Poser S, Lüer W, Bruhn H, Frahm J, Brück Y, Felgenhauer K
Department of Neurology and Neuropathology, Göttingen University, Germany.
Acta Neurol Scand. 1992 Dec;86(6):579-85. doi: 10.1111/j.1600-0404.1992.tb05490.x.
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.
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。
