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.

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.

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.

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.

Influence of Intrauterine Inflammation, Delivery, and Postnatal Feeding on the Temporal Changes of Serum Alpha 1 Acid Glycoprotein Levels in Extremely-Low-Birth-Weight Infants.

Infection remains the primary cause of death in extremely-low-birth-weight infants (ELBWIs). Alpha 1 acid glycoprotein (α1AG), an acute-phase protein, has been shown to be elevated in sporadic cases of septic ELBWIs prior to abnormal clinical signs. To delineate the roles of inflammation, delivery, and feeding in postnatal α1AG changes in ELBWIs, 75 ELBWIs of 26.5 ± 2.2 weeks of gestation born between May 2011 and August 2017 were retrospectively studied. The dependence of α1AG levels obtained on days 0−5 on the clinical variables was examined by incorporating interactions with age, followed by estimations of regression coefficients between clinical variables and α1AG levels at the early and late postnatal ages, defined by their standard deviation. Chorioamnionitis (p < 0.001), funisitis (p = 0.045), vaginal delivery (p = 0.025), enteral feeding (p = 0.022), and probiotics (p = 0.005) were associated with early α1AG elevations. Hypertensive disorder of pregnancy (p < 0.001) and gestational age (p = 0.001) were associated with late α1AG elevation; premature rupture of membranes (p < 0.001), funisitis (p = 0.021), body weight z-scores (p < 0.001), and enteral feeding (p = 0.045) were associated with late α1AG reduction. Postnatal α1AG changes in ELBWIs were associated with variables representative of age, growth, delivery, inflammation, and enteral feeding, potentially reflecting the process of sensitization to extrinsic microbes in utero, at birth, and thereafter.

Achieving good neurological outcome by combining decompressive craniectomy for acute subdural hematoma and transarterial embolization of intraperitoneal injured arteries for multiple severe trauma: a case report.

A 22-year-old woman jumped from the 4th floor of her apartment in an attempt to commit suicide. Whole-body computed tomography showed multiple injuries, including right acute subdural hematoma, left hemopneumothorax, several fractures, intraperitoneal hemorrhage, and spleen injury. Her consciousness deteriorated rapidly, and her right pupil was dilated. Furthermore, she had unstable vital signs including blood pressure of approximately 70/40 mmHg, pulse about 150/minute, respiratory rate 25/minute, and percutaneous oxygen saturation of 90% on 10 L oxygen. Intratracheal intubation and insertion of a thoracostomy tube were performed in the emergency room. Due to concomitant brain herniation and hemorrhagic shock, simultaneous decompressive craniectomy for acute subdural hematoma and transarterial embolization of intraperitoneal injured arteries were performed in our hybrid operating room. Despite rapid blood transfusions, the blood pressure did not increase. After starting embolization of the injured arteries of the spleen, the blood pressure increased, thereby making it possible to remove the acute subdural hematoma, and hemostasis was then achieved. Four hours later, the acute subdural hematoma and intracranial pressure increased again, and re-operation was performed in the normal operating room. Cranioplasty and clavicular fracture reduction were performed 14 days later. She recovered enough to talk and walk, and her consciousness stabilized. Interviews with her and her family by a psychiatrist determined that abnormal behaviors had first appeared 2 months earlier. She was diagnosed with acute and transient psychotic disorders, and treatment was started. The patient was discharged home 1 month later with mild disability of her higher-order brain function.

Near-infrared light scattering and water diffusion in newborn brains.

OBJECTIVE: MRI provides useful information regarding brain maturation and injury in newborn infants. However, MRI studies are generally restricted during acute phase, resulting in uncertainty around upstream clinical events responsible for subtle cerebral injuries. Time-resolved near-infrared spectroscopy non-invasively provides the reduced scattering coefficient ( µ s ' ), which theoretically reflects tissue structural complexity. This study aimed to test whether µ s ' values of the newborn head reflected MRI findings. METHODS: Between June 2009 and January 2015, 77 hospitalised newborn infants (31.7 ± 3.8 weeks gestation) were assessed at 38.8 ± 1.3 weeks post-conceptional age. Associations of µ s ' values with MRI scores, mean diffusivity and fractional anisotropy were assessed. RESULTS: Univariable analysis showed that µ s ' values were associated with gestational week (p = 0.035; regression coefficient [B], 0.065; 95% confidence interval [CI], 0.005-0.125), fractional anisotropy in the cortical grey matter (p = 0.020; B, -5.994; 95%CI, -11.032 to -0.957), average diffusivity in the cortical grey matter (p < 0.001; B, -4.728; 95%CI, -7.063 to -2.394) and subcortical white matter (p = 0.001; B, -2.071; 95%CI, -3.311 to -0.832), subarachnoid space (p < 0.001; B, -0.289; 95%CI, -0.376 to -0.201) and absence of brain abnormality (p = 0.042; B, -0.422; 95%CI, -0.829 to -0.015). The multivariable model to explain µ s ' values comprised average diffusivity in the subcortical white matter (p < 0.001; B, -2.066; 95%CI, -3.200 to -0.932), subarachnoid space (p < 0.001; B, -0.314; 95%CI, -0.412 to -0.216) and absence of brain abnormality (p = 0.021; B, -0.400; 95%CI, -0.739 to -0.061). INTERPRETATION: Light scattering was associated with brain structure indicated by MRI-assessed brain abnormality and diffusion-tensor-imaging-assessed water diffusivity. When serially assessed in a larger population, µ s ' values might help identify covert clinical events responsible for subtle cerebral injury.

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.

Primary Dural Mucosa-associated Lymphoid Tissue Lymphoma Mimicking Falx Meningioma: A Case Report.

An 85-year-old woman presented with ataxia and deterioration of cognitive functions. She had no history of autoimmune diseases or viral infections. Magnetic resonance imaging showed a solitary mass lesion at the cerebral falx on contrast-enhanced T1-weighted imaging. Gross total resection of the lesion involving the dura mater was performed by bifrontal craniotomy. Histological examination showed diffuse infiltration of small lymphocytes and plasma cells. There was also some proliferation of large lymphocytes with folded nuclei, high-density chromatin, and inconspicuous nucleoli. The large atypical B lymphocytes did not demonstrate diffuse dense sheet findings. Meningothelial components were not detected. Immunohistochemistry was positive for pan B-cell antigens. The analysis of the kappa/lambda ratio indicated kappa immunoglobulin light chain-restricted B-cell proliferation. The final histopathological diagnosis was mucosa-associated lymphoid tissue lymphoma. Systemic screening examinations were then performed. Histological findings of the bone marrow showed normal findings without atypical lymphocytes. A chromosomal study of the bone marrow showed 46, XX. 18F fluoro-2-deoxyglucose positron emission tomography showed high accumulations at the left pterygoid muscle and the right transverse processes of the thoracic vertebrae, and mild accumulation at the right ilium bone, which indicated disseminated lesions. One year later, thickening of the dura mater was detected. Therefore, gamma knife surgery was performed. Two years later, she was alive without neurological deterioration, and magnetic resonance imaging showed no evidence of recurrence.

A Practical Grading Scale for Predicting Outcomes of Radiosurgery for Dural Arteriovenous Fistulas: JLGK 1802 Study.

BACKGROUND AND PURPOSE: To assess the long-term outcomes of intracranial dural arteriovenous fistula (DAVF) treated with stereotactic radiosurgery (SRS) alone or embolization and SRS (Emb-SRS) and to develop a grading system for predicting DAVF obliteration. METHODS: This multi-institutional retrospective study included 200 patients with DAVF treated with SRS or Emb-SRS. We investigated the long-term obliteration rate and obliteration-associated factors. We developed a new grading system to estimate the obliteration rate. Additionally, we compared the outcomes of SRS and Emb-SRS by using propensity score matching. RESULTS: The 3- and 4-year obliteration rates were 66.3% and 78.8%, respectively. The post-SRS hemorrhage rate was 2%. In the matched cohort, the SRS and Emb-SRS groups did not differ in the rates of obliteration (P=0.54) or post-SRS hemorrhage (P=0.50). In multivariable analysis, DAVF location and cortical venous reflux (CVR) were independently associated with obliteration. The new grading system assigned 2, 1, and 0 points to DAVFs in the anterior skull base or middle fossa, DAVFs with CVR or DAVFs in the superior sagittal sinus or tentorium, and DAVFs without these factors, respectively. Using the total points, patients were stratified into the highest (0 points), intermediate (1 point), or lowest (≥2 points) obliteration rate groups that exhibited 4-year obliteration rates of 94.4%, 71.3%, and 60.4%, respectively (P<0.01). CONCLUSIONS: SRS-based therapy achieved DAVF obliteration in more than three-quarters of the patients at 4 years of age. Our grading system can stratify the obliteration rate and may guide physicians in treatment selection.

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.

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.

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.

Investigations into the efficacy of a novel extubation-aerosol shield: a cough model study.

BACKGROUND: Physicians have had to perform numerous extubation procedures during the prolonged coronavirus disease 2019 (COVID 19) pandemic. Future pandemics caused by unknown pathogen may also present a risk of exposure to infectious droplets and aerosols. AIM: This study evaluated the ability of a newly developed aerosol barrier, "Extubation-Aerosol (EA)-Shield" to provide maximum protection from aerosol exposure during extubation via an aerosolised particle count and high-quality visualisation assessments. METHODS: We employed a cough model having parameters similar to humans and used micron oil aerosol as well as titanium dioxide as aerosol tracers. Aerosol barrier techniques employing a face mask (group M) and EA-Shield (group H) were compared. FINDINGS: The primary outcome was the difference in the number of particles contacting the physician's face before and after extubation. The maximum distances of aerosol dispersal after extubation were measured as the secondary outcomes. All aerosolised particles of the two tracers were significantly smaller in group H than in group M (p < 0.05). In addition, the sagittal and axial maximum distances and sagittal areas of aerosol dispersal for 3, 5, and 10 s after extubation were significantly smaller in group H than in group M (p < 0.05). CONCLUSION: This model indicates that EA-Shield could be highly effective in reducing aerosol exposure during extubation. Therefore, we recommend using it as an aerosol barrier when an infectious aerosol risk is suspected.

Dural-based Cavernous Malformation at the Temporal Convexity Presenting with Hemorrhage in a Pregnant Woman: Case Report.

Intracranial cavernous malformations (CMs) are benign vascular malformations that arise mostly within the brain parenchyma, but occasionally from the dura mater. Here, we report an extremely rare case of a 29-year-old pregnant woman presenting with hemorrhage in a dural-based CM at the temporal convexity. She presented with headache at 38 weeks of pregnancy. Imaging showed a hemorrhagic mass lesion in the left temporal lobe. Consciousness was clear, with no apparent neurological symptoms or headache. Her baby was delivered by emergency Caesarean section. Magnetic resonance (MR) imaging revealed an enhanced lesion around the hematoma and flow void appearances. Cerebral angiography confirmed the left middle meningeal artery feeding the lesion with apparent contrast medium pooling. Surgical removal of the lesion as a single block was performed. Histological examinations were consistent with CM. The lesion was located outside the brain and attached to the dura mater of the convexity, so the final diagnosis was dural-based CM. The patient was discharged home with her baby without any neurological deficits, and no recurrence has been seen for 2 years. Dural-based CM at the temporal convexity was detected, presenting as headache induced by hemorrhage during pregnancy. The relationship between bleeding of the lesion and pregnancy remains unclear, but female hormones and vascular growth factors during pregnancy can induce morphological changes and angiogenesis in CMs.

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 Outcomes of Sporadic Vestibular Schwannomas Treated with Recent Stereotactic Radiosurgery Techniques.

PURPOSE: Vestibular schwannomas (VSs) are benign; thus, understanding long-term tumor control and late adverse radiation effects of stereotactic radiosurgery (SRS) through current radiosurgical techniques is important to inform treatment decisions. Our aim was to clarify long-term tumor control rates and incidence of late adverse radiation effects in patients with VSs followed for 5 years or longer after SRS. METHODS AND MATERIALS: Altogether, 615 patients with VSs (excluding neurofibromatosis type 2 and partially treated tumors) followed for 5 years or longer after SRS using recent radiosurgical techniques were evaluated. All patients were treated at a margin dose of less than 14 Gy. All tumors were classified into 4 categories: type A (intracanalicular tumor, 87 patients [14%]), type B (cerebellopontine angle [CPA] tumor, 325 patients [53%]), type C (CPA tumor compressing the brain stem, 138 patients [22%]), and type D (CPA tumor compressing the brain stem with a deviation of the fourth ventricle, 65 patients [11%]). Median tumor volume was 2.0 cm3 and median marginal dose was 12 Gy. RESULTS: Median follow-up period was 158 months. Actuarial 5-, 10-, and 15-year or longer local control (LC) rates were 93%, 91%, and 89%, respectively. Tumor type (P < .001, hazard ratio 2.389) and number of prior surgeries (P = .007, hazard ratio 1.698) were significant for LC. Depending on the tumor type, the actuarial 10-year LC rates were 100%, 93%, 88%, and 70% in type A, B, C, and D tumors, respectively. No patient developed persistent facial palsy. Twenty patients (3.3%) developed delayed cysts. One patient developed malignant transformation (0.2%). CONCLUSIONS: SRS is a safe and effective treatment for VSs in the long term, excluding VSs compressing the brain stem with a deviation of the fourth ventricle. Delayed cysts such as cyst formation, enlarged preexisting cysts or extratumoral cysts, and malignant transformation should be considered possible causes of long-term treatment failures.

Subarachnoid Hemorrhage After Ischemic Stroke Associated with Systemic Lupus Erythematosus and Antiphospholipid Syndrome.

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease associated with increased risk of stroke. Antiphospholipid syndrome is another autoimmune disease that frequently overlaps with SLE. We report the case of a patient presenting with subarachnoid hemorrhage after ischemic stroke associated with SLE and antiphospholipid syndrome. CASE DESCRIPTION: A 22-year-old man presented with cerebral infarction of the right corona radiata. He had no contributory past or family histories. On imaging at this time, a 4-mm fusiform aneurysm of the right anterior cerebral artery was incidentally detected. Several examinations were performed, but no abnormalities or abnormal lesions were seen on echography or whole-body computed tomography. Blood tests yielded positive results for antinuclear antibody, lupus anticoagulant, and anti-beta-2 glycoprotein І antibody. He presented 70 days later with subarachnoid hemorrhage. Cerebral angiography showed the same fusiform aneurysm without any change in shape and no new aneurysms. The balloon occlusion test was performed without any neurologic symptoms, so the right anterior cerebral artery was trapped using coils. After 6 months, he presented with new optic hyperesthesia and facial butterfly-shaped erythema and fulfilled the criteria for SLE. At 2 years after initial presentation, he showed no recurrence of either aneurysm or ischemic stroke. CONCLUSIONS: The patient's first ischemic stroke was induced by antiphospholipid syndrome and underlying SLE. Fusiform aneurysm may have resulted from focal vasculitis, with activation of SLE leading to aneurysm rupture.

Repeated Intracranial Hemorrhage After Delivery Induced by Coagulopathy Associated with Multiple Venous Malformations.

BACKGROUND: Venous malformations are classified as slow-flow vascular malformations. Coagulation abnormalities are known to be frequent among patients with venous malformations. We report a case of repeated intracranial hemorrhage after delivery, induced by coagulopathy associated with multiple venous malformations. CASE DESCRIPTION: A 28-year-old woman presented with left chronic subdural hematoma 1 month after successfully giving birth. She had a history of multiple venous malformations around the pubic region and hips. The hematoma was evacuated by burr hole surgery. Three hours later, her level of consciousness rapidly deteriorated and computed tomography showed acute epidural hematoma. The hematoma was removed immediately by craniotomy under general anesthesia. No bleeding points were apparent in the operative field. Continuous bleeding around the dura mater and subdural space were encountered, and hemostasis was not achieved by electrocoagulation. After using fresh frozen plasma, hemostasis was achieved. Level of consciousness and neurologic symptoms improved postoperatively. Magnetic resonance imaging revealed multiple venous malformations in bilateral lower extremities and the pelvis. Disseminated intravascular coagulopathy was diagnosed, and thrombomodulin and blood coagulation factor XIII were administered. She was discharged home without any neurologic deficits. CONCLUSIONS: The delivery activated localized intravascular coagulopathy in the venous malformations and induced chronic subdural hematoma. Surgical interventions then resulted in progression of the coagulopathy to disseminated intravascular coagulopathy, inducing acute epidural hematoma.