External Validation of the Modified Southwestern Aneurysm Severity Index in the Barrow Ruptured Aneurysm Trial Surgical Cohort.

BACKGROUND AND OBJECTIVES: Predicting functional outcomes after surgical management of ruptured aneurysms is essential. This study sought to validate the modified Southwestern Aneurysm Severity Index (mSASI), which predicts functional outcomes 1 year after treatment. METHODS: The surgical arm of a randomized controlled trial, the Barrow Ruptured Aneurysm Trial, was used to validate the mSASI model. mSASI scores incorporating the Hunt and Hess scale, Non-Neurological American Society of Anesthesiologists Physical Classification Status, imaging findings, and other modifiers were assigned and evaluated against the Glasgow Outcome Scale (GOS) score at 1 year. The model's performance was assessed for discrimination and calibration. Similar evaluations were constructed using the modified Rankin Scale (mRS) as the 1-year functional outcome measurement. Long-term outcomes (3, 6, 10 years) were also evaluated. RESULTS: Of 280 clinical trial patients treated surgically, 242 met the inclusion criteria. The mean age was 54.1 ± 12.9 years; 31% were men. Favorable GOS score (4-5) and mRS score (0-2) at 1 year were observed in 73.6% and 66.1% of patients, respectively. The mSASI model predicted unfavorable GOS score at 1 year with fair to good discrimination (area under the curve = 0.75, 95% CI = 0.68-0.82) and accurate calibration (R2 = 0.98). Similar results were obtained when mRS was used as the outcome measure (area under the curve = 0.75, 95% CI = 0.68-0.82; R2 = 0.95). CONCLUSION: The mSASI model was externally validated in our cohort to predict functional outcomes using the GOS or mRS scores 1 year after surgery. This index may be used for prognosticating outcomes of patients undergoing surgery for ruptured aneurysms at short-term and long-term intervals.

Significance of Early Postoperative Magnetic Resonance Imaging following Intracranial Meningioma Resection.

The significance of early postoperative magnetic resonance imaging (MRI) for meningioma resection has not yet been evaluated. We retrospectively reviewed patients with intracranial meningiomas resected at our institute between 2011 and 2021. Early postoperative MRI with contrast enhancement was routinely performed within 48 h after surgery while first follow-up MRI was performed approximately after 6 months. MRI findings were reviewed, and the risk factors for postoperative infarction and early recurrence were analyzed. Among the 245 resections performed, early postoperative MRI was performed in 200 cases. Postoperative radiological and symptomatic infarctions occurred in 54 (27%) and 17 patients (9%), respectively. Diameter > 5 cm (p = 0.015) and skull base location (p = 0.010) were independent risk factors for radiological infarctions. Follow-up postoperative MRI performed in 180 patients (90%) detected early recurrence in 24 patients (13%). Non-gross total resection was an independent risk factor for early recurrence (p < 0.0001). Additionally, early recurrence after gross total resection occurred significantly more frequently in meningiomas with dural sinus involvement than in those without (8.3% vs. 0%, p = 0.018). Thus, early postoperative MRI may enable the timely assessment of postoperative neurological deficits, especially after large skull base meningioma resections along with accurate detection of early recurrence, which is critical for meningiomas with dural sinus involvement.

Pearls & Oy-sters: Cognitive and Affective Dysfunction Caused by a Small Cerebellar Hemangioblastoma.

The primary function of the cerebellum is the coordination and regulation of movement; therefore, cerebellar tumors usually present with ataxia, dysarthria, and vertigo. Large tumors also cause elevated intracranial pressure that may lead to a disturbance of consciousness. Furthermore, it has become increasingly evident that the cerebellum plays a substantial role in cognitive and affective processing. A 44-year-old female patient presented with a 1-month history of depression and flat affect. She had no cerebellar symptoms including no coordination dysfunction or dysarthria. Cognitive function tests revealed impairments in attention, execution, and processing speed. Hamilton Depression Scale and Hospital Anxiety Depression Scale indicated moderate-to-severe depression. Magnetic resonance (MR) imaging revealed a 7-mm enhancing lesion in the culmen of the cerebellar vermis with surrounding edema. Technetium-99m ethyl cysteinate dimer single-photon emission tomography (SPECT) showed hypoperfusion in the left frontal lobe. Although she was initially treated with corticosteroids for presumed sero-negative autoimmune encephalitis, her symptoms persisted. She then underwent cerebellar lesion resection. The histologic diagnosis was hemangioblastoma. The patient's symptoms dramatically improved within 1 week of resection, including improved batteries for cognitive function and depression. Complete regression of cerebellar edema and left frontal lobe hypoperfusion was observed on MR and SPECT images, respectively. This case reiterates the crucial influence of the cerebellum on cognitive and affective function. Moreover, cognitive dysfunction may be masked in cases with focal cerebellar symptoms or elevated intracranial pressure and, consequently, not adequately evaluated.

Recurrent Sphenocavernous Meningioma.

A case of a recurrent sphenocavernous meningioma is presented. The patient is a 42-year-old male who presented with an episode of transient right-sided numbness. A magnetic resonance imaging (MRI) revealed a large left sphenocavernous meningioma. The patient underwent a frontotemporal craniotomy for tumor resection. Near total resection was achieved with minimal residual in the left cavernous sinus (CS) and orbital apex. The pathology was consistent with meningioma, World Health Organization (WHO) grade I. A follow-up MRI was done 9 months after surgery and showed a growth of the residual tumor, which was treated with intensity modulated radiotherapy. Tumor growth was detected on serial imaging over a 4-year period. Surgical resection was offered. A left frontotemporal craniotomy with pretemporal transcavernous approach was performed. The bone flap was reopened and the dura was opened in a Y-shaped fashion. The roof of the optic canal was drilled off, and the falciform ligament was opened to decompress the optic nerve. The tumor was disconnected from the anterior clinoid region (the anterior clinoid process was eroded by the tumor) and reflected off the wall of the lateral CS. Tumor was adherent to the V2 fascicles (the lateral CS wall was resected in the first surgery) and was sharply dissected off. Gross total resection was achieved. The pathology was consistent with meningioma, WHO grade I. The patient had an unremarkable postoperative course without any new neurological deficits. The link to the video can be found at: https://youtu.be/KVBVw_86JqM .

A two-stage combined anterolateral and endoscopic endonasal approach to the petroclival region: an anatomical study and clinical application.

OBJECT: The pretemporal transcavernous anterior petrosal (PTAP) approach and the combined petrosal (CP) approach have been used to resect petroclival meningiomas (PCMs). In this cadaveric anatomical study, a two-stage combined PTAP and endoscopic endonasal far medial (EEFM) approach (the PTAPE approach) was compared morphometrically to the CP approach. A case study provides a clinical example of using the PTAPE approach to treat a patient with a PCM. The key elements of the approach selection process are outlined. METHODS: Five cadaveric specimens underwent a CP approach and 5 underwent a PTAPE approach. The area of drilled clivus, length of multiple cranial nerves (CNs), and the area of brain stem exposure were measured, reported as means (standard deviations) by group, and compared. RESULTS: The total area of the clivus drilled in the PTAPE group (695.3 [121.7] mm2) was greater than in the CP group (88.7 [17.06] mm2, P < 0.01). Longer segments of CN VI were exposed via the PTAPE than the CP approach (35.6 [9.07] vs. 16.3 [6.02] mm, P < 0.01). CN XII (8.8 [1.06] mm) was exposed only in the PTAPE group. Above the pontomedullary sulcus, the total area of brain stem exposed was greater with the PTAPE than the CP approach (1003.4 [219.5] mm2 vs. 437.6 [83.7] mm2, P < 0.01). Similarly, the total exposure of the medulla was greater after the PTAPE than the CP exposure (240.2 [57.06] mm2 vs. 48.1 [19.9] mm2, P < 0.01). CONCLUSION: A combined open-endoscopic paradigm is proposed for managing large PCMs. This approach incorporates the EEFM approach to address the limitations of the PTAP and the CP approach in a systematic fashion. Understanding the anatomical findings of this study will aid in tailoring surgical approaches to patients with these complex lesions.

Quantitative Evaluation of Proliferative Potential Using Flow Cytometry Reveals Intratumoral Heterogeneity and Its Relevance to Tumor Characteristics in Vestibular Schwannomas.

This study sought to explore the existence and clinical significance of intratumoral heterogeneity of proliferative potential in vestibular schwannoma (VS). Rapid intraoperative flow cytometry was utilized with raw samples to measure the proliferative ability of VS. The proliferation index (PI) was defined as the ratio of the number of cells with greater than normal DNA content to the total number of cells. A total of 66 specimens (26 from the intrameatal portion and 40 from the cisternal portion) were obtained from 34 patients with VS. There was a moderate correlation between the PI and MIB-1 labelling index values (R = 0.57, p < 0.0001). In contrast, the patterns of heterogeneity, represented by the proportion of intrameatal PI to cisternal PI, were associated with tumor size (p = 0.03). In addition, preoperative hearing tended to be poor in cases where the intrameatal PI was higher than the cisternal PI (p = 0.06). Our data demonstrated the presence of intratumoral heterogeneity of proliferative potential in VS and its relationship with tumor characteristics. The results of this study may advocate the resection of the intrameatal portion of large VSs treated with planned subtotal resection, especially in cases of poor preoperative hearing function.

Nervus Intermedius Outcomes After Vestibular Schwannoma Surgery and Radiosurgery: A Single-Institution Experience.

OBJECTIVE: Nervus intermedius (NI) dysfunction after the management of vestibular schwannoma (VS) is underreported and is experienced by patients undergoing stereotactic radiosurgery and surgical resection. The aim of this study was to present NI outcomes in a series of patients who underwent all treatment modalities for VS and to review the existing literature. METHODS: We performed a retrospective review of all patients with VS who were treated at our institution between January 1, 2008, and December 31, 2018. Demographic data, tumor size, Koos grade, treatment modality, extent of resection, postoperative facial nerve function, and hearing function were collected. NI outcomes were determined from phone interview using a previously published functional questionnaire. RESULTS: Of 222 patients who were reviewed, 98 patients responded to the questionnaire. Patients were stratified into 3 groups: group 1, 54 patients who underwent radiation; group 2, 27 patients who underwent surgical treatment; group 3, 17 patients who underwent both radiation and surgery. Of patients, 28% presented with preoperative NI dysfunction, most commonly dry eye followed by taste dysfunction and lacrimation dysfunction. Following treatment, 79% of patients experienced NI dysfunction, most commonly dry eye. Statistical differences in dry eye and taste dysfunction were observed when comparing the treatment groups. CONCLUSIONS: NI dysfunction is common following treatment for VS and should be included in pretreatment counseling of patients, as it may impact treatment choice and quality of life. Additional studies are warranted to fully characterize NI dysfunction after treatment.

Impact of vestibular nerve preservation on facial and hearing outcomes in small vestibular schwannoma surgery: a technical feasibility study.

BACKGROUND: Management of small vestibular schwannomas (VSs) remains controversial. When surgery is chosen, the preservation of facial and cochlear nerve function is a priority. In this report, we introduce and evaluate a technique to anatomically preserve the vestibular nerves to minimize manipulation and preserve the function of the facial and cochlear nerves. METHODS: The vestibular nerve preservation technique was prospectively applied to resect small VS tumors in patients with serviceable preoperative hearing (American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) class A or B). Clinical and radiological data were recorded and analyzed. RESULTS: Ten patients met the inclusion criteria. The mean (SD) age was 40.4 (12.5) years. Follow-up ranged from 6 weeks to 2 years. The maximum tumor diameter parallel to the internal auditory canal ranged from 10 to 20 mm (mean, 14.9 (3.1) mm). There were three Koos grade 3 and seven Koos grade 2 tumors. Gross total resection was achieved in all cases. Both the facial and cochlear nerves were anatomically preserved in all cases. Postoperatively, 7 patients (70%) remained in the AAO-HNS class A or B hearing category. None of the patients had new vestibular symptoms, and all had House-Brackmann grade 1 facial function. Nervus intermedius dysfunction was observed in 1 patient preoperatively, which worsened postoperatively. Two patients had new nervus intermedius symptoms postoperatively. CONCLUSION: Improvement of facial nerve and hearing outcomes is feasible through the intentional preservation of the vestibular nerves in the resection of small VSs. Longer follow-up is required to rule out tumor recurrence.

Cerebral Hyperperfusion Syndrome After a Burr Hole Drainage Surgery for Chronic Subdural Hematoma.

BACKGROUND: Although chronic subdural hematoma (CSDH) has a good prognosis after classical minimally-invasive drainage surgery, severe complications still occur at a substantial rate. Cerebral hyperperfusion syndrome (CHS), which is a common severe complication after carotid endarterectomy or carotid artery stenting for cervical carotid artery stenosis, is rare after drainage surgery for a CSDH. CASE DESCRIPTION: We describe the case of an 82-year-old woman who presented with ipsilesional symptoms including contralateral hemiparesis and dysarthria, progressively worsening consciousness, and status epilepticus after a burr hole drainage surgery for CSDH. Magnetic resonance fluid-attenuated inversion recovery imaging showed diffuse subcortical low intensity in the ipsilesional hemisphere almost simultaneously with the appearance of the symptoms. Arterial spin labeling magnetic resonance perfusion imaging showed the abnormal increase of cerebral blood flow in the hemisphere. Continuous propofol administration and blood pressure management improved the symptoms. CONCLUSIONS: CHS can cause severe postoperative complications after drainage surgery for CSDH. Subcortical low-intensity fluid-attenuated inversion recovery imaging is a useful investigation for early detection of CHS in CSDH, and arterial spin labeling imaging is an effective minimally-invasive modality for confirming the diagnosis.

Intracranial Infantile Myofibromatosis Mimicking Malignant Brain Tumor: A Case Report and Literature Review.

BACKGROUND: Myofibroma is a fibrous tumor of infancy that sometimes affects a single patient in a multiple fashion (infantile myofibromatosis). Its intracranial involvement is extremely rare, and its clinical picture has been poorly characterized. Here we report an interesting case of myofibromatosis with an intracranial lesion that behaved like an aggressive tumor and yet demonstrated very benign pathology. CASE DESCRIPTION: A 36-year-old man had never been diagnosed with infantile myofibromatosis despite his lifelong history of multiple tumors of various diagnoses. He presented with simple partial seizure and progressive right finger paresis. A series of brain magnetic resonance imaging scans revealed a rapidly growing lesion at his left frontal convexity, which corresponded to a high uptake area on a (18)F-fluorodeoxyglucose-positron emission tomography scan, highly suspicious of malignancy. He underwent complete tumor resection and his symptoms quickly resolved postoperatively. The pathological diagnosis was myofibroma with a MIB-1 labeling index of 1%-2%. A retrospective review of his previous tumors demonstrated the same pathology, which led to the diagnosis of myofibromatosis. Follow-up magnetic resonance imaging illustrated stabilization or regression of other preexisting lesions as well as formation of a new intracranial lesion. CONCLUSIONS: The discrepancy between rapid tumor growth associated with increased uptake on metabolic imaging and benign pathologic findings with a low proliferative index is noteworthy and should be recognized in the management of an intracranial lesion in a patient with infantile myofibromatosis. Given de novo formation of a lesion in this adult patient, long-term follow-up is essential in this disease.

Degradation of Stop Codon Read-through Mutant Proteins via the Ubiquitin-Proteasome System Causes Hereditary Disorders.

During translation, stop codon read-through occasionally happens when the stop codon is misread, skipped, or mutated, resulting in the production of aberrant proteins with C-terminal extension. These extended proteins are potentially deleterious, but their regulation is poorly understood. Here we show in vitro and in vivo evidence that mouse cFLIP-L with a 46-amino acid extension encoded by a read-through mutant gene is rapidly degraded by the ubiquitin-proteasome system, causing hepatocyte apoptosis during embryogenesis. The extended peptide interacts with an E3 ubiquitin ligase, TRIM21, to induce ubiquitylation of the mutant protein. In humans, 20 read-through mutations are related to hereditary disorders, and extended peptides found in human PNPO and HSD3B2 similarly destabilize these proteins, involving TRIM21 for PNPO degradation. Our findings indicate that degradation of aberrant proteins with C-terminal extension encoded by read-through mutant genes is a mechanism for loss of function resulting in hereditary disorders.

[Analysis of Rupture during Follow-up of Unruptured Aneurysm].

OBJECT: To elucidate clinical aspects of ruptured aneurysms, we retrospectively investigated associations between risk factors and ruptured and unruptured cases during conservative management. METHODS: Two hundred and twenty-nine patients with 291 unruptured cerebral aneurysms treated between 2000 and 2012 were analyzed. Mean duration of observation was 62 months (1183.4 person-years). We investigated the following six risk factors: history of subarachnoid hemorrhage;multiplicity;location of aneurysms;aneurysm size ≤5mm;bleb or irregular forms;and follow-up period <1 year. RESULTS: Twenty-two aneurysms in 22 patients (19 women;86.4%) ruptured during this study. The annual rate of rupture was 1.86%. In ruptured cases, mean age was 66.7 years. According to univariate analysis, aneurysm size≥5mm(p=0.000), bleb or irregular form(p=0.006)and duration of observation<1 year (p =0.000) were significantly associated with aneurysmal rupture. In multivariate analysis of these factors, aneurysm size≥5mm(p =0.0188;odds ratio(OR), 3.4;95% confidence interval (CI), 1.2-9.7) and duration of observation<1 year (p=0.006;OR, 5.0;95% CI, 1.6-14.9) represented independent risk factors for aneurysm rupture. CONCLUSIONS: The results of this study were almost the same as those of the UCAS Japan study. In addition, duration of observation <1 year was a risk factor for aneurysm rupture. When we decide on surgical treatment after considering factors such as aneurysm size, form, and surgical risk, surgery should be performed as soon as possible.

Effectiveness of intraarterial administration of fasudil hydrochloride for preventing symptomatic vasospasm after subarachnoid hemorrhage.

OBJECTIVE: We examined the effect of intraarterial administration of fasudil hydrochloride (IAFC), a Rho kinase inhibitor, for the prevention of symptomatic vasospasm after SAH by evaluating cerebral circulation. METHODS: We evaluated IAFC cases of 57 sides of 38 patients (12 men and 26 women, average age 60.2 years old) diagnosed with aneurysmal subarachnoid hemorrhage (SAH) from February 2012 to November 2012. All cases were treated by clipping or coil embolization within 48 h after onset. Indication for IAFC was the existence of a spastic change on follow-up digital subtraction angiography (DSA) compared with that of onset. RESULTS: Clipping was performed in 30 cases and coil embolization in 8 cases. IAFC was performed an average of 6.6 days after onset. Color gradient mapping demonstrated reduction of the circulation time after IAFC compared with before IAFC on 39 sides, no change on 15 sides, and extension on 3 sides. Average arterial circulation time before IAFC was 2.25 ± 0.57 s and after IAFC was 1.95 ± 0.55 s. IAFC significantly shortened average arterial circulation (P = 0.005). No case developed symptomatic vasospasm after IAFC. CONCLUSION: IAFC significantly reduced the cerebral circulation time after aneurysmal SAH and might be effective for the prevention of symptomatic vasospasm.

Subdural empyema due to Lactococcus lactis cremoris: case report.

Lactococcus lactis cremoris (L. lactis cremoris) infections are very rare in humans. Only three case reports of brain abscess have been reported and the infectious routes and pathological features are still unknown. We experienced a subdural empyema due to L. lactis cremoris in an immunocompetent adult. A 33-year-old man was admitted with fever, right facial pain, left hemiparesis, and left hemianopsia. Computed tomography demonstrated low density fluid collection in the right falcotentorial subdural space. Magnetic resonance (MR) images revealed a high signal lesion on a diffusion-weighted image (DWI) and fluid attenuated inversion recovery (FLAIR) images in the right paratentorial and parafalcine subdural space, right maxillary sinus, and bilateral ethmoidal sinus. He underwent two sequential open surgeries for removal and drainage of empyema and was treated with antibiotics including meropenem and ampicillin. To our knowledge, this is the first report of subdural empyema caused by L. lactis cremoris infection. We report the case and discuss the pathological features with the previous literature.

Brain metastasis from invasive thymoma mimicking intracerebral hemorrhage: case report.

A 55-year-old man with an 8-year history of invasive thymoma presented with sudden onset of left hemiparesis. Computed tomography (CT) and magnetic resonance (MR) imaging showed a right frontal lobe intracerebral hemorrhage and the possibility of brain metastasis could not be rejected. The patient underwent removal of the hematoma. Histological examination showed brain metastasis from invasive thymoma. To the best of our knowledge, this is the first reported case of hemorrhagic brain metastasis from invasive thymoma (non-cancerous) mimicking intracerebral hemorrhage.

Symptomatic vasospasm in elderly patients with aneurysmal subarachnoid hemorrhage: comparison with nonelderly patients.

OBJECTIVE: To clarify the influence of age on the occurrence of symptomatic vasospasm (SVS), we retrospectively compared 34 elderly (over 70 years) and 71 nonelderly patients with aneurysmal subarachnoid hemorrhage (SAH). METHODS: Between 2008 and 2010, at our hospital 105 patients (Hunt and Kosnik grades I-IV) underwent aneurysm surgery within 72 h of the insult. They were divided into four groups based on their age (younger/older than 70 years) and treatment (aneurysmal clipping or coiling). In all patients, we used the same protocol, which included the delivery of intrathecal urokinase and intravenous fasudil chloride; in patients with angiographic evidence of vasospasm, we also injected fasudil chloride intra-arterially. RESULTS: Among the elderly patients, 4.3% of those treated by clipping and 9.1% of those treated by coiling experienced SVS; the comparative incidence in younger patients was 6.5% and 4.0%, respectively. The differences were not statistically significant (p = 0.40). The ratio of ventriculo peritoneal (VP) shunts was higher in the elderly patients (p = 0.00007). The incidence of favorable treatment outcomes was significantly lower in elderly patients (p = 0.00004). CONCLUSION: Under our treatment protocol, patient age did not affect the incidence of SVS. Our protocol may be effective for the prevention of SVS after aneurysmal SAH regardless of patient age.

Endoscopic surgery for traumatic acute subdural hematoma.

Traumatic acute subdural hematoma (ASDH) is generally addressed by craniotomy under general anesthesia. We report a patient whose traumatic ASDH was treated under local anesthesia by one-burr-hole endoscopic surgery. This 87-year-old woman had undergone coil embolization for a ruptured right middle-cerebral artery aneurysm and placement of a ventriculoperitoneal shunt for normal pressure hydrocephalus 5 years earlier. Upon admission, she manifested consciousness disturbance after suffering head trauma and right hemiplegia. Her Glasgow Coma Scale score was 8 (E2V2M4). Computed tomography (CT) demonstrated a thick, left-frontotemporal ASDH. Due to her advanced age and poor condition, we performed endoscopic surgery rather than craniotomy to evacuate the ASDH. Under local anesthesia, we made a burr hole in her left forehead and increased its size to 15 mm in diameter. After introducing a transparent sheath into the hematoma cavity with a rigid endoscope, the clot was evacuated with a suction tube. The arterial bleeding point was electrically coagulated. A postoperative CT scan confirmed the reduction of the hematoma. There was neither brain compression nor brain swelling. Her consciousness disturbance and right hemiplegia improved immediately. Endoscopic surgery may represent a viable method to address traumatic intracranial hematomas in some patients.