Epidemiology of post-suboccipital craniotomy headache: A multicentre retrospective study.

The detailed epidemiology and mechanism of post-craniotomy headaches are not well understood. This study aimed to establish the actual clinical incidence and causes of post-craniotomy headaches. Suboccipital craniotomy surgeries performed in six institutions within the five-year study period were included. This study included 311 patients (138 males, 173 female; mean age, 59.3 years old). A total of 145 patients (49%) experienced post-craniotomy headaches. Microvascular decompression surgery, craniectomy and facial spasms were significant risk factors for post-craniotomy headaches. In most cases, the post-craniotomy headaches disappeared within one month; however, some patients suffered from long-term headaches. The craniotomy site and the methods of dura and skull closures should be individually determined for each patient. However, to prevent post-craniotomy headaches, craniotomy, instead of craniectomy, may be considered.

[Isolated central nervous system relapse in type II enteropathy-associated T cell lymphoma].

A 75-year-old male presented with progressive lower abdominal discomfort. CT scan demonstrated hypertrophy of the intestinal wall, small bowel dilatation, and masses in the descending colon. Biopsy specimens of the jejunum and descending colon revealed widespread distribution of medium-sized atypical lymphocytes with an immunophenotype, positivity for CD3, CD8, CD56, TAI-1, granzyme B and TCRß, but negativity for CD4, CD5, CD20, CD30 and EBER-ISH. Type II enteropathy-associated T cell lymphoma (EATL; Lugano, stage IIE) was diagnosed. Subsequently, he received 6 cycles of chemotherapy with 2/3 dose CHOP and obtained complete remission. However, 18 months after the initial presentation, he presented with rapidly progressive mental deterioration. Gadolinium enhanced T1-weighted brain MR images showed multiple masses with mild heterogeneous enhancement. Brain biopsy revealed necrotic tumors composed of medium-sized atypical lymphocytes, positive for CD3, CD8, CD56, TIA-1, granzyme B and TCRß, but negative for CD4, CD20, and EBER-ISH. CT scan disclosed no evidence of systemic lymphoma relapse, indicating central nervous system relapse of EATL. Despite immediate high-dose chemotherapy with methotrexate, he died of disease progression. EATL is a rare disease with a very poor outcome, for which a validated standard treatment is still lacking. Further studies are needed to identify innovative therapies for treating EATL.

Vascular Ehlers-Danlos syndrome without the characteristic facial features: a case report.

As a type of Ehlers-Danlos syndrome (EDS), vascular EDs (vEDS) is typified by a number of characteristic facial features (eg, large eyes, small chin, sunken cheeks, thin nose and lips, lobeless ears). However, vEDs does not typically display hypermobility of the large joints and skin hyperextensibility, which are features typical of the more common forms of EDS. Thus, colonic perforation or aneurysm rupture may be the first presentation of the disease. Because both complications are associated with a reduced life expectancy for individuals with this condition, an awareness of the clinical features of vEDS is important. Here, we describe the treatment of vEDS lacking the characteristic facial attributes in a 24-year-old healthy man who presented to the emergency room with abdominal pain. Enhanced computed tomography revealed diverticula and perforation in the sigmoid colon. The lesion of the sigmoid colon perforation was removed, and Hartmann procedure was performed. During the surgery, the control of bleeding was required because of vascular fragility. Subsequent molecular and genetic analysis was performed based on the suspected diagnosis of vEDS. These analyses revealed reduced type III collagen synthesis in cultured skin fibroblasts and identified a previously undocumented mutation in the gene for a1 type III collagen, confirming the diagnosis of vEDS. After eliciting a detailed medical profile, we learned his mother had a history of extensive bruising since childhood and idiopathic hematothorax. Both were prescribed oral celiprolol. One year after admission, the patient was free of recurrent perforation. This case illustrates an awareness of the clinical characteristics of vEDS and the family history is important because of the high mortality from this condition even in young people. Importantly, genetic assays could help in determining the surgical procedure and offer benefits to relatives since this condition is inherited in an autosomal dominant manner.

Evaluation of cerebral function using iomazenil SPECT for patients with traumatic brain injury.

Traumatic brain injuries demonstrate various symptoms, including the disturbance of higher brain function, which is not visualized as a morphological lesion on magnetic resonance (MR) imaging. We examined the use of iomazenil single photon emission computed tomography (SPECT) for patients with traumatic brain injury and evaluated its diagnostic value. The study population included patients who were admitted to our hospital for traumatic brain injuries. All patients survived and were discharged from our hospital. MR imaging and iomazenil SPECT were examined during the acute and/or chronic phases. MR images were acquired using a 1.5-T clinical instrument. The T1- and T2-weighted and fluid-attenuated inversion recovery (FLAIR) axial images were evaluated. SPECT images were acquired using a multi-detector SPECT machine 3 h after the intravenous injection of 740 MBq of iomazenil. Axial, statistically analyzed images and stereotactic extraction estimation images were reconstructed and evaluated statistically based on the Z-score for each cerebral cortex. Iomazenil SPECT showed various lesions that were not demonstrated by MR imaging. Some clinical symptoms correlated with the iomazenil SPECT findings. Iomazenil SPECT is thus considered to be valuable for evaluating both brain lesions and the brain function after traumatic brain injury.

Current practices and future directions of therapeutic strategy in glioblastoma: survival benefit and indication of BNCT.

Since 1998, we are performing clinical studies on treatment of GBM using conventional fractionated photon radiation therapy (CRT), proton beam therapy (PBT) or boron neutron capture therapy (BNCT). We investigated whether these radiation modalities improves the survival of patients with GBM. Sixty-eight cases of newly diagnosed GBM have been treated in our institution. After surgery, radiation therapy was performed using CRT with a dose of 60.0-61.2 Gy (n=36), hyperfractionated PBT concomitant with fractionated photon irradiation with a total dose of 96.6 Gy (n=17), or a single fraction of BNCT (n=15). In PBT, the surrounding volume of 2 cm from main tumor mass and the volume of perifocal edema were irradiated at dose of 75.6 and 60 Gy, respectively. The median OS time of the case series of BNCT for GBM has been reported as 13-20.7 M. In this study, the median OS and median time to MR change (TTM) for all patients were 25.7 and 11.9 M, respectively. The 1- and 2-year survival rates were 85.7% and 45.5%, respectively. On the other hand, in the patients who underwent CRT and ACNU-based chemotherapy, OS and 2-year survival rate were 14.2M and 17.9%, respectively. In the patients who underwent high-dose PBT, OS and 2-year survival rate were 21.3M and 38.5%, respectively. The present small case series of selected patients showed survival benefit after BNCT. The comparison using previously reported prognostic factor-based classifications suggest that outcome of BNCT in terms of survival appeared to have non-inferiority compared to the standard therapy. With respect to the case series as a high-dose radiation trial, the outcome (OS: 9.5-25 M) of previously reported may still be comparable to that of BNCT. Randomized trials of comparably selected patients are required to demonstrate conclusively that prolonged survival is a result of this tumor-selective radiotherapy.

Boron neutron capture therapy for newly diagnosed glioblastoma.

PURPOSE: The efficacy, safety, and dose distribution of neutron capture therapy (NCT) were evaluated in 15 patients with newly diagnosed glioblastoma. METHODS AND MATERIALS: Seven patients received intraoperative NCT (protocol-1) and eight patients received external beam NCT (protocol-2). Sulfhydryl borane (5 g/body) was administered intravenously. Additionally, p-dihydroxyboryl-phenylalanine (250 mg/kg) was given in protocol-2. The external beam NCT was combined with fractionated photon irradiation. RESULTS: Four of 15 patients were alive at analysis for a mean follow-up time from diagnosis of 23.0M. Twelve of the 15 patients were followed up for more than one year, and 10 (83.3%) of the 12 patients maintained their Karnofsky Performance Status (KPS; 90 in eight patients and 100 in two patients) at 12 months. The median overall survival and the time to tumor progression (TTP) for all patients were 25.7 and 11.9 M, respectively. There was no difference in TTP between the protocol-1 (12.0 M) and protocol-2 (11.9 M). The 1- and 2-year survival rates were 80.0% and 53.3%, respectively. Three protocol-1 patients and one protocol-2 patient suffered transient orbital swelling accompanied by double vision (Grade 2); one of the three protocol-1 patients suffered post-epileptic brain swelling (Grade 4) requiring surgical intervention. CONCLUSION: It is suggested that NCT is effective for survival of newly diagnosed glioblastoma with acceptable adverse effects. Because of the limitation of the present NCT pilot study without the contemporary control arm, it is unconvincing whether the neutron capture reaction led to distinct survival benefits, and further optimized studies on less invasive external beam NCT in large series of patients are warranted.

Combined use of sodium borocaptate and buthionine sulfoximine in boron neutron capture therapy enhanced tissue boron uptake and delayed tumor growth in a rat subcutaneous tumor model.

We have previously reported that buthionine sulfoximine (BSO) enhances sodium borocaptate (BSH) uptake by down regulating glutathione (GSH) synthesis in cultured cells. This study investigated the influence of BSO on tissue BSH uptake in vivo and the efficacy of BSH-BSO-mediated boron neutron capture therapy (BNCT) on tumor growth using a Fisher-344 rat subcutaneous tumor model. With BSO supplementation, boron uptake in subcutaneous tumor, blood, skin, muscle, liver, and kidney was significantly enhanced and maintained for 12h. Tumor growth was significantly delayed by using BSO. With further improvement in experimental conditions, radiation exposure time, together with radiation damage to normal tissues, could be reduced.