TY - JOUR
T1 - A case of primary Sjogren's syndrome with CNS disease mimicking chronic progressive multiple sclerosis
AU - Watanabe, Seietsu
AU - Mochizuki, Hiroshi
AU - Nakashima, Ichirou
AU - Itoyama, Yasuto
N1 - Copyright:
Copyright 2004 Elsevier Science B.V., Amsterdam. All rights reserved.
PY - 1998/7
Y1 - 1998/7
N2 - We report a 40-year-old woman with primary Sjogren's syndrome (SIS) with slowly progressive CNS disease. At age 38, she noticed spasticity and very gradual onset of monoparesis in the left leg. She hardly walked by herself at age 40. On admission, neurological examination revealed mild slurred speech, vertical nystagmus, spasticity in the four extremities, spastic monoparesis of the left leg, exaggerated jaw jerk, hyperreflexia in all limbs except right biceps and brachioradialis reflex, and positive bilateral Hoffmann reflexes and Babinski signs. Laboratory examinations disclosed positive anti- nuclear antibody (speckled type) and anti-SS-A/Ro antibody (64 x). CSF examination revealed cell count 8/mm3, protein 42 mg/dl, 4 bands of oligoclonal band and the elevation of IgG index. MR-imaging presented multiple plaque-like lesions in white matter of cerebrum and brainstem, which did not show gadolinium enhancement. Additionally she complained of dry eyes. Lacrimal and salivary secretion tests showed hyposecretion of tears and hyposialosis. The biopsied specimen of labial minor salivary gland revealed the destruction of the ducts and periductal lymphocytic infiltrations. The diagnosis of primary SjS was confirmed. We herein report a rare case of primary SjS with CNS disease mimicking chronic progressive multiple sclerosis (MS), and discuss a difficulty in differentiating CNS disease of SiS from MS.
AB - We report a 40-year-old woman with primary Sjogren's syndrome (SIS) with slowly progressive CNS disease. At age 38, she noticed spasticity and very gradual onset of monoparesis in the left leg. She hardly walked by herself at age 40. On admission, neurological examination revealed mild slurred speech, vertical nystagmus, spasticity in the four extremities, spastic monoparesis of the left leg, exaggerated jaw jerk, hyperreflexia in all limbs except right biceps and brachioradialis reflex, and positive bilateral Hoffmann reflexes and Babinski signs. Laboratory examinations disclosed positive anti- nuclear antibody (speckled type) and anti-SS-A/Ro antibody (64 x). CSF examination revealed cell count 8/mm3, protein 42 mg/dl, 4 bands of oligoclonal band and the elevation of IgG index. MR-imaging presented multiple plaque-like lesions in white matter of cerebrum and brainstem, which did not show gadolinium enhancement. Additionally she complained of dry eyes. Lacrimal and salivary secretion tests showed hyposecretion of tears and hyposialosis. The biopsied specimen of labial minor salivary gland revealed the destruction of the ducts and periductal lymphocytic infiltrations. The diagnosis of primary SjS was confirmed. We herein report a rare case of primary SjS with CNS disease mimicking chronic progressive multiple sclerosis (MS), and discuss a difficulty in differentiating CNS disease of SiS from MS.
KW - CNS disease
KW - Chronic progressive multiple sclerosis
KW - Multiple sclerosis
KW - Sjogren's syndrome
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M3 - Article
C2 - 9868312
AN - SCOPUS:0031772452
VL - 38
SP - 658
EP - 662
JO - Clinical Neurology
JF - Clinical Neurology
SN - 0009-918X
IS - 7
ER -