Effectiveness of lidocaine infusion for status epilepticus in childhood: a retrospective multi-institutional study in Japan

We evaluated the usefulness of intravenous lidocaine therapy for managing of status epilepticus (SE) during childhood in a retrospective multi-institutional study. Questionnaires were sent to 28 hospitals concerning patients admitted for SE who were managed with lidocaine, assessing patient characteristics, treatment protocols and efficacy. In 279 treated patients, 261 SE occurrences at ages between 1 month and 15 years were analyzed. SE was classified as showing continuous, clustered, or frequently repeated seizures. Considering efficacy and side effects in combination, the usefulness of lidocaine was classified into six categories: extremely useful, useful, slightly useful, not useful, associated with deterioration, or unevaluated. In 148 SE cases (56.7%), lidocaine was rated as useful or extremely useful. Multivariate analysis indicated lidocaine was to be useful in SE with clustered and frequently repeated seizures, and SE attributable to certain acute illnesses, such as convulsions with mild gastroenteritis. Efficacy was poor when SE caused by central nervous system (CNS) infectious disease. Standard doses (approximately 2mg/kg as a bolus, 2mg/kg/h as maintenance) produced better outcomes than lower or higher doses. Poor responders to the initial bolus injection of lidocaine were less likely to respond to subsequent continuous infusion than good initial responders. We recommend lidocaine for use in SE with clustered or frequently repeated seizures, and in SE associated with benign infantile convulsion and convulsions with mild gastroenteritis. Lidocaine should be initiated with a bolus of 2mg/kg. If SE is arrested by the bolus, continuous maintenance infusion should follow; treatment should proceed to different measures when SE shows a poor response to the initial bolus of lidocaine.     

Effects of FAK ablation on cerebellar foliation, Bergmann glia positioning and climbing fiber territory on Purkinje cells

Focal adhesion kinase (FAK) is a non-receptor tyrosine kinase that is widely expressed in the brain, and plays key roles in various cellular processes in response to both extracellular and intracellular stimuli. Here, we explored the role of FAK in cerebellar development. In the mouse cerebellum, FAK was found to be distributed as tiny cytoplasmic aggregates in various neuronal and glial elements, including Purkinje cells (PCs), Bergmann glia (BG), parallel fiber (PF)-terminals and climbing fiber (CF)-terminals. The neuron/glia-specific ablation of FAK impaired cerebellar foliation, such as variable decreases in foliation sizes and the lack of intercrural and precentral fissures. Some of the BG cells became situated ectopically in the molecular layer. Furthermore, the FAK ablation altered the innervation territories of CFs and PFs on PCs. CF innervation regressed to the basal portion of proximal dendrites and somata, whereas ectopic spines protruded from proximal dendrites and PFs expanded their territory by innervating the ectopic spines. Furthermore, the persistence of surplus CFs innervating PC somata caused multiple innervation. When FAK was selectively ablated in PCs, diminished dendritic innervation and persistent somatic innervation by CFs were observed, whereas cerebellar foliation and cell positioning of BG were normally retained. These results suggest that FAK in various neuronal and glial elements is required for the formation of normal histoarchitecture and cytoarchitecture in the cerebellum, and for the construction of proper innervation territory and synaptic wiring in PCs.     

Transatrial repair of submitral left ventricular pseudoaneurysm

We report a case of deceleration injury causing traumatic dehiscence of a Medtronic Freestyle aortic bioprosthesis (Medtronic Inc, Minneapolis, MN) in a 70-year-old patient 5 years after a total root reconstruction. The patient presented with progressive exertional dyspnea 6 months after a significant impact during a hockey game. Intraoperatively, there was a posterior dehiscence of the proximal suture line and almost complete tear of the left coronary leaflet. At reoperation, the patient underwent reconstruction of the aortic root and Bentall procedure with a size 23 mechanical aortic valved conduit.     

Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5

Intravascular large B-cell lymphoma (IVLBCL) is pathologically distinct with a broad clinical spectrum and immunophenotypic heterogeneity. A series of 96 patients with IVLBCL (median age, 67 years; range, 41-85 years; 50 men) was reviewed. Anemia/thrombocytopenia (84%), hepatosplenomegaly (77%), B symptoms (76%), bone marrow involvement (75%), and hemophagocytosis (61%) were frequently observed. The International Prognostic Index score was high or high-intermediate in 92%. For 62 patients receiving anthracycline-based chemotherapies, median survival was 13 months. CD5, CD10, Bcl-6, MUM1, and Bcl-2 were positive in 38%, 13%, 26%, 95%, and 91% of tumors, respectively. All 59 CD10- IVLBCL cases examined were nongerminal center B-cell type because they lacked the Bcl-6+MUM1- immunophenotype. CD5 positivity was associated with a higher prevalence of marrow/blood involvement and thrombocytopenia and a lower frequency of neurologic abnormalities among patients with CD10-IVLBCL. Compared with 97 cases of de novo CD5+CD10-diffuse LBCL, 31 cases of CD5+CD10-IVLBCL exhibited higher frequencies of poor prognostic parameters, except age. Multivariate analysis in IVLBCL revealed that a lack of anthracycline-based chemotherapies (P<.001, hazard ratio [HR]: 9.256), age older than 60 years (P=.012, HR: 2.459), and thrombocytopenia less than 100x10(9)/L (P=.012, HR: 2.427) were independently unfavorable prognostic factors; CD5 positivity was not. Beyond immunophenotypic diversity, IVLBCL constitutes a unique group with aggressive behavior.     

Hepatic lesion of invasive thymoma: possibility of direct invasion

Thymomas grow slowly but tend to invade the surrounding tissue. We herein report four patients with hepatic lesions of thymomas, which were thought to have directly invaded the liver. These patients had received combined modality treatments throughout the clinical course, and the hepatic lesions appeared a long time after the initial treatment – 11.8 years on average. In these patients, the prognoses after the appearance of hepatic invasion were poor. Appropriate local control in the early stages of the clinical course may therefore be essential for preventing the extrathoracic spread of invasive thymoma.     

A case of stage IV advanced gastric cancer responding to TS-1/CDDP neoadjuvant chemotherapy which leads to a pathological complete response

A 72-year-old male with advanced gastric cancer (cT3N2M0H0P0CY1, cStage IV) was treated with TS-1/CDDP as neoadjuvant chemotherapy. TS-1 (60 mg/m(2)/day) was orally administered for 3 weeks followed by 2 drug free weeks as a course, and CDDP (60 mg/m(2)) was administered by intravenous drip on day 8. After the fourth course,a significant tumor reduction was obtained. Total gastrectomy, splenectomy, and D 2 type nodal dissection were performed. The histological diagnosis revealed complete disappearance of cancer cells in the stomach and all of the lymph nodes, which is a so-called pathological complete response. The patient has now been in good health without a recurrence for 24 months after surgery. This case suggests that neoadjuvant chemotherapy with TS-1/CDDP is a potential regimen for advanced gastric cancer.     

A case of liver metastasis from anorectal malignant melanoma on hemodialysis treated by chemotherapy

We report a case on hemodialysis with liver metastases from anorectal malignant melanoma treated by dacarbazine (DTIC). A 61-year-old man presented with anal bleeding. An elastic soft mass was palpated in the anal canal, and a biopsy specimen was diagnosed as anorectal malignant melanoma histologically. After introducing hemodialysis for the chronic renal failure, abdominoperineal resection was performed. Two and a half years after surgery, computed tomography showed multiple liver metastases. We chose chemotherapy consisting of DTIC 100 mg for five consecutive days every 4 weeks in addition to hemodialysis (3 times a week). After three cycles of chemotherapy, liver metastases were stable, but new lung metastases were found. After 12 cycles of chemotherapy, liver metastases became stable, but lung metastases were progressive. Subsequently, the patient died of respiratory failure 4 years after surgery, 1 year and 7 months after the diagnosis of multiple liver metastases. No severe toxicity was observed during this period. We conclude that administration of DTIC undergoing hemodialysis for malignant melanoma with renal failure seems to be useful without severe adverse events.     

A case of recurrent esophageal cancer treated by local injection of OK-432 for 8 months and the patient survived for 2 years and 3 months

It is sometimes difficult to decide a treatment strategy for postoperative recurrence of esophageal cancer. Such recurrent esophageal cancer cases often present with extremely poor prognosis. We report a case of an 85-year-old man with a massive recurrent tumor of mediastinum 3 years after esophagectomy for squamous cell carcinoma (T3N2M0, Stage III) of the intrathoracic esophagus. The operative procedures were transhiatal esophagectomy and gastric reconstruction via posterior mediastinal route. Endoscopic local injection of OK-432 and balloon dilation was given to this patient after mediastinal recurrence. This patient lived for two years and three months after recurrence without severe side effects. Local injection of OK-432 is effective as a palliative therapy for recurrent case.     

Plasma amino acids in patients with essential tremor

Essential tremor (ET) is one of the most common movement disorders. However, there are currently no accepted biomarkers for ET. This report suggested that concentration of plasma glutamic acid, aspartic acid, and taurine could be biomarkers for ET.