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 journalArticle

5 Citations (Scopus)

Abstract

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
Volume47
Issue number11
DOIs
Publication statusPublished - Nov 24 2008

Fingerprint

Motor Neurons
Motor Neuron Disease
Sialography
Sialadenitis
Quadriplegia
Passive Immunization
Germinal Center
Muscle Spasticity
Intravenous Immunoglobulins
Lip
Salivary Glands
Anti-Idiotypic Antibodies
Biopsy

All Science Journal Classification (ASJC) codes

  • Internal Medicine

Cite this

Upper motor neuron syndrome associated with subclinical Sjögren's syndrome. / Hagiwara, Koichi; Murai, Hiroyuki; Ochi, Hirofumi; Osoegawa, Manabu; Shigeto, Hiroshi; Ohyagi, Yasumasa; Kira, Jun Ichi.

In: Internal Medicine, Vol. 47, No. 11, 24.11.2008, p. 1047-1051.

Research output: Contribution to journalArticle

Hagiwara, Koichi ; Murai, Hiroyuki ; Ochi, Hirofumi ; Osoegawa, Manabu ; Shigeto, Hiroshi ; Ohyagi, Yasumasa ; Kira, Jun Ichi. / Upper motor neuron syndrome associated with subclinical Sjögren's syndrome. In: Internal Medicine. 2008 ; Vol. 47, No. 11. pp. 1047-1051.
@article{fb26b6cd050c4a95bd9938f0f90635d2,
title = "Upper motor neuron syndrome associated with subclinical Sj{\"o}gren's syndrome",
abstract = "We present two patients with primary lateral sclerosis-like upper motor neuron disease accompanying subclinical Sj{\"o}gren'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 Sj{\"o}gren'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 Sj{\"o}gren's syndrome should be sought in patients presenting with selective upper motor neuron involvement.",
author = "Koichi Hagiwara and Hiroyuki Murai and Hirofumi Ochi and Manabu Osoegawa and Hiroshi Shigeto and Yasumasa Ohyagi and Kira, {Jun Ichi}",
year = "2008",
month = "11",
day = "24",
doi = "10.2169/internalmedicine.47.0846",
language = "English",
volume = "47",
pages = "1047--1051",
journal = "Internal Medicine",
issn = "0918-2918",
publisher = "Japanese Society of Internal Medicine",
number = "11",

}

TY - JOUR

T1 - Upper motor neuron syndrome associated with subclinical Sjögren's syndrome

AU - Hagiwara, Koichi

AU - Murai, Hiroyuki

AU - Ochi, Hirofumi

AU - Osoegawa, Manabu

AU - Shigeto, Hiroshi

AU - Ohyagi, Yasumasa

AU - Kira, Jun Ichi

PY - 2008/11/24

Y1 - 2008/11/24

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=48249149938&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=48249149938&partnerID=8YFLogxK

U2 - 10.2169/internalmedicine.47.0846

DO - 10.2169/internalmedicine.47.0846

M3 - Article

C2 - 18520119

AN - SCOPUS:48249149938

VL - 47

SP - 1047

EP - 1051

JO - Internal Medicine

JF - Internal Medicine

SN - 0918-2918

IS - 11

ER -