Eagle jugular syndrome accompanied by de novo brainstem cavernous malformation: a case-based systematic review.

BACKGROUND: Eagle jugular syndrome (EJS), recently identified as a cause of cerebrovascular disease (CVD) due to venous obstruction by an elongated styloid process (SP), is reported here alongside a case of concurrent de novo cerebral cavernous malformation (CCM). This study aims to explore the potential causal relationship between EJS and de novo CCM through a comprehensive literature review. METHOD: Systematic literature reviews, spanning from 1995 to 2023, focused on EJS cases with definitive signs and symptoms and de novo CCM cases with detailed clinical characteristics. Data on the pathophysiology and clinical manifestations of EJS, as well as potential risk factors preceding de novo CCM, were collected to assess the relationship between the two conditions. RESULT: Among 14 patients from 11 articles on EJS, the most common presentation was increased intracranial hypertension (IIH), observed in 10 patients (71.4%), followed by dural sinus thrombosis in four patients (28.6%). In contrast, 30 patients from 28 articles were identified with de novo CCM, involving 37 lesions. In these cases, 13 patients developed CCM subsequent to developmental venous anomalies (43%), seven following dural arteriovenous fistula (dAVF) (23%), and two after sinus thrombosis (6%). In a specific case of de novo brainstem CCM, the development of an enlarged condylar emissary vein, indicative of venous congestion due to IJV compression by the elongated SP, was noted before the emergence of CCM. CONCLUSION: This study underscores that venous congestion, a primary result of symptomatic EJS, might lead to the development of de novo CCM. Thus, EJS could potentially be an indicator of CCM development. Further epidemiological and pathophysiological investigations focusing on venous circulation are necessary to clarify the causal relationship between EJS and CCM.

Comparison Between Cz-C3/C4 and C3-C4 Montages to Protect Against Peripheral Stimulation in Transcranial Facial Motor-Evoked Potential Monitoring.

INTRODUCTION: In facial motor-evoked potential monitoring, efforts to reduce peripheral stimulation are necessary because it can cause false-negatives. The effects of peripheral stimulation on Cz-C3/C4 and C3-C4 montages were compared. METHODS: Facial motor-evoked potentials were recorded from bilateral orbicularis oculi (Oculi) and oris (Oris) muscles. The double-train approach combining single-pulse and five-train pulse stimulation was used to determine the effect of peripheral stimulation. If the five-train pulse produced a significant waveform, it was defined as "total success." In total success cases, "true success" was defined as a case in which no waveform appeared after the single pulse at the threshold level of the five-train pulse. The total and true success rates and the threshold value of Oculi and Oris were compared between Cz-C3/C4 and C3-C4 montages. RESULTS: Thirty-six muscles each of Oculi and Oris of 18 patients were used for the analysis. True success was more likely to be obtained by the Cz-C3/C4 montage than the C3-C4 montage in Oculi (42% vs. 22%, p = 0.039). Both Oculi and Oris had higher thresholds to elicit facial motor-evoked potentials with the Cz-C3/C4 montage (Oculi: 101.7 vs. 71.4 mA, p = 0.038; Oris: 94.8 vs. 73.1 mA, p = 0.016). CONCLUSIONS: Cz-C3/4 montage is more effective at reducing peripheral stimulation compared with the C3-4 montage. This effect was primarily seen in the orbicularis oculi muscle. It should be noted that the Cz-C3/C4 montage has a higher threshold than the C3-C4 montage in facial muscles. In facial motor-evoked potential monitoring, the Cz-C3/C4 montage may be more suitable to eliminate peripheral stimulation.

Case report: Usefulness of angioscopy in determining antiplatelet drug reduction after carotid artery stenting.

We report a case in which neointima was confirmed by angioscopy and antiplatelet drug administration was reduced 2 months after carotid artery stenting (CAS). A patient in their 80s was scheduled to undergo resection for renal cancer; however, he also had right cervical internal carotid artery stenosis. Because this was a risk for general anesthesia, CAS was performed after first starting dual antiplatelet therapy. Urologically, early reduction of antiplatelet drugs was necessary for a nephrectomy. Although no obvious neointima could be identified on ultrasound 2 months after CAS, thin neointima was observed using angioscopy. Based on the above results, we reduced the antiplatelet drug administration, and then the nephrectomy was performed. Ultimately, no cerebral infarction occurred in the perioperative or postoperative periods. Angioscopy allows for visual confirmation of thin neointima. If sufficient neointima can be confirmed, antiplatelet drug reduction can be performed more safely and reliably.

Coil Embolization of Unruptured Distal Anterior Cerebral Artery Aneurysm Using a Marathon Microcatheter.

Marathon is rarely used in coil embolization for an aneurysm; particularly, there have been no reports about distal anterior cerebral artery aneurysms. We have reported a case of successful use of Marathon in coil embolization in case of a distal anterior cerebral artery aneurysm. The patient was an 83-year-old woman. She had undergone coil embolization for an unruptured distal anterior cerebral artery aneurysm, which was discovered by chance. Our initial approach involved the use of a combination of Traxcess and Excelsior SL-10, but the use of SL-10 could not follow Traxcess because the right anterior cerebral artery from the right internal carotid artery had a sharp bifurcation. However, by switching to a combination of TENROU and Marathon, we could access the aneurysm. We thereby decided to continue the use of Marathon in order to complete the coil embolization. In coil embolization for an aneurysm, Marathon was found to be useful, depending on the location of the aneurysm and access route.

A Novel Approach for Transvenous Embolization of Dural Arteriovenous Fistula Using a Balloon and a Coil as Walls: Case Presentation.

BACKGROUND: Transvenous embolization (TVE) for dural arteriovenous fistula (DAVF) is difficult depending on an accessible route. Reported herein is a case of transvenous embolization using a balloon and a coil as "walls." Case Description. A 56-year-old male patient presented with a 1-month history of mild motor aphasia. The magnetic resonance imaging showed a hemorrhagic lesion in his left temporal lobe, and the cerebral angiography showed a DAVF, with parasinus shunt points near the torcula and the left transverse sinus. Access to the shunt point was very difficult; however, TVE was performed using a balloon as a wall. Furthermore, all lesion embolization was possible using a coil as a wall. CONCLUSIONS: Using a balloon or coil as a wall during a TVE is useful.

Bilateral Magnetic Resonance Imaging-Guided Focused Ultrasound Thalamotomy for Essential Tremor.

INTRODUCTION: Essential tremor is the most common movement disorder in adults. Bilateral symptoms are typical; however, bilateral thalamotomy for essential tremor is associated with a high probability of adverse events. We retrospectively investigated the efficacy and safety of staged bilateral ventral intermediate nucleus thalamotomy for refractory essential tremor using magnetic resonance imaging-guided focused ultrasound. METHODS: We enrolled 5 consecutive patients with refractory essential tremor between September 2016 and March 2020. Patients underwent a second operation at least 1 year after the first operation. The second lesion was created asymmetrically to the first lesion. RESULTS: Mean patient age was 57.6 years, and the mean interval between the 2 operations was 27.8 months. The second lesion center was superior to the first lesion in all patients. The mean baseline, second preoperative, and second postoperative Clinical Rating Scale for Tremor total scores were 63.6, 49.2, and 21.8, respectively. The mean baseline, second preoperative, and second postoperative Clinical Rating Scale for Tremor part C scores were 18.4, 8.2, and 2.6, respectively. One patient had permanent adverse events of slight dysarthria and discomfort in the tongue. No patient experienced dysphagia or cognitive dysfunction after the second operation. Four of the 5 patients were satisfied with the results of the bilateral treatment, including the patient who had permanent adverse events. CONCLUSIONS: Magnetic resonance imaging-guided focused ultrasound is an effective method for bilateral thalamotomy when adhering to the following considerations: (1) asymmetrical lesions are created and (2) sufficient interval (>1 year) between operations.

Cerebral vasospasm after coil embolization for unruptured anterior communicating artery aneurysm: illustrative case.

BACKGROUND: Compared with several reports of cerebral vasospasm after clipping for unruptured cerebral aneurysm, only one study to date has reported cerebral vasospasm after coil embolization. Herein, the authors report a rare case of cerebral vasospasm after coil embolization for unruptured cerebral aneurysm. OBSERVATIONS: A 58-year-old woman with an unruptured anterior communicating artery aneurysm was referred to our department. Stent-assisted coil embolization was performed for the aneurysm, and no obvious adverse events were observed on cerebral angiography obtained immediately after the operation. However, the patient developed mild headache and slight restlessness soon after the operation and new-onset disorientation, left hemispatial neglect, and left hemiplegia the day after the operation. Emergency brain magnetic resonance imaging and cerebral angiography indicated vasospasm in the right middle cerebral artery, and intra-arterial injection of fasudil hydrochloride hydrate was performed to dilate the middle cerebral artery. Blood flow in the middle cerebral artery immediately improved, and she was discharged without neurological deficits 8 days after the operation. LESSONS: Immediate intervention is necessary to prevent cerebral infarction in patients with cerebral vasospasm, which may occur even after coil embolization for unruptured cerebral aneurysm.

Focused Ultrasound Thalamotomy for Refractory Essential Tremor: A Japanese Multicenter Single-Arm Study.

BACKGROUND: Several feasibility studies and a randomized, controlled, multicenter trial have demonstrated the safety and efficacy of unilateral transcranial magnetic resonance-guided focused ultrasound (FUS) lesioning of the ventral intermediate thalamic nucleus in treating essential tremor. OBJECTIVE: To evaluate the safety and efficacy of FUS thalamotomy in a Japanese patient cohort through a prospective, multicenter, single-arm confirmatory trial. METHODS: A total of 35 patients with disabling refractory essential tremor underwent unilateral FUS thalamotomy and were followed up for 12 post-treatment months. Safety was measured as the incidence and severity of treatment-related adverse events. Efficacy was measured as the tremor severity and quality of life improvements using the Clinical Rating Scale for Tremor and Questionnaire for Essential Tremor. RESULTS: The mean skull density ratio (SDR) was 0.47. There was a significant decrease in the mean postural tremor score of the treated hand from baseline to 12 mo by 56.4% (95% CI: 46.7%-66.1%; P < .001), which was maintained at last follow-up. Quality of life improved by 46.3% (mean overall Questionnaire for Essential Tremor score of 17.4 [95% CI: 12.1-22.7]) and there were no severe adverse events. The most frequent adverse event was gait disturbance and all events resolved. CONCLUSION: Unilateral FUS thalamotomy allowed significant and sustained tremor relief and improved the quality of life with an outstanding safety profile. The observed safety and efficacy of FUS thalamotomy were comparable to those reported in a previous multicenter study with a low SDR, and inclusion of the low SDR group did not affect effectiveness.

4-Hydroxyl-2-Nonenal Localized Expression Pattern in Retrieved Clots is Associated with Large Artery Atherosclerosis in Stroke Patients.

OBJECTIVES: The relationship between stroke etiology and clot pathology remains controversial. MATERIALS AND METHODS: We performed histological analysis of clots retrieved from 52 acute ischemic stroke patients using hematoxylin and eosin staining and immunohistochemistry (CD42b and oxidative/hypoxic stress markers). The correlations between clot composition and the stroke etiological group (i.e., cardioembolic, cryptogenic, or large artery atherosclerosis) were assessed. RESULTS: Of the 52 clots analyzed, there were no significant differences in histopathologic composition (e.g., white blood cells, red blood cells, fibrin, and platelets) between the 3 etiological groups (P = .92). By contrast, all large artery atherosclerosis clots showed a localized pattern with the oxidative stress marker 4-hydroxyl-2-nonenal (P < .01). From all 52 clots, 4-hydroxyl-2-nonenal expression patterns were localized in 28.8% of clots, diffuse in 57.7% of clots, and no signal in 13.5% of clots. CONCLUSIONS: A localized pattern of 4-hydroxyl-2-nonenal staining may be a novel and effective marker for large artery atherosclerosis (sensitivity 100%, specificity 82%).

Feasibility and Efficacy of a 9-Fr Balloon-Guiding Catheter Sheathless Insertion to Reduce Access Site Complications During Mechanical Thrombectomy for Acute Ischemic Stroke.

OBJECTIVE: A 9-French (Fr) sheath is routinely used during mechanical thrombectomy (MT) for treating acute ischemic stroke (AIS). However, the use of a large sheath is a risk factor for access site complications (ASCs). Previous studies focused on preventing intracranial complications, and only a few have explored ASCs. We investigated the technical feasibility and efficacy of a sheathless procedure for AIS (SPAIS) that uses a 9-Fr balloon-guiding catheter as a guiding sheath to reduce the sheath size and prevent ASCs during MT. METHODS: We retrospectively analyzed the data of 133 patients who underwent MT at our center. Patients treated between January 2015 and August 2017 received conventional treatment (C) using a 9-Fr sheath, and SPAIS was attempted in patients treated between September 2017 and October 2019. We first assessed the technical feasibility of SPAIS, and subsequently compared the incidence of ASCs between the SPAIS and C groups. Routine postsurgical ASC assessments using duplex ultrasonography were performed during the post-MT bed-rest period. RESULTS: The technical success rate of SPAIS was 97.6% (81 of 83 patients). The incidence of ASCs was significantly lower in the SPAIS group (2 of 81, 2.5%) than in the C group (7 of 52, 13.4%) (P < 0.05). Moreover, developed pseudoaneurysms in the SPAIS group showed significantly faster hemostasis than those in the C group (mean 20 minutes vs. 32 minutes; P < 0.05). CONCLUSIONS: SPAIS is a feasible technique that effectively reduces MT-associated ASCs; thus, this approach should be adopted to improve patient outcomes.

What factors impact the clinical outcome of magnetic resonance imaging-guided focused ultrasound thalamotomy for essential tremor?

OBJECTIVE: Magnetic resonance imaging-guided focused ultrasound (MRgFUS) is a novel and useful treatment for essential tremor (ET); however, the factors impacting treatment outcome are unknown. The authors conducted this study to determine the factors affecting the outcome of MRgFUS. METHODS: From May 2016 through August 2017, 15 patients with ET were admitted to Ohnishi Neurological Center and treated with MRgFUS. To determine the factors impacting treatment outcome, the authors retrospectively studied correlations between the Clinical Rating Scale for Tremor (CRST) improvement rate and age, disease duration, baseline CRST score, skull density ratio (SDR), skull volume, maximum delivered energy, or maximum temperature. RESULTS: The mean CRST score was 18.5 ± 5.8 at baseline and 4.6 ± 5.7 at 1 year. The rate of improvement in the CRST score was 80% ± 22%. Younger age and lower baseline CRST score were correlated with a higher CRST improvement rate (p = 0.025 and 0.007, respectively). To obtain a CRST improvement rate ≥ 50%, a maximum temperature ≥ 55°C was necessary. There was no correlation between SDR and CRST improvement rate (p = 0.658). A lower SDR and higher skull volume required significantly higher maximum delivered energy (p = 0.014 and 0.016, respectively). A higher maximum temperature was associated with a significantly larger lesion volume (p = 0.026). CONCLUSIONS: Younger age and lower baseline CRST score were favorable outcome factors. It is important to assess predictive factors when applying MRgFUS.

Resolution of Trigeminal Neuralgia After Transvenous Embolization of a Cavernous Sinus Dural Arteriovenous Fistula.

BACKGROUND: Trigeminal neuralgia (TN) resulting from a dural arteriovenous fistula (DAVF) are rare. A case of cavernous sinus dural arteriovenous fistula (CSDAVF) in a patient with TN that resolved immediately after transvenous embolization (TVE) is described. CASE DESCRIPTION: A 75-year-old woman presented with continuous facial pain. On time-of-flight magnetic resonance angiography (MRA), a CSDAVF was suspected, and digital subtraction angiography (DSA) showed a CSDAVF. The TN completely resolved immediately after TVE, with no recurrence. Follow-up MRA showed complete obliteration of the fistula 13 months after TVE. CONCLUSIONS: The pulsatile flow at the posteromedial part of the cavernous sinus might have compressed the upper aspect of the Gasserian ganglion, causing TN. Disappearance of pulsatile flow after TVE might result in complete resolution of TN.

Cerebral and spinal cord tanycytic ependymomas in a young adult with a mutation in the NF2 gene.

We studied one frontal lobe tumor and multiple spinal cord tumors (one in an extramedullary location) that had been resected from a 24-year-old man. The frontal lobe tumor was well demarcated and non-infiltrating, and consisted of eosinophilic, elongated fibrillary cells arranged in a fascicular pattern. A similar histology was reproduced in the spinal cord tumors, with additional areas showing standard features of ependymoma. Immunohistochemical and ultrastructural observations revealed that all the tumors were ependymal in nature with positivity for GFAP and epithelial membrane antigen and negativity for oligodendrocyte transcription factor 2, showing intra- and intercellular microrosettes, leading us to a diagnosis of tanycytic ependymoma for the frontal lobe tumor and tanycytic ependymoma with ordinary ependymomatous component for the spinal cord tumors. The spinal extramedullary tumor was a schwannoma. Importantly, a heterozygous truncating mutation in the NF2 gene was identified in the blood lymphocytes from the patient. It is known that multiple nervous system tumors can occur in neurofibromatosis type 2 (NF2), which is caused by mutation in the NF2 gene, and that occurrence of ependymoma, including the tanycytic variant, can be associated with this genetic condition. The present case provides further information about the clinicopathology of tanycytic ependymoma with details of the immunohistochemical, ultrastructural and genetic features.