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Asymmetric sensory ganglionopathy: A case series

Authors

  • Celedonio Marquez-Infante PhD,

    1. Servicio de Neurologia, Hospital Universitario Virgen del Rocío, Seville, Spain
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  • Sinéad M. Murphy MRCPI,

    1. MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London
    2. Department of Neurology, The Adelaide and Meath Hospitals Incorporating the National Children's Hospital, Dublin
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  • Liberty Mathew MBChB,

    1. MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London
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  • Ali Alsanousi PhD,

    1. MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London
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  • Michael P. Lunn FRCP,

    1. MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London
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  • Sebastian Brandner FRCPath,

    1. Division of Neuropathology and Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London
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  • Tarek A. Yousry FRCR,

    1. UCL Institute of Neurology, Department of Brain Repair and Rehabilitation, Queen Square, London
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  • Julian Blake MRCP,

    1. Department of Clinical Neurophysiology, The National Hospital for Neurology and Neurosurgery and Department of Molecular Neuroscience, UCL Institute of Neurology, London, United Kingdom
    2. Department of Clinical Neurophysiology, Norfolk and Norwich University Hospital, Norwich, United Kingdom
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  • Mary M. Reilly FRCP

    Corresponding author
    • MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London
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Correspondence to: M.M. Reilly; e-mail: m.reilly@ucl.ac.uk

ABSTRACT

Introduction

Sensory ganglionopathies are uncommon but potentially very disabling. They have heterogeneous etiologies including autoimmune, paraneoplastic, toxic, and inflammatory although many remain idiopathic despite intensive investigation. Asymmetric sensory loss is relatively common at the onset, but with time, symptoms usually spread to involve all limbs symmetrically.

Methods

We report 6 patients with a persistent strikingly asymmetrical sensory ganglionopathy with acute or subacute onset and slow progression.

Results

Peripheral nerve biopsies in 5 patients showed axonal loss without significant inflammation; a dorsal root ganglion biopsy in 1 patient showed neuronal loss and inflammatory infiltrate. Four patients received immunomodulatory treatment, but overall the response to treatment was poor.

Conclusions

Asymmetrical sensory ganglionopathies may have an inflammatory basis. Immunomodulatory therapy may be considered early in the disease course, although in this series there was a limited response to treatment. Muscle Nerve, 2013

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