Abstract
Sjogren's syndrome is an autoimmune disorder that has a prominent involvement of exocrine glands. Systemic involvement of other organs can also happen. Peripheral nervous system involvement is common and may present as axonal sensory/sensorimotor or demyelinating polyneuropathy, mononeuritis multiplex, ganglionopathy, or cranial neuritis. It can also present with transverse myelitis, especially longitudinally extensive transverse myelitis in association with aquaporin-4 antibodies. It can also mimic central nervous system (CNS) demyelinating disorders such as multiple sclerosis. The present case series highlights some uncommon neurological presentations of Sjogren's syndrome. In this series, patients presented with Miller-Fischer syndrome, recurrent Guillain-Barre syndrome, multifocal dystonia, and demyelinating disorder of CNS. None of the cases had presenting complaints of dry mouth or eyes. Special investigations such as magnetic resonance imaging (MRI), nerve conduction studies, anti-ganglioside panel, and cerebrospinal fluid analysis, depending on the clinical presentation of the cases, were conducted. Schirmer's test, tear breakup time, antinuclear antibodies (ANA) by immunofluorescent assay, ANA blot demonstrating the presence of anti-SSA (Ro) and/or anti-SSB (La) antibodies, and lip biopsy were conducted in all cases, which confirmed the diagnosis of Sjogren's syndrome. After the diagnosis was confirmed, other tests such as C- reactive protein, serum cryoglobulin, and rheumatoid factor were conducted. Treatment was initiated with steroids, followed by long-term immunosuppression with injection rituximab. Sjogren's syndrome may present with various presentations of neurological involvement, sometimes rare, and hence a high degree of suspicion is required when other usual causes are excluded.