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莱尔米特-迪克洛病:一种罕见病理改变的罕见双侧小脑病变。

The Lhermitte-Duclos disease: a rare bilateral cerebellar location of a rare pathology.

作者信息

Borni Mehdi, Kammoun Brahim, Kolsi Fatma, Abdelmouleh Souhir, Boudawara Mohamed Zaher

机构信息

Department of Neurosurgery-UHC Habib Bourguiba, Sfax, Tunisia.

出版信息

Pan Afr Med J. 2019 Jun 14;33:118. doi: 10.11604/pamj.2019.33.118.16809. eCollection 2019.

Abstract

Dysplastic gangliocytoma or Lhermitte-Duclos disease is a rare disorder characterized by a slowly progressive unilateral tumour mass of the cerebellar cortex. It is probably hamartomatous, although the exact pathogenesis remains unknown. Lhermitte-Duclos disease was recently encountered to be part of a multiple hamartoma-neoplasia complex (Cowden's syndrome). It typically presents in young adults, although it has been encountered at all ages. We present the case of bilateral cerebellar location of this pathology in a 50-year-old man presented with a progressive onset and worsening of headaches accompanied by nuchal rigidity, photophobia and nausea awakening each morning. Upon physical examination, the patient was awake with a discrete right vestibular syndrome made of positive Romberg without nystagmus. Magnetic Resonance Imaging (MRI) was performed and revealed salient "tiger stripe" appearance of the bilateral cerebellar cortex relevant to a Lhermitte-Duclos disease.

摘要

发育异常性神经节细胞瘤或勒米特-迪克洛病是一种罕见的疾病,其特征为小脑皮质出现缓慢进展的单侧肿瘤块。尽管确切的发病机制尚不清楚,但它可能是错构瘤性的。勒米特-迪克洛病最近被发现是多发性错构瘤-肿瘤综合征(考登综合征)的一部分。它通常在年轻人中出现,不过在各个年龄段都有发现。我们报告一例50岁男性双侧小脑发生这种病变的病例,该患者晨起出现头痛进行性发作且加重,伴有颈部强直、畏光和恶心。体格检查时,患者清醒,有轻微的右侧前庭综合征,表现为闭目难立征阳性但无眼球震颤。进行了磁共振成像(MRI)检查,结果显示双侧小脑皮质有显著的“虎纹”外观,符合勒米特-迪克洛病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c54c/6711701/06a82b7a045f/PAMJ-33-118-g001.jpg

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