Neurological manifestations of primary Sjögren’s syndrome

Abstract

Primary Sjögren’s syndrome (pSS) is an autoimmune connective tissue disease affecting the exocrine glands, leading to damage of their structure and impairment of their function. In the course of pSS the internal organs may be involved and the symptoms may concern any system. Neurological disorders are one of the most common extraglandular manifestations of pSS. Available literature data estimate the prevalence of neurological symptoms as about 8.5–70% of patients diagnosed with pSS. The most common neurological complication of pSS is peripheral neuropathy, and in particular sensory polyneuropathy. Central nervous system involvement is much less common. There are also reports of various symptoms connected with damage to cranial nerves and the autonomic nervous system. A careful neurological evaluation, combined with neurophysiological tests, is recommended in patients with pSS. This review summarizes the neurological manifestations of pSS, their possible pathogenic mechanisms, diagnostic evaluation and potential treatment.

Multisystem presentation of primary Sjögren syndrome

“…Therefore, patients with a confirmed type I renal tubular acidosis should be investigated fully (Box 2). 6,7,11 typically requires treatment of the underlying condition, and in the case of potassium wasting mediated by Sjögren syndrome, corticosteroids and other immunosuppressive agents. Amiloride and potassium citrate (which we used in our patient) have also been noted to be effective.…”

“…Central findings are more controversial, with a wide range of presentations from seizures, cerebellar dysfunction, psychiatric manifestations and cognitive impairments. 10,11 There are no definitive tests that can be used to conclusively confirm central involvement with Sjögren disease. Analysis of cerebral spinal fluid has been shown to have oligoclonal banding, lymphocytosis and elevated immunoglobumin levels.…”

Syringomyelia-like syndrome in neuromyelitis optica spectrum disorder complicated with Sjogren’s syndrome: a case report

“…The wide spectrum of neurological manifestations in pSS have been reported, the prevalence of neurological symptoms of pSS range from 8.5 to 70%. However, Central nervous system (CNS) involvement is not common in PSS, approximately 2 to 25%, vary in presentation from focal, multifocal to additive or progressive, accompanying with a clinical course of fixed or cumulative deficits [ 16 , 17 ]. The occurrence of myelopathy in pSS appears to be far from exceptional.…”

Peripheral Neuropathy in Connective Tissue Diseases

“…It is characterized by sicca syndrome and other extra-glandular symptoms. Peripheral nervous involvement in Sjögren syndrome (SS) is reported with variable frequency because of diverse methods for detection of neuropathy and may precede the onset of the disease or be the initial diagnostic clue [24]. The most common feature is symmetrical distal sensory neuropathy, autonomic neuropathy and trigeminal sensory neuropathy.…”