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Open AccessArticle

C-Fiber Loss as a Possible Cause of Neuropathic Pain in Schwannomatosis

1
Department of Neurology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany
2
Department of Neurology, Charité University Medicine, 10117 Berlin, Germany
3
Berlin Institute of Health, 10178 Berlin, Germany
4
Department of Neuropathology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany
5
Department of Neurophysiology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany
6
Department of Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany
7
Department of Neurosurgery, University Medical Center Tübingen, 72076 Tübingen, Germany
8
Leibniz Institute on Aging, Fritz Lipmann Institute, 07745 Jena, Germany
9
MVZ Human Genetics, 99084 Erfurt, Germany
10
Institute of Human Genetics, University of Ulm, 89081 Ulm, Germany
11
Department of Neuroradiology, University Medical Center Heidelberg, 69120 Heidelberg, Germany
12
Department of Radiology, German Cancer Research Center, 69120 Heidelberg, Germany
*
Author to whom correspondence should be addressed.
Martinistr. 52, 20246 Hamburg, Germany.
These two authors contributed equally to this publication as last authors.
Int. J. Mol. Sci. 2020, 21(10), 3569; https://doi.org/10.3390/ijms21103569
Received: 17 April 2020 / Revised: 9 May 2020 / Accepted: 15 May 2020 / Published: 18 May 2020
(This article belongs to the Section Molecular Neurobiology)
Schwannomatosis is the third form of neurofibromatosis and characterized by the occurrence of multiple schwannomas. The most prominent symptom is chronic pain. We aimed to test whether pain in schwannomatosis might be caused by small-fiber neuropathy. Twenty patients with schwannomatosis underwent neurological examination and nerve conduction studies. Levels of pain perception as well as anxiety and depression were assessed by established questionnaires. Quantitative sensory testing (QST) and laser-evoked potentials (LEP) were performed on patients and controls. Whole-body magnetic resonance imaging (wbMRI) and magnetic resonance neurography (MRN) were performed to quantify tumors and fascicular nerve lesions; skin biopsies were performed to determine intra-epidermal nerve fiber density (IENFD). All patients suffered from chronic pain without further neurological deficits. The questionnaires indicated neuropathic symptoms with significant impact on quality of life. Peripheral nerve tumors were detected in all patients by wbMRI. MRN showed additional multiple fascicular nerve lesions in 16/18 patients. LEP showed significant faster latencies compared to normal controls. Finally, IENFD was significantly reduced in 13/14 patients. Our study therefore indicates the presence of small-fiber neuropathy, predominantly of unmyelinated C-fibers. Fascicular nerve lesions are characteristic disease features that are associated with faster LEP latencies and decreased IENFD. Together these methods may facilitate differential diagnosis of schwannomatosis. View Full-Text
Keywords: Schwannomatosis; small-fiber neuropathy; pain; MR-neurography; fascicular microlesions Schwannomatosis; small-fiber neuropathy; pain; MR-neurography; fascicular microlesions
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Farschtschi, S.C.; Mainka, T.; Glatzel, M.; Hannekum, A.-L.; Hauck, M.; Gelderblom, M.; Hagel, C.; Friedrich, R.E.; Schuhmann, M.U.; Schulz, A.; Morrison, H.; Kehrer-Sawatzki, H.; Luhmann, J.; Gerloff, C.; Bendszus, M.; Bäumer, P.; Mautner, V.-F. C-Fiber Loss as a Possible Cause of Neuropathic Pain in Schwannomatosis. Int. J. Mol. Sci. 2020, 21, 3569.

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