[Intraoperative Monitoring During Microvascular Decompression].

During microvascular decompression(MVD)for hemifacial spasm(HFS), trigeminal neuralgia(TN), and glossopharyngeal neuralgia(GPN), brainstem auditory-evoked potential monitoring is widely used to preserve hearing function. In MVD for HFS, abnormal muscle response monitoring is useful for identifying the offending vessels compressing the facial nerve and confirming the completion of decompression intraoperatively. The amplitude of facial motor-evoked potential by transcranial electrical stimulation in the orbicularis oculi muscle is reported to decrease after completing MVD. The Z-L response(ZLR)probably confirms the true offending vessels by stimulating the culprit vessels; then, the ZLR could disappear after decompressing the offending vessels away from the compression sites. Spontaneous electromyographic activities obtained from the mentalis muscles by injection of saline into the facial nerve reportedly decreased after MVD compared with those before MVD. In MVD for the GPN, glossopharyngeal motor-evoked potential by transcranial electrical stimulation is used to preserve swallowing function and not to assess the completion of MVD. Because MVD for both the TN and GPN can result in normalization of the hyperactivity of the sensory nerve, it may be difficult to develop any monitoring to confirm the completion of MVD during surgery.

Association of Baseline Renal Function with Mortality in Patients with Sepsis Requiring Continuous Renal Replacement Therapy for Acute Kidney Injury: A Single-Center Retrospective Study.

INTRODUCTION: To date, the prognosis of patients with sepsis and underlying chronic kidney disease (CKD) had been poor. However, the impact of preseptic renal function on the short-term prognosis of patients with extremely severe septic shock with acute kidney injury (AKI) that requires renal replacement therapy (RRT) is unclear. METHODS: Of the septic shock cases treated at the intensive care unit for ≥48 h, 131 adults who were diagnosed as septic AKI and underwent continuous venovenous hemodiafiltration were retrospectively analyzed. The relationships of demographic, clinical, and laboratory data with mortality were evaluated, and the independent risk factors for death were identified. RESULTS: The median age of the subjects was 73 (range, 63-80) years, and 76 (58%) were men. The rate of mortality was significantly higher among patients with CKD (n = 42) than in those without CKD (n = 89) (43% vs. 22%, p < 0.016). On univariate and multivariate logistic regression analyses, the associated factors and independent predictors of death were Sequential Organ Failure Assessment score (odds ratios [ORs] 1.151, 95% confidence intervals [CIs] 1.026-1.293, p = 0.017, and OR 1.129, 95% CI 1.003-1.271, respectively); baseline estimated glomerular filtration rate (OR 0.986, 95% CI 0.975-0.997, p = 0.016, and OR 0.983, 95% CI 0.970-0.996, respectively); and lactic acid (OR 1.094, 95% CI 1.005-1.190, p = 0.038, and OR 1.110 CI 1.015-1.215, respectively). CONCLUSION: Reduced baseline renal function may be a factor for poor short-term prognosis in severe septic AKI cases requiring RRT.

[Intraoperative Monitoring during Microvascular Decompression for Hemifacial Spasm].

Brainstem auditory evoked potential(BAEP)and abnormal muscle response(AMR)monitoring are widely used during microvascular decompression(MVD)for hemifacial spasm(HFS). In BAEP monitoring, the intraoperative findings of wave V do not necessarily predict postoperative hearing function. However, if a warning sign as significant as wave V change appears, the surgeon must abort the operation or inject artificial cerebrospinal fluid into the VIII nerve. During MVD for HFS, BAEP monitoring must be performed to preserve hearing function. AMR monitoring is useful in identifying the offending vessels compressing the facial nerve and confirming the completion of decompression intraoperatively. During the operation of the offending vessels, AMR sometimes changes its onset latency and amplitude in real time. These findings allow surgeons to identify the offending vessels. Even if the AMRs remain after completion of decompression, a decrease in amplitude of more than 50% compared to baseline is predictive of postoperative loss of HFS in long-term outcomes. When the AMRs disappear after dural opening, the AMR monitoring should be continued because the AMRs sometimes reappear.

Severe drug-induced immune hemolytic anemia due to cefmetazole: A case report.

OBJECTIVE: To report a case of drug-induced immune hemolytic anemia (DIIHA) that was suspected to have been caused by cefmetazole. CASE SUMMARY: A 93-year-old woman with no previous history of liver complications underwent a contrast-enhanced computed tomography scan, which resulted in a diagnosis of acute cholecystitis. The patient experienced intravascular hemolysis and rapid progression of anemia after being exposed to 2 g/day of cefmetazole. After 48 hours of cefmetazole administration, the patient was transferred to the intensive care unit (ICU) of our facility. In view of the severe autoimmune hemolytic anemia, the patient was started on steroid immunosuppression. The patient's condition further deteriorated for 13 hours after treatment and showed increased lactic acidosis and decreased consciousness, thus, the patient was intubated and managed on a ventilator. Lactic acidosis was not easily controlled, and the patient required continuous renal replacement therapy within 15 hours of ICU admission. Blood pressure was unable to be maintained even with the use of catecholamine, and the patient subsequently died 28 hours after ICU admission. Blood taken immediately after death was used to perform a drug-dependent antibody test where DIIHA due to cefmetazole was diagnosed. CONCLUSION: If there is rapid progression of anemia following drug administration, the possibility of DIIHA needs to be considered. If DIIHA is suspected, identification and immediate discontinuation of the causal drug are essential, and a drug-dependent antibody test should be considered.

Intraoperative findings of abnormal muscle response for hemifacial spasm following botulinum neurotoxin treatment.

OBJECTIVE: This study aimed to investigate the effect of preoperative botulinum toxin (BTX) injection on intraoperative abnormal muscle response (AMR) in patients with hemifacial spasm (HFS). METHODS: A total of 104 patients (32 men, 72 women) who underwent microvascular decompression (MVD) for HFS were included in this study. A total of 62 patients without and 42 patients with preoperative BTX treatments were assigned to group A and group B, respectively. AMR recordings were obtained from the orbicularis oculi and mentalis muscles by stimulation of the marginal mandibular branch and zygomatic branch of the facial nerve, respectively. The intraoperative AMR monitoring findings and therapeutic effects were compared between groups A and B. RESULTS: The rates of the patients with unavailable AMRs recorded from the orbicularis oculi muscles in group B (38.1%) were significantly higher than those in group A (14.5%, p = 0.006). Moreover, in cases with over 4 times BTX injection, the recordings of AMR from the orbicularis oculi muscles were poorer than the cases with less BTX injection (p = 0.001). There were no significant differences in the rates of the patients with unavailable AMRs recorded from the mentalis muscles between the two groups. There were no significant differences in the surgical results obtained between the two groups. CONCLUSIONS: Preoperative BTX injections should be less than 4 times to ensure effective AMR monitoring. MVD using AMR monitoring is useful for patients with HFS who were previously treated by BTX as well as those who were not treated.

[Diagnosing Status Epilepticus].

Both diffusion-weighted MRI(DWI)modalities and continuous electroencephalography(cEEG)are useful for diagnosing status epilepticus. In case 1, DWI showed hyperintense regions in the right-sided parieto-occipital cortex during peri-ictal status. Intensity of the regions normalized after left hemiparesis improved. In status epilepticus , DWI usually depicts some hyperintense regions, such as the cerebral cortex, hippocampus, and thalamic pulvinar, where ictal brain activity and its propagation are likely occur the seizure. In case 2, cEEG led to an accurate diagnosis of non-convulsive status epilepticus due to right-sided temporal contusion. Intravenous application of levetiracetam and lacosamide alleviated the clinical symptoms and electrographic seizures. Abnormal cEEG findings during status epilepticus vary from rhythmic delta activity and epileptiform and generalized periodic discharges to ictal discharges. Accurate diagnosis of status epilepticus using MRI and cEEG can offer earlier intervention, such as prompt administration of benzodiazepines, midazolam, lorazepam, ultimately resulting in a good recovery.

Multiplex ligation-dependent probe amplification analysis is useful for detecting a copy number gain of the FGFR1 tyrosine kinase domain in dysembryoplastic neuroepithelial tumors.

PURPOSE: Dysembryoplastic neuroepithelial tumors (DNTs) are slow-growing glioneuronal tumors, and their genetic backgrounds are getting unveiled. Recently, fibroblast growth factor receptor 1 internal tandem duplication (FGFR1-ITD) of the tyrosine kinase domain (TKD) has been demonstrated by whole-genome sequencing. METHODS AND RESULTS: Here, we analyzed 22 DNTs using multiplex ligation-dependent probe amplification (MLPA) with formalin-fixed paraffin-embedded specimens and found a copy number gain in TKD of FGFR1 (13 cases, 59%), which suggested the presence of FGFR1-ITD. Another 5 DNTs harbored FGFR1 hot spot mutations including a double mutant case, and FGFR1 alterations were detected in 18 DNTs (82%). The BRAF V600E mutation, another important mutation in DNTs, was not observed. CONCLUSIONS: With recent findings of less frequent or absent FGFR1-ITD in pilocytic astrocytomas or rosette-forming glioneuronal tumors, the analysis of FGFR1 aberrations, especially FGFR1-ITD, was suggested to be helpful to discriminate DNTs from their histological mimics.

[Posterior Quadrantectomy for Infant with Refractory Epilepsy:A Case Report].

We present the case of an 11-month-old girl with linear nevus sebaceous syndrome who underwent posterior quadrantectomy(PQ)for intractable epilepsy due to cortical dysplasia extending from the temporal, parietal, and occipital lobes in the right hemisphere. Epileptic spasms started at 4 months after birth, and the frequency of her seizures gradually increased to 10 episodes per day. Electroencephalograms in the interictal periods showed hypsarrhythmia. Magnetic resonance imaging(MRI)suggested cortical dysplasia in the right temporal, parietal, and occipital lobes. Ictal single-photon emission computed tomography revealed increased cerebral blood flow in similar areas as the cortical dysplasia suggested on MRI. Several antiepileptic drugs were administered to control the epileptic spasms, without success. In addition, her developmental delay gradually became evident. Because the epileptic foci extended into the posterior region of the right hemisphere, we did not execute a focused resection, but performed a PQ. The epileptic spasms completely disappeared after surgery and her developmental delay gradually improved. Early surgical intervention via PQ is useful in patients with drug-resistant epilepsy in whom the epileptic foci have extended into the temporal, parietal, and occipital lobes. This intervention not only controls intractable seizures but also helps to facilitate normal development.

Detailed analysis of 26 cases of 1q partial duplication/triplication syndrome.

Partial 1q trisomy syndrome is a rare disorder. Because unbalanced chromosomal translocations often occur with 1q trisomy, it is difficult to determine whether patient symptoms are related to 1q trisomy or other chromosomal abnormalities. The present study evaluated genotype-phenotype correlations of 26 cases diagnosed with 1q partial trisomy syndrome. DNA microarray was used to investigate the duplication/triplication region of 16 cases. Although there was no overlapping region common to all 26 cases, the 1q41-qter region was frequently involved. One case diagnosed as a pure interstitial trisomy of chromosome 1q by G-banded karyotype analysis was instead found to be a pure partial tetrasomy by CytoScan HD Array. In four 1q trisomy syndrome cases involving translocation, the translocated partner chromosome could not be detected by DNA microarray analyzes despite G-banded karyotype analysis, because there were a limited number of probes available for the partner region. DNA microarray and G-banded karyotyping techniques were therefore shown to be compensatory diagnostic tools that should be used by clinicians who suspect chromosomal abnormalities. It is important to continue recruiting affected patients and observe and monitor their symptoms to reveal genotype-phenotype correlations and to fully understand their prognosis and identify causal regions of symptoms.

Free-running EMG monitoring during microvascular decompression for hemifacial spasm.

BACKGROUND: The aim of this work is to determine if free-running electromyography (frEMG) can detect activity before and after microvascular decompression (MVD) treatment for hemifacial spasm (HFS), and to evaluate correlations of frEMG findings with abnormal muscle responses (AMRs) or facial motor-evoked potentials (FMEPs). METHODS: To elicit nerve responses while carrying out frEMG recording before and after MVD, saline, a lactic solution, or artificial cerebrospinal fluid was injected onto the root exit zone of the facial nerve. RESULTS: Significantly higher frEMG activity was observed following saline injection than for the other solutions (p < 0.01). For frEMG activity ratios of ≥ 50 %, there was a trend towards a greater likelihood of persistent AMRs. When frEMG activity decreased after MVD in the mentalis muscles, FMEP amplitude ratios were significantly smaller than when it did not (65 vs. 94 %, p < 0.05). CONCLUSIONS: Changes in intraoperative frEMG, AMRs, and FMEPs likely reflect a component of the normalization of hyper-excitability of the facial nerve by MVD for HFS.

Long-term outcomes in patients with pineal nongerminomatous malignant germ cell tumors treated by radical resection during initial treatment combined with adjuvant therapy.

BACKGROUND: For pineal nongerminomatous malignant germ cell tumors (NGMGCTs), we mainly performed radical tumor resection during initial treatment combined with adjuvant therapy. METHODS: We retrospectively analyzed 17 patients treated for pineal NGMGCTs between 1986 and 2007 at the University of Niigata. RESULTS: Twelve patients underwent total or subtotal resection of their tumor via the occipital transtentorial approach. Five patients underwent partial resection, and four of them later underwent total resection by salvage surgery. After surgery, eight patients were treated with combined radiochemotherapy including whole-brain irradiation, two received radiation monotherapy, one had chemotherapy with local irradiation, and six were treated with chemotherapy alone. The median follow-up period for surviving patients was 179 months. The 10-year overall survival and progression-free survival rates for the radiochemotherapy group were both 75.0 % (two patients had a recurrence and died); the rates for other adjuvant therapies were 77.8 % (two died) and 22.2 % (seven had a recurrence), respectively. Radiochemotherapy was significantly associated with an increased rate of progression-free survival compared with the other adjuvant therapies (p = 0.0396). CONCLUSIONS: For pineal NGMGCTs, initial treatment strategies including gross total resection of the tumor before or after whole-brain irradiation and chemotherapy provided good therapeutic outcomes. Obtaining complete remission of the primary tumor, irrespective of the timing of surgical resection (i.e., before or after adjuvant therapies), or complete response by neoadjuvant radiochemotherapy during an initial treatment appears to be essential for improving therapeutic outcomes of intracranial NGMGCTs.

Importance of changes in abnormal muscle responses during microvascular decompression for hemifacial spasm.

Objective: To determine if compression sites of the facial nerve correlate with immediate postoperative outcomes in patients with hemifacial spasm (HFS), and if changes in the waveform of abnormal muscle response (AMR) during microvascular decompression (MVD) for HFS can predict the postoperative course. Methods: In this retrospective review, we evaluated 50 patients with HFS who underwent AMR monitoring during MVD. The ratios of amplitude and duration of AMR waveforms were computed by comparing baseline with final examinations. Vascular compression sites were categorized into four portions of the facial nerve. Postoperatively, we classified patients into two groups based on symptom relief as those whose symptoms disappeared immediately (DI group), and those whose symptoms disappeared gradually (DG group). Results: The compression sites significantly correlated with postoperative outcomes at discharge (p < 0.001) but not with outcomes after 6 months of MVD. Lower duration ratios of AMRs from the mentalis muscle were significantly associated with an increased chance of classification into the DI group based on the results of multivariate logistic regression analysis (p = 0.017). Conclusions: Relationship between compression sites and immediate outcomes could provide useful information to surgeons for predicting if symptoms will resolve over long term. Moreover, changes in AMRs recorded from the mentalis muscle could predict the postoperative course of HFS. Significance: These findings can help surgeons evaluate the changes in AMR amplitude and duration during MVD for HFS.

Pregnancy-Associated Atypical Hemolytic Uremic Syndrome Successfully Treated with Ravulizumab: A Case Report.

Pregnancy-associated atypical hemolytic-uremic syndrome (p-aHUS) refers to a pregnancy that leads to thrombotic microangiopathy (TMA). This disease is associated with adverse maternal outcomes. We encountered a case of p-aHUS, in which treatment with ravulizumab, a long-acting C5 inhibitor, resulted in a favorable clinical course and recovery of renal function. The patient was a 31-year-old woman with no apparent medical history. She developed TMA on the third postpartum day and was initially treated with steroids, plasma exchange, and hemodialysis (HD). On the seventh day of treatment initiation, she was diagnosed with p-aHUS, and treatment with ravulizumab was started. Following administration, her platelet count increased, and her acute kidney injury improved. Consequently, HD was discontinued after six sessions, and the patient was discharged on the 28th day of treatment initiation and continued her recovery at home. Similar to eculizumab, ravulizumab is an effective treatment for p-aHUS. Early administration of ravulizumab after diagnosis of p-aHUS may contribute to favorable clinical outcomes and recovery of renal function, as observed in the present case.

Pharmacological evaluation of E2730, a novel selective uncompetitive GAT1 inhibitor, on epileptiform activities in resected brain tissues from human focal cortical dysplasia ex vivo.

Focal cortical dysplasia (FCD) is an important etiology of focal epilepsy in children and adults. However, only a few preclinical models sufficiently reproduce the characteristic histopathologic features of FCD. To improve the success rate of clinical trials for antiseizure medications (ASMs) in patients with FCD, more human-relevant preclinical models are needed, and epileptic foci resected from patients are a powerful tool for this purpose. Here, we conducted ex vivo studies using epileptic foci resected from patients with FCD type II to evaluate the pharmacologic effects of the ASM candidate E2730, a selective uncompetitive inhibitor of γ-aminobutyric acid transporter 1. We used the same ex vivo assay system to assess carbamazepine (CBZ), an ASM often prescribed for focal epilepsy, as a reference. At the higher dose tested (200 µM), both E2730 and CBZ suppressed spontaneous epileptiform activities almost completely. At the lower dose (100 µM), CBZ reduced the area of brain tissue showing epileptiform activity, whereas E2730 significantly decreased the number of epileptiforms. These findings suggest that E2730-both as a single agent and in combination with CBZ-merits evaluation in clinical trials involving patients with FCD.

Relaxin-like factor (RLF)/insulin-like peptide 3 (INSL3) is secreted from testicular Leydig cells as a monomeric protein comprising three domains B-C-A with full biological activity in boars.

RLF (relaxin-like factor), also known as INSL3 (insulin-like peptide 3), is a novel member of the relaxin/insulin gene family that is expressed in testicular Leydig cells. Despite the implicated role of RLF/INSL3 in testis development, its native conformation remains unknown. In the present paper we demonstrate for the first time that boar testicular RLF/INSL3 is isolated as a monomeric structure with full biological activity. Using a series of chromatography steps, the native RLF/INSL3 was highly purified as a single peak in reverse-phase HPLC. MS/MS (tandem MS) analysis of the trypsinized sample provided 66% sequence coverage and revealed a distinct monomeric structure consisting of the B-, C- and A-domains deduced previously from the RLF/INSL3 cDNA. Moreover, the N-terminal peptide was four amino acid residues longer than predicted previously. MS analysis of the intact molecule and PMF (peptide mass fingerprinting) analysis at 100% sequence coverage confirmed this structure and indicated the existence of three site-specific disulfide bonds. RLF/INSL3 retained full bioactivity in HEK (human embryonic kidney)-293 cells expressing RXFP2 (relaxin/insulin-like family peptide receptor 2), the receptor for RLF/INSL3. Furthermore, RLF/INSL3 was found to be secreted from Leydig cells into testicular venous blood. Collectively, these results indicate that boar RLF/INSL3 is secreted from testicular Leydig cells as a B-C-A monomeric structure with full biological activity.

[Intraoperative monitoring of motor evoked potentials during glioma removal].

OBJECTIVE: To determine whether motor evoked potentials(MEPs)provide reliable monitoring of the motor system during resection of gliomas in or adjacent to the motor cortex or pyramidal tract. MATERIALS AND METHODS: MEP recording was performed during 64 operations in 55 patients harboring gliomas. Intraoperative MEP findings were classified into 3 groups:Group A was defined as having no significant MEP changes, Group B as having reversible MEP changes(?50% amplitude decrease or loss), and Group C as having irreversible changes. Postoperative motor function was evaluated according to the presence/absence of deterioration immediately after surgery and 1 month later, as compared to preoperative motor status RESULTS: Immediately after surgery, 13 of 39(33%)patients in Group A, 6 of 17(35%)in Group B, and 7 of 8(88%)in Group C experienced deterioration of motor function. One month after surgery, 4 of 39(10%)patients in Group A, 3 of 17(18%)in Group B, and 4 of 8(50%)showed deterioration of motor function. Both immediately(χ2=8.3, p<0.05)and 1 month(χ2=6.9, p<0.05)after surgery, MEP alterations correlated significantly with postoperative deterioration of motor function. Despite MEPs being stable throughout surgery(Group A), there were some patients with deterioration of motor function initially appearing to represent false negative monitoring. However, these deteriorations were confirmed to have been caused by secondary hemorrhage, venous return dysfunction, postoperative convulsion, or resection of the supplementary motor area. CONCLUSIONS: MEP monitoring provides reliable information on the motor system during glioma surgery. Although false negative MEP results may exist in some patients, most data were not influenced by intraoperative manipulation but rather were attributable to secondary postoperative events.

Acute Liver Failure After Administration of Acetaminophen at the Recommended Daily Dose in an Adult: A Case Report.

Acetaminophen may cause liver damage in a dose-dependent way: we experienced a case where an intravenous injection of 3 g/day of acetaminophen, which is less than the recommended maximum dose, was thought to have caused acute liver failure in a 73-year-old female. Four courses of postoperative adjuvant chemotherapy were given, without liver damage until the third course. After the administration of the fourth course, the patient experienced nausea and vomiting. She was admitted to the hospital with a diagnosis of enteritis a week later. At the time of admission, there was no liver impairment. For abdominal pain caused by enteritis, acetaminophen was administered intravenously over two days, totaling 4,000 mg. On the third day, acute liver failure developed, and N-acetylcysteine was administered. There was no improvement after the introduction of treatment; hence, 1,000 mg/day of steroid pulse therapy was administered. The patient's liver function started to improve, and she was discharged from the hospital two weeks later. This case suggests that the amount of acetaminophen used per unit of body weight may be unintentionally greater for adults with a small physique; thus, physicians should provide sufficient monitoring to discover side effects early and ensure there is appropriate use.

Unilateral chorea linked to cavernous haemangioma involving the putamen improved by surgery.

Unilateral chorea movements caused by cavernous haemangioma in the putamen are extremely rare. We report a case with chorea movements linked to cavernous haemangioma, localised to an area including the putamen in which pharmacotherapy was found to be ineffective. Symptoms were, however, improved by resection of the cavernous haemangioma. In cases where chorea movements linked to cavernous haemangioma, involving the putamen, prove intractable with watchful waiting or pharmacotherapy, improvement can be expected with surgical removal of the cavernous haemangioma. It is also possible to reduce the risk of complications through the use of intraoperative navigation and monitoring.

Aberrant Right Subclavian Artery Complicated by Acquired Hemophilia A and a Subclavian Artery-Esophageal Fistula after Traumatic Injury.

An aberrant right subclavian artery (ARSA) is a rare developmental anomaly wherein the right subclavian artery arises from the descending aorta as a fourth branch of the aortic arch. We present the case of ARSA in an 81-year-old woman who was injured in a motorcycle accident. The patient had a history of asymptomatic cerebral infarction, type 2 diabetes mellitus, and rheumatoid arthritis. She was diagnosed with spleen and liver injury, left renal injury, along with fractures in the rib, pelvic, vertebrae, and right tibia. On the 3rd hospitalization day, activated partial thromboplastin time (APTT) prolongation was observed, followed by sudden massive hematemesis and shock on the 39th day. We indicate sudden hematemesis and ARSA bleeding as the cause. We performed compression with a Sengstaken-Blakemore tube and coil embolization for hemostasis. Our findings show that the bleeding was mainly caused by nasogastric tube compression, prolonged APTT, and acquired hemophilia A.

Systemic Capillary Leak Syndrome Induced by Influenza Type A Infection: A Case Report.

Rhabdomyolysis accompanying influenza virus infection is a notable extrapulmonary complication. We experienced a case of influenza type A followed by rhabdomyolysis and systemic capillary leak syndrome (SCLS). A 57-year-old man with no significant past medical history was diagnosed as having influenza type A six hours after fever onset, and treatment with oseltamivir was started. Shock, rhabdomyolysis, and acute kidney injury (AKI) progressed rapidly. At 53 hours after starting the oral treatment, intensive care was initiated, including ventilation management. In the acute phase, a large-dose replacement was given for the SCLS and continuous renal replacement therapy for AKI; both eventually healed without sequelae.