Geschwind Michael D, Potter Christopher A, Sattavat Mamta, Garcia Paul A, Rosen Howard J, Miller Bruce L, DeArmond Stephen J
Departments of Neurology, University of California,San Francisco, UCSF Memory and Aging Center, Box 1207, SanFrancisco, CA 94143-1207, USA.
Alzheimer Dis Assoc Disord. 2009 Jan-Mar;23(1):82-87. doi: 10.1097/wad.0b013e31818323ef.
Diffusion-weighted (DWI) magnetic resonance imaging (MRI) is a highly sensitive and specific test for diagnosis of sporadic Jakob-Creutzfeldt disease (sCJD); however, the neuropathologic origin of DWI signal abnormalities including other clinical features has not been well defined. We describe a case of sCJD with brain MRI taken 15 days before death, which provided an opportunity to correlate clinical, electroencephalogram, MRI, and neuropathologic findings to better understand which sCJD-specific neuropathologic changes underlie the DWI abnormalities. Clinical findings correlated well with both electroencephalogram and MRI changes. Neuropathologic analysis showed that hyperintensities on DWI MRI correlated best with the vacuolation score (r=0.78, P=0.0005) and PrP(Sc) load (r=0.77; P=0.0006), followed by reactive astrocytic gliosis (r=0.63, P=0.008). This case provides further evidence that DWI abnormalities correlate well with the clinical features and with PrP(Sc) accumulation and vacuolation.
弥散加权磁共振成像（DWI）是诊断散发性克雅氏病（sCJD）的一项高度敏感且特异的检查；然而，包括其他临床特征在内的DWI信号异常的神经病理学起源尚未完全明确。我们描述了一例在死亡前15天进行脑部MRI检查的sCJD病例，该病例为关联临床、脑电图、MRI及神经病理学发现提供了契机，以更好地理解哪些sCJD特异性神经病理学改变是DWI异常的基础。临床发现与脑电图及MRI改变均密切相关。神经病理学分析显示，DWI MRI上的高信号与空泡化评分（r = 0.78，P = 0.0005）及PrP(Sc)负荷（r = 0.77；P = 0.0006）相关性最佳，其次是反应性星形细胞胶质增生（r = 0.63，P = 0.008）。该病例进一步证明DWI异常与临床特征以及PrP(Sc)积聚和空泡化密切相关。
