Abstract: BACKGROUND: Many modified surgical techniques for moyamoya disease have been developed, and each of which has some advantages and disadvantages. We report the indication for one-stage extensive indirect vascular reconstructive surgery that is our original technique for pediatric moyamoya disease.
CASE DESCRIPTION: Case 1 was a 3-year-old boy who had TIAs involving the bilateral extremities and provoked by intense crying. Case 2 was a 5-year-old girl who had TIAs involving the right extremities and provoked by intense crying. Case 3 was an 8-year-old girl who had frequent TIAs involving the bilateral extremities. Case 4 was a 3-year-old boy who had mild tetraplegia due to bilateral cerebral infarction. In all cases but case 4, preoperative investigations demonstrated same-stage moyamoya disease. In case 4, preoperative investigations demonstrated different-stage moyamoya disease in which acute progression of bilateral major arterial stenosis occurred. Hence, all patients underwent one-stage extensive indirect vascular reconstructive surgery of the bilateral cerebral hemispheres. Postoperative investigations revealed the development of rich neovascularization, disappearance of clinical symptoms in all cases.
CONCLUSIONS: We recommend one-stage extensive indirect vascular reconstructive surgery for pediatric moyamoya disease, particularly for patients younger than 5 years, when preoperative investigations reveal a same-stage case or bilateral acute progressive case.
Abstract: Although neurons and glia inevitably undergo degeneration in the core of ischemic lesions, many cells, particularly immune cells, infiltrate the core and survive in it. Such infiltrating cells may play certain roles in the regeneration and repair of damaged brain tissues. In this study, we characterized macrophage-like cells that accumulated in the ischemic core of a rat brain whose right middle cerebral artery was transiently occluded for 90 mins. Many of the accumulated macrophage-like cells expressed Iba1, a marker of macrophages/microglia, as well as NG2 chondroitin sulfate proteoglycan (NG2), which has been recognized as a marker of oligodendrocyte progenitor cells. Such macrophage-like cells were termed BINCs (brain Iba1(+)/NG2(+) cells) to distinguish them from NG2(-)/Iba1(+) or NG2(+)/Iba1(-) cells that were also present in the perilesion and the contralateral hemisphere. Electron microscopy showed the localization of NG2 along the plasma membrane of cells that had many phagosomes and irregular-shaped or reniform heterochromatin-rich nuclei, which are characteristics of monocytes/macrophages. Brain Iba1(+)/NG2(+) cells were highly proliferative and their number peaked at 7 days post-reperfusion. An immunoblot analysis of NG2 revealed the presence of two NG2s: one expressed by BINCs with a molecular weight of 300 kDa, and the other found in the contralateral hemisphere with a molecular weight of 290 kDa. Taken the various functions of NG2, BINCs may be involved in not only phagocytosis of degenerated cells but also the healing and regeneration of lesion cores.
Abstract: Resident quiescent microglia have been thought to respond rapidly to various pathologic events in the brain by proliferating and producing many bioactive substances, including proinflammatory cytokines and nitric oxide (NO). In this study, we investigated the reaction of microglia in traumatic and ischemic lesions caused by stab wounds and the transient 90-min occlusion of middle cerebral artery in a mature rat brain. Although many Iba1(+) resident microglia underwent apoptotic degeneration in the lesion core within 24 hr after the onset of the brain insult as revealed by TUNEL staining, numerous small, round, isolectin B4(+)/CD11b(+)/CD68(+) cells were localized in the lesion core. These small, round cells with diameters of 7-9 mum and polymorph nuclei expressed neutrophil-specific elastase, alkaline phosphatase, and platelet-activating factor receptor. Accordingly, they were not activated microglia but neutrophils. Immunohistochemical staining with antibodies to inducible NO synthase (iNOS) showed that most iNOS(+) cells were neutrophils. The results from spatial and kinetic analyses using RT-PCR and immunoblotting were consistent with the immunohistochemical observations. These results suggest the necessity of reevaluating the traditional view on the roles of activated microglia in severe neuropathologic events. Note that the traditional microglial markers isolectin B4, CD11b, and CD68 are not specific for microglia, particularly in a pathologic brain.
Abstract: Brain ischemia causes the death of neurons and glial cells. Such brain cells are believed to inevitably undergo degeneration in the core of ischemic lesions, whereas neurons and glial cells may survive in the region surrounding the core that is often referred to as the ischemic penumbra. However, many cells, particularly immune cells infiltrate and survive in the core. In this study, we characterized macrophage-like cells that accumulated in the ischemic core of a rat brain whose right middle cerebral artery was transiently occluded for 90 min. At 7 days post-reperfusion, we observed macrophage-like cells expressing CD200, a cell surface glycoprotein belonging to an immunoglobulin superfamily and that elicits suppressive effects on myeloid cells including microglia by interacting with the CD200 receptor (CD200R). RT-PCR and immunoblot analyses revealed the presence of CD200-mRNA and protein in the ischemic core as well as in the contralateral region. As revealed by immunohistochemistry, CD200 is located on the cell membrane of spherical Iba1(+) cells with many cytoplasmic granules. CD200(-)/Iba1(+) macrophage-like cells were also present, which have a more irregular shape than CD200(+)/Iba1(+) cells. CD200 was detected in isolated spherical Iba1(+) macrophage-like cells. Thus, CD200 is expressed in some populations of macrophage-like cells that may be responsible for the suppression of CD200R(+) myeloid cell functions in the ischemic core.
Abstract: A 43-year-old female was treated with gamma knife radiosurgery (GKS) for right frontal arteriovenous malformation (AVM) manifesting as absence seizures. Complete nidus obliteration was confirmed on angiography 4 years after GKS. However, she experienced recurrence of her previous seizures and delayed hemorrhage occurred within the treated nidus, despite absence of abnormalities by repeated angiography 81 months after GKS. She was treated conservatively and discharged home without neurological deficits. The risk of hemorrhage from obliterated AVM is significantly reduced but not eliminated after radiosurgery. Recanalization of thrombus that is too small to detect by neuroimaging may result in delayed hemorrhage.
Abstract: We report three cases of severe traumatic basal ganglia hemorrhage TBGH accompanied with multiple injuries. Case 1 was a 33-year-old man with right putaminal hematoma. Case 2 was a 51-year-old man with left putaminal hematoma. Case 3 was an 8-year-old boy with left putaminal hematoma which presented enlargement on serial CT. All patients were involved in traffic accidents and had accompanying relevant associated extracranial injuries. All cases underwent surgery to evacuate the hematoma and were treated with intentional normothermia therapy. Although intracranial pressure (ICP) could be controlled in all patients, there were two complications during the postoperative state : a disseminated intravascular coagulation (DIC) occurred in Case 1 and upper gastrointestinal (GI) bleeding brought death in Case 3. Concerning management of patients with severe TBGH, we should investigate associated injuries and perform surgical evacuation in patients with large hematoma or in those in whom there is hematoma volume enlargement or elevated ICP. Furthermore, we should take care of not only ICP control but also systemic complications including DIC and upper GI bleeding.
Abstract: PURPOSE: To present a case of kissing aneurysms of the anterior communicating artery treated with endovascular coil embolization and discuss the advantages and disadvantages of this technique compared with neck clipping.
CASE REPORT: A 48-year-old man became drowsy and was admitted to the hospital; he had right hemiparesis and aphasia. Computed tomography revealed diffuse subarachnoid hemorrhage; diagnostic angiography identified an aneurysm at the left A1-A2 junction of the anterior communicating artery and another in the distal anterior cerebral artery (ACA). Endovascular coil embolization was performed on the same day. During the procedure, the 3-mm-diameter junctional aneurysm was successfully packed with coils, but an additional aneurysm was suspected; right carotid angiography following embolization of the left aneurysm clearly showed a mirror image aneurysm of the right A1-A2 junction. The right aneurysm was treated using the same technique. The broad-necked ACA aneurysm was unsuitable for embolization, so neck clipping was performed 5 weeks later. The patient was discharged to his home following complete recovery 7 weeks after the coil embolization.
CONCLUSIONS: Kissing aneurysms are a rare and specific type of multiple aneurysms that require caution in diagnosis and surgical management. Endovascular treatment may be suitable because it does not involve dissection around the aneurysms.
Abstract: PURPOSE: To present a case of kissing aneurysms of the anterior communicating artery treated with endovascular coil embolization and discuss the advantages and disadvantages of this technique compared with neck clipping.
CASE REPORT: A 48-year-old man became drowsy and was admitted to the hospital; he had right hemiparesis and aphasia. Computed tomography revealed diffuse subarachnoid hemorrhage; diagnostic angiography identified an aneurysm at the left A1-A2 junction of the anterior communicating artery and another in the distal anterior cerebral artery (ACA). Endovascular coil embolization was performed on the same day. During the procedure, the 3-mm-diameter junctional aneurysm was successfully packed with coils, but an additional aneurysm was suspected; right carotid angiography following embolization of the left aneurysm clearly showed a mirror image aneurysm of the right A1-A2 junction. The right aneurysm was treated using the same technique. The broad-necked ACA aneurysm was unsuitable for embolization, so neck clipping was performed 5 weeks later. The patient was discharged to his home following complete recovery 7 weeks after the coil embolization.
CONCLUSIONS: Kissing aneurysms are a rare and specific type of multiple aneurysms that require caution in diagnosis and surgical management. Endovascular treatment may be suitable because it does not involve dissection around the aneurysms.
Abstract: The authors report a case of Currarino triad with a combination of anterior sacral meningocele and mature teratoma, sacral body deformity, anorectal stenosis, and tethered cord. A newborn girl suffered from vomiting, abdominal distension and constipation. Initially, a diverting colostomy was performed at the age of one month. 7 months later, at the age of 8 months, we performed posterior sagittal anorectoplasty (PSARP): As a result, extirpation of teratoma, excision of meningocele, untethering of the spinal cord, and anorectoplasty were achieved simultaneously without complication. We suggest the use of an MRI to specify the presence of anosacral and spinal cord anomalies in patients with intractable constipation and we recommend combined pediatric and neurosurgical assessment and management for all cases of Currarino triad.
Abstract: The authors report a 12-year-old girl with moyamoya disease who was treated by unilateral combined extensive indirect revascularization surgery 8 years after initial bilateral encephalo-duro-arterio-synangiosis (EDAS) and encephalo-galeo-synangiosis (EGS). When she was 4 years old, she had frequent transient ischemic attacks (TIA) involving the lower extremities and had been treated by bilateral EDAS and EGS at another hospital. Thereafter, apparent TIA disappeared, but mental retardation developed gradually, associated with an occasional sense of weakness in the left lower limb. We performed combined extensive indirect vascular reconstruction covering the area of resting low cerebral blood flow (CBF) with no response to an acetazolamide challenge test, that resulted in an improvement of mental activity and the disappearance of weakness in the left lower limb. Postoperative examination 1 year after re-operation showed rich collaterals from the external carotid artery and improvement of both resting CBF and response to an acetazolamide challenge test. Thus we suggest that, after initial revascularization for pediatric moyamoya disease, we observe clinical signs first and periodically perform MRI/MR angiography and SPECT. When poor improvement of clinical signs or CBF is observed at one year after surgery, re-operation covering the whole area of low perfusion at rest with no response to an acetazolamide challenge test should be considered.
Abstract: The authors report a case of mobile tumor of the cauda equina. A 36-year-old man complaining of lumbago and sensory disturbance of the right lower limb, was diagnosed with an L4/5 herniated lumbar disc at another hospital. After epidural analgesia, his clinical symptoms changed for the worse and he was referred to our hospital. MRI in our hospital revealed the cranial migration of a lumbar intradural extramedullary tumor, demonstrating a mobile tumor. There are 38 case reports of mobile tumors in the previous literature. As to a mechanisms of tumor mobility, in the instance where there is an abnormal dilatation of the subarachnoid space due to a deformed spinal root, changes in cerebrospinal fluid pressure after lumbar puncture or myelography might play an important role. Such mobility is rare, but should be kept in mind when the clinical symptoms change for the worse after posture change, straining and lumbar puncture or myelography.
