Upper Motor Neuron Syndrome Associated with Subclinical Sjögrens Syndrome

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概要

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• We present two patients with primary lateral sclerosis-like upper motor neuron disease accompanying subclinical Sjögrens 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 Sjögrens 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 Sjögrens syndrome should be sought in patients presenting with selective upper motor neuron involvement.

著者

• Shigeto Hiroshi

  Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University

• Osoegawa Manabu

  Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University

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