CC BY 4.0 · J Brachial Plex Peripher Nerve Inj 2019; 14(01): e9-e13
DOI: 10.1055/s-0039-1685457
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Schwannomatosis of the Spinal Accessory Nerve: A Case Report

Ramin A. Morshed
1  Department of Neurological Surgery, University of California, San Francisco, California, United States
,
Anthony T. Lee
1  Department of Neurological Surgery, University of California, San Francisco, California, United States
,
Young M. Lee
1  Department of Neurological Surgery, University of California, San Francisco, California, United States
,
Cynthia T. Chin
2  Department of Neuroradiology, University of California, San Francisco, California, United States
,
Line Jacques
1  Department of Neurological Surgery, University of California, San Francisco, California, United States
› Author Affiliations
Further Information

Publication History

10 December 2018

18 February 2019

Publication Date:
26 April 2019 (online)

  

Abstract

Schwannomatosis is a distinct syndrome characterized by multiple peripheral nerve schwannomas that can be sporadic or familial in nature. Cases affecting the lower cranial nerves are infrequent. Here, the authors present a rare case of schwannomatosis affecting the left spinal accessory nerve. Upon genetic screening, an in-frame insertion at codon p.R177 of the Sox 10 gene was observed. There were no identifiable alterations in NF1, NF2, LZTR1, and SMARCB1. This case demonstrates a rare clinical presentation of schwannomatosis in addition to a genetic aberration that has not been previously reported in this disease context.