The NORSE (new-onset refractory status epilepticus) syndrome: defining a disease entity.
The NORSE (new-onset refractory status epilepticus) syndrome: defining a disease entity.
复制标题
NORSE（新发难治性癫痫持续状态）综合征：定义疾病实体。
DOI：
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发表时间：
2005
期刊：
影响因子：
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通讯作者：
B. K. Ong
中科院分区：
文献类型：
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作者：
E. Wilder;E. Lim;Hock;V. Sharma;J. Tan;B. Chan;B. K. Ong
介绍
目的：研究一组新发难治性癫痫持续状态（NORSE综合征）的同质性患者。
材料和方法
这是一个描述性的，半前瞻性的审查，所有病例的NORSE综合征在2000年和2004年之间看到的三级保健公立医院在新加坡。进行文献综述，以识别可能的其他类似病例进行比较。
结果
7例NORSE综合征患者被确定。特征性特征为女性、年轻、既往健康状况良好、脑脊液细胞增多症（4例）、既往发热性疾病（5例）、异常延长的癫痫持续状态（平均32天）、未能进行广泛调查以揭示潜在原因、灾难性结局以及脑磁共振成像显示的颞叶和软脑膜异常。文献综述确定了12例类似患者，包括成人和儿童。
结论
根据我们的病人和文献中描述的，我们排除了NORSE综合征。需要增加对这种临床实体的认识，以帮助描述这种独特的严重疾病的潜在病因。
INTRODUCTION
To characterise a homogeneous group of patients with new-onset refractory status epilepticus (NORSE syndrome).
MATERIALS AND METHODS
This is a descriptive, semiprospective review of all cases of NORSE syndrome seen between 2000 and 2004 at a tertiary care public hospital in Singapore. A review of the literature was performed to identify possible additional similar cases for comparison.
RESULTS
Seven patients with NORSE syndrome were identified. Characterising features were female gender, young age, previous good health, cerebrospinal fluid pleocytosis (in 4), antecedent febrile illness (in 5), extraordinarily prolonged status epilepticus (average 32 days), failure of extensive investigations to reveal an underlying cause, catastrophic outcome as well as temporal lobe and leptomeningeal abnormality on brain magnetic resonance imaging. A review of the literature identified 12 similar patients, comprising both adults and children.
CONCLUSIONS
Based on our patients and those described in the literature, we characterise the NORSE syndrome. Increased recognition of this clinical entity is needed to help delineate the underlying aetiology of this unique severe illness.