[A Case Report of Surgical Resection of a Supraseller Granuloma in a Patient with Erdheim-Chester Disease].

Erdheim-Chester disease(E-CD)is a rare pathology characterized by systematic granulomatosis that occasionally involves the central nervous system. We report about a 68-year-old woman with E-CD who presented with right-side visual disturbance. Magnetic resonance imaging showed a suprasellar tumor that elevated the right optic nerve and involved the right internal carotid and right anterior choroidal arteries. The tumor was partially resected via a trans-Sylvian approach and was histologically diagnosed as a granuloma. Considering the abnormal findings of postoperative X-ray and 99 mTc bone scintigraphy of the long bones, the pathology was diagnosed as E-CD. After surgery, her right-side visual disturbances disappeared. However, 1 year later, she died of systemic infection and heart failure. Histological autopsy findings indicated numerous yellowish nodules in the heart, lung, and kidney with pericardial and pleural effusions and whole-body granulomatosis, including the brain. E-CD is a rare but critical disease. This pathological entity should be considered when encountering cases of intracranial granuloma to ensure its early diagnosis and appropriate treatment. Surgical resection of intracranial granulomas in patients with E-CD may promptly improve neurological dysfunction.

[A Case of Glioblastoma that Appeared One Year after the Removal of Subcortical Hematoma].

Subcortical bleeding from brain tumors is not rare. In the majority of cases, tumors are revealed within a few months after bleeding. We herein report a relatively rare case of glioblastoma(GBM)that appeared one year after the removal of a subcortical hematoma. A 70-year-old woman suddenly began experiencing headache, vomiting, and aphasia. CT revealed a subcortical hematoma in the left superior temporal lobe and subarachnoid bleeding. Neither aneurysms nor abnormal signs suggesting a malignant tumor were noted during cerebral angiography. The hematoma was completely removed via craniotomy, and she was discharged with no neurological deficit.(MRI performed seven months after the surgery showed neither space-occupying lesions in the left temporal lobe nor brain edema. Twelve months after the initial surgery, she had aphasia again. CT and MRI revealed an enhanced mass lesion in the left temporal lobe. Positron emission tomography findings strongly indicated the presence of a malignant tumor. Histology of the tumor after removal showed GBM HDH-1 wild-type with an MIB-1 labelling index of approximately 50%. After the surgery, she underwent extensive local radiation therapy(50 Gy)with chemotherapy(temozolomide). The pathological mechanism underlying the appearance of GBM at the site where subcortical bleeding was previously observed is unclear. GBM may have caused bleeding or may have originated from the brain tissue that was damaged during the first surgery. Follow-up using neuroimaging for one year may be needed when subcortical bleeding is observed.

Staged angioplasty for carotid artery stenosis to prevent postoperative hyperperfusion.

OBJECTIVE: Hyperperfusion (HP) is a rare but potentially devastating complication after carotid revascularization. This report describes the clinical efficacy of staged angioplasty (SAP) for carotid artery stenosis to prevent HP after carotid revascularization. METHODS: Eighteen of 143 patients with high-grade internal carotid artery stenosis scheduled for angioplasty were considered at high risk of postprocedure HP based on their severely impaired cerebral blood flow (CBF) and cerebral vasoreactivity, which were determined using single-photon emission computed tomography with acetazolamide. Nine of the high-risk patients were treated with carotid artery stenting and the other 9 were treated with SAP, which consisted of balloon angioplasty with undersized balloon catheters (Stage 1) followed by carotid artery stenting 1 to 2 months later (Stage 2). RESULTS: In the regular carotid artery stenting group, 5 of 9 patients (56%) showed HP phenomenon on single-photon emission computed tomography just after stenting, and 1 patient (11%) developed status epilepticus owing to HP. In the SAP group, none of the 8 patients treated by SAP or the 1 patient who required stent placement during the first stage owing to a wall dissection developed postprocedure HP phenomenon or HP syndrome. CONCLUSION: SAP decreased the HP phenomenon on single-photon emission computed tomography after performing these procedures in selected patients. Although additional intervention is needed, SAP is considered a relatively simple and effective method to avoid HP in patients at high risk of HP after carotid revascularization.

Hemispheric laminar necrosis as a complication of traumatic carotid-cavernous sinus fistula.

A 43-year-old woman presented with a very rare case of hemispheric laminar necrosis as a complication of traumatic carotid-cavernous sinus fistula (CCF). The patient suffered head injury and extensive burns following a car accident. Oral intubation was performed under sedation. When sedation was discontinued 17 days after injury, the patient demonstrated left hemiparesis. Magnetic resonance imaging showed laminar necrosis affecting the right cerebral hemisphere. Angiography revealed a right high-flow direct CCF. Transarterial embolization of the fistula using a detachable balloon achieved complete occlusion of the fistula. However, the left hemiparesis persisted following this intervention. Traumatic CCF may be missed in patients with disturbed consciousness, so clinicians should not overlook possibility of the triad of symptoms of CCF in patients with head injury.

Long-term maintenance of brain tumor stem cell properties under at non-adherent and adherent culture conditions.

There is increasing evidence for the presence of cancer stem-like progenitors in malignant brain tumors. This subpopulation of progenitor cells, the so-called "cancer stem cells (CSCs)", may play a pivotal role in brain tumor initiation, growth, and recurrence. Here we describe the establishment of one permanent brain tumor stem cell line that able to form new spheres after culture under adherent monolayer conditions and to recapitulate the properties of the original tumor upon transplantation into immunodeficient mice. Re-formed spheres retained their stem cell properties and isolated single CSCs from these spheres formed spheres/tumors even after long-term cultures (over 2 years). These data suggested that a small population of CSCs preserved its stem cell properties even after serial passages under non-adherent/adherent culture conditions. Evaluation of underlying metabolic events and assessment of the biological features of these viable cell lines will yield useful knowledge on the in situ behavior of brain tumors.

Culture method for the induction of neurospheres from mouse embryonic stem cells by coculture with PA6 stromal cells.

Embryonic stem (ES) cells proliferate and maintain their pluripotency for over 1 year in vitro and may therefore provide a sufficient source for cell therapies. However, most of the previously reported methods for obtaining a source for cell therapies have not been simple. We describe here a novel method for induction of neurospheres from mouse ES cells by coculturing on PA6 cells instead of the formation of embryoid bodies. The ES cells cocultured with the PA6 stromal cell line for at least 3 days were capable of differentiating into spheres. The cells in the spheres were all green fluorescent protein (GFP) positive, showing that they were derived from GFP-expressing D3-ES cells. The spheres contained nestin-positive cells. The number of spheres increased when they were cocultured with PA6 for a longer period. Sphere formation was observed even after 10 mechanical dissociations and subculturings, showing its self-renewal ability. The cells differentiated into microtubule-associated protein-2 (MAP2)-positive neuronal cells and glial fibrillary acidic protein (GFAP)-positive glial cells. gamma-Aminobutyric acid-positive cells and tyrosine hydroxylase-positive cells were also observed in the spheres. The percentages of the MAP2- or GFAP-positive cells in the sphere changed according to the period of coculture on PA6 cells. At an early stage of coculture, more neurons were generated and, at a later period, more glial cells were generated. These results suggested that neurosphere could be generated from ES cells by coculturing with PA6, and that these cells resembled neural stem cells derived from mouse fetal brain tissue.

Hepatocyte growth factor promotes neuronal differentiation of neural stem cells derived from embryonic stem cells.

We previously reported that hepatocyte growth factor (HGF) promoted proliferation of neurospheres and neuronal differentiation of neural stem cells (NSCs) derived from mouse embryonic brain. In this study, spheres from mouse embryonic stem (ES) cells were generated by floating culture following co-culture on PA6 stromal cells. In contrast to the behavior of the neurospheres derived from embryonic brain, addition of HGF to the growth medium of the floating cultures decreased the number of spheres derived from ES cells. When spheres were stained using a MAP-2 antibody, more MAP-2-positive cells were observed in spheres cultured with HGF. When HGF was added to the growth and/or differentiation medium, more MAP-2-positive cells were also obtained. These results suggest that HGF promotes neuronal differentiation of NSCs derived from ES cells.

Hepatocyte growth factor promotes proliferation and neuronal differentiation of neural stem cells from mouse embryos.

Hepatocyte growth factor (HGF), originally cloned as a hepatocyte mitogen, has recently been reported to exhibit neurotrophic activity in addition to being expressed in different parts of the nervous system. At present, the effects of HGF on neural stem cells (NSCs) are not known. In this study, we first report the promoting effect of HGF on the proliferation of neurospheres and neuronal differentiation of NSCs. Medium containing only HGF was capable of inducing neurosphere formation. Addition of HGF to medium containing fibroblast growth factor 2 or epidermal growth factor increased both the size and number of newly formed neurospheres. More neurons were also obtained when HGF was added in differentiation medium. In contrast, neurosphere numbers were reduced after repeated subculture by mechanical dissociation, suggesting that HGF-formed neurospheres comprised predominantly progenitor cells committed to neuronal or glial lines. Together, these results suggest that HGF promotes proliferation of neurospheres and neuronal differentiation of NSCs derived from mouse embyos.