Acute motor and sensory axonal neuropathy in association with primary Sjögren's syndrome: a case report.

BACKGROUND Primary Sjogren's syndrome is a chronic, autoimmune, connective tissue disorder that results from the infiltration of exocrine glands, especially the lacrimal and salivary glands, by autoantibodies. Patients with Sjogren's syndrome commonly present with dry eyes (xerophthalmia) and dry mouth (xerostomia). However, the clinical manifestations of Sjogren's syndrome can be complicated and variable due to involvement of extraglandular organ systems, such as the nervous system. The neurological manifestations of this disorder often precede those of other exocrine gland symptoms. Hence, early diagnosis of Sjogren's syndrome remains a challenge. CASE PRESENTATION We report the case of a 63-year-old woman with primary Sjogren's syndrome who presented with acute motor and sensory axonal neuropathy (AMSAN). Treatment with glucocorticoids and immunosuppressants partially improved her muscle weakness and paresthesia. CONCLUSIONS This case demonstrates the importance of early recognition and diagnosis of AMSAN in association with primary Sjogren's syndrome to achieve a favorable clinical outcome. Primary Sjogren's syndrome may be underdiagnosed because of vague symptoms of the sicca complex. Comprehensive immunological testing to evaluate this condition may be performed in patients presenting with variants of Guillain-Barre syndrome.