Central Nervous System Involvement in Primary Sjögren’s Syndrome: Narrative Review of MRI Findings

Módis, László; Aradi, Zsófia; Horváth, Ildikó; Bencze, János; Papp, Tamás; Emri, Miklós; Berényi, Ervin; Szántó, Antónia

doi:10.3390/diagnostics13010014

Cited by 4 publications

(1 citation statement)

References 71 publications

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“…2 A second brain MRI showed progression of the T2-hyperintensities within the brainstem now involving the cerebellar peduncles and the cerebellum, consistent with progressive neuro-Sjögren syndrome (Figure 1B). 3 She was treated with IV cyclophosphamide and rituximab, after which her hypersomnolence and ophthalmological symptoms completely resolved at two months.…”

Section: Case Reportsmentioning

confidence: 99%

A case of primary Sjögren syndrome masquerading as post-COVID Bickerstaff encephalitis

Lin,

Ling,

Tan

2024

NeuroAsia

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We present a case of acute external ophthalmoplegia, hypersomnolence, and ataxia occurring 5 days after an episode of SARS-CoV-2 upper respiratory tract infection. The patient’s serum returned positive for anti-GD1a antibodies. Guided by precedent cases, she was empirically started on a course of intravenous (IV) immunoglobulin for possible para-infectious Bickerstaff brainstem encephalitis (BBE). However, she failed to respond and subsequently developed autoimmune hemolytic anemia as well as renal tubular acidosis (type 1). Corroborative history from her family elucidated constitutional and sicca symptoms and she was eventually diagnosed with primary Sjögren syndrome (pSS) complicated by brainstem and cerebellar involvement. She was treated with IV cyclophosphamide and rituximab, after which her neurological deficits completely resolved at two months.

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Section: Case Reportsmentioning

confidence: 99%

A case of primary Sjögren syndrome masquerading as post-COVID Bickerstaff encephalitis

Lin,

Ling,

Tan

2024

NeuroAsia

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Neurological and Psychiatric Clinical Manifestations of Sjögren Syndrome

Popescu,

Hickernell,

Paulson

et al. 2024

Curr Neurol Neurosci Rep

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Neurological injury in primary Sjogren’s syndrome

Khalayli,

Bouri,

Wahbeh

et al. 2023

Annals of Medicine &Amp; Surgery

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Introduction: The incidence of neurological injury in primary Sjogren’s syndrome varies between 2.5 and 60%. The authors aimed to evaluate its prevalence and characteristics in patients with primary Sjogren’s syndrome in a sample of the Syrian population. Patients and methods: Forty-eight patients with primary Sjogren’s syndrome, attending outpatient clinics at Damascus Hospital between January 2020 and January 2022 in this cross-sectional study at the outpatient clinics, were interviewed and examined, and the necessary laboratory and radiological examinations were demanded. Information was collected on disease duration, onset time, and patterns of neurological symptoms. Results: Forty-eight patients, including 42 females, aged 56.1±10.3 years were enroled. Central nervous system involvement was found in 34 patients. 85% of patients had generalized nerve manifestations, while local nerve manifestations were found in 77,5% of patients. The common neurological manifestation was headaches, then cognitive disorders, and the most common pattern of headache was migraine. Beck Depression Index showed a significant increase in the apathy evaluation scale. The study of cognitive changes showed a significant increase in the Mini-Mental State Examination (MMSE) index. Carotid Doppler showed the presence of injury in 42.4% of patients. The magnetic resonance imaging showed positive findings in 21 patients and positive evoked potentials in 52% of patients. Discussion: Studies showing the prevalence of Sjogren’s neurological injury patterns are insufficient, but this was changed when the criteria for diagnosing Sjogren’s syndrome was modified, and the definition of neurological traits in the context of the syndrome was expanded. The presence of a high rate of headaches, cognitive changes, and fatigue confirms that generalized nervous system injuries are more common than local injuries. Migraine was the most common pattern of headache found in patients with the syndrome compared with other patterns such as tension headaches and headaches due to medications, especially analgesics. This was associated with the presence of anti-SSA antibodies and Raynaud’s phenomenon, which suggest that the headache mechanism may be due to vascular endothelial dysfunction or an immune-mediated inflammation injury of the neurovascular system. The changes that appeared on the MRI images suggested premotor cortex involvement rather than mesolimbic cortical impairment, and its presence was also associated with SSA antibody positivity, and it is caused by inflammation. Conclusion: Primary Sjogren’s syndrome should be considered as having any unspecified or specific neurological disorder.

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Central Nervous System Involvement in Primary Sjögren’s Syndrome: Narrative Review of MRI Findings

Cited by 4 publications

References 71 publications

A case of primary Sjögren syndrome masquerading as post-COVID Bickerstaff encephalitis

A case of primary Sjögren syndrome masquerading as post-COVID Bickerstaff encephalitis

Neurological and Psychiatric Clinical Manifestations of Sjögren Syndrome

Neurological injury in primary Sjogren’s syndrome

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