: Hypertrophic polyneuropathy

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