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急性脱髓鞘疾病。分类与非侵入性诊断。

Acute demyelinating disease. Classification and non-invasive diagnosis.

作者信息

Poser S, Lüer W, Bruhn H, Frahm J, Brück Y, Felgenhauer K

机构信息

Department of Neurology and Neuropathology, Göttingen University, Germany.

出版信息

Acta Neurol Scand. 1992 Dec;86(6):579-85. doi: 10.1111/j.1600-0404.1992.tb05490.x.

Abstract

Five young patients are described with biopsy-proven acute demyelinating disease. Two cases are classified as Schilder's disease, a particular childhood form of multiple sclerosis (MS) with atypical clinical manifestation, normal or atypical CSF-findings and large bilateral lesions in magnetic resonance imaging (MRI). Two further cases presented with a fulminant bout of MS with typical clinical picture and CSF-findings; they are classified as Marburg's disease. The last case was an acute second bout of classical MS. The biopsy seemed to be justified in 4 cases, but unnecessary in the last case. Localized proton magnetic resonance spectroscopy (MRS) performed in one case allowed us to examine the focal cerebral abnormalities directly and non-invasively. The spectra revealed a pattern typical of acute demyelination suggesting potential for a replacement of biopsy in the future.

摘要

描述了5例经活检证实的急性脱髓鞘疾病患者。2例被归类为席尔德病,这是一种特殊的儿童型多发性硬化症(MS),临床表现不典型,脑脊液检查结果正常或不典型,磁共振成像(MRI)显示双侧有大片病灶。另外2例表现为暴发性MS,具有典型的临床表现和脑脊液检查结果,被归类为马尔堡病。最后1例是经典MS的急性复发。活检在4例中似乎是合理的,但在最后1例中没有必要。对1例患者进行了局部质子磁共振波谱(MRS)检查,使我们能够直接且无创地检查局灶性脑异常。波谱显示出急性脱髓鞘的典型模式,提示未来有可能替代活检。

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