Sjogren’s syndrome: neurological features

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Sjogren’s syndrome: neurological features. Neurological involvement occurs in 20% of cases. Fully referenced neurology checklist.

Demographic features

  • Neurological involvement occurs in 20% of cases
  • These precede the diagnosis of Sjogren’s syndrome in about 80-90% of cases
  • Central nervous system (CNS) involvement occurs in more than 50% of cases
  • Peripheral nerves are involved in > 60% of cases

Variant presentations

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Central features

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Dorsal root ganglionopathy (DRG)

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Cranial mononeuropathies

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Peripheral neuropathy (PN): types

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Other features

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References

  1. Delalande S, de Seze J, Fauchais AL, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore) 2004; 83:280-291.
  2. Massara A, Bonazza S, Castellino, et al. Central nervous system involvement in Sjogren's syndrome: unusual, but not unremarkable-clinical, serological characteristics and outcomes in a large cohort of Italian patients. Rheumatology 2010; 49:1540-1549.
  3. Mori K, Iijima M, Koike H, et al. The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy. Brain 2005; 128:2518-2534.
  4. Birnbaum J. Peripheral nervous system manifestations of Sjögren syndrome: clinical patterns, diagnostic paradigms, etiopathogenesis, and therapeutic strategies. Neurologist 2010; 16:287-297.
  5. Chai J, Herrmann DN, Stanton M, Barbano RL, Logigian EL. Painful small-fiber neuropathy in Sjogren syndrome. Neurology 2005; 65:925-927.
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