Pulmonary hypertension with bronchopulmonary dysplasia: Aichi cohort study.

BACKGROUND: The incidence of pulmonary hypertension (PH) associated with bronchopulmonary dysplasia (BPD) has not been investigated in regional cohorts. The aim of this study was to clarify the incidence of PH associated with BPD in all very low birthweight infants (VLBWIs) born during the study period in Aichi Prefecture, Japan. METHODS: We conducted a retrospective observational cohort study of all VLBWIs born in Aichi Prefecture. The inclusion criteria were VLB, birth between 1 January 2015 and 31 December 2015, and admission to any neonatal intensive care unit in Aichi Prefecture. BPD28d and BPD36w were defined as the need for supplemental oxygen or any respiratory support at 28 days of age or 36 weeks of postmenstrual age (PMA). The primary outcome was the incidence of PH after 36 weeks' PMA (PH36w) in VLBWIs with BPD28d and BPD36w. The secondary outcomes were the clinical factors related to PH36w in BPD36w patients. Mann-Whitney U-test and Fisher's exact test were used for univariate analysis. Differences were considered statistically significant at P < 0.05. Risk ratio (RR) and 95% confidence interval (CI) were also evaluated. RESULTS: A total of 441 patients were analyzed. A total of 217 and 131 patients met the definition of BPD28d and BPD36w, respectively. Nine patients were diagnosed with PH36w (4.2% and 6.9% of the BPD28d and BPD36w patients, respectively). The presence of oligohydramnios (RR, 2.71; 95% CI: 1.55-4.73, P = 0.014) and sepsis (RR, 3.62; 95% CI: 1.51-8.63, P = 0.025) was significant in the PH36w patients. CONCLUSIONS: The incidence of PH36w was 4.2% and 6.9% in the BPD28d and BPD36w patients, respectively. Oligohydramnios and sepsis were significantly associated with PH36w in VLBWIs.

Predictors of long-term tumor control after stereotactic radiosurgery for Koos grade 4 vestibular schwannomas.

PURPOSE: To evaluate the predictors of long-term tumor control following stereotactic radiosurgery (SRS) for Koos grade 4 vestibular schwannomas (VSs). METHODS: Overall, 203 sporadic VS patients with compression of the brainstem were treated with SRS. The median tumor volume was 6.7 cm3 (range, 2.0-28.9 cm3) and the median marginal dose was 12 Gy (range, 9-13.5 Gy). RESULTS: The median follow-up period was 152 months (range, 12-277 months). Tumor control (TC) rates at 3, 5, and 10 years were 89%, 85%, and 82%, respectively. Operation-free survival (OFS) rates at 3, 5, and 10 years were 92%, 85%, and 83%, respectively. Middle cerebellar peduncle (MCP) compression on pre-SRS magnetic resonance imaging scans was significant for both TC (p < 0.001, hazard ratio 1.332) and OFS (p < 0.001, hazard ratio 1.306). The 3-, 5-, and 10-year OFS rates were 98%, 94%, and 92% in the low-risk group (MCP compression < 9.8 mm and > 48 years old), and 58%, 25%, and 17% in high-risk group (MCP compression ≥ 9.8 mm and ≤ 48 years old), respectively. Ten patients (4.9%) developed delayed cyst-related complications. Eleven patients (5.4%) developed newly developed or worsened trigeminal neuralgia. No patient developed persistent facial palsy as an adverse radiation effect. A ventricular peritoneal shunt was required in six patients (3%) who developed hydrocephalus after SRS. CONCLUSION: SRS is an acceptable treatment option in selected patients with Koos grade 4 VSs. Risk group classification based on patient age and MCP compression is useful in decision-making of Koos grade 4 VSs.

Long-term hearing outcomes after gamma knife surgery in patients with vestibular schwannoma with hearing preservation: evaluation in 92 patients with serial audiograms.

The treatment strategy for patients with vestibular schwannoma (VS) is controversial, and data concerning the long-term hearing outcomes > 5 years after gamma knife surgery (GKS) are limited. The long-term hearing outcomes after GKS were evaluated in VS patients with hearing preservation. Ninety-two VS patients with a pure tone average (PTA) ≤ 50 dB were evaluated. The median age was 54 years; the median tumor volume was 1.5 cm3. The tumors were treated with a median margin dose of 12 Gy and a median mean cochlear dose of 4.0 Gy. At the time of GKS, 65 patients retained a PTA of 0-30 dB, and 27 had a PTA of 31-50 dB. The median follow-up period was 106 months. At the final follow-up, 2 (2%) developed tumor progression. During the median audiogram follow-up of 83 months, the PTA was ≤ 30 dB in 22 patients (24%) and 31-50 dB in 27 patients (29%); 43 patients (47%) worsened to a PTA > 50 dB. Hearing preservation rates were 66, 57, and 44% at 3, 5, and 10 years, respectively. In multivariate analysis, the mean cochlear dose (P < 0.001) and pre-GKS PTA (P = 0.045) were significant for hearing preservation. GKS was an effective treatment option for VS patients with a PTA ≤ 50 dB. As a lower cochlear dose and better pre-GKS PTA contributed to long-term hearing preservation, prophylactic GKS before hearing deterioration or tumor growth would be a treatment of choice if patients provided informed consent.

[Meteorological Factors that Affect Stroke Onset].

BACKGROUND: To date, there have been many publications concerning relationship of meteorological condition and stroke onset. However, little is known about detailed meteorological factors that affect stroke onset. With hospital characteristics that most inpatients at our institution lived around Komaki, we examined the relationship between stroke onset and meteorological factors based on detailed meteorological data of Komaki. MATERIALS AND METHODS: Between April 1, 2012 and March 31, 2015, 1351 stroke patients admitted to Komaki City Hospital were enrolled in this study. These patients were classified into hypertensive intracranial hemorrhage(ICH), subarachnoid hemorrhage(SAH), and cerebral infarction(CI). Meteorological conditions such as season, weather, temperature, atmospheric pressure, and humidity were evaluated as factors that affect stroke onset. RESULTS: The incidence of ICH was lower in summer, and the minimum daily temperature was significantly related to ICH onset. The incidence of SAH was significantly related to the temperature difference compared to that on the previous day. When the mean temperature was lower than that on the previous day, the incidence of SAH was higher. No meteorological factor was found to affected the incidence of CI in this study. CONCLUSIONS: This study indicated that seasons and temperatures affect stroke onset.

Systemic pro-inflammatory cytokine status following therapeutic hypothermia in a piglet hypoxia-ischemia model.

BACKGROUND: Inflammatory cytokines are implicated in the pathogenesis of perinatal hypoxia-ischemia (HI). The influence of hypothermia (HT) on cytokines after HI is unclear. Our aim was to assess in a piglet asphyxia model, under normothermic (NT) and HT conditions: (i) the evolution of serum cytokines over 48 h and (ii) cerebrospinal fluid (CSF) cytokine levels at 48 h; (iii) serum pro/anti-inflammatory cytokine profile over 48 h and (iv) relation between brain injury measured by magnetic resonance spectroscopy (MRS) and brain TUNEL positive cells with serum cytokines, serum pro/anti-inflammatory cytokines and CSF cytokines. METHODS: Newborn piglets were randomized to NT (n = 5) or HT (n = 6) lasting 2-26 h after HI. Serum samples were obtained 4-6 h before, during and at 6-12 h intervals after HI; CSF was obtained at 48 h. Concentrations of interleukin (IL)-1ß, -4, -6, -8, -10 and TNF-α were measured and pro/anti-inflammatory status compared between groups. White matter and thalamic voxel lactate/N-acetyl aspartate (Lac/NAA) (a measure of both oxidative metabolism and neuronal loss) were acquired at baseline, after HI and at 24 and 36 h. RESULTS: Lac/NAA was reduced at 36 h with HT compared to NT (p = 0.013 basal ganglia and p = 0.033 white matter). HT showed lower serum TNF-α from baseline to 12 h (p < 0.05). Time-matched (acquired within 5 h of each other) serum cytokine and MRS showed correlations between Lac/NAA and serum IL-1ß and IL-10 (all p < 0.01). The pro/anti-inflammatory ratios IL-1ß/IL-10, IL-6/IL-10, IL-4/IL-10 and IL-8/IL-10 were similar in NT and HT groups until 36 h (24 h for IL-6/IL-10); after this, 36 h pro/anti-inflammatory cytokine ratios in the serum were higher in HT compared to NT (p < 0.05), indicating a pro-inflammatory cytokine surge after rewarming in the HT group. In the CSF at 48 h, IL-8 was lower in the HT group (p < 0.05). At 48 h, CSF TNF-α correlated with Lac/NAA (p = 0.02) and CSF IL-8 correlated with white matter TUNEL positive cell death (p = 0.04). CONCLUSIONS: Following cerebral HI, there was a systemic pro-inflammatory surge after rewarming in the HT group, which is counterintuitive to the putative neuroprotective effects of HT. While serum cytokines were variable, elevations in CSF inflammatory cytokines at 48 h were associated with MRS Lac/NAA and white matter cell death.

Multisession gamma knife surgery for large brain metastases.

This study aimed to explore the safety and efficacy of multisession gamma knife surgery (GKS) for large brain metastases with a volume of 10 cm3 or larger. Fifty-six patients who had 65 brain metastases with a volume of at least 10 cm3 were treated with multisession GKS. Three-session GKS at a prescription dose of 10 Gy to the tumor margin per session with a 2-week interval between doses was performed in 3 patients. Other patients were treated with 2-session GKS at a prescription dose of 10-13 Gy to the tumor margin per session with an interval of 1-4 weeks. The median tumor volume was 21 cm3. The median survival was 7 months. The 6-, 12-, and 18-month survival rates were 62, 42, and 31%, respectively. The progression-free survival rates at 6, 12, and 18 months were 93, 80, and 74%, respectively. At the time of the first and last GKS sessions, the median tumor volumes were 21 and 15 cm3, respectively, which decreased to 5 cm3 with a median follow-up period of 6 months. Seventy-four percent of evaluated patients who had pre-GKS clinical symptoms obtained symptomatic improvement in a mean interval of 2 months. Multisession GKS was a safe and effective alternative to surgical resection for patients who had brain metastases with a tumor volume of 10 cm3 or larger. Although long-term results remain unclear, multisession GKS may be a suitable palliative treatment to preserve neurological function.

Prognostic factors of hydrops fetalis with pleural effusion.

BACKGROUND: Hydrops fetalis (HF) has a low survival rate, particularly in the case of preterm birth. In addition, the severity index of HF has not been fully investigated yet. The aim of this study was to clarify the prognostic factors of HF with pleural effusion. METHODS: All live-born HF patients with pleural effusion, except for chromosomal abnormality or complex congenital heart disease, born from 2009 to 2013 in Aichi Prefecture in Japan were included. Prenatal, perinatal, and postnatal information was obtained from the medical records and was retrospectively analyzed. RESULTS: Forty-one HF patients with pleural effusion were included, and 28 patients (68%) survived. On multivariate logistic stepwise analysis, gestational birth week (OR, 0.71; 95% CI: 0.52-0.96, P = 0.027) and standard deviation (SD) score of the birthweight (OR, 1.74; 95% CI: 1.01-2.99, P = 0.045) were significant factors for postnatal death. All patients with both ≥32 gestational weeks and <3.0 birthweight SD score survived. CONCLUSIONS: Combined with the gestational weeks data, birthweight SD score may be useful to estimate the prognosis of HF with pleural effusion.

Single nucleotide polymorphisms in AGTR1, TFAP2B, and TRAF1 are not associated with the incidence of patent ductus arteriosus in Japanese preterm infants.

BACKGROUND: Persistent patent ductus arteriosus (PDA) is a frequent complication in preterm infants. Single nucleotide polymorphisms (SNP) in several genes, including angiotensin II receptor, type 1 (AGTR1), transcription factor AP-2 beta (TFAP2B) and tumor necrosis factor receptor-associated factor 1 (TRAF1), have been reported to be associated with PDA in preterm infants. The aim of this study was to evaluate the relationships between PDA in preterm infants and polymorphisms in AGTR1, TFAP2B and TRAF1 in the Japanese population. METHODS: The subjects consisted of 107 preterm infants with gestational age <32 weeks. Extremely low-birthweight infants were treated with prophylactic indomethacin during the first 24 h after birth. Five SNP, namely, rs5186 in AGTR1, rs987237 and rs6930924 in TFAP2B, and rs1056567 and rs10985070 in TRAF1, were genotyped using TaqMan SNP genotyping assays. RESULTS: There were no significant differences in the distributions of the genotypes and allele frequencies of all studied SNP between the PDA group (n = 46) and the non-PDA group (n = 61). CONCLUSIONS: There were no significant associations between the studied SNP and the incidence of PDA in Japanese preterm infants. These SNP may not be clinically important predisposing factors for PDA in Japanese preterm infants.

Effects of tolvaptan on congestive heart failure complicated with chylothorax in a neonate.

Tolvaptan is an oral vasopressin type 2 receptor antagonist that can be used for heart failure patients with hyponatremia or symptomatic congestion. Although the effects of tolvaptan in adults have been well documented, only limited information is available in children. The case of a neonate with congestive heart failure complicated with chylothorax after palliative surgery for transposition of the great arteries treated with tolvaptan is reported. Slow up-titration to 0.1 mg/kg successfully increased urine output and improved refractory congestive heart failure without hypernatremia. Subsequently, bodyweight and chylothorax decreased gradually. Moreover, the use of tolvaptan reduced the dosage of furosemide. Tolvaptan could be an alternative drug for neonates with congestive heart failure. Further large studies are needed to confirm the efficacy and identify the appropriate dose of tolvaptan in neonates.

Methyl-isobutyl amiloride reduces brain Lac/NAA, cell death and microglial activation in a perinatal asphyxia model.

Na⁺/H⁺ exchanger (NHE) blockade attenuates the detrimental consequences of ischaemia and reperfusion in myocardium and brain in adult and neonatal animal studies. Our aim was to use magnetic resonance spectroscopy (MRS) biomarkers and immunohistochemistry to investigate the cerebral effects of the NHE inhibitor, methyl isobutyl amiloride (MIA) given after severe perinatal asphyxia in the piglet. Eighteen male piglets (aged < 24 h) underwent transient global cerebral hypoxia-ischaemia and were randomized to (i) saline placebo; or (ii) 3 mg/kg intravenous MIA administered 10 min post-insult and 8 hourly thereafter. Serial phosphorus-31 (³¹P) and proton (¹H) MRS data were acquired before, during and up to 48 h after hypoxia-ischaemia and metabolite-ratio time-series Area under the Curve (AUC) calculated. At 48 h, histological and immunohistochemical assessments quantified regional tissue injury. MIA decreased thalamic lactate/N-acetylaspartate and lactate/creatine AUCs (both p < 0.05) compared with placebo. Correlating with improved cerebral energy metabolism, transferase mediated biotinylated d-UTP nick end-labelling (TUNEL) positive cell density was reduced in the MIA group in cerebral cortex, thalamus and white matter (all p < 0.05) and caspase 3 immunoreactive cells were reduced in pyriform cortex and caudate nucleus (both p < 0.05). Microglial activation was reduced in pyriform and midtemporal cortex (both p < 0.05). Treatment with MIA starting 10 min after hypoxia-ischaemia was neuroprotective in this perinatal asphyxia model.

Effect of embolization before stereotactic radiosurgery for brain arteriovenous malformations: a case-control study with propensity score matching.

OBJECTIVE: The aim of this study was to evaluate whether endovascular embolization prior to stereotactic radiosurgery (SRS) has a negative impact on nidus obliteration for patients with arteriovenous malformations (AVMs). METHODS: A total of 704 eligible patients with AVM who did not undergo prior surgery or radiotherapy were evaluated. Of these patients, 593 were treated with SRS only, and 111 were treated with embolization followed by SRS (E+SRS). Most patients in the E+SRS group (88%) underwent embolization with n-butyl-2-cyanoacrylate. In the comparison of radiosurgical outcomes between patients treated with SRS only and E+SRS, these groups were matched in a 1:1 ratio using propensity score matching to eliminate differences in basic characteristics. The primary outcome was to compare the nidus obliteration rates between the SRS-only and E+SRS groups. The secondary outcomes were the comparison of cumulative hemorrhage rates and the incidence of cyst formation or chronic encapsulated hematoma after SRS between these groups. RESULTS: In the unmatched cohorts, the actuarial 3-, 5-, and 8-year nidus obliteration rates after a single SRS session were 49.6%, 69.4%, and 74.1% in the SRS-only group, respectively, and 30.7%, 50.9%, and 68.6% in the E+SRS group, respectively (p = 0.001). In the matched cohort of 98 patients in each group, the rates were 47.1%, 62.0%, and 69.6% in the SRS-only group and 32.5%, 55.3%, and 75.0% in the E+SRS group, respectively (p = 0.24). There was no significant difference in either cumulative hemorrhage or the incidence of cyst formation or chronic encapsulated hematoma between the groups. CONCLUSIONS: Pre-SRS embolization did not affect nidus obliteration rates, cumulative hemorrhage rates, or the incidence of cyst formation or chronic encapsulated hematoma as late adverse radiation effects in patients with AVM treated with SRS.

Hemifacial spasm caused by multiple vascular attachments due to remote compression effects of a dermoid cyst in the cerebellar hemisphere: illustrative case.

BACKGROUND: Dermoid cysts located laterally in the posterior fossa are rare. The authors report the case of a dermoid cyst in the cerebellar hemisphere presenting with hemifacial spasm (HFS) caused by multiple vascular attachments due to remote compression effects. OBSERVATIONS: A 48-year-old man presented with left HFS. Computed tomography showed a mass lesion in the left cerebellar hemisphere with calcification and erosion of skull bone. Magnetic resonance imaging showed no contrast enhancement of the lesion and a dural defect. The lesion compressed the brainstem and cerebellopontine cistern, but no vascular attachments to the facial nerve were seen. Tumor removal and microvascular decompression were performed. The lesion was composed of soft tissue containing oil-like liquid and hairs, and the border of the cerebellar arachnoid was clear. There were multiple vascular attachments to the root exit zone, facial nerve, and brainstem. After displacing these arteries, the intraoperative abnormal muscle response disappeared. Histopathological findings showed stratified squamous epithelium, keratin flakes, calcifications, and hairs. The HFS disappeared completely and has remained absent for 27 months. LESSONS: The dermoid cyst originating from occipital bone compressed the cerebellar hemisphere, displacing multiple vessels and leading to HFS. Tumor removal and the removal of all vascular factors can completely resolve HFS.

Secondary aneurysmal bone cyst of the frontal bone with fibrous dysplasia showing rapid expansion: a case report.

A 19-year-old woman presented with swelling of the left forehead without pain. She did not have any relevant past or family history. Computed tomography showed destruction of the outer cortex of the frontal bone. A solitary mass lesion with a fluid collection was detected with magnetic resonance imaging. Because the swelling of the left forehead had enlarged rapidly with osteolytic changes, surgical removal of the lesion was performed. The lesion appeared to be enveloped in a fibrous capsule. The soft lesion was removed from the frontal bone. The outer frontal bone was absent, although the inner frontal bone was preserved. Then, the frontal bone was resected with margins from the edge of the erosion. The dura mater under the lesion was intact. A cranioplasty was performed using titanium mesh. On histological examination, the trabecular bones revealed irregular shapes and arrangements, indicating fibrous dysplasia. There was a continuous high-cell-concentration pathological lesion outside the fibrous dysplasia. There were numerous cells, such as mononuclear cells, osteoclast-like multinucleated giant cells, foam cells, and red blood cells. The osteoclast-like multinucleated giant cells and other cells did not show significant nuclear atypia. Immunostaining with H3.3G34W was negative, and the ubiquitin-specific peptidase 6/Tre-2 gene showed no rearrangements. The histopathological diagnosis was secondary aneurysmal bone cyst with fibrous dysplasia. Additional postsurgical therapy was not performed. There has been no evidence of recurrence of the lesion for two years.

Novel SPEG variants in a neonate with severe dilated cardiomyopathy and relatively mild hypotonia.

Striated muscle preferentially expressed protein kinase (SPEG) variants have been reported to cause centronuclear myopathy associated with cardiac diseases. The severity of skeletal muscle symptoms and cardiac symptoms are presumably related to the location of the variant. Here, we report novel SPEG compound heterozygous pathological variants in a neonate with severe dilated cardiomyopathy and relatively mild hypotonia. This report expands the genotype-phenotype correlations of patients with SPEG variants.

Xenon augmented hypothermia reduces early lactate/N-acetylaspartate and cell death in perinatal asphyxia.

OBJECTIVE: Additional treatments for therapeutic hypothermia are required to maximize neuroprotection for perinatal asphyxial encephalopathy. We assessed neuroprotective effects of combining inhaled xenon with therapeutic hypothermia after transient cerebral hypoxia-ischemia in a piglet model of perinatal asphyxia using magnetic resonance spectroscopy (MRS) biomarkers supported by immunohistochemistry. METHODS: Thirty-six newborn piglets were randomized (all groups n = 9), with intervention from 2 to 26 hours, to: (1) normothermia; (2) normothermia + 24 hours 50% inhaled xenon; (3) 24 hours hypothermia (33.5°C); or (4) 24 hours hypothermia (33.5°C) + 24 hours 50% inhaled xenon. Serial MRS was acquired before, during, and up to 48 hours after hypoxia-ischemia. RESULTS: Mean arterial blood pressure was lower in all treatment groups compared with normothermia (p < 0.01) (although >40mmHg); the combined therapy group required more fluid boluses (p < 0.05) and inotropes (p < 0.001). Compared with no intervention, both hypothermia and xenon-augmented hypothermia reduced the temporal regression slope magnitudes for phosphorus-MRS inorganic phosphate/exchangeable phosphate pool (EPP) and phosphocreatine/EPP (both p < 0.05); for lactate/N-acetylaspartate (NAA), only xenon-augmented hypothermia reduced the slope (p < 0.01). Xenon-augmented hypothermia also reduced transferase-mediated deoxyuridine triphosphate nick-end labeling (TUNEL)(+) nuclei and caspase 3 immunoreactive cells in parasagittal cortex and putamen and increased microglial ramification in midtemporal cortex compared with the no treatment group (p < 0.05). Compared with hypothermia, however, combination treatment did not reach statistical significance for any measure. Lactate/NAA showed a strong positive correlation with TUNEL; nucleotide triphosphate/EPP showed a strong negative correlation with microglial ramification (both p < 0.01). INTERPRETATION: Compared with no treatment, xenon-augmented hypothermia reduced cerebral MRS abnormalities and cell death markers in some brain regions. Compared with hypothermia, xenon-augmented hypothermia did not reach statistical significance for any measure. The safety and possible improved efficacy support phase II trials.

Endothelin receptor antagonist attenuates oxidative stress in a neonatal sepsis piglet model.

BACKGROUND: Oxidative stress (oxidant-antioxidant imbalance) plays an important role in the pathophysiology of neonatal sepsis. This study evaluated whether an antisense peptide endothelin receptor antagonist, ETR-P1/fl, could attenuate oxidative stress in a neonatal sepsis model. METHODS: A total of 18 3-d-old piglets were anesthetized and mechanically ventilated. Six piglets received cecal ligation and perforation (CLP group) for induction of sepsis. Six piglets also received continuous infusion (0.05 mg/kg/h) of ETR-P1/fl 30 min after CLP (ETR-P1/fl group). Six piglets received a sham operation. Serum total hydroperoxide (TH), biological antioxidant potentials (BAPs), oxidative stress index (OSI, calculated as TH/BAP), interleukin (IL)-6, serum glutamic oxaloacetic transaminase (GOT), and creatinine were measured before CLP and at 1, 3, and 6 h after CLP. RESULTS: CLP evoked a state of shock resulting in elevated TH, OSI, and IL-6 levels. ETR-P1/fl administration after CLP resulted in lower serum TH at 1 and 3 h after CLP, OSI at 1 and 3 h after CLP, IL-6 at 1 and 3 h after CLP, and GOT at 3 and 6 h after CLP as compared with the CLP group. CONCLUSION: ETR-P1/fl treatment significantly attenuated the elevation of serum oxidative stress markers (TH and OSI), IL-6, and GOT in a progressive neonatal sepsis CLP model.

Efficacy of Magnetic Resonance Contrast-Enhanced Vessel Wall Imaging as an Ancillary Examination for Subarachnoid Hemorrhage With Bleeding Points Difficult to Determine on Conventional Vascular Imaging: A Report of Three Cases.

Here, we have demonstrated the efficacy of magnetic resonance contrast-enhanced vessel wall imaging (CE-VWI) as an ancillary examination for subarachnoid hemorrhage with bleeding points difficult to confirm by conventional vascular imaging. Case 1 had a ruptured small aneurysm with a size of 1.8 × 1.1 mm at the origin of left anterior choroidal artery. CE-VWI showed enhancement of the apex of the aneurysm. Surgical clipping was performed by a mini-clip. In Case 2, a ruptured small aneurysm, 2.1 × 1.9 mm, was detected at right middle cerebral artery bifurcation. CE-VWI showed enhancement of the aneurysmal wall. Endovascular coil embolization was performed. In Case 3, irregular dilatation of left internal carotid artery (ICA) was detected. CE-VWI demonstrated enhancement of the dilatation wall. The lesion was deemed to be a dissection of the ICA or a blood blister-like aneurysm. Endovascular treatment using intracranial stent placement was performed, and the patient has had no rebleeding events for one and a half years. In all cases, conventional vascular imaging detected scanty morphological changes, and CE-VWI information provided reliable confirmation of the lesions as bleeding points.

Intravascular Large B-Cell Lymphoma Diagnosed by Open Brain Biopsy and Achievement of Remission After Early Initiation of Chemotherapy: Case Report.

A 60-year-old man presented with progressive disturbance of consciousness. His father had died of malignant lymphoma, while his mother and sister died of acute leukemia. Magnetic resonance imaging (MRI) revealed multiple high-intensity lesions in the bilateral cerebral hemispheres on diffusion-weighted images. Serum soluble interleukin 2 receptor was 5,640 U/mL. Screenings of blood antibodies known to rise in autoimmune diseases were all normal. Cerebrospinal fluid examinations demonstrated slight elevation of protein and glucose, while the oligoclonal band and myelin basic protein were not elevated. Biopsies of bone marrow and random skin did not show any malignant features. His consciousness gradually deteriorated over a week, with lesions in his right frontal, left temporal, and bilateral parietal lobes shown to be growing. Therefore, open brain biopsy was performed, and one block of the right frontal lesion was harvested. Histological examination revealed atypical large cells only in the capillaries. Although immunohistochemical examinations showed positive staining for CD20, they were negative for CD3. Histopathological diagnosis was intravascular large B-cell lymphoma. After undergoing six cycles of intravenous chemotherapy with rituximab, cyclophosphamide, doxorubicin, and prednisone, his consciousness and neurological symptoms improved, and he appeared to achieve remission. Two years later, there have been no apparent recurrences, and the brain lesions have disappeared.

Long-Term Risks of Hemorrhage and Adverse Radiation Effects of Stereotactic Radiosurgery for Brain Arteriovenous Malformations.

BACKGROUND: The information about long-term risks of hemorrhage and late adverse radiation effects (AREs) after stereotactic radiosurgery for brain arteriovenous malformations (AVMs) is lacking. OBJECTIVE: To evaluate the long-term risks of hemorrhage and late ARE rates in patients with AVM treated with Gamma Knife surgery (GKS). METHODS: We examined 1249 patients with AVM treated with GKS. The Spetzler-Martin grade was I in 313 patients (25%), II in 394 (32%), III in 458 (37%), and IV/V in 84 (7%). The median treatment volume was 2.5 cm3, and the median marginal dose was 20 Gy. RESULTS: The median follow-up period was 61 months. The 5- and 10-year nidus obliteration rates were 63% and 82%, respectively. The 5- and 10-year cumulative hemorrhage rates were 7% and 10%, respectively. The annual hemorrhage rate was 1.5% for the first 5 years post-GKS, which decreased to 0.5% thereafter. During the follow-up period, 42 symptomatic cyst formations/chronic encapsulated hematomas ([CFs/CEHs], 3%) and 3 radiation-induced tumors (0.2%) were observed. The 10- and 15-year cumulative CF/CEH rates were 3.7% and 9.4%, respectively. CONCLUSION: GKS is associated with reduced hemorrhage risk and high nidus obliteration rates in patients with AVM. The incidence of late AREs tended to increase over time. The most common ARE was CF/CEH, which can be safely removed; however, careful attention should be paid to the long-term development of fatal radiation-induced tumors.

Intracranial Idiopathic Acute Epidural Hematoma Presenting with a Stroke-like Attack and Rapid Neurological Deterioration: A Case Report.

A 36-year-old woman presented with sudden onset of a right-sided headache that awoke her from sleep. She had no episodes of trauma or abuse. She was initially able to speak, but fell into a coma within an hour. The right pupil was dilated, with slow pupillary reflexes to light on both sides, and she showed left hemiparalysis. Computed tomography scan showed a right acute epidural hematoma, approximately 4 cm in thickness, and there were no findings of trauma such as skin wounds, subcutaneous hematomas, or skull fractures. In the emergency room, decompression of intracranial pressure by one burr hole was performed, and her dilated right pupil improved to normal size. She was then moved to the operating room, and hematoma removal was performed by craniotomy. Her blood pressure trended downward despite rapid blood transfusion and vasopressor therapy. There were no abnormal findings apparent intraoperatively, except for oozing from the whole surface of the dura mater and epidural space. Her consciousness improved postoperatively, and her left hemiparalysis improved within a few days. No causative diseases, risk factors, or vascular abnormalities were found on laboratory and radiological surveys. Two months postoperatively, the bone flap was removed because of infection. Eight months postoperatively, a cranioplasty using artificial skull was performed, and her postoperative course was uneventful. One year after the initial surgery, she has no neurological deficits, and there has been no recurrence of epidural hematoma.