PT -期刊文章盟弥迦书约斯特盟德里克施迪AU -米歇尔•托莱达诺盟Sameer帕里克说盟Neeraj Kumar说TI -神经的Hemophagocytic Lymphohistiocytosis (S32.001) DP - 2019年4月09年TA -神经病学PG - S32.001 VI - 92 IP - 15补充4099 - //www.ez-admanager.com/content/92/15_Supp首页lement/S32.001.short 4100 - //www.ez-admanager.com/content/92/15_Supplement/S32.001.full所以Neurology2019 4月09年;92 AB -目的:描述神经参与成人hemophagocytic lymphohistiocytosis(通过)。背景:通过是一种罕见但严重的多系统疾病。通过影响神经系统和神经系统的表现很少能占主导地位。对成人的临床和影像学特点通过患者神经受累。设计/方法:使用罗切斯特的梅奥诊所,明尼苏达州数据库,患者看到通过回顾性研究进展。1996年2月至2014年12月人口统计学和临床数据进行评估。结果:99总通过患者中,14(中位年龄48年,21 - 71)被确定为神经症状归因于通过。8(57%)有神经症状疾病发作。绝大多数(64%)出现脑病但面对hemiatixia和复视。三个病人(21%)有突出的周围神经系统的参与。 Clinical course was complicated by seizures in three patients, and stroke in two (one ischemic, one hemorrhagic). In most patients, HLH was associated with either malignancy (36%), or infection (36%). Three (21.5%) had no identifiable associated systemic condition. Of the 9/14 patients who underwent lumbar puncture, the majority (89%) had elevated protein but only three (33%) had pleocytosis. Brain MRI was done in twelve patients with a wide variety of findings. Seven patients were treated with etoposide and dexamethasone, and in six, treatment was directed at the underlying condition. Only two patients were alive at the time of our review.Conclusions: In this large, single-center retrospective series, neurologic manifestations of HLH occurred in 14% of patients. Neurologic symptoms were often present at disease onset, and peripheral nerve involvement, which has only rarely been reported, occurred in 21%. Diagnosis can be delayed due to patients often having multiple medical comorbidities. In addition to standard hematologic workup, MRI and lumbar puncture are important pieces of the evaluation. Patients may improve with treatment, but overall prognosis is poor.Disclosure: Dr. Yost has nothing to disclose. Dr. Stitt has nothing to disclose. Dr. Toledano has nothing to disclose. Dr. Parikh has nothing to disclose. Dr. Kumar has nothing to disclose.

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