Upper motor neuron syndrome associated with subclinical Sjögren's syndrome

Koichi Hagiwara, Hiroyuki Murai, Hirofumi Ochi, Manabu Osoegawa, Hiroshi Shigeto, Yasumasa Ohyagi, Jun Ichi Kira

Research output: Contribution to journalArticlepeer-review

11 Citations (Scopus)


We present two patients with primary lateral sclerosis-like upper motor neuron disease accompanying subclinical Sjögren's syndrome. Both patients showed progressive spastic quadriparesis, but neither sensory involvement nor detrusor dysfunction was noted. Lower motor neuron signs were detected only in their late follow-up period. Although sicca symptom was nearly absent, salivary labial gland biopsy revealed marked sialoadenitis in both patients. They also displayed a constellation of findings that suggested an autoimmune etiology closely related to Sjögren's syndrome, including germinal center formation in one patient, and markedly elevated levels of anti-nuclear antibody with abnormal sialography in the other. Both patients showed significant neurological improvement after the initial course of intravenous immunoglobulin therapy. We suggest that the evidence for subclinical Sjögren's syndrome should be sought in patients presenting with selective upper motor neuron involvement.

Original languageEnglish
Pages (from-to)1047-1051
Number of pages5
JournalInternal Medicine
Issue number11
Publication statusPublished - 2008

All Science Journal Classification (ASJC) codes

  • Internal Medicine


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