A Presentation of Pediatric Sjogren's Syndrome with Abducens Nerve Palsy

Sjogren's syndrome is a systemic autoimmune disease that classically presents with xerophthalmia and xerostomia. However, neurological manifestations occur in 10 to 60% of patients with Sjogren's syndrome and can often precede classic sicca symptoms in Sjogren's syndrome in some cases up to several years. Rarely, cranial neuropathy can be the initial presentation. Here, we present the first case of a 15-year-old girl with left abducens palsy in the setting of a new diagnosis of Sjogren's syndrome. Comprehensive evaluation revealed elevated Sjogren's syndrome-related antigen A-60 antibody. Cerebrospinal fluid analysis was unremarkable. Radiological studies demonstrated evidence of chronic parotitis. Acute treatment included high-dose methylprednisolone and rituximab, and symptoms resolved by follow-up at 2 weeks. The most common neurological disorder of Sjogren's syndrome is pure sensory neuropathy. In pediatric Sjogren's syndrome, neurological complications are rare but include aseptic meningoencephalitis, acute disseminated encephalomyelitis, transverse myelitis, optic neuritis, and cranial neuropathies. In the circumstance of a cranial neuropathy, the trigeminal nerve is most commonly involved but oculomotor nerves can occasionally be affected. Abducens palsies have been described in four patients with Sjogren's syndrome, typically women and all middle aged or older, with our patient being the first pediatric case. Thus, it is important to consider screening for Sjogren's syndrome in the evaluation of pediatric patients with new onset of isolated cranial neuropathy even in the absence of classic sicca symptoms.