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伴有脊髓受累但无半球病变的后部可逆性脑病综合征:一例报告。

Posterior reversible encephalopathy syndrome with spinal cord involvement but without hemisphere lesions: A case report.

作者信息

Liu Lu, Dai Dawei, Cao Fan, Zhang Liming, Wang Xun

机构信息

Department of Neurology, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China.

出版信息

Medicine (Baltimore). 2019 Jan;98(2):e13649. doi: 10.1097/MD.0000000000013649.

Abstract

RATIONALE

Posterior reversible encephalopathy syndrome (PRES) was termed by Hinchey in 1996. Patients have a reversible vasogenic brain edema in imaging and acute neurological symptoms such as headache, seizures, encephalopathy, and visual disturbances when suffering from hypertension, pre-eclampsia/eclampsia, renal failure, immunosuppressive medications, autoimmune disorders, sepsis, thrombocytopaenia, hypocalcaemia, alcohol withdrawal, and many other potential causes. de Havenon A et al have proposed a new syndrome named PRES with spinal cord involvement (PRES-SCI). The patients with PRES-SCI have similar symptoms these of PRES. Patients have neurologic signs with the spinal cord involved and lesions in magnetic resonance imaging (MRI) extending to the cervicomedullary junction, usually with extreme elevation in blood pressure and a history of hypertensive retinopathy. We administrated a young patient whose condition was consistent with PRES-SCI except for the hemisphere lesions.

PATIENT CONCERNS

A 20-year-old Asian male patient was admitted for a 1 week history of blurred vision and weakness of the limbs. He has had poorly controlled hypertension for 1 year before admission. In emergency room, his blood pressure could raise to 260/140mmHg. Neurological examinations and cerebral spinal fluid tests were negative. The MRI of the brain and spinal cord showed reversible lesions in the medulla and upper cervical spinal cord that extended to the lower thoracic spine.

DIAGNOSIS

Taking into account the characteristic lesions in the MRI as well as the reversible course upon treatment, he was diagnosed PRES-SCI.

INTERVENTIONS

He was treated with medicines for the hypertension.

OUTCOMES

His symptoms rapidly improved and finally the lesions on the MRI of the brain and spianl cord disappeared.

LESSONS

Clinicians should suspect PRES-SCI when patients have mild or no neurologic signs accompanied with extreme elevation in blood pressure and lesions in spinal cord. Spinal lesions alone may be a subtype of PRES-SCI.

摘要

理论依据

后可逆性脑病综合征(PRES)由欣奇于1996年命名。患者在影像学上表现为可逆性血管源性脑水肿,且在患有高血压、先兆子痫/子痫、肾衰竭、免疫抑制药物、自身免疫性疾病、败血症、血小板减少症、低钙血症、酒精戒断以及许多其他潜在病因时,会出现头痛、癫痫发作、脑病和视觉障碍等急性神经症状。德哈维农等人提出了一种名为脊髓受累的后可逆性脑病综合征(PRES-SCI)的新综合征。PRES-SCI患者具有与PRES相似的症状。患者有脊髓受累的神经体征,磁共振成像(MRI)显示病变延伸至颈髓交界处,通常血压极度升高且有高血压视网膜病变史。我们收治了一名年轻患者,其病情除半球病变外与PRES-SCI一致。

患者情况

一名20岁亚洲男性患者因视力模糊和肢体无力1周病史入院。入院前1年他的高血压控制不佳。在急诊室,他的血压可升至260/140mmHg。神经检查和脑脊液检查均为阴性。脑部和脊髓的MRI显示延髓和上颈髓有可逆性病变,病变延伸至下胸椎。

诊断

考虑到MRI中的特征性病变以及治疗后的可逆病程,他被诊断为PRES-SCI。

干预措施

他接受了治疗高血压的药物治疗。

结果

他的症状迅速改善,最终脑部和脊髓MRI上的病变消失。

经验教训

当患者有轻度或无神经体征,伴有血压极度升高和脊髓病变时,临床医生应怀疑PRES-SCI。单独的脊髓病变可能是PRES-SCI的一种亚型。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/08fc/6336622/b82781e71498/medi-98-e13649-g001.jpg

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