Optic pathway/hypothalamic gliomas (OP/HGs) are rare astrocytic tumors that appear more commonly among young children and often are unresectable. They comprise approximately 2% of all central nervous system tumors and account for 3–5% of pediatric intracranial tumors. Initial manifestations are often visual disturbances, endocrinopathies and hypothalamic dysfunction such as the diencephalic syndrome, and sometimes hydrocephalus due to cerebrospinal fluid (CSF) outflow obstruction. In many cases, the tumors are diagnosed late in the clinical course because they silently enlarge. These tumors consist mostly of histologically benign, World Health Organization (WHO) grade I tumors represented by pilocytic astrocytomas (PA), the rest being pilomyxoid astrocytomas (PXA) – WHO grade II tumors. In young pediatric patients, however, can be seen PXA that show aggressive clinical course such as CSF dissemination. Our small series of 14 non-Neurofibromatosis type 1 (NF-1) OP/HGs PA patients underwent extended resection without any adjuvant treatments. The median age at initial treatment was 11.5 ± 6.90 years (range, 1–25 years) and median follow up 85.5 ± 25.0 months. Surgical resection for OP/HGs results in acceptable middle-term survival, tumor control and functional outcome equivalent to chemotherapy. There is, however, no longer doubt that chemotherapy with or without biopsy and as-needed debulking surgery remains the golden standard in management of OP/H. Clinical conditions and treatment plans for OP/HGs vary depending on their structure of origin.

Microvascular decompression (MVD) is a widely used, safe, and effective treatment for trigeminal neuralgia (TGN). However, the extent of application of this therapeutic method and its outcomes in Japan are currently unclear. To address these questions, the authors analyzed the use of MVD for the treatment of TGN during the 33-month period from July 2010 to March 2013, using data contained in the Diagnosis Procedure Combination database. The analysis revealed that MVD was used for the treatment of TGN in 1619 cases (608 men, 1011 women), with approximately 1.66 times more women treated than men. MVD for TGN was most frequently performed in individuals 60 to 79 years of age; of particular note was the remarkable increase in the number of women in this particular category. The overall number of procedures performed per 100,000 population/year in Japan was 0.46. The number of procedures was larger in prefectures with higher populations, with a tendency toward a higher number of MVD procedures performed in the area designated West than in the East. Discharge outcomes indicated that cure and improvement were achieved in 97.6% of cases, with a mortality rate of 0.2%, and no differences in discharge outcomes between men and women. The mean length of hospital stay in patients undergoing MVD for TGN was 14.8 days. This analysis revealed discernable trends in the use of MVD for the treatment of TGN in Japan.

Elastin encoded by elastin gene (ELN) is a crucial extracellular matrix protein responsible for arterial resilience. The objective of this study was to identify single nucleotide polymorphisms (SNPs) of ELN gene susceptible to intracranial aneurysm (IA) in Korean population. Two SNPs of ELN gene, rs2071307 (Gly422Ser) and rs2856728 (intron), were genotyped in 90 patients with IA and 90 age and frequency matched controls. Fisher’s exact test was conducted to evaluate allelic association with IA. Of the two SNPs in ELN gene, T allele of rs2856728 (intron) showed statistically significant association with increased development of IA (odds ratio [OR]: 2.34, 95% confidence interval [CI]: 1.44–3.81, P = 7.6 × 10⁻⁴). However, G allele of rs2071307 (Gly422Ser) had no significant association with the development of IA (OR: 1.27, 95% CI: 1.44–3.81, P = 0.607). Interestingly, the odds of having rs2856728 variant was approximately 2-fold higher in males than that in females (OR: 3.46 vs. 1.88, P < 0.05). However, none of SNPs showed difference between single and multiple IA in this study. This preliminary study implies that the rs2856728 variant in ELN gene polymorphisms might play crucial roles in the development and pathogenesis of IA in Korean population.

The advent of magnetic resonance imaging (MRI) enables noninvasive measurement of cerebrospinal fluid (CSF) motion, and new information about CSF motion has now been acquired. The driving force of the CSF has long been thought to be choroid plexus (CP) pulsation, but to investigate whether this phenomenon actually occurs, CSF motion was observed in the ventricular system and subarachnoid space using MRI. Eleven healthy volunteers, ranging in age from 23 to 58 years, participated in this study. The MRI sequences used were four-dimensional phase-contrast (4D-PC) and time-spatial labeling inversion pulse (t-SLIP). The 4D-PC images included sagittal images in the cranial midline, coronal images focusing on the foramen of Monro (FOM), and oblique coronal images of the trigone to quantify CSF velocity and acceleration. These values were compared and analyzed as non-parametric data using the Kolmogorov-Smirnov test and the Mann-Whitney U test. 4D-PC showed that the median CSF velocity was significantly lower in the posterior part of the lateral ventricle than in other regions. The quantitative analysis of velocity and acceleration showed that they were decreased around the CP in the trigone. Image analysis of both velocity mapping and t-SLIP showed suppressed CSF motion around the CP in the trigone. These findings cast doubt on CP pulsation being the driving force for CSF motion.

Carcinoma of the external and middle ear is a very rare disease. Despite the various treatment modalities available, its prognosis is still poor. We aimed to analyze the treatment modalities, outcomes, and validity of surgical approaches, especially in advanced tumors in the ear. Twenty-five patients with carcinoma of the external and middle ear were retrospectively analyzed. The modified Pittsburgh staging system was used for staging of the patients. Overall and disease-free survival was estimated using of Kaplan-Meier method. In our cohort of 25 patients, the majority (76%) had tumor located in external auditory meatus. The most common histologic subtype was squamous cell carcinoma (80%). More than half of patients (56%) had tumor stage IV. In the stage IV group, five patients underwent subtotal temporal bone resection and ipsilateral neck dissection. Seven patients underwent definitive radiotherapy. The remaining two patients underwent palliative chemotherapy. The 2-year overall and disease-free survival for patients with tumor stage IV was 67.7% and 57.8%, respectively. In patients with tumor stage IV, the 2-year overall survival for patients who underwent surgery was 80.0% versus 53.6% for those who underwent radiotherapy (P = 0.16). The 2-year disease-free survival for patients who underwent surgery was 80.0% versus 28.6% for those who underwent radiotherapy (P = 0.15). In the present study, the outcome of patients who received surgical treatment tended to be better than that of patients who received radiation therapy. Our results suggest that en bloc resection could be the first choice even in the advanced disease stage.

The factors that lead to the improvement of gait function in patients with diseases of the central nervous system (CNS) who use a hybrid assistive limb (HAL) are not yet fully understood. The purpose of the present study was to analyze these factors to determine the prognosis of the patients’ gait function. Patients whose CNS disease was within 180 days since onset were designated as the subacute-phase patients, and patients whose disease onset had occurred more than 180 days previously were designated as chronic-phase patients. Fifteen subacute-phase patients and 15 chronic-phase patients were given HAL training. The study analyzed how post-training walking independence in these patients was affected by the following factors: age, disease, lesion area, lower limb function, balance, period until the start of training, number of training sessions, additional rehabilitation, higher-order cognitive dysfunction, HAL model, and the use of a non-weight-bearing walking-aid. In subacute-phase patients, walking independence was related to lower limb function (r_s = 0.35). In chronic-phase patients, there was a statistically significant correlation between post-training walking independence and balance (r_s = 0.78). In addition, in patients with a severe motor dysfunction that was accompanied by inattention and global cognitive dysfunction, little improvement occurred, even with double-leg model training, because they had difficulty wearing the device. The results demonstrated that the factors that improved walking independence post HAL training differed between patients with subacute- and chronic-stage CNS diseases. The findings may serve as valuable information for future HAL training of patients with CNS diseases.

A single-center retrospective comparative study was designed to identify the risk factors for restenosis of lumbar foraminal stenosis (LFS) after microscopic foraminal decompression (MFD). 21 consecutive patients who underwent single-level MFD with an average of 19-month follow-up were divided into two study groups based on clinical outcomes; group 1 (7 patients with poor outcomes requiring revision surgery), group 2 (14 patients with good outcomes with no revision surgery required). Changes of lumbar spinal alignment on plain standing radiographs were compared and analyzed between two study groups to investigate the pathology and risk factors associated with restenosis after MFD. Preoperative disc wedging (DW) angle was significantly larger in group 1 than in group 2 (3.5 ± 1.0° vs 1.1 ± 0.2°, P < 0.01). Postoperatively, disc height (DH) and foraminal height (FH) decreased (P < 0.05), and DW deteriorated (P < 0.01) significantly in group 1, while there were no significant changes in group 2. Lumbar lordosis (LL) remarkably improved postoperatively in group 2 (24.7 ± 8.0 to 32.0 ± 7.0, P < 0.001), contrary to limited improvement in group 1 (25.1 ± 9.2 to 27.0 ± 12.0, P = 0.45). Postoperative LL is a predictive factor for restenosis after MFD. Decrease in DH or progression of DW was contributing to restenosis. LFS presenting with large DW and lumbar degenerative kyphosis should be excluded from surgical indications for MFD without instrumented fusion, considering the high recurrence rate.