An autopsied case of Sjögren's syndrome with massive necrotic and demyelinating lesions of the cerebellar white matter

White Matter Water Diffusion Changes in Primary Sjogren Syndrome

“…39 Finally, the underlying process accounting for the WM involvement might be a combination of Wallerian degeneration and demyelination, which is supported by a limited number of autopsy studies that have demonstrated both axonal degeneration and demyelination in the CNS of patients with pSS. 40,41 Similarly, decreased FA and AD and increased RD have been demonstrated in patients with neuromyelitis optica. 42,43 These changes involve multiple, major WM tracts in the normal-appearing WM, accumulate with increasing disease duration, and are probably related to both demyelination and Wallerian degeneration.…”

Central and peripheral neurological complications of primary Sjögren's syndrome

“…Focal manifestations mostly presented with stroke-like features such as aphasia, hemiplegia, hypoesthesia, cerebellar ataxia consistent with a focal vasculitis [11,29,54]. Intracerebral or subarachnoid hemorrhage may signal the presence of vasculitis Several other focal neurologic manifestations have been described in pSS including internuclear ophthalmoplegia [29], nystagmus, dystonia, athetosis, intention tremor, chorea, L-dopa resistant Parkinsonism, focal and generalized seizures [28,29], cerebellar involvement [5,11], and spastic tetraparesis [11,44,55].…”

A New Kind of and Reversible Brainstem Involvement in Primary Sjögren's Syndrome as an Initial Manifestation

“…In some case reports of pSS with CNS involvement, clinical and radiological improvement have been suggested (Hirohata, Yasukawa, Ishida, Komai, & Yamada, 2005;Kadota et al, 2002;Sanahuja et al, 2008). However, severe clinical outcomes have been also reported (Hayashi et al, 2008;Ichikawa et al, 2004;Owada et al, 2002;Yaguchi et al, 2008;Yoon et al, 2000). Our patient had the neurological findings for 7 months and the MRI findings were severe, however, her response to the treatment was very good.…”