Suzuki Stage VI Unilateral Moyamoya Disease Presenting With Massive Intraventricular Hemorrhage.

Moyamoya disease (MMD) is characterized by stenosis of the terminal portion of the internal carotid artery (ICA) and the development of collateral vessels. In late Suzuki stage MMD, ICA almost disappears, and the moyamoya vessels gradually regress. We report a case of late Suzuki stage unilateral MMD presenting with intraventricular hemorrhage. A 76-year-old woman who had previously been diagnosed with right ICA occlusive disease was referred to our hospital due to impaired consciousness. Radiological evaluation revealed massive intraventricular hemorrhage. After endoscopic hematoma removal, digital subtraction angiography (DSA) was performed to examine the vascular anatomy, which revealed numerous basal moyamoya vessels originating from the posterior cerebral artery. Three-dimensional rotational angiography identified a choroidal anastomosis originating from the posterior choroidal artery as the hemorrhage source. The patient had an RNF213 p.Arg4810Lys heterozygous variant in the germline. Based on the DSA findings, MMD was diagnosed, and the patient was transferred to a rehabilitation hospital with good postoperative consciousness. In conclusion, patients diagnosed with ICA occlusive disease may have late Suzuki stage MMD, potentially leading to major hemorrhage; therefore, antithrombotic medications should be administered with caution. In diagnosing ICA occlusive disease, the assessment of periventricular anastomosis should be considered, taking into account the possibility of MMD.

Changes in the Risk of Stroke in Dialysis Patients: A Retrospective Analysis over the Last 40 Years.

The stroke incidence in hemodialysis (HD) patients is high, but the associated factors remain largely unknown. This study aimed to analyze stroke incidence in HD patients and changes in risk factors. Data of 291 patients were retrospectively analyzed. The cumulative stroke incidences were 21.6% at 10 years and 31.5% at 20. Diabetic nephropathy (DN) significantly increased overall stroke (hazard ratio (HR), 2.24; 95% confidence interval (CI), 1.21-4.12; p = 0.001) and ischemic stroke (HR, 2.16; 95% CI, 1.00-4.64; p = 0.049). Patients treated with online HDF were less likely to have overall stroke (HR, 0.13; 95% CI, 0.03-0.56; p = 0.006) and ischemic stroke (HR, 0.08; 95% CI, 0.01-0.60; p = 0.014). DN (HR, 1.56; 95% CI, 1.08-2.27; p = 0.019) and age >80 years at HD initiation (20-49 years old; HR 0.13, 95% CI, 0.05-0.35, p < 0.001 and age 50-79 years; HR 0.42, 95% CI, 0.26-0.66, p < 0.001 (reference: age >80 years)) were significantly associated with stroke and/or death events. Over time, stroke risk increased in HD patients, due to the increasing number of DN. Although dialysis technology has advanced over time, these advances could not overcome other risk factors for stroke. Further increase in stroke and mortality due to aging remains a concern.

Risk Factors and Outcomes of Cerebral Stroke in End-Stage Renal Disease Patients Receiving Hemodialysis.

OBJECTIVE: Dialysis patients have a higher incidence of stroke, and outcomes are often poor. Diabetic nephropathy (DN) is a stroke risk-factor, but the importance is unclear in dialysis patients. This study investigated the stroke features and risk factors in hemodialysis (HD) patients. METHODS: All end-stage renal disease patients undergoing HD at Teraoka Memorial Hospital dialysis center were identified, with 195 recruited. Baseline clinical characteristics were collected, and the clinical outcomes and related factors of stroke in HD patients were retrospectively analyzed. The incidence rate of stroke and mortality were calculated using Kaplan-Meier survival analysis. Factors potentially related to stroke were analyzed by the log-rank test and Cox proportional hazards model for univariate and multivariate analysis. RESULTS: In total, 21.0% (41 of 195) patients developed stroke. The incidence rates of stroke per 1000 patient-years were 53.6, 65.2, and 34.0 in all HD patients, DN patients, and non-DN patients, respectively. The cumulative incidence rates of stroke in all HD patients, DN patients, and non-DN patients per 5 years, and per 10 years were 22.6%, 43.5%; 28.8%, 59.6%; and 17.6%, 31.1%, respectively. The incidence rate of stroke in the DN patients was significantly higher than in the non-DN patients (P = .013). DN was the significant risk factor for stroke by multivariate analysis (hazard ratio 2.63, 95% confidence interval 1.08-7.85; P = .032). CONCLUSIONS: This study revealed the trends of stroke in HD patients at a single institution in Japan. DN was shown to be a significant risk factor for stroke in HD patients.

Genetic Analysis of Ring Finger Protein 213 (RNF213) c.14576G>A in Intracranial Atherosclerosis of the Anterior and Posterior Circulations.

BACKGROUND: Intracranial atherosclerosis of the anterior circulation (anterior ICAS) and intracranial atherosclerosis of the posterior circulation (posterior ICAS) are thought to involve different pathogeneses and risk factors. Recently, we identified a genetic variant that has a significant association with ICAS. The variant was ring finger protein 213 (RNF213) c.14576G>A (rs112735431), which was originally identified as a susceptibility genetic variant for moyamoya disease (MMD). The present study investigated the association of RNF213 c.14576G>A with anterior and posterior ICAS. MATERIALS AND METHODS: A total of 221 study participants (43 with anterior ICAS, 61 with posterior ICAS, 12 with extracranial carotid atherosclerosis [ECAS], 5 with MMD, and 100 control subjects) were recruited from April 2015 to October 2015. A genetic analysis of RNF213 c.14576G>A and an association study with these cerebrovascular diseases were performed. RESULTS: RNF213 c.14576G>A was present in 10 of 43 patients in the anterior ICAS group and 4 of 5 patients in the MMD group, but was not present in the patients in the posterior ICAS and ECAS groups. c.14576G>A was found in 2 of 100 patients in the control group. RNF213 c.14576G>A showed a significant association with anterior ICAS (allele count: P = 3.9 × 10-5, odds ratio [OR] = 13.0, 95% confidence interval [CI] = 2.8-60.8; prevalence of carriers of c.14576G>A: P = 2.6 × 10-5, OR = 14.8, 95% CI = 3.1-71.3). However, RNF213 c.14576G>A showed no association with posterior ICAS. RNF213 c.14576G>A also had a significant association with MMD and had no association with ECAS. CONCLUSIONS: The genetic variant RNF213 c.14576G>A is significantly associated with anterior ICAS but not with posterior ICAS. The present findings may indicate factors involved in the pathogenesis of ICAS-related stroke.

Transoral penetration of a half-split chopstick between the basion and the dens.

A 6-year-old girl was admitted for transoral penetrating injury by a half-split chopstick. Subsequent CT imaging examinations showed that the chopstick had passed between the basion and the dens and reached the subarachnoid space just at the medulla oblongata. A sagittal reconstructed CT scan was useful for the evaluation of the injury. Imaging findings and their clinical relevance are discussed.

[MR imaging study of edema along the optic tract in patient with Rathke's cleft cyst].

We report an 80-year-old woman who was admitted to our hospital with symptoms due to diabetes insipidus. Magnetic resonance (MR) imaging demonstrated a sellar/suprasellar cystic lesion with marginal enhancement and the thick pituitary stalk. The MR imaging depicted edema spreading along the optic tract on fluid-attenuated inversion recovery (FLAIR) images. Upon neurological examination at the time of admission, there were no abnormal findings affecting the field of vision or visual acuity. In endocrinological examination, the basal plasma values of pituitary hormones were within normal range except for that of prolactin, which was 47.9 ng/ml. The preoperative diagnosis was craniopharyngioma, and the intrasellar mass was partially removed by the endoscopic transnasal transsphenoidal approach. Postoperative histopathological examination revealed Rathke's cleft cyst associated with squamous metaplasia. Lymphocytic infiltration was also confirmed in both the anterior and posterior pituitary lobe. The postoperative course was satisfactory. Edema spreading along the optic tract was reported as a characteristic MR imaging finding for diagnosis of craniopharyngiomas or optic nerve glioma. However, it is suggested that edema of the optic pathway seems to be caused not only by craniopharyngioma but also other suprasellar lesions. It was a rare case of secondary lymphocytic hypophysitis caused by Rathke's cleft with edema along the optic tract.