Professional Education

  • Doctor of Medicine, Hokkaido University (2008)
  • Doctor of Philosophy, Hokkaido University (2017)

Stanford Advisors

Research & Scholarship

Lab Affiliations


All Publications

  • Circulating miRNome profiling in Moyamoya disease-discordant monozygotic twins and endothelial microRNA expression analysis using iPS cell line BMC MEDICAL GENOMICS Uchino, H., Ito, M., Kazumata, K., Hama, Y., Hamauchi, S., Terasaka, S., Sasaki, H., Houkin, K. 2018; 11: 72


    Moyamoya disease (MMD) is characterized by progressive stenosis of intracranial arteries in the circle of Willis with unknown etiology even after the identification of a Moyamoya susceptible gene, RNF213. Recently, differences in epigenetic regulations have been investigated by a case-control study in MMD. Here, we employed a disease discordant monozygotic twin-based study design to unmask potential confounders.Circulating genome-wide microRNA (miRNome) profiling was performed in MMD-discordant monozygotic twins, non-twin-MMD patients, and non-MMD healthy volunteers by microarray followed by qPCRvalidation, using blood samples. Differential plasma-microRNAs were further quantified in endothelial cells differentiated from iPS cell lines (iPSECs) derived from another independent non-twin cohort. Lastly, their target gene expression in the iPSECs was analyzed.Microarray detected 309 plasma-microRNAs in MMD-discordant monozygotic twins that were also detected in the non-twin cohort. Principal component analysis of the plasma-microRNA expression level demonstrated distinct 2 groups separated by MMD and healthy control in the twin- and non-twin cohorts. Of these, differential upregulations of hsa-miR-6722-3p/- 328-3p were validated in the plasma of MMD (absolute log2 expression fold change (logFC) > 0.26 for the twin cohort; absolute logFC > 0.26, p < 0.05, and q < 0.15 for the non-twin cohort). In MMD derived iPSECs, hsa-miR-6722-3p/- 328-3p showed a trend of up-regulation with a 3.0- or higher expression fold change. Bioinformatics analysis revealed that 41 target genes of miR-6722-3p/- 328-3p were significantly down-regulated in MMD derived iPSECs and were involved in STAT3, IGF-1-, and PTEN-signaling, suggesting a potential microRNA-gene expression interaction between circulating plasma and endothelial cells.Our MMD-discordant monozygotic twin-based study confirmed a novel circulating microRNA signature in MMD as a potential diagnostic biomarker minimally confounded by genetic heterogeneity. The novel circulating microRNA signature can contribute for the future functional microRNA analysis to find new diagnostic and therapeutic target of MMD.

    View details for DOI 10.1186/s12920-018-0385-3

    View details for Web of Science ID 000443622300001

    View details for PubMedID 30157848

    View details for PubMedCentralID PMC6114494

  • Novel insights into symptomatology of moyamoya disease in pediatric patients: survey of symptoms suggestive of orthostatic intolerance JOURNAL OF NEUROSURGERY-PEDIATRICS Uchino, H., Kazumata, K., Ito, M., Nakayama, N., Houkin, K. 2017; 20 (5): 485–88


    OBJECTIVE A specific population of young patients with moyamoya disease (MMD) persistently experience physical symptoms not attributable to focal ischemia. These symptoms, highly suggestive of orthostatic intolerance (also termed "orthostatic dysregulation"), were investigated and reported as potential determinants of quality of life in young MMD patients. METHODS Forty-six patients (6-30 years of age) were selected from a group of 122 patients who were diagnosed with MMD before 18 years of age. The authors administered a structured questionnaire consisting of 11 items based on screening checklists published in the Japanese clinical guidelines for juvenile orthostatic dysregulation in young patients. The results were tabulated, and correlations with clinical data were explored. RESULTS Thirty-seven (80%) patients (mean age 15.9 years) responded to the questionnaire. Frequent headache, vertigo/dizziness on standing, fatigue, difficulty with getting out of bed, and motion sickness were the top 5 symptoms, resulting in 57% of patients being unable to attend school. Forty-three percent of the patients demonstrated multiple symptoms suggestive of orthostatic intolerance, even as long as 5 years after revascularization surgery. The number of symptoms was inversely associated with the number of years after surgery (p = 0.028). The number of symptoms was not associated with a history of surgery, clinical presentations, vascular involvement, cerebral perfusion, brain lesions, or history of transient ischemic attacks. CONCLUSIONS The present study provided novel insight into the symptomatology of young patients with MMD. Failure to notice nonfocal physical symptoms can significantly impair quality of life in young patients with MMD even years after successful revascularization surgery. These symptoms may serve as independent clinical markers used to assess disease outcome, although the underlying mechanisms of this disease are, as of yet, unclear.

    View details for DOI 10.3171/2017.5.PEDS17198

    View details for Web of Science ID 000414270000014

    View details for PubMedID 28862519

  • Synergistic Interactions Between Direct and Indirect Bypasses in Combined Procedures: The Significance of Indirect Bypasses in Moyamoya Disease NEUROSURGERY Uchino, H., Kim, J., Fujima, N., Kazumata, K., Ito, M., Nakayama, N., Kuroda, S., Houkin, K. 2017; 80 (2): 201–8


    Whether additional indirect bypasses effectively contribute to revascularization in combined procedures remains unclear in patients with moyamoya disease.To evaluate the longitudinal changes associated with combined procedures while following up pediatric and adult patients long term and to assess whether any other clinical factors or hemodynamic parameters affected these changes to determine an optimal surgical strategy.We studied 58 hemispheres in 43 adults and 39 hemispheres in 26 children who underwent combined revascularization for moyamoya disease. To evaluate bypass development, we assessed the sizes of the superficial temporal artery and middle meningeal artery using magnetic resonance angiography. Multivariate analysis determined the effects of multiple variables on bypass development.Indirect bypass (middle meningeal artery) development occurred in 95% and 78% of the pediatric and adult hemispheres, respectively. Of these, dual development of direct and indirect bypasses occurred in 54% of the pediatric hemispheres and in 47% of the adult hemispheres. Reciprocal superficial temporal artery regression occurred in 28% of the hemispheres during the transition from the postoperative acute phase to the chronic phase during indirect bypass development. Good indirect bypass development was associated with adult hemispheres at Suzuki stage 4 or greater (odds ratio, 7.4; 95% confidence interval, 1.4-39.4; P = .02). Disease onset type and preoperative hemodynamic parameters were not considered predictors for the development of surgical revascularization.Simultaneous direct and indirect bypass development was most frequently observed, regardless of patient age and hemodynamic status. Applying indirect bypass as an adjunct to direct bypass could maximize revascularization in adults and children.

    View details for DOI 10.1227/NEU.0000000000001201

    View details for Web of Science ID 000404511400018

    View details for PubMedID 28175916

  • Edaravone Reduces Hyperperfusion-Related Neurological Deficits in Adult Moyamoya Disease Historical Control Study STROKE Uchino, H., Nakayama, N., Kazumata, K., Kuroda, S., Houkin, K. 2016; 47 (7): 1930–32


    Postoperative hyperperfusion-related transient neurological deficits (TNDs) are frequently observed in adult patients with moyamoya disease who undergo direct bypass procedures. The present study evaluated the effect of the free radical scavenger edaravone on postoperative hyperperfusion in adult moyamoya disease.This study included 92 hemispheres in 72 adult patients who underwent direct bypass for moyamoya disease. Serial measurements of cerebral blood flow were conducted immediately after surgery and on postoperative days 2 and 7. In 40 hemispheres for 36 patients, edaravone (60 mg/d) was administered from the day of surgery to postsurgical day 7. The incidence of postoperative hyperperfusion and associated TNDs were compared with a control group that included 52 hemispheres in 36 patients.Radiological hyperperfusion was observed in 28 of 40 (70.0%) and 39 of 52 (75.0%) hemispheres in the edaravone and control groups, respectively (P=0.30). Hyperperfusion-related TND incidences were significantly lower in the edaravone group compared with the control group (12.5% versus 32.7%; P=0.024). Multivariate analysis demonstrated that edaravone administration (P=0.009) and left-sided surgery (P=0.037) were significantly correlated with hyperperfusion-related TNDs (odds ratios, 0.3 and 4.2, respectively).Perioperative administration of edaravone reduced the incidence of hyperperfusion-related TNDs after direct bypass procedures in adult patients with moyamoya disease.

    View details for DOI 10.1161/STROKEAHA.116.013304

    View details for Web of Science ID 000379844900054

    View details for PubMedID 27174526

  • A novel application of four-dimensional magnetic resonance angiography using an arterial spin labeling technique for noninvasive diagnosis of Moyamoya disease CLINICAL NEUROLOGY AND NEUROSURGERY Uchino, H., Ito, M., Fujima, N., Kazumata, K., Yamazaki, K., Nakayama, N., Kuroda, S., Houkin, K. 2015; 137: 105-111


    Noncontrast-enhanced time-resolved four-dimensional magnetic resonance angiography using an arterial spin labeling technique (ASL-4D MRA) is emerging as a next generation angiography for the management of neurovascular diseases. This study evaluated the feasibility of ASL-4D MRA for the diagnosis of Moyamoya disease (MMD) and MMD staging by using digital subtraction angiography (DSA) and time-of-flight MRA (TOF MRA) as current standards.Eleven consecutive non-operated patients who underwent DSA for the diagnosis of MMD were recruited. Two independent observers evaluated the three tests. The data were analyzed for inter-observer and inter-modality agreements on MMD stage. Nine of 22 hemispheres underwent surgical revascularization and ASL-4D MRA was repeated postoperatively.Time-resolved inflow of blood through the cerebral vessels, including moyamoya vessels, was visualized in all the 22 non-operated hemispheres. MMD stages assessed by DSA and ASL-4D MRA were completely matched in 18 hemispheres, with a significant positive correlation between these modalities (r=0.93, P<0.001). Inter-observer agreement with ASL-4D MRA (κ=0.91±0.04, P<0.001) and inter-modality agreement between ASL-4D MRA and DSA (κ=0.93±0.04, P<0.001) were both excellent. MMD stages assessed by ASL-4D MRA have also a significant positive correlation with those assessed by TOF MRA (r=0.68, P=0.004). Repeated ASL-4D MRA clearly demonstrated the bypassed arteries and changes in the dynamic flow patterns of cerebral arteries in all the nine hemispheres after surgical revascularization. Of these, postoperative focal hyperperfusion was detected by single photon emission tomography in 7 hemispheres. In five of the seven hemispheres (71%) with postoperative hyperperfusion, ASL-4D MRA demonstrated focal hyperintense signals in the bypassed arteries, although TOF MRA did not.Noninvasive ASL-4D MRA is feasible for the diagnosis of MMD staging. This next generation angiography may be useful for monitoring disease evolution and treatment response in cerebral arteries after revascularization surgery in MMD.

    View details for DOI 10.1016/j.clineuro.2015.07.003

    View details for Web of Science ID 000361921000022

    View details for PubMedID 26185929

  • Intraoperative assessment of cortical perfusion by indocyanine green videoangiography in surgical revascularization for moyamoya disease ACTA NEUROCHIRURGICA Uchino, H., Kazumata, K., Ito, M., Nakayama, N., Kuroda, S., Houkin, K. 2014; 156 (9): 1753–60


    Postoperative hyperperfusion is a potential complication of the direct bypass procedure for moyamoya disease (MMD). However, no reliable modality is available yet for the prediction of postoperative hyperperfusion during surgery for MMD. This study aimed to investigate whether semiquantitative analysis of indocyanine green (ICG) videoangiography could contribute to the prediction of postoperative hyperperfusion in MMD.This study included 12 hemispheres from 10 patients who underwent surgical revascularization for MMD. Intraoperative ICG videoangiography was performed before and after a direct bypass procedure. The ICG intensity-time curves were semiquantitatively analyzed to evaluate cortical perfusion by calculating the blood flow index (BFI). Using single-photon emission computed tomography (SPECT), postoperative cerebral blood flow measurements were performed thrice: immediately, and 2 and 7 days after surgery.BFI significantly increased from 21.3 ± 10.5 to 38.4 ± 20.0 after bypass procedures in all the hemispheres (p < 0.01). The ratio of BFI before and after the bypass procedure was 2.4 ± 2.0, ranging from 0.5 to 8.0. Postoperative hyperperfusion was observed in nine of the 12 operated hemispheres within 7 days after surgery. Of these, three hemispheres developed hyperperfusion immediately after surgery. In the adult cases, the increase in the ratio of BFI after bypass was significantly greater in those who developed hyperperfusion immediately after surgery than in those who did not (6.5 ± 0.5 vs. 1.8 ± 2.1, p < 0.01). In contrast, no significant increase in BFI was observed in the pediatric MMD patients who experienced immediate hyperperfusion. No correlation between the changes in BFI and the occurrence of delayed hyperperfusion was observed.Our results suggest that semiquantitative analysis of BFI by intraoperative ICG videoangiography is useful in evaluating changes in cortical perfusion after bypass procedures for MMD and can predict the occurrence of early-onset hyperperfusion in MMD patients after direct bypass.

    View details for DOI 10.1007/s00701-014-2161-2

    View details for Web of Science ID 000340602400017

    View details for PubMedID 24973201

  • Semiquantitative analysis of indocyanine green videoangiography for cortical perfusion assessment in superficial temporal artery to middle cerebral artery anastomosis ACTA NEUROCHIRURGICA Uchino, H., Nakamura, T., Houkin, K., Murata, J., Saito, H., Kuroda, S. 2013; 155 (4): 599–605


    Postoperative hyperperfusion may lead to severe neurological complications after superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis. However, there are no reliable modalities to predict the occurrence of postoperative hyperperfusion during surgery. The purpose of this study is to evaluate whether a semiquantitative analysis of indocyanine green (ICG) videoangiography could be useful in predicting postoperative hyperperfusion after STA-MCA anastomosis.This study included seven patients who underwent STA-MCA anastomosis due to occlusive carotid artery diseases. During surgery, ICG videoangiography was performed before and after bypass procedures, and ICG intensity-time curves were semiquantitatively analyzed to evaluate hemodynamic changes by calculating maximum intensity, time to peak (TTP), and blood flow index (BFI).Maximum intensity significantly increased from 252.6 ± 132.5 to 351.7 ± 151.9 after bypass (p < 0.001). TTP was significantly shortened from 12.9 ± 4.4 s to 9.8 ± 3.7 s (p < 0.001). Furthermore, BFI significantly increased from 33.9 ± 28.1 to 74.6 ± 88.4 (p < 0.05). Postoperative hyperperfusion was observed in five of seven patients 1 day after surgery. The ratio of BFI before and after bypass procedures was significantly higher in patients with postoperative hyperperfusion than those without, 2.5 ± 1.1 and 1.5 ± 0.4, respectively (p = 0.013).These findings suggest that semiquantitative analysis of ICG videoangiography is helpful in predicting occurrence of hyperperfusion after STA-MCA anastomosis in patients with occlusive carotid artery diseases.

    View details for DOI 10.1007/s00701-012-1575-y

    View details for Web of Science ID 000316282900007

    View details for PubMedID 23287901

  • Intraoperative Dual Monitoring During Carotid Endarterectomy Using Motor Evoked Potentials and Near-Infrared Spectroscopy WORLD NEUROSURGERY Uchino, H., Nakamura, T., Kuroda, S., Houkin, K., Murata, J., Saito, H. 2012; 78 (6): 651–57


    Carotid endarterectomy (CEA) is a useful procedure to prevent subsequent ischemic stroke in patients with severe stenosis of internal carotid artery. However, lowering of morbidity is still essential to keep its clinical significance. This study aimed to evaluate the validity of dual monitoring using transcranial motor evoked potential (MEP) and near-infrared spectroscopy (NIRS) during CEA.Transcranial MEP and NIRS monitoring were conducted in 20 consecutive CEAs. MEP was recorded in the contralateral extremities. Regional cerebral saturation of oxygen (rSO(2)) was continuously measured in the ipsilateral forehead. The changes of MEP amplitudes and rSO(2) during cross-clamping of carotid artery were compared in each case.The amplitudes of MEP significantly decreased when rSO(2) reduced to more than 20% during carotid clamping. There was a significant correlation between the changes of MEP amplitude and rSO(2) during carotid clamping in a quadratic manner (P < .001, r = 0.821). However, NIRS could not detect critical cerebral ischemia in 1 patient with cerebral infarction in the ipsilateral frontal lobe. On the other hand, MEP could not identify it in 1 patient with severe motor deficit. No perioperative complication occurred.These findings strongly suggest that both MEP and NIRS can detect critical cerebral ischemia during CEA in most patients. Dual MEP and NIRS monitoring may further increase the sensitivity to identify it, being valuable to prevent perioperative complications due to cerebral ischemia during CEA.

    View details for DOI 10.1016/j.wneu.2011.10.039

    View details for Web of Science ID 000312950100030

    View details for PubMedID 22120560

  • Predictors and clinical features of postoperative hyperperfusion after surgical revascularization for moyamoya disease: a serial single photon emission CT/positron emission tomography study. Stroke Uchino, H., Kuroda, S., Hirata, K., Shiga, T., Houkin, K., Tamaki, N. 2012; 43 (10): 2610–16


    Clinical features and pathophysiology of postoperative hyperperfusion in moyamoya disease are still unclear. This study was aimed to clarify the incidence and time course of postoperative hyperperfusion and to determine the independent predictors of postoperative hyperperfusion in moyamoya disease.This prospective study included 41 patients who underwent surgical revascularization for moyamoya disease. Using (15)O-gas positron emission tomography, hemodynamic and metabolic parameters were quantified before surgery. Using single photon emission computed tomography, cerebral blood flow was serially measured just after surgery and on 2 and 7 days postsurgery. A multivariate logistic regression analysis was conducted to test the effect of multiple variables on postoperative hyperperfusion.Postoperative hyperperfusion was observed in 29 (50.0%) of 58 operated hemispheres. The incidence of both radiological and symptomatic hyperperfusion was significantly higher in adult patients than in pediatric ones (P=0.026 and P=0.0037, respectively). Hyperperfusion just after surgery more often led to subsequent neurological deficits (P=0.033). A multivariate analysis revealed that preoperative cerebral blood volume increase was an independent predictor of both radiological and symptomatic hyperperfusion after surgery in adult moyamoya disease (OR, 6.6 and 12.3, respectively).Postoperative hyperperfusion after surgical revascularization is not rare in moyamoya disease. Adult patients with a cerebral blood volume increase may be at high risk for radiological and symptomatic hyperperfusion after surgery. Careful perioperative management would reduce surgical complications and improve long-term outcome in moyamoya disease.

    View details for DOI 10.1161/STROKEAHA.112.654723

    View details for PubMedID 22871684

  • Persistent Primitive Olfactory Artery as Novel Collateral Channel to the Anterior Cerebral Artery in Moyamoya Disease. Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association Kamo, T., Uchino, H., Saito, H., Kashiwazaki, D., Akioka, N., Kuwayama, N., Kuroda, S. 2018


    BACKGROUND: There are a variety of collateral routes to compensate persistent cerebral ischemia in moyamoya disease. However, there is no report presenting the persistent primitive olfactory artery (POA) as a spontaneous collateral route to the anterior cerebral artery (ACA) in moyamoya disease.METHODS: We precisely examined cerebral angiography in 84 patients with moyamoya disease to identify the collateral channel through the persistent POA. Its anatomy was evaluated on pre- and postoperative angiography.RESULTS: Of 84 patients, four (4.8%) had spontaneous collateral channel through the persistent POA. All of these four hemispheres were categorized into Stage 5. In all four patients, the collateral blood flow arose from the ophthalmic artery and run to the persistent POA through the ethmoidal moyamoya. The persistent POA provided collateral blood flow from the ophthalmic artery to the ACA in all four patients. Superficial temporal artery to middle cerebral artery anastomosis and encephalo-duro-myo-arterio-pericranial synangiosis was performed in three of four patients. After surgery, the collateral channel through the persistent POA completely disappeared or markedly regressed, suggesting a significant improvement of cerebral hemodynamics in the territory of not only the MCA but also the ACA.CONCLUSION: The persistent POA can potentially provide collateral blood flow to the ACA in about 5% of patients with moyamoya disease, and should be recognized as a novel collateral channel in moyamoya disease. The persistent POA may be useful to evaluate therapeutic effects of surgical revascularization on the ACA territory.

    View details for DOI 10.1016/j.jstrokecerebrovasdis.2018.10.013

    View details for PubMedID 30409746

  • Cerebral Hyperperfusion Syndrome After Revascularization Surgery in Moyamoya Disease: Region-Symptom Mapping and Estimating a Critical Threshold WORLD NEUROSURGERY Kazumata, K., Uchino, H., Tokairin, K., Ito, M., Shiga, T., Osanai, T., Kawabori, M. 2018; 114: E388–E395


    Cerebral hyperperfusion complicates the postoperative course of patients with moyamoya disease after direct revascularization surgery. There is no clear distinction between cerebral hyperperfusion syndrome and benign postoperative increase in regional cerebral blood flow (rCBF).The present study aimed to determine clinically relevant changes in rCBF, anatomical correlations, and factors associated with transient neurologic symptoms after revascularization surgery in moyamoya disease.Whole-brain voxel-based perfusion mapping was used to identify regions involved in cerebral hyperperfusion and quantify the changes in 105 hemispheric surgeries with the use of single-photon computed tomography acquired on postoperative day 7. The changes in rCBF were quantitatively analyzed, and associations with cerebral hyperperfusion syndrome were determined.Transient neurologic symptoms appeared with rCBF increase in 37.9% of adults. Speech impairments were associated with an increase in rCBF in the operculo-insula region. Cheiro-oral syndrome was associated with the posterior insula as well as the prefrontal region. A receiver operating curve analysis yielded transient neurologic symptoms with maximum accuracy at >15.5% increase from baseline. Age and preoperative rCBF were independently associated with transient neurologic symptoms (P < 0.001).Areas showing rCBF increase during the experience of transient neurologic symptoms were spatially compatible with the known functional anatomy of the brain. An increase of approximately 15% from baseline was found to be critical, which is a far lower threshold than what has been reported previously. Increasing age was significantly associated with the occurrence of symptomatic hyperperfusion. Furthermore, patients with preserved rCBF also showed symptomatic hyperperfusion.

    View details for DOI 10.1016/j.wneu.2018.02.190

    View details for Web of Science ID 000432959600043

    View details for PubMedID 29530706

  • Factors influencing cerebral aneurysm obliteration and reliability of indocyanine green video-angiography ACTA NEUROCHIRURGICA Gekka, M., Nakayama, N., Uchino, H., Houkin, K. 2018; 160 (2): 269–76


    Indocyanine green video-angiography (ICG-V) is commonly used for intraoperative confirmation of aneurysm obliteration following clipping. However, direct puncture of the aneurysm wall occasionally results in blood leakage in patients for whom ICG-V has indicated complete closure. Therefore, the present study aimed to determine the reliability of ICG-V for confirming complete aneurysm closure, and to elucidate the factors underlying aneurysm obliteration and the occurrence of false-negative ICG-V findings.Between June 2012 and June 2016, 89 patients (107 aneurysms total) undergoing aneurysm clipping were examined using ICG-V to confirm aneurysm closure. In ICG-V-negative cases, further confirmation of complete aneurysm closure was obtained via direct puncture of the aneurysm wall, except in cases where this procedure was deemed unsafe. To elucidate the possible causes of ICG-V inaccuracies, positive, negative, and false-negative ICG-V findings were compared in terms of aneurysm location (maximum height and length), neck width (parallel and orthogonal directions to the branching vessels), wall thickness around the neck, bifurcation angle, and direction of the clipping closure line. Statistical analyses were performed using the Welsh's t test and Chi-square test.Intraoperative ICG-V detected seven cases of incomplete aneurysm closure (6.5%), defined as positive ICG-V findings. Following direct aneurysm wall puncture, nine patients (8.4%) exhibited false-negative ICG-V findings. A Chi-square test revealed that false-negative ICG-V findings were significantly influenced by the presence of heterogeneous arteriosclerosis, and wall thickening at the clipping site, which were subjectively defined by the surgeon and confirmed by an independent observer, depending on the wall color and hardness, respectively.Although ICG-V is useful for intraoperative confirmation of aneurysm obliteration, our findings further highlight the risk of false-negative ICG-V findings. Acknowledgement of risk factors is crucial for efficient detection of false-negative ICG-V findings.

    View details for DOI 10.1007/s00701-017-3379-6

    View details for Web of Science ID 000419961200008

    View details for PubMedID 29134343

  • Postoperative Intracerebral Hemorrhage After Combined Revascularization Surgery in Moyamoya Disease: Profiles and Clinical Associations. World neurosurgery Tokairin, K., Kazumata, K., Uchino, H., Ito, M., Ono, K., Tatezawa, R., Shindo, T., Kawabori, M., Nakayama, N., Houkin, K. 2018; 120: e593–e600


    In combined revascularization surgery for patients with moyamoya disease, intracerebral hemorrhage (ICH) during the postoperative acute phase is a rarely observed but severe complication. Its clinical features remain unclear because of its low incidence rate. The aim of this study was to clarify the clinical characteristics of immediate postoperative ICH.The frequency, onset timing, and hematoma location of patients who demonstrated immediate postoperative ICH were investigated in 201 consecutive surgeries performed in 134 patients. Associations between immediate postoperative ICH and demographics, clinical presentation type, perioperative blood pressure (BP), and neuroimaging data were analyzed.Postoperative ICH was observed in 6 cases (2.99%; mean age, 46.0 ± 7.6 years). The onset timing of ICH was within 24 hours after surgery in most patients (83.3%). Hematomas were located at the subcortical lesion beneath the anastomosed cortex (n = 5) and caudate head (n = 1). Three cases (50.0%) required hematoma evacuation. A higher age at surgery was associated with postoperative ICH (P = 0.046). In adult cases (132 surgeries, 65.7%), hemorrhagic presentation at onset (P = 0.0027) and an increase in BP from pre- to postoperative stage (systolic BP increase: P = 0.0058, diastolic BP increase: P = 0.0274) were significantly associated with postoperative ICH.The results suggest that older patients, with hemorrhagic presentation and greater postoperative BP increase, should be carefully managed to avoid postoperative ICH. Immediate hematoma evacuation may be effective in preventing devastating outcomes after postoperative ICH.

    View details for DOI 10.1016/j.wneu.2018.08.132

    View details for PubMedID 30165232

  • Efficacy of Carotid Endarterectomy for Mild (<50%) Symptomatic Carotid Stenosis with Unstable Plaque. World neurosurgery Kashiwazaki, D., Shiraishi, K., Yamamoto, S., Kamo, T., Uchino, H., Saito, H., Akioka, N., Kuwayama, N., Noguchi, K., Kuroda, S. 2018


    Carotid endarterectomy (CEA) is known to reduce stroke risk in patients with symptomatic, moderate to severe carotid stenosis but has no apparent impact in patients with symptomatic, mild (less than 50%) carotid stenosis. However, recent development of noninvasive imaging modalities has shown that a certain subgroup of patients are at high risk for further ischemic events despite antiplatelet therapy. This study, therefore, aimed to clarify the patients' clinical features and explore the impact of CEA for them.This prospective cohort study included 74 patients who underwent CEA for symptomatic carotid stenosis between April 2012 and December 2016. Of these, 16 (22%) had mild (less than 50%) carotid stenosis. Their demographic, radiologic, intraoperative, and pathologic findings were precisely analyzed, and their outcome after CEA was examined for 38.5 ± 13.3 months.Of these 16 patients, 12 had already been treated with antiplatelets against previous ischemic cerebrovascular or coronary artery diseases. Plaque magnetic resonance imaging revealed that all patients had vulnerable plaque, including lipid-rich plaque (n = 6) and intraplaque hemorrhage (n = 10). Intraoperative observations confirmed this. Histologic analysis revealed that inflammatory cells and fragile angiogenesis were widely found in the specimens. Only 1 patient experienced transient (less than 30 days) neurologic deficit after CEA, and none of them repeated cerebrovascular events during the follow-up period.It is not rare the patients who are at high risk for subsequent ischemic events because of vulnerable plaque despite mild (less than 50%) carotid stenosis. Magnetic resonance imaging is quite useful to noninvasively detect such vulnerable plaque. CEA is a promising procedure to treat these patients.

    View details for DOI 10.1016/j.wneu.2018.09.013

    View details for PubMedID 30244188

  • Downregulation of Apolipoprotein-E and Apolipoprotein-J in Moyamoya Disease-A Proteome Analysis of Cerebrospinal Fluid JOURNAL OF STROKE & CEREBROVASCULAR DISEASES Kashiwazaki, D., Uchino, H., Kuroda, S. 2017; 26 (12): 2981–87


    Genetic factors are closely involved in the etiology of moyamoya disease (MMD). However, its postgenomic mechanisms are still unknown. This study was aimed to identify specific biomarkers in the cerebrospinal fluid (CSF) of patients with MMD, using quantitative proteome technique.This study included 10 patients with MMD and 4 controls. The CSF was collected without blood contamination during surgery. A comparative 2-dimensional gel electrophoresis study (2D-PAGE) was performed. Protein spots that showed significant differences between moyamoya patients and controls were selected for further analysis by mass spectrometry.On 2D-PAGE, 2 proteins were significantly upregulated, and 2 other proteins were downregulated in the CSF of MMD. Further mass spectrometry analysis revealed that haptoglobin and α-1-B-glycoprotein (A1BG) were upregulated. On the other hand, apolipoprotein-E (apoE), apoE precursor, and apolipoprotein-J (apoJ) were significantly downregulated in the CSF of MMD. The observed probability-based MOWSE score was 72 for haptoglobin (P <.05), 521 for A1BG (P <.05), 62 for apoE (P <.05), 72 for apoE precursor (P <.05), and 112 for apoJ (P <.05).Although the role of A1BG in the central nervous system is still unknown, the overexpressed haptoglobin may indicate the inflammation and/or angiogenesis in MMD. The downregulation of apoE and apoJ strongly suggests a critical role of lipid metabolism in the development and progression of MMD. These proteins may be novel biomarkers in shedding light on the pathogenesis of MMD, although further studies would be warranted.

    View details for DOI 10.1016/j.jstrokecerebrovasdis.2017.07.028

    View details for Web of Science ID 000417202400042

    View details for PubMedID 28843803

  • Mapping altered brain connectivity and its clinical associations in adult moyamoya disease: A resting-state functional MRI study PLOS ONE Kazumata, K., Tha, K., Uchino, H., Ito, M., Nakayama, N., Abumiya, T. 2017; 12 (8): e0182759


    Detection of subtle ischemic injuries in moyamoya disease may enable optimization of timing of revascularization surgery, and could potentially improve functional outcomes. Resting-state functional magnetic resonance imaging (rs-fMRI) is widely used to study functional organization of the brain, but it remains unclear whether rs-fMRI could elucidate distinct characteristics in moyamoya disease. Here, we aimed to determine changes in a conventional rs-fMRI measure and analyze any associations with clinical symptoms and cerebral hemodynamics. Thirty-one adults with moyamoya disease and 25 adult controls underwent rs-fMRI, in which we measured brain connectivity via temporal correlations of low-frequency BOLD signals. We identified the extent of between-group differences with multivoxel pattern analysis. Seed-based analysis was performed to determine associations with vascular lesions, symptoms, and regional cerebral blood flow (rCBF). There was significantly altered connectivity in the precentral gyrus, operculo-insular region, precuneus, cingulate cortex, and middle frontal gyrus in moyamoya disease. There was reduced connectivity in the left insula, left precuneus, right precentral, and right middle frontal regions, which form part of the salience, default mode, motor, and central executive networks, respectively. Patients with ischemic motor-related symptoms showed significantly decreased connectivity in precentral homotopic regions compared with those without, while there were no differences in vascular lesions or rCBF. Connectivity between the right occipital and left hippocampus was significantly associated with cognitive performance and posterior cerebral artery involvement. Our results demonstrate distinct alterations in the temporal correlations of low-frequency BOLD signals, predominantly in resting-state networks in moyamoya disease. Additionally, rs-fMRI measures were associated with ischemic motor-related symptoms and cognitive performance in the patients. Thus, rs-fMRI may offer a useful non-invasive method of acquiring additional information beyond cerebral perfusion as part of clinical investigations in patients with moyamoya disease.

    View details for DOI 10.1371/journal.pone.0182759

    View details for Web of Science ID 000406944300052

    View details for PubMedID 28783763

    View details for PubMedCentralID PMC5544229

  • Topographic changes in cerebral blood flow and reduced white matter integrity in the first 2 weeks following revascularization surgery in adult moyamoya disease JOURNAL OF NEUROSURGERY Kazumata, K., Tha, K., Uchino, H., Shiga, T., Shichinohe, H., Ito, M., Nakayama, N., Abumiya, T. 2017; 127 (2): 260–69


    OBJECTIVE After revascularization surgery, hyperperfusion and ischemia are associated with morbidity and mortality in adult moyamoya disease (MMD). However, structural changes within the brain following revascularization surgery, especially in the early postsurgical period, have not been thoroughly studied. Such knowledge may enable improved monitoring and clinical management of hyperperfusion and ischemia in MMD. Thus, the objective of this study was to investigate the topographic and temporal profiles of cerebral perfusion and related white matter microstructural changes following revascularization surgery in adult MMD. METHODS The authors analyzed 20 consecutive surgeries performed in 17 adults. Diffusion imaging in parallel with serial measurements of regional cerebral blood flow (rCBF) using SPECT was performed. Both voxel-based and region-of-interest analyses were performed, comparing neuroimaging parameters of postoperative hemispheres with those of preoperative hemispheres at 4 different time points within 2 weeks after surgery. RESULTS Voxel-based analysis showed a distinct topographic pattern of cerebral perfusion, characterized by increased rCBF in the basal ganglia for the first several days and gradually increased rCBF in the lateral prefrontal cortex over 1 week (p < 0.001). Decreased rCBF was also observed in the lateral prefrontal cortex, occipital lobe, and cerebellum contralateral to the surgical hemisphere (p < 0.001). Reduced fractional anisotropy (FA) and axial diffusivity (AD), as well as increased radial diffusivity (RD), were demonstrated in both the anterior and posterior limbs of the internal capsule (p < 0.001). Diffusion parameters demonstrated the greatest changes in both FA and RD on Days 1-2 and in AD on Days 3-6; FA, RD, and AD recovered to preoperative levels on Day 14. Patients with transient neurological deteriorations (TNDs), as compared with those without, demonstrated greater increases in rCBF in both the lateral prefrontal cortex and striatum as well as smaller FAs in the posterior limb of the internal capsule (p < 0.05). CONCLUSIONS The excessively increased rCBF and the recovery process were heterogeneous across brain regions, demonstrating a distinct topographic pattern during the initial 2 weeks following revascularization surgery in MMD. Temporary impairments in the deep white matter tract and immediate postoperative ischemia were also identified. The study results characterized postoperative brain perfusion as well as the impact of revascularization surgery on the brain microstructure. Notably, rCBF and white matter changes correlated to TNDs, suggesting that these changes represent potential neuroimaging markers for tracking tissue structural changes associated with hyperperfusion during the acute postoperative period following revascularization surgery for MMD.

    View details for DOI 10.3171/2016.6.JNS16653

    View details for Web of Science ID 000406569200005

    View details for PubMedID 27588593

  • Direct Anastomosis Using Occipital Artery for Additional Revascularization in Moyamoya Disease After Combined Superficial Temporal Artery-Middle Cerebral Artery and Indirect Bypass OPERATIVE NEUROSURGERY Kazumata, K., Kamiyama, H., Saito, H., Maruichi, K., Ito, M., Uchino, H., Nakayama, N., Kuroda, S., Houkin, K. 2017; 13 (2): 213–22


    The posterior cerebral artery (PCA) is involved in approximately 30% of moyamoya disease (MMD) cases. However, there have been insufficient reports describing revascularization techniques in the posterior portion of the brain, particularly of direct anastomosis.To perform a technical assessment in patients with MMD who underwent either occipital artery (OA)-PCA bypass or OA-middle cerebral artery (MCA) bypass.A total of 428 revascularization procedures in 368 patients were retrospectively assessed by reviewing clinical charts and radiological data.Ten patients (3.5%) were treated with direct bypass after the anterior revascularization with a median interval of 30 months (range, 5 months-16 years). Seven patients were < 18 years of age (average age, 17.5 ± 15.6 years). Preoperative symptoms included transient motor deficits involving the lower extremities (n = 5), visual disturbances (n = 6), and cerebral infarctions (n = 6). A favorable outcome (modified Rankin Scale score < 3) was achieved in 9 of these 10 patients. Direct anastomosis was performed in 3 hemispheres with an OA-MCA bypass and in 8 hemispheres with an OA-PCA bypass. Patency of the direct bypass was confirmed on angiogram in 7 of 7 patients who underwent conventional angiogram performed within 1 year after the surgery. None of the 10 patients demonstrated cerebral infarctions after the posterior revascularization.In MMD, symptomatic PCA regression after anterior revascularization was found predominantly in children and young adults. Direct anastomosis in the posterior portion of the brain can be successfully achieved and is effective in preventing ischemic events.

    View details for DOI 10.1227/NEU.0000000000001346

    View details for Web of Science ID 000404126300015

    View details for PubMedID 28927214

  • Proposal for a Prospective Registry for Moyamoya Disease in Japan NEUROLOGIA MEDICO-CHIRURGICA Kazumata, K., Ito, M., Uchino, H., Nishihara, H., Houkin, K. 2017; 57 (2): 66-72


    The number of clinical research papers published worldwide on moyamoya disease (MMD) has increased recently. However, the majority of the literature comprises retrospective single-center studies collecting data on small numbers of patients. Several multi-center studies are ongoing in Japan; however, the current data are insufficient for comprehensively outlining the various characteristics of MMD. To enhance our knowledge on epidemiologic, vascular, and genetic aspects of MMD, a prospective multicenter registry will be established in Japan that will help to streamline clinical research as well as improve clinical treatments and long-term outcomes. Patients with MMD or secondary moyamoya syndrome referred to the participating centers will be invited to the registry. Demographic and physiological parameters, along with neuroimaging data will be collected chronologically. Clinical events, including neurological, medical, and surgical interventions will be recorded. Whole blood samples will be collected. Extra- and intra-cranial vascular tissue, and/or cerebrospinal fluid will also be collected from patients who undergo surgical revascularization. These biospecimens will be stored at the repositories and utilized for genome-wide association studies for identifying genetic variants, as well as tissue-specific proteomic, and/or molecular analyses. Ethics approval will be obtained at all facilities collecting biospecimens. The registry will provide descriptive statistics on functional outcomes, surgical techniques used, medications, and neurological events stratified according to patients' clinical characteristics. We expect this study to provide novel insights in the management of MMD patients and design better therapies.

    View details for DOI 10.2176/

    View details for Web of Science ID 000396088000002

    View details for PubMedID 28070115

  • Cellular Functions and Gene and Protein Expression Profiles in Endothelial Cells Derived from Moyamoya Disease-Specific iPS Cells PLOS ONE Hamauchi, S., Shichinohe, H., Uchino, H., Yamaguchi, S., Nakayama, N., Kazumata, K., Osanai, T., Abumiya, T., Houkin, K., Era, T. 2016; 11 (9): e0163561


    Moyamoya disease (MMD) is a slow, progressive steno-occlusive disease, arising in the terminal portions of the cerebral internal carotid artery. However, the functions and characteristics of the endothelial cells (ECs) in MMD are unknown. We analyzed these features using induced pluripotent stem cell (iPSC)-derived ECs.iPSC lines were established from the peripheral blood of three patients with MMD carrying the variant RNF213 R4810K, and three healthy persons used as controls. After the endothelial differentiation of iPSCs, CD31+CD144+ cells were purified as ECs using a cell sorter. We analyzed their proliferation, angiogenesis, and responses to some angiogenic factors, namely VEGF, bFGF, TGF-β, and BMP4. The ECs were also analyzed using DNA microarray and proteomics to perform comprehensive gene and protein expression analysis.Angiogenesis was significantly impaired in MMD regardless of the presence of any angiogenic factor. On the contrary, endothelial proliferation was not significant between control- and MMD-derived cells. Regarding DNA microarray, pathway analysis illustrated that extracellular matrix (ECM) receptor-related genes, including integrin β3, were significantly downregulated in MMD. Proteomic analysis revealed that cytoskeleton-related proteins were downregulated and splicing regulation-related proteins were upregulated in MMD.Downregulation of ECM receptor-related genes may be associated with impaired angiogenic activity in ECs derived from iPSCs from patients with MMD. Upregulation of splicing regulation-related proteins implied differences in splicing patterns between control and MMD ECs.

    View details for DOI 10.1371/journal.pone.0163561

    View details for Web of Science ID 000383893500046

    View details for PubMedID 27662211

    View details for PubMedCentralID PMC5035048

  • Characteristics of Diffusional Kurtosis in Chronic Ischemia of Adult Moyamoya Disease: Comparing Diffusional Kurtosis and Diffusion Tensor Imaging AMERICAN JOURNAL OF NEURORADIOLOGY Kazumata, K., Tha, K. K., Narita, H., Ito, Y. M., Shichinohe, H., Ito, M., Uchino, H., Abumiya, T. 2016; 37 (8): 1432–39


    Detecting microstructural changes due to chronic ischemia potentially enables early identification of patients at risk of cognitive impairment. In this study, diffusional kurtosis imaging and diffusion tensor imaging were used to investigate whether the former provides additional information regarding microstructural changes in the gray and white matter of adult patients with Moyamoya disease.MR imaging (diffusional kurtosis imaging and DTI) was performed in 23 adult patients with Moyamoya disease and 23 age-matched controls. Three parameters were extracted from diffusional kurtosis imaging (mean kurtosis, axial kurtosis, and radial kurtosis), and 4, from DTI (fractional anisotropy, radial diffusivity, mean diffusivity, and axial diffusivity). Voxelwise analysis for these parameters was performed in the normal-appearing brain parenchyma. The association of these parameters with neuropsychological performance was also evaluated.Voxelwise analysis revealed the greatest differences in fractional anisotropy, followed, in order, by radial diffusivity, mean diffusivity, and mean kurtosis. In patients, diffusional kurtosis imaging parameters were decreased in the dorsal deep white matter such as the corona radiata and superior longitudinal fasciculus (P < .01), including areas without DTI abnormality. Superior longitudinal fasciculus fiber-crossing areas showed weak correlations between diffusional kurtosis imaging and DTI parameters compared with tissues with a single-fiber direction (eg, the corpus callosum). Diffusional kurtosis imaging parameters were associated with general intelligence and frontal lobe performance.Although DTI revealed extensive white matter changes, diffusional kurtosis imaging additionally demonstrated microstructural changes in ischemia-prone deep white matter with abundant fiber crossings. Thus, diffusional kurtosis imaging may be a useful adjunct for detecting subtle chronic ischemic injuries.

    View details for DOI 10.3174/ajnr.A4728

    View details for Web of Science ID 000381507400009

    View details for PubMedID 27012294

  • Investigating Brain Network Characteristics Interrupted by Covert White Matter Injury in Patients with Moyamoya Disease: Insights from Graph Theoretical Analysis WORLD NEUROSURGERY Kazumata, K., Khin Khin Tha, Narita, H., Shichinohe, H., Ito, M., Uchino, H., Abumiya, T. 2016; 89: 654-+


    Chronic ischemia in adult moyamoya disease (MMD) reduces the integrity of normal-appearing white matter (WM). We investigated whether covert WM impairment alters large-scale brain networks and specific neural circuits associated with neurocognitive dysfunction in MMD. Forty-six participants (control, n = 23; MMD, n = 23) were examined using diffusion tensor imaging and streamline tractography. Structural connectivity among 90 cortical and subcortical brain regions was evaluated using the mean fractional anisotropy along the fiber tracts. Graph theoretical analysis was used to measure network parameters and inter-regional connectivity. Global network parameters were reduced in patients with MMD, including cluster coefficient (controls vs. MMD: 3.62 ± 0.24 vs. 3.26 ± 0.36; P < 0.0001), characteristic path length (controls vs. MMD: 1.20 ± 0.02 vs. 1.17 ± 0.01; P < 0.001), and small-world property (controls vs. MMD: 3.07 ± 0.18 vs. 2.83 ± 0.27; P < 0.001). Reduced pairwise connectivity was found in prefrontal neural circuits within the middle/inferior frontal gyrus; supplementary motor area; and insular, inferior temporal, and dorsal cingulate cortices. Covert WM microstructural changes in patients with MMD alter large-scale brain networks, as well as lateral prefrontal neural circuits. Evaluation of structural connectivity may be useful to assess the severity of chronic ischemic injury from a network perspective.

    View details for DOI 10.1016/j.wneu.2015.11.100

    View details for Web of Science ID 000376448700087

    View details for PubMedID 26724619

  • Familial moyamoya disease in two Turkish siblings with same polymorphism in RNF213 gene but different clinical features CHILDS NERVOUS SYSTEM Bayram, A., Yilmaz, E., Per, H., Ito, M., Uchino, H., Doganay, S., Houkin, K., Unal, E. 2016; 32 (3): 569–73


    Moyamoya disease is an uncommon, progressive, and occlusive cerebrovascular disorder, predominantly affecting the terminal segment of the internal carotid arteries and its main branches. This occlusion results at the formation of a compensatory collateral arterial network (moyamoya vessels) developing at the base of the brain. The c.14576G>A variant in ring finger protein 213 (RNF213) was recently reported as a susceptibility gene for moyamoya disease.We describe two Turkish pediatric siblings with moyamoya disease born to consanguineous, unaffected Turkish parents.The first patient (proband) is a 2-year-old boy who presented with afebrile focal seizures, moderate psychomotor retardation, paresis in the left upper and lower extremity, multiple infarctions of the brain, stenosis of the bilateral internal carotid artery and the middle cerebral artery, and stenosis of the right posterior cerebral artery. The second patient is a 10-year-old girl who is an elder sister of proband. She showed normal psychomotor development, millimetric signal enhancement without diffusion limitation of the brain, and stenosis of the bilateral internal carotid artery.We herein report pediatric sibling patients of moyamoya disease who have homozygous wild-type c.14576G>A variant in RNF213, showing different clinical course and disease severity. This is the first report of pediatric siblings with moyamoya disease from Turkey validating the genetic background of most frequent variant in East Asian patients with moyamoya disease.

    View details for DOI 10.1007/s00381-015-2871-7

    View details for Web of Science ID 000371383400025

    View details for PubMedID 26277359

  • Postoperative Acute Submandibular Sialadenitis after Neurosurgery: Two Case Reports and a Review of the Literature. NMC case report journal Uchino, H., Motegi, H., Kobayashi, H., Kamoshima, Y., Kazumata, K., Terasaka, S., Houkin, K. 2016; 3 (1): 1–4


    This article details two case reports and reviews the extant literature concerning acute postoperative submandibular sialadenitis occurring in the side contralateral to the operated site after neurosurgery. Although its precise pathogenesis remains to be elucidated, it is likely related to intraoperative compression of the submandibular gland and surrounding tissues, caused by head positioning and the endotracheal tube. Submandibular swelling rapidly deteriorates after surgery, and emergent airway protection is required in most cases in order to avoid fatal airway obstruction. To avoid serious sequelae, we should be aware of acute submandibular sialadenitis that occurs contralateral to the surgical side, which, although rare, is more probable after posterior fossa surgery. When it occurs, early airway protection is crucial, and the following conservative treatment could provide a good prognosis.

    View details for DOI 10.2176/

    View details for PubMedID 28663986

    View details for PubMedCentralID PMC5386152

  • Irregular Neointimal Lining with Prominent Proliferative Activity After Carotid Patch Angioplasty: An Autopsy Case Report WORLD NEUROSURGERY Ito, M., Niiya, Y., Uchino, H., Nakayama, N., Mabuchi, S., Houkin, K. 2014; 82 (1-2)


    Although the healing response after carotid balloon injury and carotid patch angioplasty injury has been well-documented in animal models, there is limited information about this process after carotid endarterectomy (CEA) in human patients.We describe the autopsy results of a 79-year-old man who died 18 days after CEA with patch angioplasty. The treated carotid artery had an adequate luminal diameter. Elastica-Masson staining revealed that the treated portion was covered with neointima but the patch graft was exposed to the arterial lumen at 18 days after CEA. Immunohistochemistry staining for alpha-smooth muscle actin (α-SMA), von-Willebrand factor, and vascular endothelial growth factor receptor-2 revealed that the neointima was mainly composed of α-SMA-positive cells. In addition, the α-SMA-rich neointima had many more Ki-67-positive cells than did the internal carotid artery intima in the area beyond the CEA-treated portion.This case report is the first to describe an entire carotid artery specimen in the acute stage after CEA with patch angioplasty. These findings suggest that an α-SMA-rich neointima with prominent proliferative activity covers the inner surface of the treated carotid artery, but patch grafts are left uncovered. The intrinsic arterial wall may have an important role in intimal regeneration after CEA, although the nature of the neointima and the mechanism by which it regulates proliferative activity remain unclarified.

    View details for DOI 10.1016/j.wneu.2013.06.022

    View details for Web of Science ID 000342911400149

    View details for PubMedID 23851228

  • Transient crossed cerebellar diaschisis due to cerebral hyperperfusion following surgical revascularization for moyamoya disease: case report. Neurologia medico-chirurgica Hokari, M., Kuroda, S., Simoda, Y., Uchino, H., Hirata, K., Shiga, T., Nakayama, N., Houkin, K., Tamaki, N. 2012; 52 (5): 350–53


    Crossed cerebellar diaschisis (CCD) often occurs after ischemic or hemorrhagic stroke that damages the cortico-ponto-cerebellar pathway. However, CCD due to cerebral hyperperfusion following cerebrovascular reconstruction is rare. A 61-year-old woman presented with transient CCD due to cerebral hyperperfusion following bypass surgery for adult moyamoya disease. She developed transient weakness of the right extremities and was diagnosed with moyamoya disease. First, she underwent superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis with indirect synangiosis on the left. Postoperative course was uneventful. Subsequently, she underwent STA-MCA anastomosis with indirect synangiosis on the right. She complained of mild headache on the right, and single photon emission computed tomography (SPECT) performed on the 7th postoperative day demonstrated hyperperfusion in the right frontal and temporal lobes associated with hypoperfusion in the left cerebellum. Magnetic resonance (MR) imaging demonstrated no new lesions and MR angiography showed patent STA-MCA bypass. Subsequent SPECT showed disappearance of both hyperperfusion and CCD. This case strongly suggests that cerebral hyperperfusion after bypass surgery for moyamoya disease may cause transient CCD. Although the clinical significance is still obscure, this phenomenon indicates the cortico-ponto-cerebellar pathway is interrupted due to hyperperfusion, suggesting the development of hyperperfusion syndrome. Careful observation of cerebral hemodynamics after bypass surgery is warranted to avoid hyperperfusion-related complications.

    View details for PubMedID 22688075

  • [Multiple infectious intracranial lesions of Mycobacterium genavense in an immunocompromised patient]. Brain and nerve = Shinkei kenkyu no shinpo Uchino, H., Terasaka, S., Yamaguchi, S., Kobayashi, H., Kawai, K., Kubota, K., Ooe, S., Houkin, K. 2011; 63 (1): 79–83


    Nontuberculous mycobacteria rarely infect the central nervous system. Recently, Mycobacterium genavense, nontuberculous mycobacterium (NTM), has been identified as a significant pathogen in patients. In this report, we describe multiple intracranial lesions caused by M. genavense in an immunocompromised host. A 50-year-old man presented with dysarthria, aphasia, and right hemiparesis. He had a primary immunodeficiency and a M. genavense infection in the thoracic and abdominal lymph nodes. Magnetic resonance imaging revealed multiple intracranial masses in the subcortical regions with extensive perifocal edema. Laboratory investigations and cultures of cerebrospinal fluid provided no evidence of disseminated infection. We obtained a biopsy sample via a small craniotomy using neuronavigation, and NTM infection was confirmed on analysis of the specimen. He was treated with antimycobacterial agents, and the clinical symptoms and radiological findings improved. Although a surgical procedure bears the potential risk of infection, especially in an immunocompromised patient, a brain biopsy was necessary for definitive diagnosis in this case and it aided in the administration of appropriate treatment.

    View details for PubMedID 21228452

  • [Flow diversion by double-overlapping-stent for fusiform vertebral artery aneurysm: a case report]. No shinkei geka. Neurological surgery Uchino, H., Asano, T., Nakayama, N., Kuroda, S., Houkin, K. 2011; 39 (1): 59–63


    The authors present the case of a 61-year-old male with a de novo fusiform vertebral artery aneurysm, probably due to non-traumatic dissection. He underwent flow diversion therapy, using a double overlapping technique, because the origin of the contralateral vertebral artery was stenotic. Placement of two stents resulted in marked reduction of blood flow in the aneurysm. Postoperative course was uneventful. Follow-up 3D-CT angiography revealed a patent blood flow in the stent and a gradual progression of intra-aneurysmal thrombosis. Flow diversion therapy can potentially obliterate the complicated cerebral aneurysm with the preservation of blood flow in the parent artery, and would be one of the important therapeutic options in patients with complicated aneurysms in which conventional strategies such as neck clipping, endovascular coiling and parent artery occlusion are not feasible or contraindicated.

    View details for PubMedID 21270480

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