[A case of chondroblastoma arising from the temporal bone].

A 33-year-old male sustained hearing disturbance in the left ear that exacerbated over a period of three years. The patient was referred to the department of otorhynolaryngology for severe stenosis of the left external auditory canal, where neuroimaging study revealed a huge tumor in the left temporal fossa. On first examination, he showed a significant facial nerve paresis and conductive hearing loss. CT scans identified a 4.5×4.5×4.5 cm mass with intralesional calcification and extensive bony destruction in the squamous and petrous parts of the temporal bone and middle cranial fossa floor. MR imaging demonstrated the tumor of heterogenous intensity on T1-and hypointensity on T2-weighted image. The patient underwent gross total resection of the lesion via frontotemporal craniotomy. The bony and ligamentous structures around the temporomandibular joint appeared mostly intact and did not need any reconstructive surgery after tumor resection. Postoperatively the patient's facial nerve paresis showed a transient exacerbation which resolved gradually, while hearing disturbance did not improve. Histological findings of the tumor were consistent with the qualities of chondroblastoma. We should assume chondroblastoma as differential diagnosis when we encounter a temporal bone tumor that is curable by surgical resection.

[Simultaneous presentation of Tolosa-Hunt syndrome and oculomotor nerve palsy due to the nonruptured internal carotid-posterior communicating artery aneurysm: a case report].

A 45-year-old female developed mild dysesthesia and swelling, followed by ptosisand trigeminal pain, in the right side of the face. Her past medical history was unremarkable, and she had not been aware of any infectious sign. A local otolaryngologist administered glucocorticoid therapy that resolved the face pain, but the ptosis persisted. Neurological examination found complete right oculomotor nerve paresis and mild sensory loss in the first and second segments of the right trigeminal nerve. Blood examination found no abnormalities. Neuroimaging revealed a saccular aneurysm at the branching site of the posterior communicating artery, projecting posteriorly and adjacent to the dorsum sellae, without other intracranial abnormalities. Cerebral angiography demonstrated poor opacification of the superior ophthalmic vein and cavernous sinus on the right side. The patient underwent coil embolization under a diagnosis of symptomatic aneurysm, but her oculomotor neuropathy was only partially improved. We thought that the impairment of the oculomotor function by inflammatory reaction in the cavernous sinus and mechanical compression by the aneurysm had already persisted for too long for post-treatment recovery. We think that the simultaneous occurrence of Tolosa-Hunt syndrome and oculomotor nerve palsy may have resulted because trigeminal neuralgia had increased the blood pressure to induce rapid growth of the preexisting aneurysm, or the inflammatory reaction in the cavernous sinus had promoted the growth of the aneurysm, or that the association was by chance.

Spinal instrumentation for interfacet locking injuries of the subaxial cervical spine.

A retrospective analysis of our surgical management of traumatic interfacet locking was performed. Eleven interfacet locking injuries were surgically treated. An anterior procedure was performed in five patients, posterior fixation in three and a combined procedure in three. Five facet locks were reduced by preoperative skull traction. After general anesthesia at surgery, another two cases were corrected manually. Surgical fixation using spinal instrumentation was performed. One patient treated with posterior fixation required an additional anterior procedure because of a delayed disc herniation. Spinal instrumentation avoided a halo vest. The anterior approach may be selected in patients who are reduced manually, while a combined procedure should be performed in patients with irreducible facet dislocation with disc herniation. Delayed symptomatic disc herniation may occur when only posterior fixation is performed.

Spinal angiolipoma in a pregnant woman presenting with acute epidural hemorrhage.

A 26-year-old woman in week 31 of pregnancy presented to the emergency room with acute onset of paraplegia. Her medical history was unremarkable. Neurological examination revealed complete paraplegia, total sensory loss below the T7 dermatome, and significant vesicorectal dysfunction. MRI revealed an intraspinal mass from T3 to T4, which was hyperintense on both T1-weighted and T2-weighted images. Blood examination found no abnormality. She underwent emergent hemilaminectomy and removal of the hematoma. Intraoperatively, unusually ectatic venous vessels were found adhered to the lower surface of the epidural clot. No concurrent vascular malformations were identified and the dura mater was intact. The histological diagnosis was angiolipoma. Postoperatively her neurological deficits showed remarkable improvement, and she gave birth to a healthy baby. Spinal angiolipoma in a pregnant woman may be complicated with acute epidural hemorrhage. Emergent surgical evacuation can be performed safely with a good functional prognosis.

Intramedullary primitive neuroectodermal tumor presenting with rapidly-progressive cauda equina syndrome.

A 39-year-old male presented with gait disturbances with rapid deterioration for 2 weeks. Neurological examination found paraparesis, sensory loss in the L1-S5 dermatomes, and vesicorectal dysfunction. Magnetic resonance (MR) imaging revealed a fusiform intramedullary tumor at T12-L1 levels with heterogeneous enhancement. The patient underwent microsurgical tumor resection. A myelotomy exposed a highly vascular tumor that was subtotally resected. Histological examination demonstrated hypercellular tumor accompanied by significant cell atypism and mitotic figures. Immunohistochemical staining was positive for glial fibrillary acidic protein, S-100 protein, synaptophysin, and INI-1, consistent with primitive neuroectodermal tumor (PNET). Postoperatively, the patient underwent irradiation to the whole craniospinal axis. He experienced local recurrence 7 months after surgery. MR imaging performed at 10 months revealed holocord progression and intracranial dissemination. The patient died 13 months after the onset of the disease. PNET should be considered in the differential diagnosis of an intramedullary spinal cord tumor.

Infantile chronic subdural hematoma infected by Escherichia coli--case report.

A 6-month-old boy presented with a rare case of infected subdural hemorrhage manifesting as sustained fever and focal seizure. The boy had been well without contributory medical history. Physical examination found no neurological impairment with intact superficial appearance and soft fontanels. The parents denied recent head trauma or shaking injury. Blood examination was normal except for white blood cell count of 19200/microl and C-reactive protein level of 6.7 mg/dl. Bacterial culture of nasal swab, urine, stool, and venous blood samples was negative. Cerebrospinal fluid examination showed normal findings. Cranial computed tomography revealed an expansive subdural fluid collection in the right frontotemporal region. Magnetic resonance imaging showed the lesion as hypointense on T1- and hyperintense on T2-weighted images with intense enhancement of the outer membrane. The patient underwent burr-hole drainage, which identified the subdural hematoma encapsulated in a thick outer membrane and intermingled with pus material. Culture of the pus identified Escherichia coli. The patient received antibiotic therapy for 8 weeks that resulted in complete resolution of the infection. We assumed that preexisting subdural hematoma formed after minor head trauma was followed by hematogenous infection by E. coli. Infected subdural hematoma is possible in infants presenting with subdural hemorrhage with clinical symptoms of bacteremia.

Huge supratentorial extraventricular anaplastic ependymoma presenting with massive calcification--case report.

A 15-year-old boy presented with an anaplastic supratentorial ependymoma causing massive intratumoral calcification, without contributory medical and family history, and manifesting as persistent headache for 2 months. Physical examination found no neurological deficit except for visual defect in the right lower quadrant, with intact visual acuity. Blood examination showed no abnormalities. Cranial computed tomography revealed a huge calcified mass in the left parietooccipital lobe, with extensive perilesional brain edema. Cranial radiography showed diffuse and symmetrical thinning of the calvarial bone. Magnetic resonance imaging confirmed the tumor as an assembly of medullated masses with extraventricular location, 7 x 6.5 x 6.5 cm in diameter, and appearing as heterogeneous intensity on both T(1)- and T(2)-weighted images with inhomogeneous enhancement except for the central cores. The patient underwent tumor resection. Intraoperative findings revealed that the cortical veins overlying the tumor were reddish and moderately engorged. The hypervascular tumor, entirely extraventricular in location, was totally resected without neurological deterioration. Histological examination revealed that the tumor was highly cellular with hyperchromatic nuclei and cell atypia. Necrosis, mitotic figures, and perivascular pseudorosette formations were frequently seen. Immunohistochemical study showed positive staining for glial fibrillary acidic protein, S-100 protein, vimentin, and epithelial membrane antigen, but negative for synaptophysin. The MIB-1 labeling index was 26.5%. The findings were compatible with anaplastic ependymoma (World Health Organization classification grade 3). Ependymoma should be included in the differential diagnosis of a supratentorially located, extraventricular mass with massive intratumoral calcification.

Arteriovenous fistula arising from the persistent primitive olfactory artery with dual supply from the bilateral anterior ethmoidal arteries.

A 59-year-old male presented with generalized seizure. The patient had not been aware of any traumatic head injuries or preceding infection, and had no contributory medical history. On admission, he was alert and well oriented, without neurological impairment or headache. He was afebrile and blood examination showed no abnormal findings. Computed tomography revealed an irregular intracerebral hematoma, 3 x 1.5 cm in diameter, in the left rectal gyrus. Cerebral angiography showed an arteriovenous fistula (AVF) in the anterior cranial fossa supplied only by the persistent primitive olfactory artery (PPOA) originating from the anterior cerebral artery, forming a shunt to an ascending cortical vein, and drained by the superior sagittal sinus. The patient underwent endovascular obliteration of the AVF via the transarterial route. Immediately after successful isolation, angiography showed that the bilateral anterior ethmoidal arteries supplied the AVF. The feeding branches from the left anterior ethmoidal artery were completely occluded via the ophthalmic artery, but introduction of the catheter into the right ophthalmic artery markedly decreased the stump pressure. Follow-up angiography performed at 3 and 8 weeks following embolization showed spontaneous resolution of the residual AVF without findings of recanalization or new abnormal channels. AVF arising in the anterior cranial fossa may be associated with an unusual pattern of the blood supply when including the PPOA.

Analysis of neuronal cell death in the cerebral cortex of H-Tx rats with compensated hydrocephalus.

OBJECT: Some cases of compensatory hydrocephalus have been reported in which cognitive deficiency progresses despite the absence of progressive ventricular dilation. In this study, the differentially expressed genes in compensated hydrocephalic H-Tx rat cortices were determined. A molecular mechanism that induces neuronal death in the cerebral cortex of compensated hydrocephalus is proposed. METHODS: The cerebral cortices of 8-week-old H-Tx rats with spontaneously arrested hydrocephalus (hH-Tx) and nonhydrocephalic H-Tx (nH-Tx) control rats were subjected to cDNA microarray analysis followed by canonical pathway analysis. RESULTS: In the hH-Tx rats, many genes in the amyloidal processing pathway showed altered expression, including Akt3 and p38 MAPK. These latter genes are involved in tau protein phosphorylation, and their increased expression in hydrocephalus was confirmed by real-time polymerase chain reaction analysis. Immunohistological and immunoblot analysis revealed elevated phosphorylated tau expression in the cerebral cortex neurons of the hH-Tx rats. CONCLUSIONS: The accumulation of phosphorylated tau protein in the cerebral cortex may be one of the mechanisms by which later cognitive dysfunction develops in patients with compensated hydrocephalus. More work needs to be done to determine if the accumulation of phosphorylated tau in the cortex can help predict which patients may decompensate thus requiring more aggressive treatment for compensated hydrocephalus.