An unusual manifestation of Sjögren syndrome encephalitis

Iwai, Kenji; Amo, Kiyoko; Kuki, Ichiro; Fukuoka, Masataka; Kim, Kiyohiro; Yamairi, Chiharu; Togawa, Masao

doi:10.1016/j.braindev.2018.08.004

Cited by 7 publications

(3 citation statements)

References 5 publications

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“…9 There are a few reports in which Sjogren's syndrome was initially diagnosed as a case of encephalitis and the individual later developed sicca symptoms. 6,7 Our patient was significantly encephalopathic from a combination of TTP, encephalitis as evidenced on her images, and intermittent seizures. When her mental status improved, she denied complaints of sicca symptoms however she will need to be monitored closely as an outpatient in the future.…”

Section: Discussionmentioning

confidence: 72%

“…3 There are additionally a few cases describing encephalitis as the initial presentation of Sjogren's syndrome. 6,7 This case is a unique presentation of undiagnosed likely Sjogren's syndrome complicated by a prolonged immune response to COVID-19 outside of the acute infection, with acquired TTP and encephalitis as the initial diagnoses that led to both etiologies' discovery.…”

Section: Discussionmentioning

confidence: 99%

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An Immunologic Storm: A Case of Encephalitis and Thrombotic Thrombocytopenic Purpura With Underlying Likely Sjogren’s Syndrome Induced by a COVID-19 Immune Response

Kasturiarachi

Alsbrook

Crook

et al. 2022

The Neurohospitalist

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Introduction There are rare cases of Sjogren’s syndrome presenting with manifestations of encephalitis. There are also rare patients with Sjogren’s presenting with acute thrombotic thrombocytopenic purpura (TTP). There are no cases of both occurring together as the only symptoms of the syndrome. During the COVID-19 pandemic, more cases of autoimmunity are being described given its robust immune response. It is important to keep a wide differential about these varying clinical presentations. Case Presentation Our patient is a 19-year-old female with a history of menorrhagia, recent COVID-19 infection, and remote suicidal ideation. She presented with headaches, vomiting, and psychosis. Her labs found platelets of 12,000 and she was soon discovered to have TTP. She was found to have contrast enhancing lesions scattered in her left hemisphere on magnetic resonance imaging as well as seizures. Her workup was negative for infection, but labs revealed a positive antinuclear antibody, elevated anti-Ro antibody (anti-SSA) and anti-La antibody (anti-SSB), and elevated COVID-19 antibodies. She was treated with antiepileptics, pulse dose steroids for 5 days, plasmapheresis, and weekly rituximab for 4 weeks. She had significant clinical improvement. Conclusion Sjogren’s syndrome can have varying presentations including TTP with or without encephalitis as a presenting feature. Autoimmunity can also be triggered from COVID-19 infection.

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Section: Discussionmentioning

confidence: 72%

Section: Discussionmentioning

confidence: 99%

An Immunologic Storm: A Case of Encephalitis and Thrombotic Thrombocytopenic Purpura With Underlying Likely Sjogren’s Syndrome Induced by a COVID-19 Immune Response

Kasturiarachi

Alsbrook

Crook

et al. 2022

The Neurohospitalist

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“…Sjögren's syndrome is an autoimmune inflammatory disease affecting primarily lacrimal and salivary glands, which causes the pathognomonic sicca syndrome with dry eyes and dry mouth (1). However, extraglandular manifestations and especially neurologic symptoms are increasingly reported (2)(3)(4)(5). Among those, involvement of the peripheral nervous system is the most prevalent [up to 62% of symptomatic patients (6)], while cranial nerve affection has been described for up to 41% in neurologic cohorts.…”

Section: Introductionmentioning

confidence: 99%

Hearing dysfunction in patients with Neuro-Sjögren: a cross-sectional study

et al. 2020

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Background: Sjögren's syndrome is an immunologically mediated disease with salivary and lacrimal gland destruction characterised by typical sicca symptoms of dry mouth and eyes. Awareness of extraglandular neurological manifestations such as polyneuropathy and affection of cranial nerves is rising. Hearing loss as consequence of involvement of the vestibulocochlear nerve presents a severe disability. The exact prevalence and nature of hearing dysfunction in patients with Neuro-Sjögren has been insufficiently evaluated to date. Methods: Thirty patients with Sjögren's syndrome (ACR-EULAR classification criteria) and polyneuropathy were included in the study in the time period between 11/2016 and 03/2018. The median age was 59 years and 57% were females. Auditory function was investigated by pure tone audiometry, Freiburg speech comprehension audiometry, transient evoked otoacoustic emissions and brainstem evoked response audiometry.Results: Pure tone audiometry revealed hearing loss in 10/30 patients (33%) with severity ranging from mild in most patients (60%) to severe in 10%. In addition, pathological audiometric test findings showed retrocochlear auditory dysfunction in 14 further patients. In total, 24/30 patients (80%) showed pathological test results on audiometric testing suggesting hearing dysfunction. Conclusions:In conclusion, our results show that hearing dysfunction as a possible consequence of cranial neuropathy in patients with Neuro-Sjögren has been underestimated in previous studies.

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Anti-Sjögren’s-syndrome-related antigen A autoantibodies (Anti-SSA antibody) and meningoencephalitis: Sjögren's syndrome waiting to be unveiled? A case series and review of literature

Mittal

Kaur

et al. 2020

Rheumatol Int

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An unusual manifestation of Sjögren syndrome encephalitis

Cited by 7 publications

References 5 publications

An Immunologic Storm: A Case of Encephalitis and Thrombotic Thrombocytopenic Purpura With Underlying Likely Sjogren’s Syndrome Induced by a COVID-19 Immune Response

An Immunologic Storm: A Case of Encephalitis and Thrombotic Thrombocytopenic Purpura With Underlying Likely Sjogren’s Syndrome Induced by a COVID-19 Immune Response

Hearing dysfunction in patients with Neuro-Sjögren: a cross-sectional study

Anti-Sjögren’s-syndrome-related antigen A autoantibodies (Anti-SSA antibody) and meningoencephalitis: Sjögren's syndrome waiting to be unveiled? A case series and review of literature

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