Neurosciences Department, Monash Medical Centre, Clayton, Australia.
Neurosciences Department, Monash Medical Centre, Clayton, Australia.
J Clin Neurosci. 2019 Nov;69:279-280. doi: 10.1016/j.jocn.2019.08.005. Epub 2019 Aug 22.
A 23-year old man was found to have a Chiari Type 1 malformation and cerebellar atrophy. While this association has previously been described, the remote cerebellar atrophy is difficult to explain. We believe the answer lies with our finding of signal hyperintensity on MR imaging at the level of the inferior olives. This suggest hypertrophic olivary degeneration, caused by trans-synaptic degeneration following disruption to the Guillain-Mollaret triangle. Propagation of this process to the cerebellar Purkinje cells occurs in some cases. We describe a case in support of this hypothesis and review previously published evidence.
一名 23 岁男性被发现存在 Chiari Ⅰ型畸形和小脑萎缩。虽然这种关联以前已经被描述过，但远端小脑萎缩很难解释。我们认为答案在于我们在磁共振成像上发现橄榄下部水平的信号高信号。这表明肥厚性橄榄变性是由于吉兰-巴雷三角中断导致的突触间变性引起的。在某些情况下，这种过程会传播到小脑浦肯野细胞。我们描述了一个支持该假说的病例，并回顾了以前发表的证据。
