[Congenital Dermal Sinus with Dermoid Cyst Complicated by Brain Abscess: A Case Report].

Congenital dermal sinus(CDS)is a rare entity of spinal dysraphism, caused by the focal failure of disjunction leading to adhesion between the cutaneous and neural ectoderm. Some reports found that tumors, such as dermoid and epidermoid cysts, meningitis and subdural abscess are often complicated by CDS. A 1-year-old girl was referred to our department for CDS with a dermoid cyst complicated by brain and subdural abscesses. Diffusion weighted imaging and gadolinium-diethylenetriamine penta-aceticacid administration revealed ring-enhancing lesions, suggesting brain abscess, in the left temporal lobe, and subdural abscesses on the bilateral middle cranial bases. T1-and T2-weighted lumbar magnetic resonance imaging revealed CDS. With a preoperative diagnosis of CDS with brain and subdural abscesses, resection of CDS was performed after draining the brain abscess twice. The CDS extended into the spinal canal and it was completely exercised. We then performed sub-total resection of the dermoid cyst in the subdural space. Recent reports showed that dermoid cysts are related to CDS infection and deterioration of the infection, as seen in this case. Complication of dermoid cysts is an important consideration during CDS treatment. While the mechanism of the development of brain abscess in CDS patients has been unclear, this rare but important case revealed the mechanism to be the direct spread of inflammation via CDS.

A Case of Holocord Leptomeningeal Dissemination from Cerebellar Hemangioblastoma without von Hippel-Lindau Disease.

Hemangioblastoma disseminated along leptomeninges from the solitary cranial lesion without von Hippel-Lindau (VHL) disease is a quite rare instance with 23 cases reported in 40 years. We add a new case and discuss these rare instances. A 55-year-old female underwent surgery for total removal of cerebellar hemangioblastoma. Twenty months later, magnetic resonance (MR) images of the spinal cord revealed a tumor compressing the thoracic cord at T3-4 level which was removed en bloc by emergent spinal surgery. However, paraplegia and bowel bladder dysfunction recurred 5 months after the spinal surgery. Spine MR images showed diffuse enhancement of subarachnoid space. Exploratory surgery disclosed that the enhanced lesion was disseminated hemangioblastoma. After whole spinal irradiation, she was transferred to a palliative care hospital. Even after complete removal, possibility of leptomeningeal dissemination demands continuous follow-up. The mechanism of seeding of hemangioblastoma remains unclear, but attention must be paid to avoid spreading tumor cells during surgery because all the disseminated cases had precedent cranial surgery.

A Case of Metastatic Choriocarcinoma With Intracerebral Hemorrhage Caused by a Ruptured Pseudoaneurysm.

A rare cause of cerebral hemorrhage is the metastasis of choriocarcinoma from gynecology. Herein, we report a case of a patient with brain metastasis of choriocarcinoma with cerebral hemorrhage. A 14-year-old female who had undergone surgery for a hydatidiform molar pregnancy presented with a disturbance of consciousness due to cerebral hemorrhage. Imaging studies revealed the presence of a cerebral aneurysm and several mass lesions in the lung field, and high serum beta-human chorionic gonadotropin level was confirmed. Thus, we suspected cerebral hemorrhage caused by brain metastasis of choriocarcinoma. She went into a coma, and an emergency craniotomy was performed to remove the hematoma and aneurysm. The pathology of the aneurysm was pseudoaneurysm due to the rupture of the vascular wall caused by increasing metastatic cells from choriocarcinoma in the cerebrovascular wall. Therefore, multidrug chemotherapy was immediately initiated. The choriocarcinoma, including the metastatic lesions, is in remission. To improve the outcome of choriocarcinoma, it must be diagnosed early, and treatment should be immediately started. Moreover, neurosurgeons should be aware of such diseases and consider them as one of the differential diagnoses, particularly in females of reproductive age with cerebral hemorrhage.

Volumetric-Modulated Arc Therapy for Giant-Cell Tumor of Temporal Bone: An Illustrative Case Report.

Giant-cell tumor of the skull is extremely rare. Surgery is the main treatment for this disease, but not all cases are suitable for complete resection. In this report, we present the clinical features of a case of giant-cell tumor of temporal bone that demonstrated good outcome after radiation therapy (RT) using volumetric-modulated arc therapy (VMAT). The patient was a 55-year-old man with giant-cell tumor of temporal bone who received surgery as the first treatment. Three months after the initial surgery, the tumor regrew, and the patient received surgical resection again. Although second partial resection was undergone, it regrew. Therefore, 36 months after initial surgery, RT was conducted. The prescribed dose was 54 Gy in 1.8 Gy fractions using VMAT. The tumor began to shrink from 4 months after the initiation of RT and kept shrinking slowly and gradually. At the last follow-up, there was no evidence of local recurrence. There was no report about VMAT for giant-cell tumor of the skull, and no report revealed the radiographic details after recent radiation techniques. Therefore, this case report was meaningful in describing the details and response during and after VMAT for giant-cell tumor of temporal bone. The adjuvant RT using VMAT seemed to demonstrate a sufficient local control benefit without severe adverse effects in our case with giant-cell tumor of temporal bone.

Dural Arteriovenous Fistula-Derived Parkinsonism Symptoms: A Case Report with Literature Review.

Objective: Parkinsonism caused by dural arteriovenous fistula (DAVF) is very rare, however, it is reversible by endovascular treatment. We herein report a case of parkinsonism caused by DAVF with review of previous literature. Case Presentation: An 87-year-old woman with parkinsonism and dementia was admitted to our hospital with disturbance of consciousness and aggravated parkinsonism symptoms. Plain CT revealed low-density areas in the brainstem and left cerebellar peduncle. Magnetic resonance images revealed hyperintense lesions on FLAIR, which had elevated apparent diffusion coefficient (ADC) values, in the same lesion of plain CT. However, no edematous change was detected. CT angiograms revealed obstruction of the left transverse and sigmoid sinuses. Dilations of the left superior petrosal sinus, left petrosal vein, and pontine veins were also noted. A low-density area on plain CT had a contrast effect. Cerebral angiography revealed a DAVF involving the left transverse sinus and fed by the left occipital and left middle meningeal arteries. Transarterial embolization (TAE) with Onyx obliterated the DAVF, and parkinsonism symptoms gradually improved. We reviewed 21 DAVF-derived parkinsonism cases, most of which were treated by TAE. Recent cases were treated with Onyx. In many cases, parkinsonism improved after endovascular treatment. Conclusion: DAVF-derived parkinsonism is rare but treatable by endovascular therapy. DAVF should be one of the differential diagnosis of the parkinsonisms.

A Patient Who Underwent Mechanical Thrombectomy for Acute Occlusion of the Vertebral and Radial Arteries.

Objective: We report a case of acute occlusion of the vertebral artery and radial artery. We performed mechanical thrombectomy for the radial artery following mechanical thrombectomy for the vertebral artery. Case Presentation: A 73-year-old woman developed sudden-onset dizziness and dysesthesia of the left finger, and was taken to our hospital. Atrial fibrillation was observed. Image inspection revealed acute cerebral infarction of the left lateral medulla and left cerebellar hemisphere, and occlusion of the vertebral and radial arteries. Mechanical thrombectomy for the left vertebral artery occlusion was performed after intravenous recombinant tissue plasminogen activator (rt-PA), and then mechanical thrombectomy was performed for the left radial artery occlusion. Conclusion: This case suggests that it is possible to guide the system to the radial artery and to perform thrombectomy using existing intracranial endovascular treatment devices.

Angioscopic Findings in 3 Patients Who Required Retreatment After Carotid Artery Stenting.

BACKGROUND: Use of angioscopy to directly observe the stent lumen in the chronic phase after carotid artery stenting (CAS) has not been reported to date. Here we report 3 patients in whom angioscopy helped confirm the stent lumen during retreatment after CAS. CASE DESCRIPTION: Case 1 required retreatment for stent shortening that occurred 1 month after the first CAS. Preprocedure angioscopy showed the presence of neointima, which could not be revealed by intravascular ultrasound (IVUS). In case 2, which required repeat CAS for distal progressive stenosis of the internal carotid artery, the neointima was observed on the stent surface and was more pronounced on the distal side. In case 3, retreatment was necessary for recurrent ischemic stroke caused by stent restenosis; preprocedure angioscopy showed an unstable plaque, which was not detected as vulnerable by IVUS, protruding into the stent lumen, with partial ulceration and bleeding. CONCLUSIONS: Compared with IVUS, angioscopy enables a more detailed observation of the stent lumen. Although angioscopy is relatively invasive, its use in evaluating changes in the stent lumen after CAS should be clarified by accumulation of reported cases.

Intravenous administration of bone marrow stromal cells increases survivin and Bcl-2 protein expression and improves sensorimotor function following ischemia in rats.

Intravenous administration of bone marrow stromal cells (MSCs) in animal models with focal cerebral ischemia has been found to be effective in attenuating neuronal damage. We examined whether intravenously transplanted MSCs alters expression of apoptosis-related proteins. Fisher-344 rats were subjected to 90-min middle cerebral artery occlusion (MCAO). The experimental groups were: (I) vehicle group, with intravenous injection of phosphate-buffered saline (PBS) 3h after MCAO; and (II) transplant group, with intravenous injection of MSCs (3x10(6)cells) 3h after MCAO. Neurological function of rats was evaluated using modified neurological severity score (mNSS) and Rotor-rod Motor Test (RMT). Rats were sacrificed on 1st, 3rd and 7th days of MCAO, and coronal brain sections were stained immunohistochemically to identify the apoptosis-related proteins, namely survivin and Bcl-2. We also examined Terminal Deoxynucleotidyl Transferase-Mediated dUTP-biotin Nick End Labeling (TUNEL)-positive cells on 3rd day of MCAO. Functional recovery according to mNSS and RMT was significantly better in the transplant group as compared with the vehicle group (P<0.05). Immunohistochemical analysis revealed significant expression of survivin on 3rd day and Bcl-2 on 1st and 3rd days in the transplant group. The vehicle group displayed significantly more TUNEL-positive cells than the transplant group on 3rd day (P<0.05). These results suggest that intravenous transplantation of MSCs prevents down-regulation of survivin and Bcl-2 preventing apoptosis and cell death in the ischemic brain leading to motor and sensory function recovery.

Generation of bone marrow-derived neural cells in serum-free monolayer culture.

Bone marrow-derived cells (BMCs) are reported to trans-differentiate into neural lineages, and are expected to offer a valuable resource for treating severe diseases of the central nervous system. BMCs that show neural differentiation may differ morphologically from typical marrow stromal cells. The present study aimed to obtain efficient generation of cells with neural features from bone marrow. Serum-free culture was applied to BMCs to prevent the growth of differentiated cells. Using basic fibroblast growth factor and extracellular matrix, rodent BMCs capable of proliferation and neural differentiation expanded in monolayer form. Cultured cells were small, round or spindle-shaped, and expressed nestin. Under neural differentiation conditions, cells developed long, thin cellular processes with branches, and expressed neuronal and glial phenotypes. Intracerebrally transplanted BMCs survived and migrated into the subcortex of syngenic animals.

The characteristics of human cranial bone marrow mesenchymal stem cells.

Recently, cell-based therapy has attracted attention for treatment of central nervous system (CNS) disorders. Bone marrow-derived mesenchymal stem cells (BMSCs) are considered to have good engraftment potential. Therefore, more efficient and less invasive methods to obtain donor cells are required. Here, we established human BMSCs from cranial bone waste (cBMSCs) obtained following routine neurosurgical procedures. cBMSCs and cells obtained from the iliac crest (iBMSCs, standard BMSCs) showed expression of cell surface markers associated with mesenchymal stem cells and multipotency traits such as differentiation into osteogenic and adipogenic lineages. cBMSCs showed higher expression of the neural crest-associated mRNAs p75, Slug, and Snail than iBMSCs. Neurogenic induced cells from cBMSCs expressed the neural markers nestin, Pax6, neurofilament (NF)-L, and NF-M as seen with RT-PCR, and NF-M protein as seen with western blotting at higher levels than cells from iBMSCs. Immunostaining showed a significantly greater proportion of NF-M-positive cells in the population of induced cBMSCs compared with the population of iBMSCs. Thus, cBMSCs showed a greater tendency to differentiate into neuron-like cells than iBMSCs.

Simulated microgravity maintains the undifferentiated state and enhances the neural repair potential of bone marrow stromal cells.

Recently, regenerative medicine with bone marrow stromal cells (BMSCs) has gained significant attention for the treatment of central nervous system diseases. Here, we investigated the activity of BMSCs under simulated microgravity conditions. Mouse BMSCs (mBMSCs) were isolated from C57BL/6 mice and harvested in 1G condition. Subjects were divided into 4 groups: cultured under simulated microgravity and 1G condition in growth medium and neural differentiation medium. After 7 days of culture, the mBMSCs were used for morphological analysis, reverse transcription (RT)-polymerase chain reaction, immunostaining analysis, and grafting. Neural-induced mBMSCs cultured under 1G conditions exhibited neural differentiation, whereas those cultured under simulated microgravity did not. Moreover, under simulated microgravity conditions, mBMSCs could be cultured in an undifferentiated state. Next, we intravenously injected cells into a mouse model of cerebral contusion. Graft mBMSCs cultured under simulated microgravity exhibited greater survival in the damaged region, and the motor function of the grafted mice improved significantly. mBMSCs cultured under simulated microgravity expressed CXCR4 on their cell membrane. Our study indicates that culturing cells under simulated microgravity enhances their survival rate by maintaining an undifferentiated state of cells, making this a potentially attractive method for culturing donor cells to be used in grafting.

Effects of simulated microgravity on proliferation and chemosensitivity in malignant glioma cells.

A three-dimensional (3D) clinostat is a device for generating multidirectional G force, resulting in an environment with an average of 10(-3)G. We cultured human malignant glioma cell lines in a 3D-clinostat (CL group) and examined the growth properties and chemosensitivity of the cells compared to cells cultured under normal 1G conditions (C group). The growth rate was significantly inhibited in the CL group, but without cell cycle change. Mitochondrial activity was also inhibited in the CL group. Thus, inhibition of malignant glioma proliferation occurred that could be attributed to deceleration of mitosis. Chemosensitivity to cisplatin (cis-diamminedichloroplatinum(II), CDDP) in the CL group was significantly enhanced compared to the C group. This method has significant potential as a treatment of malignant gliomas and a tool for understanding developmental biology.

Novel electrical stimulation sets the cultured myoblast contractile function to 'on'.

OBJECTIVE: In the present study, the effect of electrical stimulation was examined for the ability to induce morphological, physiological, and molecular biological effects on myoblasts during cell differentiation. METHODS: L6 rat myoblasts were electrically stimulated by newly developed methods on culture days 6, 8, 10 and 12. RESULTS: This electrical stimulation accelerated the appearance of myotubes, and subsequently produced spontaneously contracting muscle fibers. Measurement of membrane potential showed that the contracting cell had functional ion channels and gap junctional intercellular communication. In the electrically stimulated cells, an enhanced expression of MyoD family and M-cadherin was also observed. Expression of connexin 43 was increased and maintained at a high level in the electrically stimulated cells. CONCLUSION: This is the first demonstration of in vitro induction of myoblasts in spontaneously contractile muscle fibers by intermittent stimulation. This novel method for induction of myoblast differentiation represents an important advance in cell therapy.