[A case of isolated CNS sarcoidosis with diffuse confluent high intensity lesions at bilateral deep white matter].

No to shinkei = Brain and nerve Pub Date : 2006-07-01
Kohji Maeda, Yasushi Kita, Satoshi Uehara, Osamu Yamasaki, Mitsue Rikimaru, Naoki Saji, Masayasu Tabuti, Masaru Furumoto
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Abstract

We reported a 60-year-old woman who suffered from isolated neurosarcodosis. She was presenting comprehensive dysfunction and intermittent high fever. In several months she gradually developed dysorientation, amnesia, dementia. However, no focal sign such as paralysis or sensory disturbance was demonstrated. Her blood chemistry showed normal including ACE (angiotensin converting enzyme) and lysozyme. Cerebrospinal fluid revealed elevated mononuclear cells, protein, and decreased glucose level. At first we treated with antibiotics including antiviral drugs in suspect of the infectious encephalomeningitis. But no improvement was observed. The elevation of ACE in spinal fluid made us suspect of neurosarcoidosis. So intravenous predonizoron (1,000 mg) was given, improving. Her high fever and mental disturbance improved. Second spinal fluid showed improvement. During the course her brain MRI revealed new bilateral diffuse confluent high intensity lesions at the deep white matters. Brain biopsy of deep matter at the right anterior lobe showed noncaseating granuloma. Since systemic work-up to detect sarcoidosis did not reveal lesions other than CNS, we considered this patient as having isolated CNS sarcoidosis.

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[孤立性中枢神经系统结节病伴双侧深部白质弥漫性融合性高强度病变1例]。
我们报告了一位60岁的女性,她患有孤立的神经结节病。她表现出全面功能障碍和间歇性高烧。几个月后,她逐渐出现了定向障碍、健忘症、痴呆。然而,没有局灶性症状,如麻痹或感觉障碍。她的血液化学正常,包括血管紧张素转换酶和溶菌酶。脑脊液显示单核细胞、蛋白升高,葡萄糖水平降低。起初我们用抗生素治疗,包括抗病毒药物,怀疑是传染性脑脑膜炎。但没有观察到任何改善。脊髓液中ACE升高使我们怀疑神经结节病。因此给予静脉注射predonizoron (1000 mg),病情有所改善。她的高烧和精神障碍有所改善。第二次脊髓液显示改善。在此过程中,她的脑部MRI显示在深部白质出现新的双侧弥漫性融合性高强度病变。右前叶深部脑组织活检显示非干酪化肉芽肿。由于检测结节病的系统检查未发现除中枢神经系统以外的病变,我们认为该患者患有孤立的中枢神经系统结节病。
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