Sjogren syndrome: neurologic complications

Veronica P Cipriani MD MS (

Dr. Cipriani of the University of Chicago Medical Center received consulting fees from Genetech as an advisory board participant

)
Francesc Graus MD PhD, editor. (Dr. Graus of the University of Barcelona has no relevant financial relationships to disclose.)
Originally released June 28, 2006; last updated June 30, 2018; expires June 30, 2021

This article includes discussion of the neurologic complications of Sjögren syndrome, Gougerot-Sjögren syndrome, and Sicca complex. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Neurologic manifestations occur in 20% to 27% of patients with Sjögren syndrome, often preceding the diagnosis of this systemic autoimmune disease. The peripheral nervous system, skeletal muscles, and central nervous system may be involved. Sjögren syndrome has been associated with neuromyelitis optica with positive serum aquaporin autoantibody. The symptoms of neurologic Sjögren syndrome may also mimic multiple sclerosis. HTLV-1 infection and vitamin B12 deficiency can complicate Sjögren myeloneuropathies. In this article, the author reviews the clinical presentations and postulated pathogenesis of these complications and offers current treatment recommendations.

Key points

 

• Sjögren syndrome is a common autoimmune disease, particularly among postmenopausal women, and it is manifested by dry mouth, dry eyes, fatigue, and arthralgias.

 

• Neurologic symptoms occur in 20% to 27% of patients with Sjögren syndrome due to involvement of cranial nerves (Bell palsy, trigeminal neuralgia, diplopia), peripheral nerves (sensorimotor neuropathies), skeletal muscles (fibromyalgia, polymyositis), and the central nervous system.

 

• Sjögren syndrome can mimic the symptoms and radiographic features of multiple sclerosis.

 

• Sjögren syndrome is closely associated with neuromyelitis optica, a demyelinating disease of the central nervous system caused by antibodies to aquaporin-4 (NMO IgG).

 

• A high index of suspicion is required given the pleomorphic manifestations and the fact that neurologic symptoms often precede the clinical diagnosis of Sjögren syndrome.

Historical note and terminology

In 1933, Henrik Sjögren described the association of keratoconjunctivitis sicca (filamentary keratitis) with arthritis (Sjögren 1933). Morgan and Castleman noted the histopathological commonality between the keratitis described by Sjögren and the glandular enlargement described by Mikulicz (Morgan and Castleman 1953; Mikulicz 1892). By 1973, the term “Sjögren syndrome” became widely accepted as these disorders were considered variants of the same process (Mason et al 1973). The name Gougerot-Sjögren syndrome is commonly used in the French literature (de Seze et al 2005) given that Henry Gougerot first reported, in Paris, the typical symptoms of xerostomia and xerophthalmia due to atrophy of salivary and lachrymal glands (Gougerot 1925).

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