A case of acute kidney injury and Fanconi syndrome while taking multiple supplements, including Red Yeast Rice Cholesterol Help®.

A 62-year-old man visited his primary care physician with the complaints of loss of appetite and fatigue. He was admitted to our hospital based on a diagnosis of acute kidney injury, Fanconi syndrome as indicated by hypokalemia, hypouricemia, hypophosphatemia, elevated glucose levels in urine, and aminoaciduria. He had been taking multiple supplements, including Red Yeast Rice Cholesterol Help®, for one and a half years. After admission, all the supplements were stopped. Blood samples were collected; however, the samples were negative for diseases that could cause Fanconi syndrome. Renal biopsy revealed renal proximal tubular damage, mainly characterized by simplification of the proximal tubular epithelium. The mycotoxin, citrinin, which is reported to be produced by the mold used for producing red yeast rice, but not the mold Monascus pilosus used for Red Yeast Rice Cholesterol Help®, reportedly causes proximal tubular damage. However, although the causative agent has not been identified, it was thought that a substance similar to citrinin, produced by the mold used for Red Yeast Rice Cholesterol Help®, caused proximal tubular damage, leading to acute kidney injury and Fanconi syndrome. Hence, all supplements were stopped, and the patient was treated with oral potassium and phosphorus preparations, leading to gradual recovery of his kidney function. We herein report the first case of acute kidney injury and Fanconi syndrome in a patient taking multiple health supplements, including Red Yeast Rice Cholesterol Help®. Early discontinuation of the oral supplements was probably useful in improving the patient's kidney function.

The Protective Role of KANK1 in Podocyte Injury.

Approximately 30% of steroid-resistant nephrotic syndromes are attributed to monogenic disorders that involve 27 genes. Mutations in KANK family members have also been linked to nephrotic syndrome; however, the precise mechanism remains elusive. To investigate this, podocyte-specific Kank1 knockout mice were generated to examine phenotypic changes. In the initial assessment under normal conditions, Kank1 knockout mice showed no significant differences in the urinary albumin-creatinine ratio, blood urea nitrogen, serum creatinine levels, or histological features compared to controls. However, following kidney injury with adriamycin, podocyte-specific Kank1 knockout mice exhibited a significantly higher albumin-creatinine ratio and a significantly greater sclerotic index than control mice. Electron microscopy revealed more extensive foot process effacement in the knockout mice than in control mice. In addition, KANK1-deficient human podocytes showed increased detachment and apoptosis following adriamycin exposure. These findings suggest that KANK1 may play a protective role in mitigating podocyte damage under pathological conditions.

Nephrotic syndrome with acute kidney injury due to combination therapy of immune checkpoint inhibitors: a case report and review of the literature.

BACKGROUND: Recent studies have focused on immune checkpoint inhibitors. Renal complications associated with the use of immune checkpoint inhibitors are uncommon compared with other immune-related adverse events. Acute interstitial nephritis accounts for most of these renal complications, with nephrotic syndrome quite rare. We herein report a case of nephrotic syndrome associated with immune checkpoint inhibitors that was more severe than that in previous cases. By comparing this case with previous reports, the possible reasons for the particular severity of this case are discussed. CASE PRESENTATION: A 75-year-old man developed nephrotic syndrome with acute kidney injury after the first combination therapy of nivolumab and ipilimumab for malignant pleural mesothelioma. The results of a kidney biopsy indicated minimal change disease with mild atherosclerosis, acute interstitial nephritis, and fusion of nearly all podocyte foot processes. Nivolumab and ipilimumab therapy were stopped, and treatment with corticosteroids was initiated. We investigated previously reported cases of nephrotic syndrome using immune checkpoint inhibitors. Seventeen cases of immune checkpoint inhibitor-related nephrotic syndrome, including ours, have been reported. Two of the 17 patients with immune checkpoint inhibitor-related nephrotic syndrome required hemodialysis treatment for acute kidney injury. Unlike many previously reported cases, the present patient was administered two different immune checkpoint inhibitors, which may be one of the reasons for the development of severe nephrotic syndrome. CONCLUSIONS: In addition to previously reported risk factors, immune checkpoint inhibitor combination therapy can exacerbate nephrotic syndrome compared to immune checkpoint inhibitor monotherapy.

A case of carcinoid syndrome probably exacerbated by hemodialysis in which prochlorperazine maleate was effective.

Carcinoid syndrome is caused by the release of serotonin and other substances, which commonly occurs due to liver metastasis of neuroendocrine tumors. It rarely occurs due to liver metastasis of neuroendocrine carcinoma. We report the case of a patient with liver metastasis of neuroendocrine carcinoma who suffered from acute abdominal pain and diarrhea triggered by hemodialysis. Various differential diagnoses were considered, but we concluded these symptoms to be probably caused by exacerbation of carcinoid syndrome, as the serum 5HIAA level was markedly elevated, and a drug with anti-serotonin activity was effective. Prochlorperazine maleate, which has anti-serotonin activity, was effective for these symptoms, and the patient was able to continue maintenance hemodialysis, which contributed to his quality of life and prognosis. We speculated the mechanism of carcinoid exacerbation was that substances such as serotonin had entered the systemic circulation via the increased extrahepatic shunt of the portal venous blood flow, entering the inferior vena cava and that this condition had been triggered by hemodialysis via the same mechanism as portal systemic encephalopathy.

High-flow arteriovenous fistula in X-linked Alport syndrome: a case report.

Most male X-linked Alport syndrome patients with COL4A5 nonsense mutations experience end-stage kidney failure by 30 years old. Although there is no definition of high-flow arteriovenous fistula, access blood flows greater than 2000 mL/min might predict the occurrence of high-output heart failure. A 50-year-old Japanese man had suffered from proteinuria at 4 years old and sensorineural hearing loss and a lenticular lens at 20 years old. He had started to receive hemodialysis treatment due to end-stage kidney disease at 22 years old. A genetic test confirmed a novel hemizygous nonsense variant COL4A5 c.2980G > T (p.Gly994Ter), and he was diagnosed with X-linked Alport syndrome. COL4A5 c.2980G > T was considered "pathogenic" according to the American College of Medical Genetics and Genomics guidelines and in vitro experiments. Shortness of breath on exertion was exaggerated, his brachial artery blood flow was over 4,236-4,353 mL/min, his cardiac output was 5,874 mL/min, and he needed radial artery banding at 51 years old. After radial artery banding surgery, the brachial artery blood flow decreased to 987-1,236 mL/min, and echocardiography showed a cardiac output at 5100 mL/min with improved E' and E/E'. His shortness of breath on exertion improved gradually. Although rare, high-output heart failure due to high-flow arteriovenous fistula should be kept in mind as a complication in X-linked Alport syndrome patients, and our patient was successfully treated with radial artery banding surgery.

Evaluation of the Immunological Response of Childhood Cancer Patients Treated with a Personalized Peptide Vaccine for Refractory Soft Tissue Tumor: A Four-Case Series.

This case series aimed to evaluate the peptide-specific immunoglobulin G (IgG) response, clinical effectiveness, and the safety of a personalized peptide vaccine (PPV) in four children with refractory solid cancer. Although the pre-vaccination IgG responses were suppressed, IgG levels against the vaccinated peptides after 12 vaccinations were increased in all three cases who received at least 12 vaccinations. Vaccination-related adverse effects were grade 1 injection-site local skin lesions. One patient, whose diagnosis was relapsed rhabdomyosarcoma, remains in sustained remission after 37 months. Although the pre-vaccination immune response in this patient was low, IgG levels against 2 of the 4 peptide vaccines were increased after the sixth vaccination, followed by a strong increase at the eighteenth vaccination against all 4 peptides, with a >100-fold increase vs. 2 peptides. The remaining three patients exhibited progressive disease and eventually died of their original cancer. The results of the current case series suggest that in cases of childhood solid tumors, when the tumor is controlled at the time of entry PPV may have some consolidation effect. Therefore, PPV could be a new immunotherapy modality for refractory childhood solid tumors.

Membranous nephropathy associated with multicentric Castleman's disease that was successfully treated with tocilizumab: a case report and review of the literature.

BACKGROUND: Multicentric Castleman's disease is a life-threatening disorder involving a systemic inflammatory response and multiple organ failure caused by the overproduction of interleukin-6. Although renal complications of Castleman's disease include AA amyloidosis, thrombotic microangiopathy, and membranoproliferative glomerulonephritis, membranous nephropathy is relatively rare. We experienced a case of secondary membranous nephropathy associated with Castleman's disease. CASE PRESENTATION: The patient was a 43-year-old Japanese man who had shown a high zinc sulfate value in turbidity test, polyclonal hypergammaglobulinemia, anemia, and proteinuria. A physical examination revealed diffuse lymphadenopathy, an enlarged spleen and papulae of the body trunk. A skin biopsy of a papule on the patient's back showed plasma cells in the perivascular area and he was diagnosed with multicentric Castleman's disease, plasma cell variant. Kidney biopsy showed the appearance of bubbling in the glomerular basement membranes in Periodic acid methenamine silver stain and electron microscopy revealed electron dense deposits within and outside the glomerular basement membranes. Since immunofluorescence study showed predominant granular deposition of IgG1 and IgG2, he was diagnosed with secondary membranous nephropathy associated with Castleman's disease. He was initially treated with prednisolone alone, however his biochemical abnormalities did not improve. After intravenous tocilizumab (700 mg every 2 weeks) was started, his C-reactive protein elevation, anemia, and polyclonal gammopathy improved. Furthermore, his urinary protein level declined from 1.58 g/gCr to 0.13 g/gCr. The prednisolone dose was gradually tapered, then discontinued. He has been stable without a recurrence of proteinuria for more than 6 months. CONCLUSIONS: Tocilizumab might be a treatment option for secondary membranous nephropathy associated with Castleman's disease.

A Kidney Transplant Recipient with Recurrent Henoch-Schönlein Purpura Nephritis Successfully Treated with Steroid Pulse Therapy and Epipharyngeal Abrasive Therapy.

There is no specific treatment for recurrent Henoch-Schönlein purpura nephritis (HSPN) in a transplanted kidney. We herein report a case of a kidney transplant recipient with recurrent HSPN that was successfully treated with steroid pulse therapy and epipharyngeal abrasive therapy (EAT). A 39-year-old Japanese man developed HSPN 4 years ago and had to start hemodialysis after 2 months despite receiving steroid pulse therapy followed by oral prednisolone, plasma exchange therapy, and cyclophosphamide pulse therapy. He had undergone tonsillectomy 3 years earlier in the hopes of achieving a better outcome of a planned kidney transplantation and received a living-donor kidney transplantation from his mother 1 year earlier. Although there were no abnormalities in the renal function or urinalysis 2 months after transplantation, a routine kidney allograft biopsy revealed evidence of mesangial proliferation and cellular crescent formation. Mesangial deposition for IgA and C3 was noted, and he was diagnosed with recurrent HSPN histologically. Since the renal function and urinalysis findings deteriorated 5 months after transplantation, 2 courses of steroid pulse therapy were performed but were ineffective. EAT using 0.5% zinc chloride solution once per day was combined with the third course of steroid pulse therapy, as there were signs of chronic epipharyngitis. His renal function recovered 3 months after daily EAT and has been stable for 1.5 years since transplantation. Daily EAT continued for >3 months might be a suitable strategy for treating recurrent HSPN in cases of kidney transplantation.

Acute paradoxical brain herniation after decompressive craniectomy for severe traumatic brain injury: A case report.

BACKGROUND: Sinking skin flap syndrome or paradoxical brain herniation is an uncommon neurosurgical complication, which usually occurs in the chronic phase after decompressive craniectomy. We report a unique case presenting with these complications immediately after decompressive craniectomy for severe traumatic brain injury. CASE DESCRIPTION: A 65-year-old man had a right acute subdural hematoma (SDH), contusion of the right temporal lobe, and diffuse traumatic subarachnoid hemorrhage with midline shift to the left side. He underwent an emergency evacuation of the right SDH with a right decompressive frontotemporal craniectomy. Immediately after the operation, his neurological and computed tomography (CT) findings had improved. However, within 1 h after the surgery, his neurological signs deteriorated. An additional follow-up CT showed a marked midline shift to the left, i.e., paradoxical brain herniation, and his skin flap overlying the decompressive site was markedly sunken. We immediately performed an urgent cranioplasty with the right temporal lobectomy. He responded well to the procedure. We suspected that a cerebrospinal fluid leak had caused this phenomenon. CONCLUSION: Decompressive craniectomy for severe traumatic brain injury can lead to sinking skin flap syndrome and/or paradoxical brain herniation even in the acute phase. We believe that immediate cranioplasty allows the reversal of such neurosurgical complications.

Acute kidney injury due to thin basement membrane disease mimicking Deferasirox nephrotoxicity: a case report.

BACKGROUND: Although the renal toxicity of Deferasirox, an oral iron chelator, has been reported to be mild, there have been reports of acute interstitial nephritis or Fanconi syndrome due to this agent. Thin basement membrane disease (TBMD) is a hereditary disease characterized primarily by hematuria, with gross hematuria also observed in about 7% of cases. We herein report a case of TBMD that presented with acute kidney injury and gross hematuria during treatment with Deferasirox. CASE PRESENTATION: The patient was a 63-year-old man who had been diagnosed with myelodysplastic syndrome 6 years ago. He had started taking Deferasirox at 125 mg due to post-transfusion iron overload 6 months ago. Deferasirox was then increased to 1000 mg three months ago. When the serum creatinine level increased, Deferasirox was reduced to 500 mg three weeks before hospitalization. Although the serum creatinine level decreased once, he developed a fever and macroscopic hematuria one week before hospitalization. The serum creatinine level increased again, and Deferasirox was stopped four days before hospitalization. He was admitted for the evaluation of acute kidney injury and gross hematuria. Treatment with temporary hemodialysis was required, and a kidney biopsy was performed on the eighth day of admission. Although there was no major abnormality in the glomeruli, the leakage of red blood cells into the Bowman's space was observed. Erythrocyte cast formation was observed in the tubular lumen, which was associated with acute tubular necrosis. The results of an electron microscopic study were compatible with TBMD. CONCLUSION: Although Deferasirox is known to be nephrotoxic, gross hematuria is relatively rare. When we encounter a case of acute kidney injury with gross hematuria during treatment with Deferasirox, TBMD should be considered as a possible cause of gross hematuria.

Granulomatous interstitial nephritis due to chronic lymphocytic leukemia: a case report.

BACKGROUND: Renal failure due to the infiltration of chronic lymphocytic leukemia (CLL) cells into the tubulointerstitial area of the kidney is uncommon. Furthermore, granulomatous interstitial nephritis (GIN) is a rare histological diagnosis in patients undergoing a renal biopsy. We herein report a case of GIN due to the diffuse infiltration of CLL cells in a patient who developed progressive renal failure. CASE PRESENTATION: The patient was a 55-year-old man who had been diagnosed with CLL 4 years earlier and who had been followed up without treatment. Although his serum creatinine level had remained normal for three and a half years, it started to increase in the six months prior to his presentation. A urinalysis showed mild proteinuria without any hematuria at the time of presentation. A renal biopsy revealed the diffuse infiltration of CLL cells into the tubulointerstitial area with non-caseating epithelioid cell granulomas. Despite cyclophosphamide treatment, his renal function did not improve, and he ultimately required maintenance hemodialysis. CONCLUSION: When progressive renal failure is combined with CLL, GIN due to the direct infiltration of CLL cells should be considered as a differential diagnosis.

A bioinformatics-to-clinic sequential approach to analysis of prostate cancer biomarkers using TCGA datasets and clinical samples: a new method for precision oncology?

Biomarker-driven cancer therapy has met with significant clinical success. Identification of a biomarker implicated in a malignant phenotype and linked to poor clinical outcome is required if we are to develop these types of therapies. A subset of prostate adenocarcinoma (PACa) cases are treatment-resistant, making them an attractive target for such an approach. To identify target molecules implicated in shorter survival of patients with PACa, we established a bioinformatics-to-clinic sequential analysis approach, beginning with 2-step in silico analysis of a TCGA dataset for localized PACa. The effect of candidate genes identified by in silico analysis on survival was then assessed using biopsy specimens taken at the time of initial diagnosis of localized and metastatic PACa. We identified PEG10 as a candidate biomarker. Data from clinical samples suggested that increased expression of PEG10 at the time of initial diagnosis was linked to shorter survival time. Interestingly, PEG10 overexpression also correlated with expression of chromogranin A and synaptophysin, markers for neuroendocrine prostate cancer, a type of treatment-resistant prostate cancer. These results indicate that PEG10 is a novel biomarker for shorter survival of patients with PACa. Also, PEG10 expression at the time of initial diagnosis may predict focal neuroendocrine differentiation of PACa. Thus, PEG10 may be an attractive target for biomarker-driven cancer therapy. Thus, bioinformatics-to-clinic sequential analysis is a valid tool for identifying targets for precision oncology.

OUTCOME OF CLIVAL CHORDOMAS AFTER SKULL BASE SURGERIES WITH MEAN FOLLOW-UP OF 10 YEARS.

BACKGROUND AND OBJECTIVE: Skull base chordomas are clinically malignant because of the difficulty of total removal, high recurrence rate, and occasional drop metastasis. Although aggressive surgical resection and postoperative radiation have been recommended, the long-term outcome remains unsatisfactory. METHODS: From 1992 to 2011, we treated 24 patients with skull base chordoma using aggressive surgical removal as a principal strategy. Skull base approaches were selected according to tumor extension to remove the tumor and surrounding bone as completely as possible. After surgery, all patients were closely observed with MRI to find small and localized recurrent tumors, which were treated with gamma-knife radiosurgery or surgical resection. The mean postoperative follow-up duration was 10.2 years (range, 1-17.2 years). RESULTS: The 5-, 10-, and 15-year overall survival rates were 86%, 72%, and 72%, respectively. The 5- and 10-year progression-free survival rates were 47% and 35%, respectively. Tumor extension to the brainstem and partial tumor removal were the factors related to poor survival. CONCLUSIONS: Our results suggest that aggressive surgical removal improves the long-term outcome of patients with skull base chordoma. We would like to emphasize that skull base chordomas should be aggressively removed using various skull base approaches.

Monocytes infiltrate the pancreas via the MCP-1/CCR2 pathway and differentiate into stellate cells.

Recent studies have shown that monocytes possess pluripotent plasticity. We previously reported that monocytes could differentiate into hepatic stellate cells. Although stellate cells are also present in the pancreas, their origin remains unclear. An accumulation of enhanced green fluorescent protein (EGFP)(+)CD45(-) cells was observed in the pancreases and livers of chimeric mice, which were transplanted with a single hematopoietic stem cell isolated from EGFP-transgenic mice and treated with carbon tetrachloride (CCl4). Because the vast majority of EGFP(+)CD45(-) cells in the pancreas expressed stellate cell-associated antigens such as vimentin, desmin, glial fibrillary acidic protein, procollagen-I, and α-smooth muscle actin, they were characterized as pancreatic stellate cells (PaSCs). EGFP(+) PaSCs were also observed in CCl4-treated mice adoptively transferred with monocytes but not with other cell lineages isolated from EGFP-transgenic mice. The expression of monocyte chemoattractant protein-1 (MCP-1) and angiotensin II (Ang II) increased in the pancreas of CCl4-treated mice and their respective receptors, C-C chemokine receptor 2 (CCR2) and Ang II type 1 receptor (AT1R), were expressed on Ly6C(high) monocytes isolated from EGFP-transgenic mice. We examined the effect of an AT1R antagonist, irbesartan, which is also a CCR2 antagonist, on the migration of monocytes into the pancreas. Monocytes migrated toward MCP-1 but not Ang II in vitro. Irbesartan inhibited not only their in vitro chemotaxis but also in vivo migration of adoptively transferred monocytes from peripheral blood into the pancreas. Irbesartan treatment significantly reduced the numbers of EGFP(+)F4/80(+)CCR2(+) monocytic cells and EGFP(+) PaSCs in the pancreas of CCl4-treated chimeric mice receiving EGFP(+) bone marrow cells. A specific CCR2 antagonist RS504393 inhibited the occurrence of EGFP(+) PaSCs in injured mice. We propose that CCR2(+) monocytes migrate into the pancreas possibly via the MCP-1/CCR2 pathway and give rise to PaSCs.

The 'one and a half round microcatheterization technique' for stent-assisted coil embolization of intracranial aneurysm: technical case series.

BACKGROUND: Stent-assisted coil embolization is useful for wide-necked, large and giant aneurysms, and is effective for avoiding coil herniation. However, the mobility of the microcatheter is often restricted, resulting in deviated or unbalanced coiling. In order to prevent this insufficient coiling, the authors devised a method for microcatheterization, the 'one and a half round microcatheterization technique'. This technique is based on the formation of a one and a half round loop by the microcatheter along the aneurysmal wall. Furthermore, this technique can be supplemented with the double-catheter technique. METHODS: From July 2010 to July 2012, the authors used this technique for 20 aneurysms in 20 patients (6 men and 14 women; mean age 61.7 years). The one and a half round microcatheterization technique was used alone in 12 cases and was supplemented with the double-catheter technique in eight. The clinical and angiographic results were retrospectively evaluated. RESULTS: The average aneurysm size was 16.7 mm; 12 aneurysms (60%) were located at the internal carotid artery, 5 (25%) at the basilar artery and 3 (15%) at the vertebral artery. Immediate angiographic results showed complete obliteration in 6 aneurysms (30%) and residual neck in 10 (50%), leaving 4 residual aneurysms (20%). This technique was useful and acceptably safe for packing the aneurysmal sac entirely. During an average follow-up of 20.5 months, 13 of the 18 aneurysms (72%) were stable or had improved, although 3 (16%) required retreatment. CONCLUSIONS: The one and a half round microcatheterization technique provides dense coil packing for stent-assisted embolization of large or giant aneurysms.

Primary CNS lymphoma arising in the region of the optic nerve presenting as loss of vision: 2 case reports, including a patient with a massive intracerebral hemorrhage.

We report 2 cases of primary central nervous system (CNS) lymphoma arising in the region of the optic nerve. For both patients, diagnosis of lymphoma was impossible without histological examination because of the rarity of the lymphoma location. The first case involved an 84-year-old woman who developed loss of vision and hypopituitarism. Intraoperative finding was optic glioma; histological diagnosis was diffuse large B cell lymphoma, however. The second case involved a 67-year-old man who developed loss of vision. The pre-surgical diagnosis was optic nerve neuritis; this was then revised to granuloma. The tumor arose in the optic nerve. Methotrexate and rituximab were administered and the patient remained in complete remission for 3 years. However, a sudden intratumoral hemorrhage occurred. Although most of the lymphoma cells obtained from the initial surgery were negative for vascular endothelial growth factor (VEGF) immunoreactivity, high levels of VEGF immunoreactivity in lymphoma cells was detected in the specimen obtained after intratumoral bleeding at recurrence, and correlation between VEGF reactivity and tumor recurrence was suggested. To date, primary CNS lymphomas with intracerebral hemorrhage have been reported in 3 cases only, and a correlation between intratumoral hemorrhage and the degree of VEGF expression has been suggested. VEGF also might have predictive significance for recurrence.

[Surgical seeding of an anaplastic ependymoma].

Ependymomas can relapse at the primary site and disseminate to the spinal cord. Furthermore, extraneural metastases are rare. We present a case of anaplastic ependymoma with extraneural metastasis, possibly caused by surgical seeding. An 18-year-old female was referred to us after experiencing frequent convulsive attacks. At age 9 years, she underwent partial resection of a left frontal anaplastic ependymoma, followed by 45 Gy radiation. At age 15 years, a recurrent tumor was totally removed. Three years later, magnetic resonance images revealed a left frontal recurrent tumor and a new parietal lesion. Our preoperative diagnosis of the parietal tumor was a radiation-induced meningioma. However, intraoperative pathological examinations revealed the lesion to be an anaplastic ependymoma. The tumor, which was located in the epidural space and had invaded extensively into the cranial bone, was gross totally removed. Although extremely rare, surgical seeding of anaplastic ependymomas should be considered as a differential diagnosis for recurrent tumors in the surgical field.

[Systematic review of complications for proper informed consent (9) periprocedural complications of carotid artery stenting: a review article].

BACKGROUND: Carotid artery stenting(CAS)is a common procedure for the treatment of high surgical risk patients with carotid artery stenosis. Recent clinical study failed to show the non-inferiority of CAS to carotid endarterectomy(CEA)because of higher complication rates of CAS. However the result of a multicenter case study in Japan with tailored application of adequate devices including stents and protection devices revealed the safety of CAS in Japan. In this article the overall review in the previous reports of CAS are reviewed focusing on the complications and clinical outcome. METHOD: Five randomized controlled trials concerning CAS versus CEA and 14 case series of CAS were reviewed, and the rates of periprocedural complications of CAS including death, stroke, myocardial infarction, hypotension and bradycardia, cranial nerve palsy, major vascular injury, cholesterol crystal embolization and hyperperfusion syndrome were summarized. RESULT: Mortality within 30 days of procedures was 0.9%. The rate of any stroke was 6.0% from the five RCT results, but a recent 14-case series reported any stroke rate at 3-4% and severe stroke rate at 1-2% respectively. The rates of acute myocardial infarction, hypotension, and bradycardia were 1%, 4%, and 4-12% respectively. The rate of bleeding complication of the access root was about 2% and non-bleeding was 1-2%. Cholesterol crystal embolization occurred in 0.2%. Incidence of hyperperfusion syndrome occurred in 1.5%, and intracranial hemorrhage in 0.8%. CONCLUSION: Historically, the rapid improvement of devices and technical skills have contributed to the better clinical results. Also the lessons from complication cases have led to more appropriate indication and perfect protection to avoid adverse events. Therefore the rate of complication is always improving and those written in this article may not reflect the present real number. This article should be quoted with the previous reference as well as the newest data according to the operator's own experiences on taking informed consent.

Motor evoked potential monitoring of the vagus nerve with transcranial electrical stimulation during skull base surgeries.

OBJECT: Dysphasia is one of the most serious complications of skull base surgeries and results from damage to the brainstem and/or cranial nerves involved in swallowing. Here, the authors propose a method to monitor the function of the vagus nerve using endotracheal tube surface electrodes and transcranial electrical stimulation during skull base surgeries. METHODS: Fifteen patients with skull base or brainstem tumors were enrolled. The authors used surface electrodes of an endotracheal tube to record compound electromyographic responses from the vocalis muscle. Motor neurons were stimulated using corkscrew electrodes placed subdermally on the scalp at C3 and C4. During surgery, the operator received a warning when the amplitude of the vagal motor evoked potential (MEP) decreased to less than 50% of the control level. After surgery, swallowing function was assessed clinically using grading criteria. RESULTS: In 5 patients, vagal MEP amplitude permanently deteriorated to less than 50% of the control level on the right side when meningiomas were dissected from the pons or basilar artery, or when a schwannoma was dissected from the vagal rootlets. These 5 patients had postoperative dysphagia. At 4 weeks after surgery, 2 patients still had dysphagia. In 2 patients, vagal MEPs of one side transiently disappeared when the tumors were dissected from the brainstem or the vagal rootlets. After surgery, both patients had dysphagia, which recovered in 4 weeks. In 7 patients, MEP amplitude was consistent, maintaining more than 50% of the control level throughout the operative procedures. After surgery all 7 patients were neurologically intact with normal swallowing function. CONCLUSIONS: Vagal MEP monitoring with transcranial electrical stimulation and endotracheal tube electrode recording was a safe and effective method to provide continuous real-time information on the integrity of both the supranuclear and infranuclear vagal pathway. This method is useful to prevent intraoperative injury of the brainstem corticobulbar tract or the vagal rootlets and to avoid the postoperative dysphagia that is often associated with brainstem or skull base surgeries.