Differential, size-dependent: sensory neuron involvement in the painful and ataxic forms of primary Sjogren%26apos;s syndrome-associated neuropathy

作者：Kawagashira Yuichi; Koike Haruki^*; Fujioka Yusuke; Hashimoto Rina; Tomita Minoru; Morozumi Saori; Iijima Masahiro; Katsuno Masahisa; Tanaka Fumiaki; Sobue Gen

来源：Journal of the Neurological Sciences, 2012, 319(1-2): 139-146.

DOI：10.1016/j.jns.2012.05.022

• Primary Sjogren%26apos
• s syndrome
• Painful neuropathy
• Ataxic neuropathy
• Ganglionitis
• Cytotoxic autoimmunity

Primary Sjogren%26apos;s syndrome (pSS)-associated neuropathy manifests a wide variety of peripheral neuropathies that may show overlap among the neuropathic forms. In this report, we describe histopathological findings of two autopsy cases with pSS-associated neuropathy; one of them manifested the painful form and another showed ataxic form. The population of dorsal root ganglion (DRG) neurons and the density of myelinated fibers in the dorsal spinal root were variably reduced among spinal segments in both forms. In the painful form, there was a prominent reduction of small neurons, while in the ataxic form, large neurons were predominately lost In accordance with the degree of the DRG cell loss, the modality of nerve fiber loss in the dorsal spinal roots and sural nerve correlated well with the corresponding DRG neuron loss. Prominent CD8 + T lymphocyte infiltration was present in the DRG, sympathetic ganglion, epineurial and perineurial space throughout the peripheral nerve trunks, and visceral organs, including the submandibular gland of both forms. %26lt;br%26gt;Although the size of affected DRG neurons is different, these two forms share a similar causal mechanism, namely, cytotoxic autoimmunity to ganglion neurons, which may be one of a continuum of etiological factors. This hypothesis may have an impact on therapeutic approach.

• 出版日期2012-8-15