Preliminary Study of Neurocognitive Dysfunction in Adult Moyamoya Disease and Improvement after Superficial Temporal Artery-Middle Cerebral Artery Bypass

OBJECTIVE: Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease of unknown etiology. In addition, the neurocognitive impairment of adults with MMD is infrequently reported and, to date, has not been well described. We attempted to determine both the neurocognitive profile of adult moyamoya disease and whether a superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis can improve the neurocognitive impairment in exhibiting hemodynamic disturbance without stroke. METHODS: From September 2010 through November 2012, 12 patients with angiographically diagnosed MMD underwent STA-MCA anastomosis for hemodynamic impairment. Patients with hypoperfusion and impaired cerebrovascular reserve (CVR) capacity but without evidence of ischemic stroke underwent a cognitive function test, the Seoul Neuropsychological Screening Battery (SNSB). Five patients agreed to undergo a follow-up SNSB test. Data from preoperative and postoperative neurocognitive function tests were compared and analyzed. RESULTS: Five of 12 patients were enrolled. The median age was 45 years (range, 24-55 years). A comparison of preoperative to postoperative status of SNSB, memory domain, especially delayed recall showed significant improvement. Although most of the domains showed improvement after surgery, the results were not statistically significant. CONCLUSION: In our preliminary study, large proportions of adult patients with MMD demonstrate disruption of cognitive function. This suggests the possibility of chronic hypoperfusion as a primary cause of the neurocognitive impairment. When preoperative and postoperative status of cognitive function was compared, memory domain showed remarkable improvement. Although further study is needed, neurocognitive impairment may be an indication for earlier intervention with reperfusion procedures that can improve cognitive function.

Hemorrhagic Recurrence in Diffuse Astrocytoma without Malignant Transformation

Although uncommon, hemorrhage can be a complication of low grade glioma with an unfavorable prognosis such as transformation to higher grade glioma. To our knowledge, hemorrhagic recurrence of World Health Organization Grade II, diffuse astrocytoma without malignant transformation has not been reported. Thus, we report a case of diffuse astrocytoma with hemorrhagic recurrence without malignant transformation. The patient had undergone craniotomy and tumor removal 7 years previously. Annual follow-up MRIs had shown evidence of slow tumor recurrence. With the sudden onset of seizure, the patient was diagnosed as hemorrhagic recurrence and underwent second tumor removal highly suspecting malignant change into higher grade glioma. Histopathology confirmed diffuse astrocytoma without malignant changes. As the patient's postoperative condition was excellent, we plan to withhold chemotherapy and radiation therapy for use as a later treatment option.