Langerhans cell histiocytosis presenting as a rapidly evolving frontotemporal syndrome

被引：6

作者：

Garg, Divyani ^{[1
]}

Pedapati, Radhakrishna ^{[1
]}

Nakra, Tripti ^{[2
]}

Singh, Rajesh Kumar ^{[1
]}

Prabhakar, Anuj ^{[3
]}

Dash, Deepa ^{[1
]}

Bhatia, Rohit ^{[1
]}

Tripathi, Manjari ^{[1
]}

机构：

[1] All India Inst Med Sci, Dept Neurol, New Delhi, India

[2] All India Inst Med Sci, Dept Neuropathol, New Delhi, India

[3] All India Inst Med Sci, Dept Neuroradiol, New Delhi, India

来源：

NEUROLOGICAL SCIENCES | 2019年 / 40卷 / 05期

关键词：

Langerhans cell histiocytosis; Hypothalamus; Cognition; Dementia;

D O I：

10.1007/s10072-019-3709-y

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Langerhans cell histiocytosis (LCH) is a rare disorder in adults which usually manifests with involvement of multiple organ systems, including the central nervous system. We describe an unusual case of biopsy-proven LCH presenting with frontotemporal-dominant cognitive impairment with hypothalamic involvement, along with multisystem disease. We propose that the dementia was probably an immune-mediated process triggered by LCH which responded dramatically to high-dose steroids.

引用

页码：1055 / 1058

页数：4

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