Riaz G, Campbell W W, Carr J, Ghatak N
Department of Neurology, St Louis University Medical Center.
Arch Neurol. 1990 Apr;47(4):472-4. doi: 10.1001/archneur.1990.00530040130030.
A patient with a 2-year history of progressive ataxia and tingling in the right hand had prominent facial myokymia. Magnetic resonance imaging revealed syringomyelia and syringobulbia. After successful syringosubarachnoid shunting, the patient died of massive pulmonary embolism. Postmortem examination revealed a syrinx involving the spinal cord and lower half of the medulla; neither the facial nucleus nor facial nerve fibers were directly involved. We hypothesize that interruption of aberrant corticobulbar fibers in the medulla produced disinhibition of a rhythmic neural generator in the facial nucleus.
一名有2年进行性共济失调和右手刺痛病史的患者出现明显的面部肌束震颤。磁共振成像显示脊髓空洞症和延髓空洞症。在成功进行脊髓蛛网膜下腔分流术后，患者死于大面积肺栓塞。尸检发现脊髓空洞累及脊髓和延髓下半部；面神经核和面神经纤维均未直接受累。我们推测，延髓中异常皮质延髓纤维的中断导致面神经核中节律性神经发生器的去抑制。
