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Scholars Journal of Medical Case Reports | Volume-14 | Issue-05
Combined Central and Peripheral Nervous System Involvement Revealing Primary Sjögren’s Syndrome
I. Khoussar, W. Rhandour, R. Felk, N. Oubelkacem, N. Alami, Z. Khammar, R. Berrady.
Published: May 20, 2026 |
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Pages: 1105-1107
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Abstract
Background: Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease primarily characterized by lymphocytic infiltration of exocrine glands leading to sicca symptoms. However, neurological manifestations may occur and can occasionally precede the classical glandular features, posing significant diagnostic challenges. Case presentation: We report the case of a 57-year-old woman who presented with progressive headaches and lower limb paresthesia evolving over five months. Neurological examination revealed distal sensory deficits without motor impairment. Brain magnetic resonance imaging (MRI) demonstrated bilateral capsulo-thalamic lesions associated with non-specific subcortical white matter hyperintensities. Electroneuromyography revealed axonal sensory neuropathy of the lower limbs. Immunological investigations showed positive antinuclear antibodies (ANA, 1 :640) along with anti-SSA and anti-SSB antibodies. Histopathological examination of a minor salivary gland biopsy confirmed focal lymphocytic sialadenitis (grade IV). A diagnosis of primary Sjögren’s syndrome with combined central and peripheral nervous system involvement was established. The patient was treated with high-dose corticosteroids followed by cyclophosphamide, resulting in partial clinical improvement. Conclusion: This case highlights the heterogeneous neurological spectrum of primary Sjögren’s syndrome and emphasizes the importance of considering this diagnosis in patients presenting with unexplained neurological symptoms, particularly when associated with sicca features.


