Cerebellar degeneration in primary Sjӧgren syndrome

Abstract

Neurological manifestations are reported as a consequence of primary Sjӧgren syndrome (PSS). Any part of the brain and peripheral nervous system can be involved in PSS. However, cerebellar degeneration and atrophy associated with PSS have been rarely reported. Our report describes a 22-year-old woman who presented with cerebellar ataxia, arthritis and arthralgia. Evaluation of her symptoms, autoantibodies and salivary gland pathology was in favour of the diagnosis of Sjögren syndrome. Also, her brain MRI revealed cerebellar degeneration. There are only four patients reported to be affected by cerebellar atrophy associated with PSS. Administration of high doses of methylprednisolone and cyclophosphamide leads to substantial improvement in the cerebellar symptoms of this case. In addition, after 2 months of follow-up, the patient's ataxia recovered significantly. It could be concluded that in addition to neurological degenerative disorders, in some cases cerebellar atrophy could also be associated with autoimmune conditions such as PSS.

Anti-Ro/SSA Antibodies May Be Responsible for Cerebellar Degeneration in Sjogren’s Syndrome

“…The most notable finding in the literature review was the positive rate of autoantibody detection ( Table 1 ). Although anti-Ro/SSA antibodies in the serum were positive in all patients, anti-La/SSB antibodies in the serum were negative in five of the 14 patients [ 7 , 12 , 16 - 18 ]. In addition, our patient 2, who had SjS with PNS involvement but no CNS involvement, was negative for both anti-Ro/SSA and anti-La/SSB antibodies in the CSF.…”

“…We found 14 patients with previously reported SjS, including our patient 1, who also had developed cerebellar degeneration [6][7][8][9][10][11][12][13][14][15][16][17][18]. The characteristics of cerebellar degeneration in patients with SjS are provided in Table 1.…”

Anti-Ro/SAA Autoantibodies Might Represent the Antineuronal Antibodies Responsible for Cerebellar Degeneration in Sjögren's Syndrome 

“…The most notable nding in this literature review was the positive rate of autoantibody detection (Table). Although anti-Ro/SSA antibodies in the serum were positive in all patients, anti-La/SSB antibodies in the serum were negative in 5 of 14 patients [5,10,[15][16][17]. Additionally, Patient 2 of our study, who had SjS with a peripheral neuropathy but no CNS involvement, was negative for both anti-Ro/SSA and anti-La/SSB antibodies in the CSF.…”

“…Nanri K [11] 2011/F/84 / IVIG Kim MJ [12] 2012/F/46 / Pre HCQ Chen YW [13] 2013/F/44 / NP Pre Sharma R [14] 2014/M/64 / NP Pre CTX Farhat E [15] 2016/F/30 / NP Pre CTX Maciel R [16] 2017/F/36 / Pre CTX Heidary M [17] 2018/F/22 / Pre CTX Our case 2020/M/36 / Pre IVIG *Whether the diagnosis of SjS had been made prior to the onset of cerebellar degeneration. Abbreviations: M: male; F: female; NP: not performed; ANA: antinuclear antibody; Pre: prednisolone; IVIG: intravenous immunoglobulin; HCQ: hydroxychloroquine; CTX: cyclophosphamide; R: remission; I: Improvement; S: stable; P: progression.…”

“…There were 14 patients with previously reported SjS, including Patient 1 in our study, who also developed cerebellar degeneration [5][6][7][8][9][10][11][12][13][14][15][16][17]. The characteristics of cerebellar degeneration in patients with SjS are shown in the Table. The average age of onset was 48.9 ± 19.4 years.…”