Impact of immediate general anesthesia in the emergency room on prevention of rebleeding after subarachnoid hemorrhage.

BACKGROUND: Aneurysm rebleeding is fatal in patients with aneurysmal subarachnoid hemorrhage (aSAH). We aimed to investigate whether immediate general anesthesia (iGA) management in the emergency room, upon arrival, prevents rebleeding after admission and reduces mortality following aSAH. METHODS: The clinical data of 3033 patients with World Federation of Neurosurgical Societies (WFNS) grade 1, 2, or 3 aSAH from the Nagasaki SAH Registry Study between 2001 and 2018 were retrospectively analyzed. iGA was defined as sedation and analgesia using intravenous anesthetics and opioids combined with intubation induction. We calculated crude and adjusted odds ratios to evaluate the associations between iGA and the risk of rebleeding/death using multivariable logistic regression models with fully conditional specification for multiple imputations. In the analysis of the relationship between iGA and death, we excluded patients with aSAH who died within 3 days after the onset of symptoms. RESULTS: Of the 3033 patients with aSAH who met the eligibility criteria, 175 patients (5.8%) received iGA (mean age, 62.4 years; 49 were male). Heart disease, WFNS grade, and lack of iGA were independently associated with rebleeding in the multivariable analysis with multiple imputations. Among the 3033 patients, 15 were excluded due to death within 3 days after the onset of symptoms. After excluding these cases, our analysis revealed that age, diabetes mellitus, history of cerebrovascular disease, WFNS grade, Fisher grade, lack of iGA, rebleeding, postoperative rebleeding, no shunt operation, and symptomatic spasm were independently associated with mortality. CONCLUSIONS: Management by iGA was associated with a 0.28-fold decrease in the risks of both rebleeding and mortality in patients with aSAH, even after adjusting for the patient's history of diseases, comorbidities, and aSAH status. Thus, iGA can be a treatment for the prevention of rebleeding before aneurysmal obliteration treatment.

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.

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.

Ruptured aneurysm-induced pituitary apoplexy: illustrative case.

BACKGROUND: Pituitary apoplexy associated with aneurysmal rupture is extremely rare and may be misdiagnosed as primary pituitary adenoma apoplexy. The authors present a case of a patient with pituitary apoplexy caused by rupture of an anterior cerebral artery aneurysm embedded within a giant pituitary adenoma, and they review the relevant literature. OBSERVATIONS: A 78-year-old man experienced sudden headache with progressive vision loss. Magnetic resonance imaging (MRI) revealed a giant pituitary tumor with abnormal signal intensity. Magnetic resonance angiography immediately before surgery showed a right A1 segment aneurysm, suggesting coexisting pituitary apoplexy and ruptured aneurysm. The patient underwent urgent transsphenoidal surgery for pituitary apoplexy. The tumor was partially removed, but the perianeurysmal component was left behind. Subsequent cerebral angiography showed a 5-mm right A1 aneurysm with a bleb that was successfully embolized with coils. Retrospective review of preoperative dynamic MRI showed extravasation of contrast medium from the ruptured aneurysm into the pituitary adenoma. Histopathologic examination showed gonadotroph adenoma with hemorrhagic necrosis. Postoperatively, the patient's visual function improved. LESSONS: MRI identification of pituitary apoplexy caused by aneurysmal rupture has not been reported previously. Aneurysmal rupture should be considered in the differential diagnosis of pituitary apoplexy. When a ruptured aneurysm is encountered, the authors recommend treating it before addressing pituitary apoplexy.

Vasospasm and Hydrocephalus Following Subarachnoid Hemorrhage Are Less Frequent in Coil Embolization Than in Clipping.

Objective: The effects of treatment methods for ruptured aneurysms on the incidence of vasospasm and normal pressure hydrocephalus (NPH) following subarachnoid hemorrhage (SAH) are controversial. We retrospectively examined the Nagasaki SAH registry data, and the complication rates of symptomatic vasospasm and NPH were analyzed based on the treatment methods. Methods: Between January 2015 and December 2017, 800 SAH patients were registered from 18 hospitals, and their age, sex, World Federation of Neurological Societies (WFNS) grade, Fisher group, size and location of cerebral aneurysms, treatment methods, incidence of symptomatic vasospasm and shunt-dependent hydrocephalus, and prognosis (discharge or 3 months later) were retrospectively analyzed. The effects of treatment methods for the ruptured aneurysm on the incidence of symptomatic vasospasm and shunt-dependent hydrocephalus were then statistically analyzed. Results: The mean age was 66.2 years old. There were 245 (30.6%) male patients and 555 (69.3%) female patients. Cerebral aneurysms were identified in 708 patients (87.5%) and surgical treatments were performed for 620. Neck clipping was employed in 416 patients (67.1%) and coil embolization was employed in 180 (29.0%). Symptomatic vasospasm developed in 118 (28.4%) in the clipping group and 30 (16.7%) in the coiling group (P = 0.0024). NPH developed in 148 (35.6%) in the clipping group and 42 (23.3%) in the coiling group (P = 0.0032). Vasospasm was listed as a major factor for an unfavorable outcome in 23 patients (8.9%) and as a minor factor in 33 (13.3%). NPH was listed as a major factor for an unfavorable outcome in 19 patients (3.5%) and as a minor factor in 46 (18.5%). Conclusions: The multicenter registry study demonstrated lower incidences of both symptomatic vasospasms and NPH in the coiling group than in the clipping group. This superiority may result in better outcomes in the coiling group.

Characteristics of aneurysmal subarachnoid hemorrhage associated with rheumatic disease.

Spontaneous subarachnoid hemorrhage (SAH) occurs due to intracranial aneurysm rupture in most cases. Rheumatic disease may cause vessel wall inflammation, which can increase the risk of rupture. However, the characteristics of SAH with rheumatic disease are unknown. This study aimed to evaluate SAH features in patients with rheumatic disease. We retrospectively analyzed clinical data of 5066 patients from the Nagasaki SAH Registry Study who had been diagnosed with aneurysmal SAH between 2001 and 2018. We evaluated the SAH characteristics in patients with rheumatic disease using multivariable logistic regression analysis. In total, 102 patients (2.0%, 11 men and 91 women, median age 69.0 [57.0-75.5]) had rheumatic disease. In these patients, univariate logistic regression analysis showed that sex, hypertension, family history of SAH, smoking history, World Federation of Neurosurgical Societies grade on admission, aneurysm size, multiple aneurysms, treatment, and symptomatic spasms were associated with SAH. Multivariable logistic regression analysis showed that characteristics independently associated with SAH in rheumatic disease were female sex (odds ratio [OR] 3.38; 95% confidence interval [CI] 1.81-6.93, P < 0.001), hypertension (OR 0.60; 95% CI 0.40-0.90, P = 0.012), family history of SAH (OR 0.18; 95% CI 0.01-0.80, P = 0.020), small ruptured aneurysms (OR 1.50; 95% CI 1.02-2.24, P = 0.048), and multiple aneurysms (OR 1.69; 95% CI 1.09-2.58, P = 0.021) in comparison with SAH without rheumatic disease. In conclusion, SAH in patients with rheumatic disease was characterized by small multiple aneurysms, regardless of the low incidence of hypertension and family history of SAH.

First Bite Syndrome Following Carotid Endarterectomy.

A 67-year-old man with a high position carotid plaque presented with severe pain in ipsilateral parotid region several days after carotid endarterectomy (CEA). The pain occurred at the first bite of each meal and resolved as further bite. We diagnosed the pain as first bite syndrome (FBS). FBS is infrequent but known as a complication associated with parapharyngeal space surgery. The pain is characterized by sharp pain in the parotid region associated with mastication. The cause is unclear but thought to the result from sympathetic denervation of the parotid gland, followed by parasympathetic nerve hypersensitivity. Only five cases associated with carotid endarterectomy (CEA) have been reported. We should be in mind that CEA for high position plaque is one of the risk factors to cause FBS associated with CEA. Neurologists and vascular surgeons as well as otolaryngologists should all be informed FBS as one of the complications after carotid endarterectomy.

[Anomalous Origin of the Ophthalmic Artery from the Anterior Cerebral Artery Associated with the Paraclinoid Internal Carotid Artery Aneurysm].

The ophthalmic artery usually arises from the supraclinoid portion of the internal carotid artery. Here, we present an extremely rare case of abnormal origin of the ophthalmic artery from the anterior cerebral artery associated with the paraclinoid internal carotid artery aneurysm. As the embryology of the ophthalmic artery is complex, this case provides additional insight into the variation of the ophthalmic artery.

Traumatic Middle Meningeal Arteriovenous Fistula of Non-fractured Site Detected by Four-dimensional Computed Tomography Angiography: A Case Report.

Middle meningeal arteriovenous fistula (MMAVF) of a non-fractured site is extremely rare, and the clinical characteristics are still unclear. We report a case of delayed onset of venous infarction due to an MMAVF following a fall accident. A 69-year-old man sustained multiple trauma due to a fall accident. Head computed tomography (CT) showed traumatic subarachnoid hemorrhage, a left subdural hematoma, and skull fracture in his right temporal bone, all of which were managed conservatively. Five days after his admission, he suddenly exhibited total aphasia and right hemiparesis. Emergent CT revealed sporadic low-density areas in his left cerebral hemisphere, and four-dimensional CT angiography (4D-CTA) showed dilatation of the left middle meningeal artery and early venous drainage in the cavernous sinus and anterior temporal diploic vein (ATDV). A series of hemodynamics of 4D-CTA revealed early venous filling of ATDV interrelated with retrograde upward flow to high convexity in the venous phase. The MMAVF was successfully obliterated by transarterial coil embolization. We herein describe this case of MMAVF in which 4D-CTA was useful for the diagnosis.

[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.

Hyperhomocysteinemia Causes Severe Intraoperative Thrombotic Tendency in Superficial Temporal Artery-middle Cerebral Artery Bypass.

CASE: Two years ago, annual magnetic resonance imaging for unruptured right internal carotid artery aneurysm of a 47-year-old woman detected a cerebral infarct in her right occipital lobe which was unknown etiology and antiplatelet therapy was initiated. She presented with sensory disorder of her left fingers 4 months ago. Infarction in right parieto-occipital cortex and severe stenosis of right middle cerebral artery was revealed. Her laboratory test was normal except remarkably high homocysteine value. Regardless of dual anti-platelet therapy, she suffered from repeated minor stroke and the stenosis was progressing. Therefore, right superficial temporal artery - middle cerebral artery bypass was undertaken. Aspirin and clopidogrel were withdrawn 1 week before the surgery. Two branches were anastomosed with 2 separate frontal M4 branches. Although patency was confirmed immediately after the anastomosis, thrombus formation was revealed after 10 minutes. We needed to perform removal of the thrombus and re-anastomosis twice. Intraoperative administration of aspirin and ozagrel alleviated thrombotic tendency. After surgery, antiplatelet therapy and supplementation with folate and vitamin B were performed. Her postoperative course was uneventful and patency of both anastomoses was confirmed. DISCUSSION: Controversy still exists regarding preoperative antiplatelet therapy before superficial temporal artery-middle cerebral artery bypass, and folates and B6-12 vitamins supplementation for hyperhomocysteinemia. Considering intraoperative thrombo tendency in our case, it is recommended to evaluate the homocysteine level before bypass surgery for intracranial stenosis especially for young patients or patients with unknown etiology. Before bypass surgery of the patient with hyperhomocysteinemia, continuation of perioperative antiplatelet drugs and supplementation with folates and B6-12 vitamins are mandatory.

[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.

Impact of perioperative aneurysm rebleeding after subarachnoid hemorrhage.

OBJECTIVE: Aneurysm rebleeding is a major cause of death and morbidity in patients with aneurysmal subarachnoid hemorrhage (SAH). Recognizing the predictors of rebleeding might help to identify patients who will benefit from acute management. This study was performed to investigate the predictors of aneurysm rebleeding and their impact on clinical outcomes in the preoperative, intraoperative, and postoperative periods. METHODS: The incidence of rebleeding, demographic data, and clinical data from 4933 patients with aneurysmal SAH beginning in the year 2000 were retrospectively analyzed in the Nagasaki SAH Registry Study. The authors performed multiple logistic regression analyses to identify the risk factors contributing to rebleeding and outcome after SAH. RESULTS: Preoperative rebleeding occurred in 7.2% of patients. Patient age (p = 0.01), multiple aneurysms (p < 0.01), aneurysm size (p < 0.0001), and heart disease (p = 0.03) were significantly associated with preoperative rebleeding. Conversely, intraoperative rebleeding occurred in 11.2% of patients. Aneurysm location (anterior communicating artery [ACoA]), family history (p = 0.02), preoperative rebleeding (p < 0.01), and clipping/coiling (p < 0.0001) were significantly associated with intraoperative rebleeding. Interaction analysis showed that clipping significantly affected intraoperative rebleeding at the ACoA (OR 4.00; 95% CI 1.82-8.80; p < 0.001). Postoperative rebleeding occurred in 2.4% of patients. Coiling/clipping (p < 0.0001) and intraoperative rebleeding (p < 0.01) were significantly associated with postoperative rebleeding. Rebleeding in all time periods examined significantly contributed to the clinical outcome after SAH. CONCLUSIONS: Aneurysm rebleeding after SAH has specific characteristics in the preoperative, intraoperative, and postoperative periods, and all of these characteristics contribute to the clinical outcome. The ACoA has a higher risk of intraoperative rebleeding, and endovascular coiling could be a good candidate in terms of techniques for preventing intraoperative rebleeding, although complete aneurysm obliteration should be accomplished.

Arterial Spin Labeling Magnetic Resonance Imaging Can Identify the Occlusion Site and Collateral Perfusion in Patients with Acute Ischemic Stroke: Comparison with Digital Subtraction Angiography.

BACKGROUND AND OBJECTIVES: Determining the occlusion site and collateral blood flow is important in acute ischemic stroke. The purpose of the current study was to test whether arterial spin labeling (ASL) magnetic resonance imaging (MRI) could be used to identify the occlusion site and collateral perfusion, using digital subtraction angiography (DSA) as a gold standard. METHOD: Data from 521 consecutive patients who presented with acute ischemic stroke at our institution from January 2012 to September 2014 were retrospectively reviewed. Image data were included in this study if: (1) the patient presented symptoms of acute ischemic stroke; (2) MRI was performed within 24 h of symptom onset; and (3) DSA following MRI was performed (n = 32 patients). We defined proximal intra-arterial sign (IAS) on ASL as enlarged circular or linear bright hyperintense signal within the occluded artery and distal IAS as enlarged circular or linear bright hyperintense signals within arteries inside or surrounding the affected region. The presence or absence of the proximal IAS and distal IAS were assessed, along with their inter-rater agreement and consistency with the presence of occlusion site and collateral flow on DSA images. RESULTS: The sensitivity and specificity for identifying occlusion site with ASL were 82.8 and 100%, respectively. Those for identifying collateral flow with ASL were 96.7 and 50%, respectively. The inter-rater reliability was excellent for proximal IAS (κ = 0.92; 95% CI 0.76-1.00) and substantial for distal IAS detection (κ = 0.78; 95% CI 0.38-1.00). CONCLUSIONS: Proximal IAS and distal IAS on ASL imaging can provide important diagnostic clues for the detection of arterial occlusion sites and collateral perfusion in patients with acute ischemic stroke.

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.

[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.