Timing of prescription of angiotensin-converting enzyme inhibitors/angiotensin receptor blockers in patients hospitalized for acute heart failure with reduced/mildly reduced ejection fraction: a retrospective analysis.

Although angiotensin-converting enzyme inhibitors (ACEis) and angiotensin II receptor blockers (ARBs) play critical roles in the treatment of heart failure with reduced or mildly reduced ejection fraction (HFrEF/HFmrEF; left-ventricular ejection fraction ≤ 50%), the ideal timing for initiation in patients with acute heart failure (AHF) is unclear. We sought to clarify the timing and safety of ACEi/ARB prescription relative to hemodynamic stabilization (pre or post) in patients hospitalized with acute HFrEF/HFmrEF. This was a retrospective, observational analysis of electronic data of patients hospitalized for AHF at 17 Japanese hospitals. Among 9107 patients hospitalized with AHF, 2648 had HFrEF/HFmrEF, and 83.0% met the hemodynamic stabilization criteria within 10 days of admission. During hospitalization, 63.5% of patients with HFrEF/HFmrEF were prescribed an ACEi/ARB, 79.4% of which were prescribed pre-stabilization. In a multivariable analysis, patients treated with an ACEi/ARB pre-stabilization were more likely to have comorbid hypertension, diabetes mellitus, or ischemic heart disease. ACEi/ARB prescription timing was not associated with adverse events, including hypotension and renal impairment, and early prescription was associated with a lower incidence of subsequent worsening of HF. In clinical practice, more hospitalized patients with AHF received an ACEi/ARB before compared with after hemodynamic stabilization, and no safety concerns were observed. Moreover, early prescription may be associated with a lower incidence of worsening HF.

[A Case of Primary Adenocarcinoma of the Duodenal Bulb with Laparoscopic Distal Gastrectomy].

A 66-year-old male presented with a torose lesion at the duodenal bulb, detected via endogastroduodenoscopy(EGD) during a medical check-up. It was histopathologically diagnosed as a low-grade adenoma. He was referred to the Department of Gastroenterology for follow-up observation. An endoscopic mucosal resection(EMR)was performed due to the increasing tumor size. The pathological findings of the resected specimen showed a tubular adenoma. The patient was then followed up as an outpatient. Two months later, a follow-up EGD revealed a mass lesion, suspected to be a remnant tumor. A laparoscopic distal gastrectomy, #3, #4sb, #5, #6 dissection, and Billroth Ⅱ+Braun anastomosis reconstruction were performed. Pathological examination showed a tubular adenocarcinoma in adenoma, tub1, with depth M, and no lymph node metastasis. Non-papillary duodenal carcinoma is a rare disease that has no established guidelines for radical surgery and the extent of lymph node dissection. Pancreaticoduodenectomy is often performed in advanced cases. However, due to the increasing number of patients and the risk of complications, limited resection should be considered as an alternative management option.

A 95-gene signature stratifies recurrence risk of invasive disease in ER-positive, HER2-negative, node-negative breast cancer with intermediate 21-gene signature recurrence scores.

PURPOSE: A subset of patients with intermediate 21-gene signature assay recurrence score may benefit from adjuvant chemoendocrine therapy, but a predictive strategy is needed to identify such patients. The 95-gene signature assay was tested to stratify patients with intermediate RS into high (95GC-H) and low (95GC-L) groups that were associated with invasive recurrence risk. METHODS: Patients with ER-positive, HER2-negative, node-negative breast cancer and RS 11-25 who underwent definitive surgery and adjuvant endocrine therapy without any cytotoxic agents were included. RNA was extracted from archived formalin-fixed, paraffin-embedded samples, and 95-gene signature was calculated. RESULTS: 206 patients had RS of 11-25 (95GC-L, N = 163; 95GC-H, N = 43). In Cox proportional hazards model, 95GC-H was significantly associated with shorter time to recurrence than was 95GC-L (HR 5.94; 95%CI 1.81-19.53; P = 0.005). The correlation between 95-gene signature and 21-gene signature assay scores was not strong (correlation coefficient r = 0.27), which might suggest that 95-gene signature reflects biological characteristics differing from what 21-gene signature shows. CONCLUSIONS: The 95-gene signature stratifies patients with ER-positive, HER2-negative, node-negative invasive breast cancer and intermediate RS of 11-25 into high and low groups that are associated with recurrence risk of invasive disease. Further retrospective analysis in the prospectively accrued TAILORx population is warranted to confirm that 95-gene signature can identify patients who would benefit from adjuvant chemoendocrine therapy.

[A Case of Long-Term Survival after Multidisciplinary Treatment for Neuroendocrine Carcinoma of the Anal Canal].

A 75-year-old man presented to a local clinic with anal pain, and a palpable anal tumor on was found on digital examination of the rectum. A biopsy led to the diagnosis of neuroendocrine carcinoma. Besides the anal tumor, an right-inguinal lymph node was revealed on computed tomography(CT). Positron emission tomography-CT showed abnormal uptake in the 2 regions. He was diagnosed with lymph node metastases from anal canal carcinoma, and an abdominoperineal resection was performed. The resected specimen included the anal canal tumor with a size of 27×18 mm in diameter. On immunohistochemistry, the anal canal tumor was strongly positive for synaptophysin and positive for chromogranin A, with a Ki- 67 positivity index of 70%. After the surgery, he was administered chemotherapy with 4 courses of cisplatin and CPT-11. One year after the surgery, CT revealed lymph node recurrence. Therefore, cisplatin and CPT-11 therapy was repeated. After 11 courses of the cisplatin and CPT-11 treatment, tumor regrowth was still detected. The treatment protocol was changed to an amrubicin monotherapy regimen. However, the patient's general condition worsened after the therapies, and he died 38 months after the surgery.

[A Case of Long-Term Survival after Chemotherapy for Gastric Cancer with Peritoneal Dissemination].

The patient was a 58-year-old man who had undergone wide gastrectomy for gastric ulcer at 22 years of age. Endoscopic examination revealed an advanced type 3 gastric cancer in the anastomotic region. We performed total gastrectomy and D1 lymph node dissection because of the bleeding from the tumor, although peritoneal dissemination was found during the surgery. A post-operative pathological diagnosis of gastric cancer pT4b(SI, abdominal wall)N0M1(PER), pStage Ⅳ, was made. After the surgery, he was administered chemotherapy with S-1 and cisplatin. After 9 courses of the treatment, the treatment protocol was changed to an S-1 therapy regimen because of general fatigue. Four years and 8 months after the surgery, the tumor marker had increased, and CT scans revealed a dissemination nodule at the left back side of the bladder. Therefore, PTX plus Rmab therapy was administered as a second-line chemotherapy. Treatment with PTX plus Rmab resulted in tumor reduction, with an improvement of the QOL of the patient; partial response was maintained for 12 months. After 16 courses of the PTX plus Rmab treatment, tumor regrowth was detected. The treatment protocol was changed again to a nivolumab regimen. After 4 courses, the tumor marker was normalized, and CT scans revealed that the peritoneal dissemination had shrunk. Although the prognosis of gastric cancer with dissemination is very poor, it is possible to prolong survival with chemotherapy.

[Laparoscopic Surgical Treatment of Differentiated Intramucosal Gastric Cancer with Lymph Node Metastasis-A Case Report].

A 78‒year‒old man was admitted to our hospital with the chief complaint of 5 kg weight loss in 6 months. An esophagogastroduodenoscopy revealed a 0‒Ⅱa lesion in the posterior wall of the antrum, and biopsy findings showed a well‒differentiated adenocarcinoma. Endoscopic ultrasonography did not show an obvious invasion of the submucosal layer. Contrast‒ enhanced abdominal computed tomography(CT)revealed an enlargement of the #11p lymph node to approximately 30 mm, and positron emission tomography(PET)‒CT showed an accumulation in the same lymph node. Since no other apparent distant metastases were observed, laparoscopic distal gastrectomy and D2 dissection were performed. The postoperative pathological diagnosis was L, 7×8 mm, 0‒Ⅱa, tub1, pT1a, ly0, v0, pPM0(73 mm), pDM0(35 mm), N2, and pStage ⅡA. We report this case because the successful laparoscopic resection of a differentiated gastric mucosal cancer with lymph node metastasis has been considered to be extremely rare.

Reelin transiently promotes N-cadherin-dependent neuronal adhesion during mouse cortical development.

Reelin is an essential glycoprotein for the establishment of the highly organized six-layered structure of neurons of the mammalian neocortex. Although the role of Reelin in the control of neuronal migration has been extensively studied at the molecular level, the mechanisms underlying Reelin-dependent neuronal layer organization are not yet fully understood. In this study, we directly showed that Reelin promotes adhesion among dissociated neocortical neurons in culture. The Reelin-mediated neuronal aggregation occurs in an N-cadherin-dependent manner, both in vivo and in vitro. Unexpectedly, however, in a rotation culture of dissociated neocortical cells that gradually reaggregated over time, we found that it was the neural progenitor cells [radial glial cells (RGCs)], rather than the neurons, that tended to form clusters in the presence of Reelin. Mathematical modeling suggested that this clustering of RGCs could be recapitulated if the Reelin-dependent promotion of neuronal adhesion were to occur only transiently. Thus, we directly measured the adhesive force between neurons and N-cadherin by atomic force microscopy, and found that Reelin indeed enhanced the adhesiveness of neurons to N-cadherin; this enhanced adhesiveness began to be observed at 30 min after Reelin stimulation, but declined by 3 h. These results suggest that Reelin transiently (and not persistently) promotes N-cadherin-mediated neuronal aggregation. When N-cadherin and stabilized ß-catenin were overexpressed in the migrating neurons, the transfected neurons were abnormally distributed in the superficial region of the neocortex, suggesting that appropriate regulation of N-cadherin-mediated adhesion is important for correct positioning of the neurons during neocortical development.

[A Case of HER2-Positive Gastric Cancer with Multiple Liver Metastases Treated with Curative Conversion Therapy after Chemotherapy].

A 66-year-old man underwent chemotherapy with S-1 plus cisplatin plus trastuzumab to treat advanced gastric cancer that was diagnosed as cStage Ⅳ adenocarcinoma(T3N1M1[P0, CYX, H1]). After 8 courses, liver metastases were absent on contrast-enhanced MRI. The patient underwent a laparoscopic distal gastrectomy with D2 lymphadenectomy. The gross appearance of the surgically resected specimen showed a shrunk gastric tumor measuring 1 mm. The postoperative course was uneventful, and the patient has been well, receiving maintenance chemotherapy of S-1 plus trastuzumab without evidence of recurrence for 15 months following the operation. Conversion surgery following chemotherapy might be an effective treatment for patients with advanced gastric cancer; however, further studies are needed to establish this treatment strategy.

Effect of exercise therapy combined with branched-chain amino acid supplementation on muscle strength in elderly women after total hip arthroplasty: a randomized controlled trial.

BACKGROUND AND OBJECTIVES: Many patients develop a prolonged decrease of muscle strength after total hip arthroplasty (THA) despite their reconstructed hip joint. Physical exercise combined with branched-chain amino acid (BCAA) supplementation has been reported to improve muscle strength in elderly persons with sarcopenia. However, the effect of BCAA supplementation in patients after THA is unknown. This study examined the effects of BCAA supplementation combined with exercise therapy on the improvement of physical function in elderly patients after THA. METHODS AND STUDY DESIGN: The subjects were 31 elderly women who underwent THA. The participants were randomly assigned to two groups: BCAA (n=18) and control (n=13). The combined therapy was carried out for one month after THA. For the exercise intervention, a 3-set physical exercise program was conducted. For the nutritional intervention, the participants consumed 3.4 g of BCAA supplement or 1.2 g of starch immediately after the exercise intervention. RESULTS: BCAA supplementation combined with muscle strengthening exercises had a significant effect on knee extension strength of the contralateral side and on upper arm cross-sectional area. The improvement ratio of knee extension strength before and after intervention on the operated side was also significantly higher in the BCAA group. CONCLUSIONS: BCAA supplementation is effective for patients to improve the strength of some muscles when combined with physical exercises, but hip abductor muscle strength of the operated leg did not improve. A future study is needed to determine the efficacy of this combined therapy for hip abductor muscle strength.

[Advantages and Disadvantages of Carotid Endarterectomy and Carotid Artery Stenting:Notes from a Hybrid Neurosurgeon].

OBJECTIVE: Carotid endarterectomy(CEA)is well-established and performed for carotid artery stenosis all over the world. However, some severe complications and pitfalls have been reported. Carotid artery stenting(CAS)in high-surgical-risk patients is considered an effective alternative to CEA. We reviewed our clinical experience of CEA and CAS and discuss the advantages and disadvantages of each treatment. MATERIALS AND METHODS: For 13 years, we performed CEA for 133 patients(135 lesions)and CAS for 127 patients with carotid artery stenosis(129 lesions). CAS is indicated for the CEA high-risk patients. Perioperative imaging studies and post-operative statuses were evaluated especially in cases with complications. RESULTS: In our CEA series, ischemic stroke occurred in 3 cases, myocardial infarction in 1, cranial nerve palsy in 2, internal carotid artery occlusion in 1, wound hematoma in 2, and hyperperfusion syndrome in 4. The procedure was halted in one case because of an internal shunt problem. All cases of CAS were successfully performed except for one case complicated with myocardial infarction. Ischemic stroke occurred in 7 cases, hyperperfusion syndrome in 2, stent occlusion in 2, and puncture site hematoma in 2. Restenosis appeared in CAS significantly more than in CEA. CONCLUSIONS: CEA is a first-line treatment for carotid artery stenosis. However, complications of CEA may result in severe situations. Recognizing pitfalls and careful perioperative management is required. The results of CAS are comparable to those of CEA. Radiological evaluation for the presence of plaque and appropriate selection of an embolus protection device are important.

[A Case of Carotid Endarterectomy for the Internal Carotid Artery Stenosis Associated with Twisted Internal Carotid Artery].

Twisted carotid artery bifurcation is a variant in which the internal carotid artery(ICA)courses mediall to the external carotid artery(ECA). Here, we report a case of ICA stenosis, which is a complication of twisted ICA, treated with carotid endarterectomy(CEA). A 75-year-old woman was brought to our hospital with left-sided hemiparesis. MRI diffusion-weighted image revealed right frontal lobe infarction and high-grade stenosis at the origin of the right ICA. The ICA was located inside in relation to the ECA, manifesting as twisted ICA. We diagnosed her with cerebral infarction, caused by the ICA stenosis, and performed CEA. Afcer administering general anesthesia, the bifurcation of the right common carotid artery was exposed. ICA was located inside, and ECA was located outside. The superior thyroid artery originated from ECA and coursed in front of the ICA. After arteriotomy, internal shunting was performed. The plaque was removed en bloc and the arteriotomy was closed. Throughout the operation, the position of the arteries was retained while the procedures were performed. The postoperative course was uneventful, and the imaging study revealed that the stenosis was resolved. It is important to confirm which artery is an ICA in a case of twisted ICA during CEA. The etiology can be congenital or acquired. In conclusion, CEA was performed safely for ICA stenosis that manifested as twisted ICA with some tips.

[A Case of Embolic Infarction Associated with Accessory Middle Cerebral Artery and Treated Using Mechanical Thrombectomy].

The accessory middle cerebral artery(AMCA)is an anatomical variant of the MCA. It originates from the anterior cerebral artery, reaches the sylvian fissure, and supplies the territory of the middle cerebral artery. We report a case of embolic infarction associated with the AMCA that was treated using mechanical thrombectomy. An 82-year-old man with chronic atrial fibrillation experienced a sudden onset of left hemiparesis and was brought to our hospital. Magnetic resonance imaging showed a right temporal lobe, parietal lobe, and basal ganglion infarction, and indicated right internal carotid artery(ICA)occlusion. Mechanical thrombectomy using a Penumbra system was performed with complete recanalization. Final angiography revealed the existence of the AMCA, and the thrombus was located at the right ICA C2 portion to the main MCA. There have been twelve reported cases of ischemic stroke associated with the AMCA, including the present case. We summarized the data from the reported cases of ischemic stroke with an AMCA and evaluated their clinical characteristics and the pitfalls of endovascular treatment. These cases suggest that the AMCA may play a role in collateral flow around the main MCA occlusion, but provides insufficient collateral blood supply. Owing to the anatomical characteristics of the AMCA, we should pay attention to possible complications of endovascular treatment.

Early Diagnosis of Infective Endocarditis by Brain T2*-Weighted Magnetic Resonance Imaging.

BACKGROUND: Because infective endocarditis (IE) carries a high risk of morbidity and mortality, rapid diagnosis and effective treatment are essential to achieving a good patient outcome. However, the diagnosis of IE is often difficult in patients presenting with nonspecific clinical manifestations. An association between IE and hypointense signal spots on brain T2*-weighted magnetic resonance imaging (MRI) has been reported, but the clinical significance remains unclear.Methods and Results:To assess the clinical importance of silent lesions in the brains of IE patients, hypointense signal spots detected on their brain T2*-weighted MRI scans were investigated in a retrospective review of 44 consecutive patients with definite or suspected IE evaluated by MRI between June 2006 and January 2014. Hypointense signal spots on T2*-weighted MRI were detected in 37 (84%) patients; of these, 21 (46%) had ischemic lesions, 10 (22%) had subarachnoid hemorrhage, 4 (9%) had intraparenchymal hemorrhage, and 4 (9%) had infectious aneurysm. The hypointense signal spots on T2*-weighted images were preferentially distributed in cortical areas. CONCLUSIONS: T2*-weighted hypointense signal spots are highly frequent in patients with IE and their presence may be informative in the monitoring of IE-associated brain lesions, even those that are neurologically asymptomatic. The strong association between IE and T2*-weighted hypointense signal spots supports the need to consider additional criteria in the diagnosis of IE.

Revascularization for Aplastic or Twiglike Middle Cerebral Artery: A Case Report.

An aplastic or twiglike middle cerebral artery (Ap/T-MCA) is a congenital anomaly related to interference of normal embryonic development of the middle cerebral artery (MCA). This condition may cause ischemic and hemorrhagic strokes as a result of hypoperfusion, vulnerable collateral circulation, and associated aneurysms. We reported a case of Ap/T-MCA with transient ischemic attack, which was successfully treated with revascularization. A 19-year-old woman with a history of transient left upper limb hemiparesis, dysesthesia, and headache visited our hospital. Digital subtraction angiography showed an occlusion in the M1 segment of the MCA and abnormal vascular networks distal to the occlusion site. The patient underwent direct and indirect revascularizations, including superficial temporal artery-to-MCA bypass and encephalomyosynangiosis. She was discharged home without any neurological deficit. Magnetic resonance angiography at 12 months after revascularization showed regression of blood flow signals at the twigs network. In spite of congenital anomaly, revascularization for an Ap/T-MCA may be useful to reduce collateral stress in microvascular networks and to prevent future stroke in selected patients.

Hippocampal pyramidal neurons switch from a multipolar migration mode to a novel "climbing" migration mode during development.

The hippocampus plays important roles in brain functions. Despite the importance of hippocampal functions, recent analyses of neuronal migration have mainly been performed on the cerebral neocortex, and the cellular mechanisms responsible for the formation of the hippocampus are not yet completely understood. Moreover, why a prolonged time is required for hippocampal neurons to complete their migration has been unexplainable for several decades. We analyzed the migratory profile of neurons in the developing mouse hippocampal CA1 region and found that the hippocampal pyramidal neurons generated near the ventricle became postmitotic multipolar cells and accumulated in the multipolar cell accumulation zone (MAZ) in the late stage of development. The hippocampal neurons passed through the pyramidal layer by a unique mode of migration. Their leading processes were highly branched and made contact with many radial fibers. Time-lapse imaging revealed that the migrating cells changed their scaffolds from the original radial fibers to other radial fibers, and as a result they proceed in a zigzag manner, with long intervals. The migrating cells in the hippocampus reminded us of "rock climbers" that instead of using their hands to pull up their bodies were using their leading processes to pull up their cell bodies. Because this mode of migration had never been described, we called it the "climbing" mode. The change from the "climbing" mode in the hippocampus to the "locomotion" mode in the neocortex may have contributed to the brain expansion during evolution.

Multicolor Fluorescence Writing Based on Host-Guest Interactions and Force-Induced Fluorescence-Color Memory.

A new strategy is reported for multicolor fluorescence writing on thin solid films with mechanical forces. This concept is illustrated by the use of a green-fluorescent pentiptycene derivative 1, which forms variably colored fluorescent exciplexes: a change from yellow to red was observed with anilines, and fluorescence quenching (a change to black) occurred in the presence of benzoquinone. Mechanical forces, such as grinding and shearing, induced a crystalline-to-amorphous phase transition in both the pristine and guest-adsorbed solids that led to a change in the fluorescence color (mechanofluorochromism) and a memory of the resulting color. Fluorescence drawings of five or more colors were created on glass or paper and could be readily erased by exposure to air and dichloromethane fumes. The structural and mechanistic aspects of the observations are also discussed.

Photoinduced color change and photomechanical effect of naphthalene diimides bearing alkylamine moieties in the solid state.

Photoinduced color change of naphthalene diimides (NDIs) bearing alkylamine moieties has been observed in the solid state. The color change is attributed to the generation of a NDI radical-anion species, which may be formed through a photoinduced electron-transfer process from the alkylamine moiety to the NDI. The photosensitivity of NDIs is highly dependent on the structures of the alkylamine moieties. Crystallographic analysis, kinetic analysis, UV/Vis/NIR spectroscopic measurements, and analysis of the photoproduct suggested that a radical anion was formed through an irreversible process initiated by proton abstraction between an amine radical cation and the neutral amine moiety. The radical anions formed stacks including mixed-valence stacks and radical-anion stacks, as shown by the broad absorption bands in near-IR spectra. These photosensitive NDIs also showed crystal bending upon photoirradiation, which may be associated with a change in the intermolecular distance of the NDI stacks by the formation of monomeric radical anions, mixed-valence stacks, and radical-anion stacks.

[Timing of helicopter transportation for patients presenting with subarachnoid hemorrhage on isolated islands].

Cerebral aneurysm re-rupture following subarachnoid hemorrhage(SAH)is a serious problem that is related with poor outcome. It is generally said that re-rupture occurs within 6 hours of the initial SAH;in the acute stage, strict management is needed even in the period before hospitalization. The aim of this study was to confirm whether patients on isolated islands should be transferred by helicopter > 6 hours after the initial SAH. Here we reviewed 125 cases of SAH in the isolated islands of Nagasaki prefecture between January 2007 and December 2012 who were transferred to Nagasaki Medical Center by helicopter as a result of consultation via TeleStroke(41 men, 84 women;mean age, 65.76 years). Re-rupture was observed in seven patients(5.6%), five of whom were diagnosed with re-rupture in a prior hospital on the isolated island. No patients demonstrated clinical deterioration during transport. Early helicopter transportation under adequate sedation and control of blood pressure within 6 hours is safe, and patients should be transferred as quickly as possible during the day. On the other hand, at night, flight safety must first be considered. Patients in stable clinical condition may be transferred the next day. We should pay special attention to patients with SAH and intracerebral hemorrhage, severe SAH, or vertebral artery dissecting aneurysm because their condition may gradually become more serious even if initially stable.

Early awareness of cerebrospinal fluid hypovolemia after craniotomy for microsurgical aneurysmal clipping.

BACKGROUND: Mild cerebrospinal fluid (CSF) hypovolemia is a well-known clinical entity, but critical CSF hypovolemia that can cause transtentorial herniation is an unusual and rare clinical entity that occurs after craniotomy. We investigated CSF hypovolemia after microsurgical aneurysmal clipping for subarachnoid hemorrhage (SAH). METHOD: This study included 144 consecutive patients with SAH. Lumbar drainage (LD) was inserted after general anesthesia or postoperatively as a standard perioperative protocol. CSF hypovolemia diagnosis was based on three criteria. RESULTS: Eleven patients (7.6%) were diagnosed with CSF hypovolemia according to diagnostic criteria in a postoperative range of 0-8 days. In all patients, signs or symptoms of CSF hypovolemia improved within 24 hours by clamping LD and using the Trendelenburg position. CONCLUSIONS: As a cause of acute clinical deterioration after aneurysmal clipping, CSF hypovolemia is likely under-recognized, and may actually be misdiagnosed as vasospasm or brain swelling. We should always take the etiology of CSF hypovolemia into consideration, and especially pay attention in patients with pneumocephalus and subdural fluid collection alongside brain sag on computed tomography. These patients are at higher risk developing of pressure gradients between their cranial and spinal compartments, and therefore, brain sagging after LD, than after ventricular drainage. We should be vigilant to strictly manage LD so as not to produce high pressure gradients.

[A rare case of developmental venous anomaly with diffuse arteriovenous shunt in the posterior fossa presented as massive cerebellar hemorrhage].

Developmental venous anomalies(DVAs), that have been previously called venous malformation, are extreme variations of normal transmedullary veins that are necessary for the drainage of white and gray matter. They are becoming the most commonly encountered intracranial vascular lesions in central nervous system imaging. Most DVAs are asymptomatic or uncomplicated, and surgery is no longer considered necessary. The author describes a rare case of an 8-year-old patient presenting with massive cerebellar hemorrhage due to DVA with diffuse arteriovenous(A-V)shunt. Cerebral angiography demonstrated diffuse A-V shunt from the basilar artery runs into the bilateral basal vein of Rosenthal through the enlarged transpontine vein. Caput medusae-like appearance was visualized, although the finding of nidus was not obvious. Moreover, three-dimensional computed tomography angiography demonstrated stenosis in part of the drainage routes. Then, we supposed that this lesion was a transitional form between a DVA and an arteriovenous malformation, and massive cerebellar hemorrhage might be caused by secondary venous hypertension due to venous stenosis. A careful follow-up should be made, because the prognosis of DVA with A-V shunt has not been fully elucidated.