2017 Volume 39 Issue 4 Pages 287-291
In this article, a rare occurrence of a ruptured cerebral aneurysm complicated by glycogen storage disease (GSD) type 1a has been reported. A 34-year-old woman who suffered from GSD type 1a presented with a sudden disturbance in consciousness and left hemiparesis presented to the hospital. The computed tomography (CT) imaging of the patient revealed subarachnoid hemorrhage due to the rupture of a right middle cerebral artery saccular aneurysm. Neck clipping of the ruptured aneurysm was performed on the day of symptom onset. As an excessive brain swelling was encountered during the surgery, the external decompression was performed. During and post-surgery, intensive medical treatment was provided, especially for preventing hypoglycemia and metabolic (lactic) acidosis. The disturbance in consciousness and left hemiparesis gradually improved. Three months after the surgery, the patient was able to walk with a cane. GSD type 2 is known to manifest as cerebral arterial disorders, including aneurysms. However, to the best of our knowledge, there are no reports on concurrent GSD type 1a and cerebral aneurysms in the literature. Regarding the pathogenesis of the cerebral aneurysm in this case, the authors speculate that hyperlipidemia and hyperuricemia associated with GSD type 1a may have contributed to the formation of the aneurysm through atherosclerosis or inflammation in the arterial wall. The lactic acidosis during and after the clipping surgery may have contributed to excessive brain edema. Providing intensive care to control blood glucose levels and to avoid metabolic acidosis is thought to be important for preventing secondary cerebral damages, especially in the perioperative period.
