2023年9月22日 · Hereditary spastic paraplegia (HSP) refers to a heterogeneous group of neurodegenerative conditions characterized by progressive degeneration of the corticospinal tracts and posterior column of the spinal cord.

In patients with spastic paraparesis, MRI is essential, initially to rule out usual causes of paraplegia such as compressive, inflammatory, infectious, or vascular myelopathies. Once the diagnosis of HSP is the most likely, neuroimaging may help in establishing the subtype.

In our study, we aimed to better characterize brain and spine MR imaging findings in a series of patients with HSP. MATERIALS AND METHODS: Nine patients from 4 different Lebanese families with the autosomal recessive form of HSP were included in the study. All patients underwent brain and whole-spine MR imaging.

2019年1月15日 · In patients with spastic paraparesis, MRI is essential, initially to rule out usual causes of paraplegia such as compressive, inflammatory, infectious, or vascular myelopathies. Once the diagnosis of HSP is the most likely, neuroimaging may help in establishing the subtype.

Various types of HSP have been reported, without a clear genotype–phenotype correlation, so there are many conditions that mimic HSP. We describe a patient with diagnosed and genetically confirmed hereditary spastic paraplegia type 11, with typical clinical symptoms and characteristic MRI findings, such as “ears of the lynx” sign and ...

2022年11月12日 · 遗传性痉挛性截瘫（hereditary spastic paraplegia，HSP），又称Strümpell-Lorrain病，是一组具有显著临床和遗传学异质性的神经退行性疾病。 HSP主要的临床特征表现为双下肢缓慢进行性无力和痉挛性截瘫，发病率约为1.8/100000[2-3]。 图2 遗传性痉挛性截瘫患者 图源来自于腾讯医典. 发病机制. HSP主要病理改变为皮质脊髓束及后柱最长的下行性运动纤维远端轴索的变性，伴或不伴脱髓鞘改变，主要由囊泡转运、轴索运输、细胞器形成、线粒体功能异常、 …

2019年1月16日 · Some neuroradiological signs have been reported in specific subtypes of HSP and are therefore helpful to guide genetic testing/interpretation. In addition, advanced MRI techniques enable...

2025年3月5日 · 本文对hsp的临床特点、基因分型、诊断与鉴别诊断等进行综述。 ... 近年来，越来越多的文献认识到先进的mri技术在检测als患者umn变性中的作用，特别是在高和超高磁场强度下的t2*加权成像可以在单个受试者水平上揭示umn的参与。 ...

2021年11月9日 · Our findings define the MRI spectrum of AP-4-HSP, providing opportunities for early diagnosis, identification of individuals at risk for complications, and a window into the role of the AP-4 complex in brain development and neurodegeneration.

2022年9月5日 · Hereditary spastic paraplegias (HSP) include a clinically and genetically heterogeneous group of conditions. Novel imaging modalities have been increasingly applied to HSP cohorts, which help to develop monitoring markers …