A Spontaneous Extracranial Internal Carotid Artery Dissection with Autosomal Dominant Polycystic Kidney Disease: A Case Report and Literature Review.

Background and Objectives: Non-cystic manifestation of autosomal dominant polycystic kidney disease (ADPKD) is an important risk factor for cerebral aneurysms. In this report, we describe a rare spontaneous internal carotid artery (ICA) dissection in a patient with ADPKD. Observations: A 38-year-old woman with a history of ADPKD and acute myocardial infarction due to coronary artery dissection experienced severe spontaneous pain on the left side of her neck. Magnetic resonance imaging (MRI) revealed a severe left ICA stenosis localized at its origin. Carotid plaque MRI showed that the stenotic lesion was due to a subacute intramural hematoma. Close follow-up by an imaging study was performed under the diagnosis of spontaneous extracranial ICA dissection, and spontaneous regression of the intramural hematoma was observed uneventfully. Conclusions: When patients with a history of ADPKD present with severe neck pain, it is crucial to consider the possibility of a spontaneous ICA dissection. A carotid plaque MRI is beneficial in the differential diagnosis. Conservative management may benefit patients without ischemic symptoms.

[A Case of Dural Arteriovenous Fistula at the Craniocervical Junction with High Signal Intensity Localized to the Thoracic Spinal Cord on T2-weighted MRI].

An 82-year-old man presented with lower extremity weakness, paresthesia, and gait disturbance. At the previous hospital, spinal MRI had demonstrated a high-intensity area confined to the thoracic spinal cord on T2-weighted images and prominent dorsal venous flow voids that were suggestive of a thoracolumbar dural arteriovenous fistula(dAVF). Spinal digital subtraction angiography(DSA)failed to detect the shunt point. MRDSA of the head revealed a dAVF at the craniocervical junction(CCJ). Cranial DSA demonstrated feeders from the left vertebral artery and left radicular artery, a shunting point on the dura at the CCJ, and drainers into the anterior and posterior spinal veins descending to the sacral level. Surgery improved his symptoms, and the abnormal imaging findings were resolved. Observation of a dAVF at the CCJ with myelopathy is rare, and the diagnosis of this type of dAVF might be challenging. MRDSA might be a useful tool for detecting this uncommon dAVF.

[Open Surgery Following Transarterial Embolization for Cavernous Sinus Dural Arteriovenous Fistula].

Cavernous sinus(CS)dural arteriovenous fistula with cortical venous drainage often have a malignant presentation and require urgent treatment. Endovascular embolization is the primary treatment of choice, but failure to catheterize the CS may occur due to vessel tortuosity, hypoplasia, or stenosis. In the absence of venous access to the lesion, a careful surgical approach should be considered. Here we describe a patient who underwent open surgery following an unsuccessful transvenous embolization due difficulties in venous access.

[Middle Cerebral Artery Fenestration with M2 Fusion Detected during Aneurysm Clipping:A Case Report].

We report a unique case of middle cerebral artery fenestration with M2 fusion detected during an aneurysm clipping. A 72-year-old woman was diagnosed with an unruptured aneurysm of the right middle cerebral artery, and middle cerebral artery fenestration with M2 fusion was detected during the aneurysm clipping surgery. It was difficult to identify this unusual vascular anomaly before the surgery. Therefore, neurosurgeons should be cautious of such hidden vascular anomalies and be prepared for the possible complications during surgery.

[A Case of Moyamoya Disease Associated with SAPHO Syndrome].

Moyamoya disease is a unique occlusive disease of the internal carotid artery(ICA)with moyamoya vessels that can lead to transient ischemic attacks and hemorrhagic stroke. When other inherited or acquired disorders and conditions occur in conjunction with moyamoya disease, the syndrome is known as quasi-moyamoya disease. We report the case of a 34-year-old woman with a past history of SAPHO(Synovitis-Acne-Pustulosis-Hyperostosis-Osteomyelitis)syndrome, who presented with arm weakness and headache. Magnetic resonance angiography revealed severe terminal stenosis of the left ICA with moyamoya vessels, and she was diagnosed as moyamoya disease associated with SAPHO syndrome. She underwent superficial temporal artery-middle cerebral artery anastomosis and her arm weakness improved postoperatively. Postoperative course was uneventful, although she showed transient right paresthesia, which improved with conservative therapy. Autoimmune response could contribute to the development of this moyamoya disease, and we discuss its mechanism with a literature review.

[Internal carotid artery stenosis with repeated ischemic stroke recurrence due to mechanical stimulation by the hyoid bone and thyroid cartilage: a case report].

We present a case of internal carotid artery (ICA) stenosis caused by mechanical stimulation by the hyoid bone (HB) and thyroid cartilage (TC). A 78-year-old man with a history of right ICA stenting four years previously was admitted for abrupt onset of dysarthria and left hemiparesis and diagnosed with ischemic stroke by magnetic resonance imaging. Three-dimensional computed tomographic angiography revealed internal carotid in-stent restenosis. Furthermore, the HB and TC contacted with the right ICA. Treatment involved antiplatelet therapy, partial HB and TC resection, and carotid artery restenting. Posttreatmently, the ICA was restored and stenosis improved. Since restenosis may occur posttreatmently in patients with carotid artery stenosis caused by mechanical stimulation of the HB and TC, it is necessary to consider treatments including not only carotid artery stenting but also partial bone structures resection and carotid endarterectomy.

Ruptured basilar artery perforator aneurysm: a novel mechanism of pure subarachnoid hemorrhage in moyamoya disease. Illustrative case.

BACKGROUND: Pure subarachnoid hemorrhage (SAH) in patients with moyamoya disease is a rare occurrence. Three underlying mechanisms have been described previously, except for ruptured aneurysm of the circle of Willis. Herein, the authors describe a novel mechanism: rupture of a perforator aneurysm in moyamoya disease. OBSERVATIONS: A 51-year-old man experienced sudden onset of severe headache and vomiting. Computed tomography showed diffuse SAH. Digital subtraction angiography (DSA) showed unilateral moyamoya disease without remarkable etiology of SAH. The patient underwent conservative management with antihypertensive agents. The second DSA on day 17 revealed a slow-filling aneurysm emerging from the basilar top perforating artery. The diagnosis of SAH due to unknown origin was changed to ruptured basilar artery perforator aneurysm (BAPA). The third follow-up DSA on day 159 revealed the resolution of BAPA. LESSONS: In the case of pure SAH, it is crucial to consider the possibility of perforator aneurysms due to hemodynamic stress caused by moyamoya disease. Repeated DSA is essential for detecting the lesion.

Impact of Pre-operative Embolization With Onyx for Brain Arteriovenous Malformation Surgery.

Objective: To clarify the safety and efficacy of pre-operative embolization using Onyx liquid embolic agent (Onyx; ev3) compared with N-butyl cyanoacrylate (NBCA; Cordis Neurovascular, Inc.) or coils in cerebral arteriovenous malformation (AVM) surgery. Methods: This was a retrospective review of a prospectively collected clinical database of brain AVMs treated at our institute from January 2005 to March 2021. A total of 38 consecutive patients who underwent AVM resection after pre-operative embolization were included. Based on pre-operative embolization materials, the patients were divided into the pre-Onyx group (n = 16), in which NBCA or coils were used for embolization, and the Onyx group (n = 22). Patient characteristics and treatment results were compared between the two groups. Results: Patient characteristics were comparable between the two groups in terms of age, sex, and rupture status. While the Spetzler-Martin grade was also similar between the two groups, the location of the AVM nidus in the eloquent area was slightly higher in patients in the Onyx group (72.7%) than in patients in the pre-Onyx group (43.8%) (P = 0.09). The embolization rate was higher in the pre-Onyx group (mean: 63.0%; range: 12.7-100%) than in the Onyx group (mean: 50.0%; range: 15.8-100%), but the difference was not statistically significant (P = 0.06). The time needed for surgical removal was shorter in the Onyx group (mean: 354.8 min; range: 144-884 min) than in the pre-Onyx group (mean: 457.9 min; range: 240-1,294 min); however, this difference was not statistically significant (P = 0.13). The amount of intraoperative bleeding was significantly lower in the Onyx group (mean: 129.8 ml; range: 20-540 mL) than in the pre-Onyx group (mean: 448.8 mL; range: 120-1,550 ml) (P = 0.0008). The surgical complication rates were comparable between the two groups (pre-Onyx group, 18.8%; Onyx group, 4.5%; P = 0.29). Conclusions: Pre-operative embolization with Onyx can significantly reduce the amount of intraoperative bleeding in AVM resection and may contribute to safe AVM surgery.

Hypointensity of draining veins on susceptibility-weighted magnetic resonance images might indicate normal venous flow and a lower risk of intracerebral hemorrhage in patients with intracranial arteriovenous shunt(s).

Patients with intracranial arteriovenous shunt(s) have a risk of intracerebral hemorrhage (ICH). We investigated the signal intensity of draining veins on susceptibility-weighted imaging (SWI) and the status of venous drainage shown by digital subtraction angiography (DSA). We then evaluated whether the signal intensity of draining veins on SWI is related to normal venous flow (NVF) and/or ICH. We analyzed SWI and DSA in 10 consecutive patients with intracranial arteriovenous shunt(s). Opacification of draining veins in the normal venous phase by DSA was judged as NVF. We evaluated the relationship between the intensity of draining veins on SWI and the presence of NVF before and after treatment. The relationship between the intensity of draining veins on SWI and the presence of ICH surrounding the draining veins was also evaluated. Of 10 patients with untreated arteriovenous shunt(s), two had arteriovenous malformation and eight had a dural arteriovenous fistula with cortical venous reflux. We analyzed 26 draining veins before treatment. In preoperative analysis, draining veins with hypointensity were significantly more likely to show NVF than were draining veins with isointensity or hyperintensity (45.5% vs. 0.0%, P = 0.007). While 69.2% of the areas surrounding draining veins with isointensity or hyperintensity showed ICH, no veins with hypointensity showed ICH (P = 0.011, odds ratio 0.036; 95% confidence interval 0.0017-0.80). In conclusion, draining veins with hypointensity on SWI may contain NVF, despite arteriovenous shunting. The areas surrounding these veins might have a lower risk of ICH because of less venous hypertension.

Impact of Mechanical Thrombectomy Device on Thrombus Histology in Acute Embolic Stroke.

BACKGROUND: Although many studies have evaluated the retrieved thrombus to assess the cause of stroke after mechanical thrombectomy for acute large vessel occlusion, the results remain controversial. We investigated the hypothesis that histology of the retrieved thrombus is enhanced by mechanical thrombectomy devices. METHODS: Thrombi were collected from consecutive patients who had undergone endovascular mechanical recanalization for large intracranial vessel occlusion. The mechanical thrombectomy device used was either an aspiration catheter or a stent retriever. The hematoxylin and eosin-stained specimens were quantitatively analyzed with respect to the relative fractions of the main constituents (erythrocytes and fibrin). Clinical and radiologic findings were also evaluated. RESULTS: Of 65 patients, an aspiration catheter was used in 27, and a stent retriever was used in 38. The presence of a preoperative susceptibility vessel sign on magnetic resonance imaging was not correlated with the percentage of erythrocytes. Thrombus cross-sectional area was larger in the aspiration group than in the stent group (P < 0.01). Conversely, the percentage of the fibrin component was higher in the stent group (P < 0.001). Preoperative intravenous administration of recombinant tissue plasminogen activator reduced thrombus cross-sectional area with a trend of increased percentage of fibrin and reduced percentage of erythrocyte in the stent group. CONCLUSIONS: Histologic differences in retrieved thrombi are enhanced by mechanical thrombectomy devices. Stent retrievers may crush the thrombus, which may have a synergistic effect with recombinant tissue plasminogen activator. Histology of the retrieved thrombi might be different from histology of the original thrombi.

Impact of basal ganglia damage after successful endovascular recanalization for acute ischemic stroke involving lenticulostriate arteries.

OBJECTIVE: Regional ischemic vulnerability of the brain reportedly differs between the cortex and basal ganglia and has been poorly assessed in the setting of endovascular mechanical thrombectomy. This study was conducted to determine the fate of an ischemic basal ganglia and its contribution to the clinical outcome after successful endovascular recanalization for acute ischemic stroke with large vessel occlusion involving the lenticulostriate arteries. METHODS: Clinical and radiological findings were retrospectively analyzed in consecutive patients with acute ischemic stroke characterized by large vessel occlusion involving the lenticulostriate arteries. Mechanical thrombectomy was performed in all patients using a stent retriever. The fate of ischemic basal ganglia based on location (lentiform nucleus, caudate nucleus, and internal capsule) and insular cortex was assessed according to the Alberta Stroke Programme Early CT Score (ASPECTS). RESULTS: Of 170 patients with large intracranial vessel occlusion who achieved successful endovascular recanalization, defined as a thrombolysis in cerebral infarction grade of ≥ 2B, involvement of the lenticulostriate arteries was seen in 55 patients (internal carotid artery, n = 35; proximal middle cerebral artery, n = 20). Preoperative infarction was detected in the lentiform nucleus (66.7%), internal capsule (11.1%), and caudate nucleus (33.3%), all of which showed secondary advancement despite successful recanalization (85.4%, 27.3%, and 54.5%, respectively; p < 0.05). Lenticulostriate arteries with a lateral proximal and/or medial proximal origin significantly affected the development of mature infarction in the lentiform nucleus. Postoperative hemorrhagic transformation was detected in 25 of 55 patients, mostly in the lentiform nucleus. Involvement of insular ribbon infarction was significantly high in patients with hemorrhagic transformation in the basal ganglia. Age, initial National Institutes of Health Stroke Scale (NIHSS) score, initial ASPECTS, postoperative ASPECTS, postoperative infarction in the insular ribbon, and lesions in the middle cerebral artery area (M1-M6) were significantly different between patients with good and poor modified Rankin Scale scores. Interestingly, no differences were detected in postoperative infarction or hemorrhagic transformation in the basal ganglia. Multivariate analysis showed that only age (p = 0.02, OR 0.88) and the initial NIHSS score (p = 0.01, OR 0.86) independently affected favorable clinical outcomes. CONCLUSIONS: The basal ganglia are vulnerable and readily develop secondary infarction and hemorrhagic transformation despite successful recanalization. However, this does not have a significant impact on the clinical outcome of acute ischemic stroke with large vessel occlusion involving the lenticulostriate arteries.

The Cross-Sectional Area of Paraspinal Muscles Predicts the Efficacy of Deep Drain Stimulation for Camptocormia.

BACKGROUND: Camptocormia, a disturbance of posture, is a well-described clinical feature of PD and other parkinsonian syndromes. Previous reports have shown that DBS of the subthalamic nucleus (STN) or globus pallidus internus is effective in treating camptocormia. However, the efficacy of DBS for camptocormia varies. OBJECTIVE: To determine a clinical marker for selecting an appropriate therapy for camptocormia, a disabling manifestation of Parkinson's disease (PD) that has a variable response to systemic and local therapies. METHODS: We obtained pre-operative lumbar magnetic resonance imaging of 14 consecutive PD patients with camptocormia who underwent subthalamic nucleus deep brain stimulation (STN-DBS) in this retrospective-designed study. Lumbar MRI was performed three to six months prior to the operation. We measured the cross-sectional area (CSA) and width of each participant's paraspinal muscles. RESULTS: Four (28.6%) patients were effective (EF), five (35.7%) were partially effective (PE), and five (35.7%) were non-effective (NE) to STN-DBS. The lumbar paraspinal CSA and width were significantly larger in the EF group than in the PE and NE groups. CONCLUSIONS: The CSA of paraspinal muscles and erector spinae width can be good predictive markers for improving camptocormia in patients with PD after deep brain stimulation.

De Novo Vertebral Artery Dissection after Endovascular Trapping for Ruptured Dissecting Internal Carotid Artery Aneurysm: Case Report.

The authors present an extremely rare case of a 54-year-old female patient with subarachnoid hemorrhage due to a rupture of a dissecting internal carotid artery (ICA) aneurysm, who developed de novo vertebral artery dissection in the spasm period after endovascular trapping of the ICA. Interestingly, postoperative cardiopulmonary monitoring showed high global end-diastolic volume index and mean arterial pressure, which could contribute to this de novo dissection via hemodynamic stress in the cerebral circulation. Spontaneous intracranial artery dissection of more than two arteries is rare, and we believe this is the first case of de novo dissection occurring on a circulating vessel different from that of the initial dissection. The clinical implications are discussed in relation to postoperative hemodynamic stress with a review of the literature.