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Small Fiber Neuropathy and Related Syndromes: Pain and Neurodegeneration pp 99–107Cite as

Small Fiber Pathology and Functional Impairment in Syndromes of Predominantly Large Fiber Neuropathy

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Abstract

In peripheral neuropathies with sensory ataxia or weakness of distal limbs, traditionally nerve conduction studies are the first-line tests, and these neuropathies are classified as large fiber neuropathy including (1) inflammatory neuropathies of Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and polyneuropathy associated with organomegaly, endocrinopathy, monoclonal gammopathy, and skin hyperpigmentation (POEMS), (2) hereditary neuropathies of Charcot-Marie-Tooth (CMT) disease encompassing different genotypes, and (3) chemotherapy-induced peripheral neuropathy (CIPN). Patients with this phenotype of large fiber neuropathy frequently have symptoms and signs suggesting small fiber involvement, i.e., small fiber nerve degeneration and functional impairment. With the applications of skin biopsy for measuring intraepidermal nerve fiber density (IENFD), quantitative sensory testing for assessing thermal thresholds, and autonomic function tests for examining sympathetic and parasympathetic axis, concomitant small fiber pathology and functional impairment (small fiber pathology or syndrome) were documented in some of these neuropathies traditionally considered as pure large fiber neuropathy. Not only serving as a criterion of small fiber pathology, parameters of small fiber examinations also provide a surrogate marker of disease activities and progression reflecting generalized ambulation difficulty and correlated with patient-reported outcome measure of quality of life.

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Authors and Affiliations

  1. Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan

    Chi-Chao Chao & Sung-Tsang Hsieh

  2. Graduate Institute of Molecular Medicine, National Taiwan University College of Medicine, Taipei, Taiwan

    Chun-Liang Pan

  3. Center of Precision Medicine, National Taiwan University College of Medicine, Taipei, Taiwan

    Chun-Liang Pan & Sung-Tsang Hsieh

  4. Department of Anatomy and Cell Biology, National Taiwan University College of Medicine, Taipei, Taiwan

    Sung-Tsang Hsieh

  5. Graduate Institute of Brain and Mind Sciences, National Taiwan University College of Medicine, Taipei, Taiwan

    Sung-Tsang Hsieh

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  1. Chi-Chao Chao
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  2. Chun-Liang Pan
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  3. Sung-Tsang Hsieh
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Correspondence to Sung-Tsang Hsieh .

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Editors and Affiliations

  1. Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan

    Sung-Tsang Hsieh

  2. Department of Neurology, Imperial College London, Hammersmith Hospital, London, UK

    Praveen Anand

  3. Department of Neurology, Harvard Medical School, Beth Israel Deaconess Medical Center, Boston, MA, USA

    Christopher H. Gibbons

  4. Neurologische Klinik, Universitätsklinikum Würzburg , Würzburg, Germany

    Claudia Sommer

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Chao, CC., Pan, CL., Hsieh, ST. (2019). Small Fiber Pathology and Functional Impairment in Syndromes of Predominantly Large Fiber Neuropathy. In: Hsieh, ST., Anand, P., Gibbons, C., Sommer, C. (eds) Small Fiber Neuropathy and Related Syndromes: Pain and Neurodegeneration. Springer, Singapore. https://doi.org/10.1007/978-981-13-3546-4_9

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