C-fiber loss as a possible cause of neuropathic pain in schwannomatosis

• Verfasst von: Farschtschi, Said [VerfasserIn]
• Verfasst von: Mainka, Tina [VerfasserIn]
• Verfasst von: Glatzel, Markus [VerfasserIn]
• Verfasst von: Hannekum, Anna-Lena [VerfasserIn]
• Verfasst von: Hauck, Michael [VerfasserIn]
• Verfasst von: Gelderblom, Mathias [VerfasserIn]
• Verfasst von: Hagel, Christian [VerfasserIn]
• Verfasst von: Friedrich, Reinhard E. [VerfasserIn]
• Verfasst von: Schuhmann, Martin U. [VerfasserIn]
• Verfasst von: Schulz, Alexander [VerfasserIn]
• Verfasst von: Morrison, Helen [VerfasserIn]
• Verfasst von: Kehrer-Sawatzki, Hildegard [VerfasserIn]
• Verfasst von: Luhmann, Jan [VerfasserIn]
• Verfasst von: Gerloff, Christian [VerfasserIn]
• Verfasst von: Bendszus, Martin [VerfasserIn]
• Verfasst von: Bäumer, Philipp [VerfasserIn]
• Verfasst von: Mautner, Victor-Felix [VerfasserIn]
• Titel: C-fiber loss as a possible cause of neuropathic pain in schwannomatosis
• Verf.angabe: Said C. Farschtschi, Tina Mainka, Markus Glatzel, Anna-Lena Hannekum, Michael Hauck, Mathias Gelderblom, Christian Hagel, Reinhard E. Friedrich, Martin U. Schuhmann, Alexander Schulz, Helen Morrison, Hildegard Kehrer-Sawatzki, Jan Luhmann, Christian Gerloff, Martin Bendszus, Philipp Bäumer and Victor-Felix Mautner
• E-Jahr: 2020
• Jahr: 18 May 2020
• Umfang: 17 S.
• Fussnoten: Gesehen am 23.09.2020
• Titel Quelle: Enthalten in: International journal of molecular sciences
• Ort Quelle: Basel : Molecular Diversity Preservation International, 2000
• Jahr Quelle: 2020
• Band/Heft Quelle: 21(2020,10) Artikel-Nummer 3569, 17 Seiten
• ISSN Quelle: 1422-0067
• ISSN Quelle: 1661-6596
• DOI: doi:10.3390/ijms21103569
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: diagnostic-criteria
• Sach-SW: expression
• Sach-SW: fascicular microlesions
• Sach-SW: lesions
• Sach-SW: MR-neurography
• Sach-SW: mutations
• Sach-SW: pain
• Sach-SW: schwann-cells
• Sach-SW: Schwannomatosis
• Sach-SW: small-fiber neuropathy
• Sach-SW: smarcb1
• K10plus-PPN: 1733616152

Abstract

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.