Is the atlas size associated with the pathophysiology of symptomatic spinal canal stenosis at the C1 level?

Narrow cervical spinal canal is an important risk factor for the development of cervical myelopathy. Patients with this disease often present with congenital narrowness of the cervical spinal canal. While there are studies on patients with subaxial spinal canal stenosis (SAS), few examined the coexistence of congenital narrow spinal canal in patients with cervical myelopathy at the C1 level. We investigated the characteristics of patients with C1 stenosis (C1S) with special reference to the size of the atlas. Thirteen patients (8 men, 5 women, mean age 76 years) with C1S were retrospectively analyzed and their clinical characteristics and radiological findings were compared with 27 SAS patients and with 26 age-, sex-, and body habitus-matched asymptomatic individuals. Of the 13 C1S patients, 6 presented with a retro-odontoid pseudotumor, 5 with atlantoaxial subluxation, and 2 with ossification or calcification of the transverse ligament; they were significantly older and shorter, and their body weight was significantly lower than in SAS patients (p < 0.001). Their average C1 anteroposterior- and spinal canal diameter was 26.9 ±â€¯2.4 mm and 12.8 ±â€¯4.1 mm, respectively and significantly smaller than in patients with subaxial stenosis (p = 0.004). These measurements were also statistically smaller than in the controls, even after matching for age, gender, height, and body weight (p < 0.05). In patients with C1S, the atlas size was significantly smaller than in SAS patients and asymptomatic controls, indicating an association between a small atlas size and symptomatic spinal canal stenosis at the C1 level.

Concurrent occurrence of an inherited 16p13.11 microduplication and a de novo 19p13.3 microdeletion involving MAP2K2 in a patient with developmental delay, distinctive facial features, and lambdoid synostosis.

A female patient presented with developmental delay, distinctive facial features, and congenital anomalies, including a heart defect and premature lambdoid synostosis. The patient showed a paternally inherited 16p13.11 microduplication and a de novo 19p13.3 microdeletion involving the mitogen-activated protein kinase kinase 2 gene (MAP2K2), in which mutations cause the cardio-facio-cutaneous (CFC) syndrome. Reports of patients with overlapping 19p13.3 microdeletions of this region describe similar clinical manifestations including distinctive facial features: prominent forehead, horizontal/down-slanting palpebral fissures, long midface, pointed chin/angular jaw, sparse eyebrows, and underdeveloped cheekbones. Some of these findings overlapped to that of the patients with 16p13.11 microduplications and CFC syndrome. Although craniosynostosis was occasionally observed in patients with dominant-negative mutations in RAS/MAP kinase signaling genes (RASopathies) related to CFC syndrome, it was also reported in two patients with 16p13.11 microduplications. Genetic contributions of both chromosomal aberrations were discussed.

A multicenter phase I/II study of the BCNU implant (Gliadel(®) Wafer) for Japanese patients with malignant gliomas.

Carmustine (BCNU) implants (Gliadel(®) Wafer, Eisai Inc., New Jersey, USA) for the treatment of malignant gliomas (MGs) were shown to enhance overall survival in comparison to placebo in controlled clinical trials in the United States and Europe. A prospective, multicenter phase I/II study involving Japanese patients with MGs was performed to evaluate the efficacy, safety, and pharmacokinetics of BCNU implants. The study enrolled 16 patients with newly diagnosed MGs and 8 patients with recurrent MGs. After the insertion of BCNU implants (8 sheets maximum, 61.6 mg BCNU) into the removal cavity, various chemotherapies (including temozolomide) and radiotherapies were applied. After placement, overall and progression-free survival rates and whole blood BCNU levels were evaluated. In patients with newly diagnosed MGs, the overall survival rates at 12 months and 24 months were 100.0% and 68.8%, and the progression-free survival rate at 12 months was 62.5%. In patients with recurrent MGs, the progression-free survival rate at 6 months was 37.5%. There were no grade 4 or higher adverse events noted due to BCNU implants, and grade 3 events were observed in 5 of 24 patients (20.8%). Whole blood BCNU levels reached a peak of 19.4 ng/mL approximately 3 hours after insertion, which was lower than 1/600 of the peak BCNU level recorded after intravenous injections. These levels decreased to less than the detection limit (2.00 ng/mL) after 24 hours. The results of this study involving Japanese patients are comparable to those of previous studies in the United States and Europe.

Effect of thrombin concentration on the adhesion strength and clinical application of fibrin glue-soaked sponge.

Fibrin glue-soaked gelatin sponge (FGGS) has been used for tissue sealing in neurosurgical practice, but too rapid clotting of fibrin glue occasionally prevents good fixation of FGGS. Dilution of thrombin may provide adequate manipulation time between mixing fibrinogen and thrombin on gelatin sponge and application into the tissue defects. The present study characterized the effect of thrombin dilution on the adhesion strength of FGGS and retrospectively assessed the clinical usage of the dilution for filling dead space or sealing arachnoid defect in 255 cases who underwent transsphenoidal surgery for the last 66 months. FGGS was prepared using three different concentrations of thrombin: 250 (standard), 50 (1:5 dilution), and 25 (1:10 dilution) units/ml, and incubated for three different periods (5, 20, and 60 seconds). FGGSs were applied over two adjacently positioned porcine skins placed on two metallic plates. The adhesion strength was evaluated by measuring maximum tensile strength during pulling out the sliding plate at a constant rate of displacement. The maximum adhesion strength was greater for FGGS with 1:10 diluted thrombin solution than for FGGS prepared with higher concentrations (p < 0.05). Adhesion strength did not decay for 20 seconds after the mixture. Only four of 255 cases (1.6%) required second reconstruction of sella floor due to the cerebrospinal fluid leakage. FGGS prepared with diluted thrombin solution can provide adequate adhesion strength for clinical use.

Delayed tentorial herniation after crainoplasty with polymethylmethacrylate: a rare complication.

We report the case of a 49-year-old man who underwent a craniectomy for severe head trauma and subsequent cranioplasty with polymethylmethacrylate. He was discharged with moderate right-hand weakness. Five years after the cranioplasty he experienced double vision and slight right hemiparesis. Magnetic resonance imaging (MRI) revealed signs of tentorial herniation but no specific space-occupying lesions. The hand-moulded polymethylmethacrylate bone flap was found to be uneven and protrusions on the inner surface of the bone graft slightly compressed the brain below. His symptoms improved dramatically after a second cranioplasty using a ceramic implant. Although some complications including infection and cosmetic problems have been reported, tentorial herniation during late follow-up as a specific complication of cranioplasty has not been documented previously. We attribute his neurological improvement to the release of compression from the initial graft and to the consequent restoration of cerebrospinal fluid dynamics.