Adenomyosis-associated recurrent acute cerebral infarction mimicking Trousseau's syndrome: A case study and review of literature.

Background: Adenomyosis is a common and benign uterine disease. Acute cerebral infarction (CI) associated with adenomyosis is rarely reported and difficult to treat. We experienced successful treatment for this disease. Case Description: A 50-year-old woman presented with a 2-day history of visual disturbance. Magnetic resonance imaging showed multiple tiny diffusion-weighted high-density spots on several lobes. No common risk factors for stroke were detected. Cancer antigen 125 level was 999 U/mL, along with massively expanded uterus and adnexa. Based on the diagnosis of benign adenomyosis, Xa inhibitor and GnRH agonists were administered for CI and adenomyosis, respectively. Acute CI recurred 7 days after admission. We suspected a relationship between infarction and adenomyosis and concluded hysterectomy as a proper treatment strategy based on the literature. Eighteen months after hysterectomy, no recurrence of CI without anti-thrombus medications has been detected. Conclusion: Hysterectomy is a radical therapy that is effective in preventing acute CI due to adenomyosis associated with ischemic symptoms.

A questionnaire-based survey to evaluate and improve the current HHT medical and social condition in Japan.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a genetic systemic vascular disease affecting multiple organs and shows recurrent intractable symptoms. This disease has not been widely recognized in Japan until recently. Both diagnosed HHT patients and potential ones have faced difficulties because of the unfamiliarity with the disease in Japan. To evaluate the effect and degree of such a Japanese situation, a questionnaire-based survey was executed in this study. METHODS: This survey was carried out among the members of HHT Japan Association. The organization consisted of 102 members (as of 6/2019), mainly HHT patients and their family members. A questionnaire was used to gather demographic data, the effort to reach the diagnosis, and information regarding current patients' and their families' medical managements. RESULTS: Of the 102 questionnaires distributed, we have got 56 responses. The participants were mostly female (30) with an average age of 55.4 ± 14.8 (mean ± standard deviation [SD]) years. The average age of males was 53.5 ± 16.4. Relatively many HHT patients were born in huge cities such as Tokyo, Osaka, and Fukuoka Prefecture (n = 4 to 8 patients). The duration between the initial symptoms and the definite diagnosis was 8.8 ± 10.9 years. The number of hospitals involved in the final diagnosis was 2.38 ± 1.83. More than 70% of patients now have to visit at least two departments and 24% of HHT patients did not want their family to screen for HHT. CONCLUSION: HHT medical practice in Japan should be further modified, for example, by establishing HHT centers and educating primary care physicians and HHT patients.

Novel method to evaluate the risk of tumor adhesions and post-operative hemorrhage of meningiomas using 320 row CT-DSA: A clinical research study.

OBJECTIVE: Meningioma is an extra-axial tumor that forms adhesions toward the brain surface in the course of its growth. Predicting adhesions between the tumor and the brain surface leads to better predictions of surgical results. There are few studies on brain-tumor adhesions or postoperative hemorrhage. This study aimed to assess tumor vascularity of the dura and cerebral surface, and predict surgical outcomes using four-dimensional computed tomography angiography (4D CTA). PATIENTS AND METHODS: Using a dynamic contrast CT, we conducted a retrospective study of 27 patients with convexity (n = 15), falx (n = 6), and parasagittal (n = 6) meningiomas treated in our hospital from January 2016 to September 2018. We set the region of interest on the dural layer and cerebral surface side of meningiomas and calculated the mean CT value in each region. Distribution of blood flow in the tumor was classified into two groups: A, which has a higher CT value of the dural side than that of the brain surface side at every timing, and B, which meets the criteria other than those in group A. Demographic data, preoperative characteristic images, and postoperative complications were compared between the groups. RESULTS: Twelve and 15 patients were classified into groups A and B, respectively. The extent of adhesions against the cerebral cortex in group A was significantly less severe compared with that in group B (p = 0.038). The rate of postoperative hemorrhage occurrence in group B (53%) was significantly higher than that in group A (8%) (p = 0.04). There were no significant differences in the other preoperative characteristic images or perioperative parameters between groups A and B. CONCLUSION: A 320-row dynamic contrast CT scanner can detect meningiomas with a high probability of severe adhesion toward the brain surface and postoperative intraparenchymal hematoma.

Silent MRA: arterial spin labeling magnetic resonant angiography with ultra-short time echo assessing cerebral arteriovenous malformation.

PURPOSE: MR angiography using the silent MR angiography algorithm (silent MRA), which combines arterial spin labeling and an ultrashort time echo, has not been used for the evaluation of cerebral arteriovenous malformations (CAVMs). We aimed to determine the usefulness of silent MRA for the evaluation of CAVMs. METHODS: Twenty-nine CAVMs of 28 consecutive patients diagnosed by 4D CT angiography or digital subtraction angiography, who underwent both time-of-flight (TOF) MRA and silent MRA, were enrolled. Two observers independently assessed the TOF-MRA and silent MRA images of CAVMs. Micro AVM was defined as AVM with a nidus diameter less than 10 mm. The detection rate, visualization of the components, and accuracy of Spetzler-Martin grade were evaluated with statistical software R. RESULTS: For all 29 CAVMs, 23 (79%) lesions were detected for TOF-MRA and all for silent MRA. Of 10 micro AVMs, only 4 (40%) lesions were detectable on TOF-MRA and all (100%) on silent MRA. The visibility of the nidus and drainer was significantly better for silent MRA than TOF-MRA (p < 0.001), while there was no significant difference in the feeder between the two sequences. The accuracy rates of the Spetzler-Martin grade for the TOF and silent MRA were 38% (11/29) and 79.3% (23/29), respectively (p < 0.001). CONCLUSIONS: Silent MRA is useful for evaluating CAVM components and detecting micro AVM.

Hemodynamic stress distribution identified by SPECT reflects ischemic symptoms of Moyamoya disease patients.

This study aimed to validate the hypothesis that the ratio of cerebral blood flow (CBF) at rest in the lenticular nucleus (LN) territory to that in the middle cerebral artery (MCA) territory is higher in symptomatic Moyamoya disease (MMD) patients than in asymptomatic MMD patients. This was a retrospective observational study of adult patients with documented MMD who underwent single-photon emission computed tomography (SPECT) and had been examined at the Department of Neurosurgery of Keio University Hospital during a 10-year period (2006-2016). The diagnosis was made on the basis of typical imaging findings. We classified unoperated MMD patients into three groups: class I, no evidence of stenosis or occlusion hemispheres and without symptoms in unilateral MMD patients; class II, hemispheres with stenosis or occlusion but without ischemic symptoms; and class III, hemispheres with evidence of stenosis or occlusion associated with ischemic symptoms. Hemodynamic stress distribution (hdSD) was defined as the ratio of CBF in one LN to the CBF in the peripheral MCA; this was obtained by SPECT at rest. We compared the values of CBF and hdSD among the groups. A total of 173 adult patients were diagnosed with MMD from January 1, 2006, to January 1, 2016. Among them, 85 MMD patients underwent SPECT studies. After excluding inappropriate cases, 144 hemispheres were included in our analysis. hdSD was significantly higher (p < 0.001) in hemispheres with ischemic symptoms (class III, mean hdSD = 1.1; 36 sides) than in those without symptoms (class II, mean hdSD = 1.03; 82 sides). However, CBF at rest in the MCA or LN was not significantly associated with ischemic symptoms. The optimal threshold for hdSD to have ischemic symptoms was 1.040 (area under the curve; 74% sensitivity 91.7% and specificity 54.9%). We used SPECT to investigate cerebral blood from MMD patients and found that high hdSD values were predictive of ischemic symptom development in these patients.

A Novel Flow Dynamics Study of the Intracranial Veins Using Whole Brain Four-Dimensional Computed Tomography Angiography.

BACKGROUND: The flow dynamics of the intracranial venous channels are fundamentally important for understanding intracranial physiology and pathophysiology. However, the method clinically applicable to the evaluation of the flow dynamics of the intracranial venous system has not been well described in the reported data. We have developed a new method to evaluate intracranial venous flow direction and velocity using 4-dimensional (4D) computed tomography angiography (CTA). The aim of the present study was to verify the accuracy and validity of 4D-CTA in a clinical setting. METHODS: We retrospectively analyzed 97 veins from 26 patients (16 cases of arteriovenous shunt disease, 9 intracranial tumor cases, and 1 cerebral aneurysm case) who had undergone both 4D-CTA and conventional digital subtraction angiography (DSA). Using 4D-CTA, we analyzed the time-density curve with gamma distribution extrapolation and obtained the direction of the flow and flow velocity of each vein. The direction of the flow in 4D-CTA was also collated with that obtained using conventional DSA to verify the experimental method. RESULTS: The direction of the flow determined by 4D-CTA was consistent with that of conventional DSA in 94.8% of cases. The average venous flow velocity was 64.3 mm/second and 81.8 mm/second, respectively, in the antegrade and retrograde channels affected by arteriovenous shunts. CONCLUSIONS: The present flow analysis using 4D-CTA enabled us to evaluate the direction and velocity of intracranial venous flow. Other than some limitations, the presented method is reliable and its potential for application in clinical settings is promising.

The Diffuse and Severe Traumatic Subarachnoid Hemorrhage Being Hard to Distinguish to Aneurysmal Subarachnoid Hemorrhage.

BACKGROUND: In primary intracerebral hemorrhage, several studies showed that contrast extravasation (CEV) is reported to be an indicator of delayed hematoma expansion, emergent hematoma removal and poor prognosis. On the contrary in head trauma, few researches validated the effectiveness of CEV because of other influences such as subarachnoid hemorrhage (SAH), contusion or brain swelling. The authors experienced a patient showing diffuse SAH caused by traumatic mechanism with acute subdural hematoma (ASDH). In the angiography, a notable rare image of CEV was found and emergency operation mainly to cease the bleeding points was performed. PATIENT PRESENTATION: A 70-year-old man was found being comatose and brought to emergency room. Computed tomography (CT) revealed diffuse SAH with left ASDH. Computed tomography angiography (CTA) also did not clarify any abnormal vessel structure except for slightly dilatation at the part of internal carotid artery, suggesting tiny ruptured aneurysm. The authors performed a cerebral angiography and resulted in no aneurysm or arteriovenous shunt detection but revealed the extravasation from middle cerebral artery and middle meningeal artery. Follow-up CT revealed increased left ASDH though 5 hours have already elapsed. The authors performed surgical intervention to halt the bleeding. CONCLUSION: The authors experienced a notably rare image of the extravasation from middle cerebral artery and middle meningeal artery in the cerebral angiography. When an obscure arterial abnormality is suspected on CTA for the unknown origin SAH with ASDH, the authors may have an affirmative attitude toward performing conventional cerebral angiography, which is most promising modality to detect the source of bleeding including CEV.

Rush progression and fatal result of septic shock related to central line catheter infection in cirrhosis patient with brain stroke.

BACKGROUND: Catheter-related blood stream infection (CRBSI) is one of the most common intractable healthcare-associated infections because catheters can be easily contaminated by resistant bacteria, and is associated with a high mortality. Central lines are currently used for administering medication to patients with severe stroke, and may thus cause CRBSI. CASE PRESENTATION: A 71-year-old woman with cirrhosis presented with subarachnoid hemorrhage (SAH) that was treated by clipping surgery. On postoperative day (POD) 38, sudden high fever (40.3 °C) was detected; the patient died a few hours later. Blood and central line cultures were positive for Klebsiella pneumoniae that may have caused CRBSI and endotoxin shock. In this case, the duration from fever detection to death was notably short. Additionally, inflammatory markers such as white blood cells (WBC) or C-reactive protein (CRP) were almost within normal ranges, even a few hours after fever was detected and before death. Cirrhosis was considered to be the cause of these phenomena. CONCLUSION: The timely diagnosis and complete treatment of patients with liver cirrhosis who develop CRBSI are highly challenging. We suggest that clinicians should rigorously apply preventive measures and strengthen CRBSI monitoring, especially in cirrhosis-associated cases.

Primary Solitary Intracranial Malignant Melanoma: A Systematic Review of Literature.

INTRODUCTION: Primary solitary intracranial malignant melanoma (PIMM) is extremely rare. In 1992, an extensive review of 81 patients with PIMM was undertaken. Imaging studies, microsurgery, and adjuvant therapy have developed considerably over the last 25 years, and targeted therapy recently has been proven successful for metastatic melanoma. These factors could influence current and future clinical PIMM results. METHODS: We undertook a literature search of PIMM patients since 1992. RESULTS: We reviewed 49 cases of PIMM. The mean age was 45.8 years. No significant sex difference was found. Intracranial hypertension and focal neurologic deficits were commonly observed around 70% and 40%, respectively. There were no significant differences of survival period according to tumor sites. Surgeries were performed in 42 of 49 patients with PIMM reviewed (92%). The mean survival of the gross total removal group was significantly longer than that of surgical results (>22 months vs. 12 months (interquartile range: 5-22 months; P = 0.026). For adjuvant therapy, 9 patients underwent chemotherapy and 18 patients underwent radiotherapy postoperatively There was no significant difference in survival period between with and without adjuvant therapies. Leptomeningeal enhancement diagnosed in the initial MRI, was the worst prognostic factor. CONCLUSIONS: Gross total removal of the PIMM was the most promising treatment. Currently adjuvant therapy has not been associated with the survival period. To improve clinical outcome, immunotherapy and targeted therapies are likely to become more important.

Preoperative Assessment of Pathologic Subtypes of Meningioma and Solitary Fibrous Tumor/Hemangiopericytoma Using Dynamic Computed Tomography: A Clinical Research Study.

BACKGROUND: Solitary fibrous tumors (SFTs)/hemangiopericytomas (HPCs) are highly vascularized tumors well known for malignant, invasive, and highly vascular features. To date, several studies have reported the preoperative imaging findings of SFTs/HPCs. In this study, computed tomography (CT) tumor values acquired from dynamic CT scan were selected to determine the tumor pathology of highly vascular tumors, such as SFTs/HPCs. METHODS: We conducted a retrospective study on patients with pathologically diagnosed meningiomas and SFTs/HPCs who had undergone a dynamic contrast CT scan. We assessed and compared the CT values of these tumors according to the pathology. RESULTS: From a total of 34 patients, 30 patients with meningiomas and 4 patients with HPCs were included. The mean CT values of SFTs/HPCs and angiomatous meningioma were statistically significantly higher than those of the other meningioma subtypes (P = 0.003). We also performed receiver operating characteristic curve analyses to detect an appropriate cutoff point for the CT value to differentiate tumor pathology, and the calculated threshold was 161 Hounsfield units (HU) (sensitivity, 100%; specificity, 75%; area under the curve, 0.87; 95%, CI 0.75-0.99). CONCLUSIONS: This study showed that obtaining a CT value is useful in determining highly vascular tumor pathology preoperatively. When considering neurosurgical extra-axial tumor removal, and when the CT value of tumors is >161 HU, then highly vascular tumors such as SFTs/HPCs or angiomatous meningiomas are likely, and this should be considered prior to surgical intervention and for risk assessment.

Successful Use of Covered Stent for Carotid Artery Injury with Active Medial Projecting Extravasation.

BACKGROUND: Nontraumatic carotid artery injury with active extravasation, or carotid blowout syndrome (CBS), is relatively rare and highly difficult to treat because it is difficult to approach the lesions owing to anatomic factors. It also involves quick progression and a risk of cerebral embolization caused by thrombi and carotid artery occlusion. Recently, covered stents were revealed to be effective for CBS. However, they have several disadvantages, such as their costs, rebleeding complications, or cerebral embolic risks. A firm selection method of CBS types that are appropriate for covered-stent therapy is expected. CASE DESCRIPTION: A 38-year-old man with esophageal cancer presented with massive hematemesis. Computed tomography revealed active extravasation from the left common carotid artery with medial projection. Initially, the open direct approach failed, which resulted in further bleeding and transient cardiopulmonary arrest. With tentative hemostasis using manual finger compression, emergency angiography was performed, and a covered stent, Fluency 8 mm × 60 mm, was placed at the rupture point. He was transferred to the rehabilitation hospital 36 days after admission with a modified Rankin score of 2 without major complications. CONCLUSION: CBS cases having rupture points around the clavicle and having medial projection extravasation should be treated by covered stent placement under tentative hemostasis using manual finger pressure rather than conventional open surgical treatment.

Safe Burr Hole Surgery for Chronic Subdural Hematoma Using Dabigatran with Idarucizumab.

BACKGROUND: Chronic subdural hematoma (CSDH) is a common intracranial hematoma. The number of patients who undergo anticoagulant therapy including a direct oral anticoagulant (DOAC) is expected to increase. Recently, idarucizumab, the antidote for dabigatran, which is a DOAC, has been developed. We successfully treated CSDH with dabigatran using emergency burr hole surgery and idarucizumab. CASE DESCRIPTION: A 79-year-old Japanese man severely hit his head and visited the emergency department. Computed tomography (CT) showed tiny traumatic acute subdural hematoma, for which he was admitted. At that time, atrial fibrillation was newly detected, for which dabigatran, having a specific antidote (idarucizumab), was chosen and started 2 weeks after the discharge. Two months after the trauma episode, he revisited the emergency department because of acute left upper and lower limb motor weakness. CT revealed a midline shifted CSDH. Considering rush course of motor weakness and shifted brain, we performed emergency surgery using an antidote for dabigatran, idarucizumab. He was discharged 5 days after surgery without any complications or excessive perioperative hemorrhage. CONCLUSION: Dabigatran should be used for atrial fibrillation detected after head trauma. Emergency surgery can be safely performed for CSDH with dabigatran using idarucizumab.

A case report of surgical management of hemangiopericytoma at the foramen magnum.

BACKGROUND: Hemangiopericytoma (HPC) is a highly vascularized mesenchymal tumor known for its high rates of recurrence and metastasis. The extent of tumor removal is known to be the most trustful prognostic factor. Skull base HPCs are challenging to treat because of the difficulty of the surgical approach and proximity to vital vascular and neuronal structures. We successfully treated a case of HPC at the ventral foramen magnum through surgical gross tumor removal via a far-lateral transcondylar approach. CASE DESCRIPTION: A 38-year-old male complained of neck pain and bilateral paresthesia of his shoulders for 2 months, for which he was referred to our hospital. A magnetic resonance image (MRI) showed a 20 mm diameter mass at the ventral foramen magnum, which compressed his medulla oblongata. The tumor was gross totally removed via a far-lateral transcondylar approach. During the surgery, marked bleeding disturbed the surgical field until the main feeding artery from the direction of the dura mater was coagulated and cut. A relatively wide surgical field and a transcondylar approach were helpful to control the bleeding. The pathological examination revealed the tumor to be a HPC. After an uneventful recovery period of 9 days, the patient was discharged without neurological sequelae. CONCLUSION: We successfully and completely removed an HPC near the foramen magnum, employing a wide surgical field and a transcondylar approach to help control bleeding. When the tumor is suspected preoperatively to be a hemangiocytoma or vascular-rich tumor, a surgical approach that can secure a wide surgical field should be selected.

Unusual Magnetic Resonance Imaging Findings of a Glioblastoma Arising During Treatment with Lenvatinib for Thyroid Cancer.

BACKGROUND: Glioblastoma (GBM) is a lesion radiologically characterized by magnetic resonance imaging findings, such as ring enhancement with extensive perifocal edema and a butterfly appearance extending into the bilateral lobes. However, these characteristic findings could be changed by antiangiogenic therapy, with decreased contrast enhancement and improved perifocal edema. Herein, we report a case of GBM that arose during treatment with a tyrosine kinase inhibitor for another cancer. CASE DESCRIPTION: A 57-year-old man presented with seizures. Until the seizure onset, he had been treated with the multireceptor tyrosine kinase inhibitor lenvatinib for 4 years for thyroid cancer and its metastasis to the thoracic vertebral body. Magnetic resonance imaging revealed a slightly high intensity lesion in the left frontal base area on T2-weighted or fluid-attenuated inversion recovery images, and the lesion showed only faint enhancement on T1-weighted images after gadolinium administration. Total resection was performed and the histopathologic diagnosis was GBM. However, grade IV histology was observed in only a limited area, and most of the specimen showed lower grade histology with moderate vascularization that lacked microvascular proliferation. CONCLUSIONS: Lenvatinib, which is antiangiogenic, might have affected the radiologic characteristics, as well as the pathology of the tumor. Brain tumors arising during treatment with receptor tyrosine kinases for other cancers could show atypical imaging findings.

Late-Onset Massive Epistaxis due to a Ruptured Traumatic Internal Carotid Artery Aneurysm: A Case Report.

A traumatic internal carotid artery (ICA) aneurysm is rare and difficult to treat. Trapping of ICA is commonly performed owing to the difficulty of directly approaching ICA aneurysms. Recently, coiling the aneurysm itself was recommended if possible. However, it is controversial which of methods are best to completely treat aneurysm. We present the case of a 74-year-old man, who had experienced a head injury 8 years previously, with recurrent severe epistaxis. An ICA aneurysm was detected on computed tomography. The trapping and bypass was planned. However, sudden epistaxis occurred, we performed trapping to stop the bleeding and save his life. After the operation, no right ICA or aneurysm was detected. However, severe epistaxis recurred two months after the operation. In the second operation, a ligation of the common -/- external carotid artery and a severance of an ICA portion between the ophthalmic artery and the aneurysm were insufficient to stop the bleeding. This case indicates ICA trapping, even if a trapping portion is below an ophthalmic artery, is insufficient to treat an ICA aneurysm. ICA aneurysms should be suspected when a patient present with recurrent -/- massive epistaxis, who has a head injury history, even if it is far past.

Spontaneous Intraventricular Pneumocephalus.

BACKGROUD: Pneumocephalus without a known underlying cause is defined as spontaneous pneumocephalus. Few patients of intraventricular pneumocephalus have been reported. PATIENT PRESENTATION: An 84-year-old man presented with dysarthria and incontinence. Computed tomography revealed an intraventricular pneumocephalus, thinning in the petrous bone, fluid in the air cells, and cleft in temporal lobe. A right subtemporal extradural approach was taken to detect bone-/-dural defects, and a reconstruction was performed using a musculo-pericranial flap. CONCLUSION: This is the first patient of an isolated intraventricular spontaneous pneumocephalus without any other site air involved. Surgical approaches to repair such bone and dura defects should be considered an appropriate option.

Histamine H2-Blocker and Proton Pump Inhibitor Use and the Risk of Pneumonia in Acute Stroke: A Retrospective Analysis on Susceptible Patients.

BACKGROUND: Although histamine H2-blockers (H2B) and proton pump inhibitors (PPI) are used commonly to prevent gastrointestinal bleeding in acute stroke, they are implicated in the increased risk of pneumonia in other disease populations. In acute stroke, the presence of distinctive risk factors of pneumonia, including dysphagia and impaired consciousness, makes inclusive analysis vulnerable to confounding. Our aim was to assess whether acid-suppressive drugs increase pneumonia in acute stroke in a population controlled for confounding. METHODS: We analyzed acute stroke patients admitted to a tertiary care hospital. To minimize confounding, we only included subjects who could not feed orally during 14 days of hospitalization. Exposure was defined as H2B or PPI, given in days; the outcome was development of pneumonia within this period. The incidence was calculated from the total number of pneumonias divided by the sum of person-days at risk. We additionally performed multivariate Poisson regression and propensity score analyses, although the restriction largely eliminated the need for multivariate adjustment. RESULTS: A total of 132 pneumonias occurred in 3582 person-days. The incidence was 3.69%/person-day (95% confidence interval (CI); 3.03-4.37%/day). All subjects had dysphagia. Stroke severity and consciousness disturbances were well-balanced between the groups exposed to H2B, PPI, or none. The relative risk (RR) compared with the unexposed was 1.22 in H2B (95%CI; 0.83-1.81) and 2.07 in PPI (95% CI; 1.13-3.62). The RR of PPI compared with H2B was 1.69 (95%CI; 0.95-2.89). In multivariate regression analysis, the RRs of H2B and PPI were 1.24 (95% CI; 0.85-1.81) and 2.00 (95% CI; 1.12-3.57), respectively; in propensity score analyses they were 1.17 (95% CI; 0.89-1.54) and 2.13 (95% CI; 1.60-2.84). CONCLUSIONS: The results of this study suggested that prophylactic acid-suppressive therapy with PPI may have to be avoided in acute stroke patients susceptible to pneumonia.

Thyrotropin-producing pituitary adenoma simultaneously existing with Graves' disease: a case report.

BACKGROUND: Thyrotropin-producing pituitary tumor is relatively rare. In particular, concurrent cases associated with Graves' disease are extremely rare and only nine cases have been reported so far. We describe a case of a thyrotropin-producing pituitary adenoma concomitant with Graves' disease, which was successfully treated. CASE PRESENTATION: A 40-year-old Japanese woman presented with mild signs of hyperthyroidism. She had positive anti-thyroid-stimulating hormone receptor antibody, anti-thyroglobulin antibody, and anti-thyroid peroxidase antibody. Her levels of serum thyroid-stimulating hormone, which ranged from low to normal in the presence of high levels of serum free thyroid hormones, were considered to be close to a state of syndrome of inappropriate secretion of thyroid-stimulating hormone. Magnetic resonance imaging showed a macropituitary tumor. The coexistence of thyrotropin-producing pituitary adenoma and Graves' disease was suspected. Initial therapy included anti-thyroid medication, which was immediately discontinued due to worsening symptoms. Subsequently, surgical therapy for the pituitary tumor was conducted, and her levels of free thyroid hormones, including the thyroid-stimulating hormone, became normal. On postoperative examination, her anti-thyroid-stimulating hormone receptor antibody levels decreased, and the anti-thyroglobulin antibody became negative. The coexistence of thyrotropin-producing pituitary adenoma and Graves' disease is rarely reported. The diagnosis of this condition is complicated, and the appropriate treatment strategy has not been clearly established. CONCLUSIONS: This case suggests that physicians should consider the coexistence of thyrotropin-producing pituitary adenoma with Graves' disease in cases in which thyroid-stimulating hormone values range from low to normal in the presence of thyrotoxicosis, and the surgical treatment of thyrotropin-producing pituitary adenoma could be the first-line therapy in patients with both thyrotropin-producing pituitary adenoma and Graves' disease.

The Coexistence of Extradural Arteriovenous Fistula and Soft Tissue Arteriovenous Malformation Within the Same Metamere.

BACKGROUND: Spinal arteriovenous shunts are a common spinal vascular disorder. However, they can have a misleading clinical presentation and poor prognosis. They are classified into 4 types according to shunt points and drainage route, among which extradural arteriovenous fistula (eAVF) is the most rare, comprising only 1% of all spinal arteriovenous shunts. We report an extremely rare case of coexistent eAVF at the craniocervical junction and soft tissue arteriovenous malformation within the same metamere. CASE DESCRIPTION: A 30-year-old man presented with neck pain. T2-weighted magnetic resonance imaging revealed a flow void at the right craniocervical junction compressing the spinal cord. Angiography revealed eAVF at the craniocervical junction. The patient was treated via transvenous embolization. After the procedure, shunt flow and symptoms markedly decreased. During the operation, an extracranial arteriovenous malformation under the occipital skin was detected immediately after occluding the shunt point. CONCLUSIONS: This case indicates that transvenous embolization can be an effective treatment for eAVF and supports some hypotheses of arteriovenous malformation development. Additionally, it emphasizes the importance of examining a lesion with multiple modalities, including angiography, three-dimensional angiography, and cone-beam computed tomography, to understand the anatomy of the lesion.