Long-term imaging course of Chiari malformation type I due to fibrous dysplasia/McCune-Albright syndrome.

INTRODUCTION: McCune-Albright syndrome (MAS) and fibrous dysplasia (FD) have been reported to cause Chiari type I malformation (CM1) and skull base invagination (BI). CASE: A 6-year-old girl was diagnosed with MAS and FD. She was diagnosed with CM1 at age 8 years, and the syringomyelia had gradually increased by age 20 years. We performed foramen magnum decompression and C1 laminectomy, and the syringomyelia stopped spreading after surgery. DISCUSSION: This patient underwent long-term radiological observation and morphological evaluations, which revealed that the skull thickening was progressing, while the posterior cranial fossa volume (PCFV) remained unchanged for 14 years. Therefore, although PCFV did not decrease, it was considered to be relatively inadequate due to the increase in brain volume with growth, resulting in posterior fossa overcrowding, causing CM1. CONCLUSION: In patients with FD/MAS, long-term evaluation of bone thickening, odontoid position, and PCFV is necessary.

[Spinal Cord Tumors:Classification, Treatment, and Prognosis].

Spinal cord tumors are classified into three types according to the site of origin: epidural tumors, intradural extramedullary tumors, and intramedullary tumors. It is important to understand the common tumors at each site. Most intradural extramedullary tumors are benign schwannomas or meningiomas and need surgical removal. For intramedullary tumors, it is important to determine the type of tumor and the site of origin before selecting the surgical approach.

Concurrent Ossification of the Posterior Atlantoaxial Interlaminar Membrane and Atlas Hypoplasia: A Case Report.

An onset of cervical myelopathy due to ossification of the posterior atlantoaxial membrane (PAAM) is extremely rare in older patients, and its clinical characteristics are still unclear. We report an onset of ossification of PAAM with congenital atlas hypoplasia in an 81-year-old man who presented with a 2-year history of progressive cervical myelopathy. Cervical computed tomography (CT) revealed canal stenosis secondary to a hypoplastic posterior arch of the atlas with a diameter of 20.3 mm between the anterior and posterior process. Magnetic resonance imaging showed marked spinal cord compression at the level of C1-2 secondary to atlas hypoplasia as well as ossification of PAAM. The patient underwent laminectomy of C1 and partial C2, as well as removal of the ossification, without atlantoaxial fusion. His neurological status improved 1 year postoperatively. In older patients, cervical myelopathy secondary to PAAM ossification, in the absence of trauma and atlantoaxial instability, may be induced by age-related pathophysiology associated with congenital atlas hypoplasia.

Bow Hunter's Stroke Due to Stretching of the Vertebral Artery Fenestration: A Case Report.

Bow Hunter's syndrome is an unusual symptomatic vertebrobasilar insufficiency resulting from intermittent mechanical compression of the vertebral artery, and is rarely a trigger for cerebral infarction following thrombus formation on the damaged endothelial vessels (Bow Hunter's stroke). The authors present an extremely rare case of a 45-year-old man showing Bow Hunter's stroke due to congenital vertebral artery fenestration stretching and sliding between C1 and C2 after head rotation to the right. Congenital vertebral artery anomaly rarely causes cerebral infarction, but could cause embolic strokes by mechanical stretching without bony abnormalities.

Syringomyelia and arachnoid cysts associated with spinal arachnoiditis following subarachnoid hemorrhage.

A 66-year-old woman with primary Sjogren syndrome developed syringomyelia following two episodes of subarachnoid hemorrhage (SAH) due to the rupture of basilar artery aneurysms. Gait disturbance and abnormal sensation with pain over the foot and abdomen appeared 3 years after the last SAH. Magnetic resonance (MR) imaging revealed a syringomyelia throughout the thoracic cord, from the T2 to T11 levels. In addition, the thoracic cord was compressed by multiple arachnoid cysts in the ventral side of spinal cord. Computed tomography myelography revealed complete block of cerebrospinal fluid (CSF) flow at the T7 level. Surgery for microlysis of the adhesions and restoration of the CSF flow pathway was performed. Postoperatively, leg motor function slowly improved and she could walk unaided. However, abdominal paresthesia was persisted. Postoperative MR imaging revealed diminished size of the syrinxes. We should recognize syringomyelia and arachnoid cysts due to adhesive arachnoiditis as a late complication of SAH. Microlysis of the adhesions focusing on the lesion thought to be the cause of the symptoms is one of the choices to treat massive syringomyelia and arachnoid cysts associated with arachnoiditis following SAH.

[Remote cerebellar hemorrhage after cervical spinal surgery: two case reports and literature review].

Remote cerebellar hemorrhage (RCH) following spinal surgery is extremely rare. We present two cases of RCH following cervical spinal surgery. The first case is a 71-year-old female. She underwent surgery for atlantoaxial subluxation and a subcutaneous suction drain was placed. Three hours after surgery, she became comatose and computed tomography (CT) revealed RCH. The second case is a 51-year-old female. She underwent surgery for ossification of posterior longitudinal ligament in the cervical spine. Intraoperatively, the dura was opened unintentionally and a subcutaneous suction drain was placed. She complained of a severe headache and nausea postoperatively, and CT obtained two days after the surgery demonstrated RCH. In both cases, drains contained much fluid, which was assumed to be consistent with cerebrospinal fluid. Both cases were managed conservatively and displayed no cerebellar symptoms on discharge. Rapid loss of a great amount of cerebrospinal fluid might be the causative factor of RCH. RCH might occur after any type of spinal surgery with dural tear or intradural manipulation. Early diagnosis is particularly important for the treatment of RCH following spinal surgery and spinal drainage might be useful to manage cerebrospinal fluid leakage.

Positional vertebral artery compression and vertebrobasilar insufficiency due to a herniated cervical disc.

The authors report a case of vertebrobasilar insufficiency caused by vertebral artery (VA) compression due to a herniated cervical disc, which was surgically treated with the aid of intraoperative angiography. This 78-year-old man visited the hospital because of syncope following head rotation. Admission CT scans revealed a calcified mass adjacent to the right lateral process of the C-4 spine. Cervical angiography demonstrated an obstruction of the right VA at this region on rotation of the head to the right. The operation revealed a cervical disc protruding toward the right VA. The disc was surgically removed, and then the decompression of the right VA was confirmed on intraoperative angiography studies. A histopathological examination showed fibrohyaline cartilage, indicating an ossified intervertebral disc. The postoperative course was uneventful, and he has not experienced any syncope since treatment. A cervical disc herniation could be a cause of vertebrobasilar insufficiency by exerting positional compression of the VA. Intraoperative angiography could be quite useful to confirm this condition during decompression surgery for a cervical VA.

Remote cerebellar hemorrhage following thoracic spinal surgery.

A 51-year-old man underwent surgery for ossification of the ligamentum flavum at the T9-T10 levels. Intraoperatively, the dura was opened unintentionally and a subcutaneous suction drain was placed. The patient complained of severe headache and nausea postoperatively. Brain computed tomography obtained 3 days after the surgery demonstrated remote cerebellar hemorrhage and hydrocephalus. Suboccipital decompression, C1 laminectomy, and ventriculostomy were performed and his symptoms subsided 2 months later. Remote cerebellar hemorrhage following spinal surgery is extremely rare, but may occur after any type of spinal surgery resulting in dural tear or intradural manipulation. Early diagnosis is particularly important for the treatment of remote cerebellar hemorrhage following spinal surgery.

[Efficacy of antimicrobial prophylaxis in neurosurgical operations].

OBJECT: It is reported that antimicrobial prophylaxis (AMP) reduces the incidence of surgical site infection (SSI) in neurological surgery. However, a great deal of variation exists regarding the type of antibiotics, dose, timing and duration. In this study, the authors analyzed the incidence of SSI comparing two different AMP protocols. CLINICAL MATERIALS AND METHODS: Five hundred and fifty patients who had undergone neurosurgeries at our institute between April 2005 and August 2007 were reviewed retrospectively. They were divided into the protocol F (309 patients with two or more days AMP) and the protocol P (241 patients with one-day AMP). RESULTS: Baseline characteristics were not statistically different between two protocols. The overall rate of SSI was 1.5%. Although SSI showed a trend of low SSI incidence in the protocol P (0.8%), this was not statistically significant compared with that in the protocol F (1.9%). CONCLUSIONS: The one-day (< 24 hours) administration of AMP is enough to prevent SSI in neurological surgery.

[Spontaneous cervical epidural hematoma presenting with hemiparesis following neck extension: a case report].

A 42-year-old woman suddenly developed weakness in her left extremities when stretching her neck two days after the onset of a nuchal pain. Because computed tomography (CT) of the brain did not show any apparent lesion, the patient had initially been treated as having a cerebral infarction until magnetic resonance imaging (MRI) of the cervical spine revealed a presence of a cervical epidural hematoma the next day. She was therefore transferred to our hospital, and a neurological examination showed moderate left hemiparesis, dissociated sensory loss under C6 on the right side, urinary incontinence, and left miosis and ptosis. A CT of the cervical spine demonstrated an anteriorly located left-sided epidural hematoma extending from C4 to C7. The T2-weighted MRI revealed hyperintense lesions around the gray matter on the left side that were compressed by the epidural hematoma. The patient underwent an emergent laminoplasty from C3 to C7. Although her neurological signs were consistent with Brown-Sequard syndrome, which was associated with left-sided Homer's sign, they gradually and completely subsided following surgery. The authors therefore emphasize that cervical lesions should be considered in the differential diagnosis in patients with acute onset of hemiparesis.

Expression of the constitutively activated RelA/NF-kappaB in human astrocytic tumors and the in vitro implication in the regulation of urokinase-type plasminogen activator, migration, and invasion.

Although malignant gliomas are highly invasive tumors, a characteristic that contributes to the commonly observed therapeutic failures and local disease recurrences, the molecular events that regulate invasion in these tumors remain poorly understood. Because the transcription factor RelA/NF-kappaB has been shown to regulate invasion during several cellular processes, we have examined immunohistochemically expression of the constitutively activated RelA/NF-kappaB in tissues obtained from 49 astrocytic tumors [8 diffuse astrocytomas, 9 anaplastic astrocytomas (AAs) and 32 glioblastomas (GBMs)]. In addition, we examined the in vitro effects of antisense oligonucleotides and curcumin on the expression and activation of RelA/NF-kappaB, urokinase-type plasminogen activator (u-PA) expression, migration, and invasion in the T98G glioma cell line. Expression of the constitutively activated RelA/NF-kappaB was observed in 2 (25%) of 8 cases of diffuse astrocytomas, 5 (55.6%) of 9 cases of AAs, and 30 (93.8%) of 32 cases of GBMs. This expression was significantly correlated with the malignant potential in astrocytic tumors (P < 0.001). Moreover, antisense oligonucleotides and curcumin inhibited phorbol-12-myristate-13-acetate (PMA)-induced RelA/NF-kappaB expression or activation (or both), down-regulated u-PA expression, and reduced the migration and invasive potentials of T98G glioma cells. Thus, the expression of constitutively activated RelA/NF-kappaB is associated with malignancy potential in astrocytic tumors and may play a critical role in the regulation of u-PA expression and invasiveness in gliomas. RelA/NF-kappaB may therefore be an intriguing candidate for studies aimed at understanding and prevention of the invasiveness of gliomas.

Immunohistochemical expression of E-cadherin in metastatic brain tumors.

The adhesion molecule E-cadherin has been shown to influence malignant transformation of tumors, including local and distant metastases. We examined the expression of E-cadherin to determine its relationship to the development of metastasis in metastatic brain tumors. Immunohistochemistry for E-cadherin and Ki-67 was carried out in 76 formalin-fixed, paraffin-embedded archival specimens of metastatic brain tumors and in 14 corresponding available primary tumors from patients who received treatment for metastatic brain tumors. The primary tumors were mainly lung cancers (51.3%), followed by gastrointestinal tumors (28.9%). E-cadherin was expressed in 62 (81.5%) of 76 cases examined. In metastatic adenocarcinomas, a consistent tendency for E-cadherin expression was noted, regardless of the degree of differentiation or the extent of spread of the disease (P = 0.04). There was a direct correlation between E-cadherin expression and high MIB-1 index in all metastatic brain tumors (P = 0.0007). Pairwise analysis in 14 primary tumors and the corresponding metastatic specimens revealed high E-cadherin and MIB-1 staining in metastatic brain tumors. These results provide a unique association between E-cadherin, systemic metastasis, and proliferation potential in metastatic brain tumors.

Relationship between expression of O6-methylguanine-DNA methyltransferase, glutathione-S-transferase pi in glioblastoma and the survival of the patients treated with nimustine hydrochloride: an immunohistochemical analysis.

Drug resistance is one of the important factors that determine tumor response to chemotherapy. Several candidates for resistance to various chemotherapeutic agents have been elucidated. O6-methylguanine-DNA methyltransferase (MGMT) removes methylation damage induced by nitrosourea from the O6 position of DNA guanines before cell injury. Glutathione-S-transferase (GST) pi is also involved in nitrosourea resistance. We examined the expression of MGMT and GST pi in 18 glioblastomas (GBM) using immunohistochemistry and compared the results with patients' survival after administration of 1-(4-amino-2-methyl-5-pyrimidinyl) methyl-3-(2-chloroethyl)-3-nitrosourea hydrochloride (ACNU)-based chemotherapy. According to the Kaplan-Meier's method, although median progression free survival (PFS) of eight patients whose tumors retained high MGMT (3+ approximately 2+), and 10 patients whose tumors showed low MGMT expression (1+ approximately 0) were nine and 15 months, respectively (p = 0.09), median overall survival (OS) of the two groups were 12 and 22 months, respectively, which were significantly different (p = 0.01). GST pi expression in GBM was not a prognostic factor. It is suggested that GBM with strong staining of MGMT activity may show more resistance to ACNU-based chemotherapy compared to that with low MGMT. The simple immunohistochemical analysis of MGMT in GBM can be a useful method to determine whether ACNU or another treatment regimen should be recommended.