Sjogren syndrome: neurologic complications

Gustavo C Roman MD (Dr. Roman, Director of the Alzheimer Center of Excellence at the Methodist Neurological Institute in Houston, Texas, has no relevant financial relationships to disclose.)
Pedro J Ruiz MD (Dr. Ruiz of the California Pacific Medical Center in San Francisco, California, has no relevant financial relationships to disclose.)
Raymond P Roos MD, editor. (Dr. Roos of the University of Chicago owns stock in Amgen, Best Doctors, Express Scripts, Ionis, and Merck.)
Originally released June 28, 2006; last updated June 3, 2012; expires June 3, 2015
Notice: This article has expired and is therefore not available for CME credit.

This article includes discussion of the neurologic complications of Sjogren 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 can mimic the symptoms of multiple sclerosis, particularly neuromyelitis optica with positive serum aquaporin autoantibody. HTLV-1 infection and vitamin B12 deficiency can complicate Sjögren myeloneuropathies. In this article, the authors review the clinical presentations and postulated pathogenesis of these complications and offer 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 of multiple sclerosis, particularly neuromyelitis optica with positive serum aquaporin autoantibody.

 

• HTLV-1 infection and vitamin B12 deficiency can complicate Sjögren myeloneuropathies.

 

• 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 (Sjogren 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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