Résumé
@#Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant, late-onset, slowly progressive cerebellar ataxia due to a pathological CAG repeat expansion in CACNA1A. Inflammation may be involved in the pathogenesis and progression of the trinucleotide repeat expansion disorder. We report a rare case of a 59-year-old woman with SCA6 who developed neuromyelitis optica spectrum disorder (NMOSD). In our case, this combination is coincidental but suggests that an inflammatory response to an unstable CAG repeat may contribute to NMOSD pathogenesis
Résumé
Previous studies have shown that patients with tandem occlusions involving extracranial internal carotid artery (ICA) and middle cerebral artery (MCA) have lower likelihood of recanalization by standard intravenous thrombolytic therapy. A 70-years-old man with a history of hypertension was admitted because of left hemiplegia and drowsiness which developed 47 minutes ago. On neurologic examination, he was drowsy and showed neglect syndrome as well as left hemiplegia. Brain CT angiography showed tandem occlusions at right extracranial ICA and proximal MCA. The administration of intravenous (IV) tissue plasminogen activator (tPA) did not improve his symptoms. We performed angioplasty and stenting for proximal ICA occlusion followed by mechanical thrombolysis for MCA occlusion. After the endovascular treatment, the MCA was recanalized and the patient recovered to show only mild left side weakness. This case shows successful treatment of hyperacute ischemic infarction from tendem occlusion of right MCA and proximal ICA with endovascular treatment following IV tPA thrombolysis.