2011
DOI: 10.1007/s00296-011-1824-z
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A case of primary Sjögren’s syndrome presenting primarily with central nervous system vasculitic involvement

Abstract: Sjögren's syndrome is primarily a chronic systemic autoimmune disease that affects exocrine organs. Neurologic symptoms frequently present as peripheral neuropathy due to small vessel vasculitis. Type and prevalence of central nervous system involvement are still controversial. In this report, we present a 35-year-old woman with primary Sjögren's syndrome with central nervous system vasculitic involvement.

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Cited by 21 publications
(14 citation statements)
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“…While callosal lesions may occur,70 71 they are relatively infrequent68 69 72 compared with MS. In one study, CC/subcallosal lesions were present in only 8% of patients with SLE or Sjogren's syndrome,58 and in another study, only 3 out of 25 patients with Sjogren's syndrome with white matter abnormalities had CC involvement 69.…”
Section: Non-demyelinating Neuroinflammatory Diseasesmentioning
confidence: 99%
“…While callosal lesions may occur,70 71 they are relatively infrequent68 69 72 compared with MS. In one study, CC/subcallosal lesions were present in only 8% of patients with SLE or Sjogren's syndrome,58 and in another study, only 3 out of 25 patients with Sjogren's syndrome with white matter abnormalities had CC involvement 69.…”
Section: Non-demyelinating Neuroinflammatory Diseasesmentioning
confidence: 99%
“…In our research, the main neurological manifestation at the beginning was headache in both kinds of vasculitides, though severe headache was more commonly observed in PCNSV. Although headache is a nonspecific symptom for ARD, CNS vasculitis should be searched for these patients if they present it [17][18][19][20][21][22]. In our study, focal and non-focal neurological manifestations were similar in both groups of vasculitides, except for the focal motor deficit which was significantly more frequent in PCNSV.…”
Section: Discussionmentioning
confidence: 44%
“…However, pSS is not a vasculitic disease, although sometimes small vessel vasculitis may occur in cases with high disease activity. Vasculitis of larger vessels is only occasionally described in cerebral angiography studies in patients with pSS . Another factor weighing against vasculitis as a pathogenic player is our previous finding that the pSS patients do not have more cerebral infarcts or higher WMH scores than age‐ and gender‐matched healthy subjects .…”
Section: Discussionmentioning
confidence: 98%