Showing papers in "Neurologia Medico-chirurgica in 2012"
TL;DR: This English version of the second edition of the Japanese Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus was made to share ideas with the international community and to promote international research on iNPH.
Abstract: Among the various disorders manifesting dementia, gait disturbance, and urinary incontinence in the elderly population, idiopathic normal pressure hydrocephalus (iNPH) is becoming of great importance. After the publication of the first edition of the Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus in 2004 (the English version was published in 2008), clinical awareness of iNPH has risen dramatically, and the number of shunt surgeries has increased rapidly across Japan. Clinical and basic research on iNPH has increased significantly, and more high-level evidence has since been generated. The second edition of the Japanese Guidelines was thus published in July 2011, to provide a series of timely evidence-based recommendations related to iNPH. The revision of the Guidelines has been undertaken by a multidisciplinary expert working group of the Japanese Society of Normal Pressure Hydrocephalus in conjunction with the Japanese Ministry of Health, Labour and Welfare research project on "Studies on the epidemiology, pathophysiology, and treatment of normal pressure hydrocephalus." This English version of the second edition of the Guidelines was made to share these ideas with the international community and to promote international research on iNPH.
508 citations
TL;DR: The present study investigated the distribution of variant p.R4810K in RNF213 in 2,508 participants from East and Southeast Asian countries using a TaqMan probe to find the number of patients with moyamoya disease, which was considered to be 53,800 in East Asian populations.
Abstract: Moyamoya disease is an idiopathic vascular disorder of the intracranial arteries. Ring finger 213 (RNF213) was previously identified as the strongest susceptibility gene for moyamoya disease in East Asian people by a genome-wide linkage analysis and exome analysis. The coding variant p.R4810K in RNF213 was strongly associated with moyamoya disease in the Japanese (odds ratio: 338.94, p = 1.05 × 10(-100)) and Korean (odds ratio: 135.63, p = 7.59 × 10(-27)) populations, and much less strongly associated in the Chinese population (odds ratio: 14.70, p = 2.63 × 10(-5)). The present study investigated the distribution of variant p.R4810K in RNF213 in 2,508 participants from East and Southeast Asian countries using a TaqMan probe. p.R4810K was detected at an allele frequency of about 1.00% in 4 of 11 investigated locations in China. In contrast, p.R4810K was detected homogeneously at relatively high frequencies of 1.00-1.72% in all investigated locations in Korea and Japan, including Okinawa. p.R4810K was not detected in Southeast Asian populations. The population susceptible to moyamoya disease was estimated to be 16.16 million people in East Asian countries, including 11.41 million Chinese, 1.36 million Korean, and 3.39 million Japanese people. The number of patients with moyamoya disease, which was estimated at approximately one per 300 carriers of p.R4810K, was considered to be 53,800 in East Asian populations.
100 citations
TL;DR: Direct/indirect revascularization surgery is a safe and effective treatment for moyamoya disease, although the issue of bleeding/re-bleeding remains to be solved.
Abstract: Moyamoya disease is a chronic, occlusive cerebrovascular disease with unknown etiology characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Recent advances in molecular biology and genetic research have provided better understanding of the pathophysiology of moyamoya disease, but surgical revascularization still remains the preferred treatment for this entity. The present study investigated the clinical course of 106 consecutive patients with moyamoya disease who underwent superficial temporal artery-middle cerebral artery anastomosis with indirect pial synangiosis in 150 hemispheres. The outcomes of surgery on the operated hemisphere were favorable, with no cerebrovascular event during the outpatient follow-up period (mean 58.4 months) in 89.3% (134/150). Two patients suffered hemorrhagic events on the operated hemisphere during the follow-up period (2/150, 1.33%), one of whom suffered deteriorated neurological status after hemorrhage. Despite the favorable long-term outcome, the incidence of temporary neurological deterioration due to cerebral hyperperfusion was 18.0% (27/150), but no patients suffered permanent neurological deterioration directly caused by hyperperfusion. In conclusion, direct/indirect revascularization surgery is a safe and effective treatment for moyamoya disease, although the issue of bleeding/re-bleeding remains to be solved. Postoperative cerebral hyperperfusion and peri-operative infarction are potential complications of this procedure, so we recommend intensive postoperative care and cerebral blood flow measurement in the acute stage, because the management of hyperperfusion is contradictory to that of ischemia.
95 citations
TL;DR: These guidelines are approved by The Japan Neurosurgical Society and the part on anesthetic management and language assessment during awake craniotomy is approved by the Neuropsychology Association of Japan.
Abstract: These guidelines are approved by The Japan Neurosurgical Society. The part on anesthetic management is approved by the Japan Society of Anesthesiology and the part on language assessment during awake craniotomy is approved by the Neuropsychology Association of Japan. Copyright of this English version of the Guidelines is reserved by The Japan Neurosurgical Society. Correspondence to: Takamasa Kayama, MD, Department of Neurosurgery, Yamagata University Faculty of Medicine, 2–2–2 Iida-nishi, Yamagata, Yamagata 990–9585, Japan. Phone: +81–23–628–5349; FAX: +81–23–628–5351. Neurol Med Chir (Tokyo) 52, 119¿141, 2012
79 citations
TL;DR: Posture and postural stability motor subscores were improved at 3 months after SCS, and gait had significantly improved at3 months and 1 year after surgery, and Timed 10-Meter Walk tests demonstrated that patient gait was significantly improvedat 3 months and 12 months after surgery.
Abstract: Patients with advanced Parkinson's disease (PD) often present with axial symptoms, including abnormal posture, postural instability, and gait disorder. Although spinal cord stimulation (SCS) is effective for pain, little is known about the effect of SCS on motor function in PD patients. The present study investigated the effect of SCS on posture and gait in 15 PD patients, 5 men and 10 women aged 63-79 years (mean 71.1 years), with low back pain and leg pain who received SCS. A visual analog scale (VAS) was used for pain evaluation pre- and postoperatively. The Unified Parkinson's Disease Rating Scale, Timed Up and Go tests, and Timed 10-Meter Walk tests were used to evaluate motor function and activities of daily living of patients. Preoperative mean VAS score was 8.9 (range 7.8-10), which showed significant postoperative improvement at 3 months to mean VAS score of 2.0 (range 0-3.3). The improvements in VAS scores persisted at 12 months after surgery with mean VAS score of 2.3 (range 0-4). Posture and postural stability motor subscores were improved at 3 months after SCS, and gait had significantly improved at 3 months and 1 year after surgery. Timed 10-Meter Walk tests also demonstrated that patient gait was significantly improved at 3 months and 12 months after surgery. Most advanced stage PD patients suffer considerable pain that causes abnormal posture and gait disturbance. SCS is expected to lead to both amelioration of pain and improvement of motor function in such patients.
76 citations
TL;DR: Members of the Guidelines Committee on the Management of Severe Head Injury contributed to the 2nd Japanese version of the Injury Prevention Guidelines.
Abstract: Members of the Guidelines Committee on the Management of Severe Head Injury contributed to the 2nd Japanese version: Chairman Minoru SHIGEMORI Members Toshiaki ABE, Tohru ARUGA, Takeki OGAWA, Hiroshi OKUDERA, Junichi ONO, Takehide ONUMA, Yoichi KATAYAMA, Nobuyuki KAWAI, Tatsuro KAWAMATA, Eiji KOHMURA, Toshisuke SAKAKI, Tetsuya SAKAMOTO, Tatsuya SASAKI, Akira SATO, Toshiyuki SHIOGAI, Katsuji SHIMA, Kazuo SUGIURA, Yoshio TAKASATO, Takashi TOKUTOMI, Hiroki TOMITA, Izumi TOYODA, Seigo NAGAO, Hiroshi NAKAMURA, Young-soo PARK, Mitsunori MATSUMAE, Tamotsu MIKI, Yasushi MIYAKE, Hisayuki MURAI, Shigeyuki MURAKAMI, Akira YAMAURA, Tarumi YAMAKI, Kazuo YAMADA, and Toshiki YOSHIMINE
74 citations
TL;DR: The aim of this paper is to review research on the pathogenesis of moyamoya disease to identify milestones in the direction of its true solution and to suggest the "double hit hypothesis" is probably the best explanation for the complicated pathology and epidemiology of this disease.
Abstract: Research on moyamoya disease has progressed remarkably in the past several decades. Indeed, many new facts concerning the epidemiology of the disease have been revealed and surgical treatments have been drastically improved. However, despite extensive research, the mechanism of moyamoya disease is still unknown. Consequently, the cardinal treatment of this disease has not yet been developed. For further clarification of its etiology, innovative studies are therefore indispensable. The aim of this paper is to review research on the pathogenesis of moyamoya disease to identify milestones in the direction of its true solution. Many hypotheses of the pathogenesis of moyamoya disease have been proposed in the past half century, including infection (viral and bacterial), autoimmune disorders, proteins abnormality, and gene abnormality. Some of these are now considered to be historical achievements. Others, however, can be still subjected to contemporary research. Currently, several genetic abnormalities are considered to offer the most probable hypothesis. In addition, interesting papers have been presented on the role of the endothelial progenitor cell on the pathogenesis of moyamoya disease. Intuitively, however, it appears that a single theory cannot always explain the pathogenesis of this disease adequately. In other words, the complex mechanism of several factors may comprehensively explain the formation of moyamoya disease. The "double hit hypothesis" is probably the best explanation for the complicated pathology and epidemiology of this disease.
72 citations
TL;DR: To clarify the clinical features of moyamoya disease in Japan, 941 patients with definite moyAMoya disease were analyzed from the databases constructed by the Research Committee on Moyamoya Disease, established by the Ministry of Health, Labour and Welfare.
Abstract: To clarify the clinical features of moyamoya disease in Japan, 941 patients with definite moyamoya disease were analyzed from the databases constructed by the Research Committee on Moyamoya Disease, established by the Ministry of Health, Labour and Welfare. Moyamoya disease occurs much more frequently among women than men, with a female-to-male ratio of 1.98. A family history of the disease was observed in 14.9%. The age at onset was characterized by two peaks: one at 5-9 years and another lower peak at around 40 years. Initial clinical features were transient ischemic attack in 46%, infarction in 20%, hemorrhage in 21%, headache in 6%, and epilepsy in 4%. The distribution of the age at onset showed one peak at around 40 years in patients with hemorrhage but two peaks in patients with ischemia.
70 citations
TL;DR: Combined bypass procedure, especially superficial temporal artery to middle cerebral artery anastomosis and indirect bypass, encephalo-duro-myo-arterio-pericranial synangiosis, is a safe and effective option to improve the short- and long-term outcome in patients with moyamoya disease.
Abstract: This review describes the basic concepts of surgical revascularization for moyamoya disease, including direct and indirect bypass surgery. Direct bypass surgery can improve cerebral hemodynamics and reduce further ischemic events immediately after surgery, but may be technically challenging in some pediatric patients. Indirect bypass surgery is simple and has widely been used. However, its beneficial effects can be achieved 3 to 4 months after surgery, and surgical design is quite important to determine the extent of surgical collateral pathways. Combined bypass procedure, especially superficial temporal artery (STA) to middle cerebral artery anastomosis and indirect bypass, encephalo-duro-myo-arterio-pericranial synangiosis, is a safe and effective option to improve the short- and long-term outcome in patients with moyamoya disease. Alternative techniques are also described for specific cases with profound cerebral ischemia in the anterior cerebral artery or posterior cerebral artery territory. Special techniques to safely complete bypass surgery and avoid perioperative complications are presented, including methods to prevent delayed wound healing, to avoid facial nerve palsy after surgery, and to preserve the STA and middle meningeal artery during skin incision and craniotomy. Finally, the importance of careful management of patients is emphasized to reduce the incidence of perioperative complications, including ischemic stroke and hyperperfusion syndrome.
69 citations
TL;DR: TCM develops frequently in SAH patients presenting with NPE, and transthoracic ultrasonography should be conducted routinely in that population, and patients with ruptured posterior circulation aneurysm may have elevated risk of developing NPE-TCM.
Abstract: Patients with poor-grade subarachnoid hemorrhage (SAH) are often complicated with acute cardiopulmonary dysfunctions, particularly neurogenic pulmonary edema (NPE) and takotsubo-like cardiomyopathy (TCM). This study retrospectively investigated the incidence, demographics, clinical characteristics, and outcomes of patients with SAH complicated with both NPE and TCM (NPE-TCM). The effects of aneurysm location and other clinical variables on the incidence of NPE-TCM were also investigated. Among 234 SAH patients treated during 5-year period, 16 (7%) presented with NPE, and transthoracic ultrasonography revealed that 14 of these 16 patients (88%) also had TCM. All 14 patients with NPE-TCM had poor-grade SAH (World Federation of Neurosurgical Societies grades IV and V). Ruptured posterior circulation aneurysm was predictive of NPE-TCM, but other clinical variables were not. Eight of the 14 patients with NPE-TCM could undergo treatment for ruptured aneurysm. Long-term outcomes were favorable in 5 of the 8 patients. Grade IV SAH patients had significantly better outcomes than grade V patients. TCM develops frequently in SAH patients presenting with NPE, and transthoracic ultrasonography should be conducted routinely in that population. Patients with ruptured posterior circulation aneurysm may have elevated risk of developing NPE-TCM. Endovascular obliteration of the aneurysm may be preferable to open surgery, but the optimal treatment modality needs to be evaluated further. Considering the limited number of SAH patients complicated with NPE-TCM, a multi-center cooperative study may be required.
57 citations
TL;DR: A potential role for VPA as an adjuvant therapy for patients with malignant glioma is suggested by in vitro and in vivo antiangiogenic effects of HDACIs, valproic acid.
Abstract: Antiangiogenic strategy is promising for malignant glioma. Histone deacetylase inhibitors (HDACIs) are unique anticancer agents that exhibit antiangiogenic effects. The in vitro and in vivo antiangiogenic effects of HDACIs, valproic acid (VPA), were investigated in malignant glioma in the brain. In vitro, VPA preferentially inhibited endothelial cell proliferation compared to glioma cell proliferation at the optimum concentration in a dose-dependent manner. VPA reduced vascular endothelial growth factor (VEGF) secretion of glioma cells in a dose-dependent manner under both normoxic and hypoxic conditions. VPA was also found to inhibit tube formation in the angiogenesis assay. In vivo, treatment with VPA combined with irinotecan reduced the number of vessels expressing factor VIII in the brain tumor model. VPA inhibits glioma angiogenesis by direct (inhibition of endothelial cell proliferation and tube formation) and indirect (decreased secretion of VEGF by glioma cells) mechanisms. These data suggest a potential role for VPA as an adjuvant therapy for patients with malignant glioma.
TL;DR: Tumor progression appears in the wall of the resection cavity or within 2 cm from its margin in approximately half of patients, and overall survival was significantly longer in patients with regional or marginal tumor progression, and constituted 90% and 54% at 1 and 2 years after surgery, respectively, compared to 75% and 0%.
Abstract: The present retrospective study evaluated the recurrence patterns after aggressive surgical removal of intracranial glioblastomas in 43 consecutive adult patients. The resection rate of the enhanced lesion on magnetic resonance imaging was 100% and 95-99% in 22 and 21 cases, respectively. All patients received postoperative fractionated radiotherapy (60 Gy in 30 fractions) with additional chemotherapy (25 cases) or vaccine therapy (18 cases). During follow-up (median 17 months), tumor recurrence was identified in 33 patients, most frequently regional within the wall of the resection cavity (20 cases). No clinical factor differed significantly between the groups of patients with regional or marginal tumor progression (N = 22) and patients with distant or multiple recurrences (N = 8). Progression-free survival did not differ significantly between these two groups (p = 0.27). However, overall survival was significantly longer (p = 0.04) in patients with regional or marginal tumor progression, and constituted 90% and 54% at 1 and 2 years after surgery, respectively, compared to 75% and 0% in patients with distant or multiple recurrences. Aggressive surgical resection and adjuvant management of intracranial glioblastoma may change its recurrence pattern. Tumor progression appears in the wall of the resection cavity or within 2 cm from its margin in approximately half of patients.
TL;DR: EDAGS is a very reliable alternative to combined bypass in adult MMD, however, randomized clinical trials are needed to assess the long-term efficacy of any bypass surgery in adult patients with MMD.
Abstract: Combined superficial temporal artery-middle cerebral artery anastomosis and encephalo-duro-arterio-galeo-synangiosis (EDAGS) were retrospectively compared with indirect bypass, EDAGS with or without inversion, in 134 hemispheres of 96 adult patients with non-hemorrhagic moyamoya disease (MMD) in terms of angiographic findings, perioperative complications, and clinical outcome. Angiographic revascularization seemed to be better in the combined bypass group compared with the EDAGS group (p = 0.045), but perioperative complication tended to be slightly more common in the combined bypass group. No statistical differences were found in clinical outcome. EDAGS is a very reliable alternative to combined bypass in adult MMD. However, randomized clinical trials are needed to assess the long-term efficacy of any bypass surgery in adult patients with MMD.
TL;DR: Presurgical embolization using NBCA made tumor removal safe and reduced bleeding volume in posterior fossa HBs, and was clearly less in the NBCA group than in the GDC ± PVA group.
Abstract: Intracranial hemangioblastomas (HBs) are hypervascular neoplasms mainly located in the posterior fossa of the central nervous system. Preoperative embolization of the feeding arteries is one proposal for reduction of intraoperative hemorrhage, although indications for the procedures should be evaluated carefully due to the potential complications. This retrospective study investigated clinical outcomes and complications of 15 patients with HBs in the posterior fossa to evaluate the safety and effectiveness of endovascular procedures as well as angiographical procedures. Surgical excision without presurgical embolization was performed in 8 cases, and excision with presurgical embolization was performed in 7 cases, using Guglielmi detachable coils with or without polyvinyl alcohol (GDC ± PVA) in 4 cases and only n-butyl 2-cyanoacrylate (NBCA) in 3 cases. The embolization was applied for selected cases in which feeding arteries were located in a deep site and hard to coagulate surgically. Partial embolization was achieved in 5 cases, and all feeders were successfully embolized in 2 cases. Total removal was achieved in 12 cases, and subtotal/partial removal was achieved in 3 cases. Subarachnoid hemorrhage with intratumoral hemorrhage occurred in 1 case during the angiographic procedure and in 1 case during the embolization procedures. The mean volume of intraoperative blood loss was clearly less in the NBCA group than in the GDC ± PVA group. HBs are mainly located in the posterior cranial fossa, so the risk of severe clinical complication may be high if vascular problems occur. In our series, presurgical embolization using NBCA made tumor removal safe and reduced bleeding volume in posterior fossa HBs.
TL;DR: Cilengitide is a novel integrin antagonist for the treatment of glioblastoma, and the multimodal anti-glioma effects are based on its cytotoxic,Anti-angiogenic, anti-invasive, and synergetic effects.
Abstract: Malignant glioma is the most common primary brain tumor and accounts for the majority of diagnoses. Treatment has involved a combination of surgery, radiation, and chemotherapy, yet these modalities rarely extend the life of the patient to more than one year from diagnosis. Integrins are expressed in tumor cells and tumor endothelial cells, and are important in angiogenesis and invasion in glioma. avb 3a ndavb5 integrins regulate cell adhesion, and inhibitors of these integrins suppress tumor growth in certain pre-clinical models. Several integrin-targeted drugs are in clinical trials as potential compounds for the treatment of cancer. Among them, cilengitide is a novel integrin antagonist for the treatment of glioblastoma. The multimodal anti-glioma effects are based on its cytotoxic, anti-angiogenic, anti-invasive, and synergetic effects. Preclinical studies showed a promising synergy between cilengitide and radiochemotherapy in order to normalize tumor vasculature and attenuate tumor invasion. Cilengitide is currently being assessed in phase III trials for patients with glioblastoma multiforme and in phase II trials for other types of cancers, demonstrating promising therapeutic outcomes to date. The results of these and other clinical studies are expected with great hope and interest. A more clear understanding of the benefits and pitfalls of each approach can then lead to the design of strategies to derive maximal benefit from these therapies.
TL;DR: Although the incidence of perinatal neurological events is low when MMD has been diagnosed, careful monitoring is required in light of the potential for stroke, and further efforts to establish management guidelines are required to ensure safer childbirth in patients with MMD.
Abstract: Stroke during pregnancy associated with moyamoya disease (MMD) has been reported sporadically, but no systematic surveys have been undertaken. To reveal the current clinical situation, the authors conducted Japan's first nationwide survey of pregnancy and delivery associated with MMD. A questionnaire was sent to all 270 perinatal medical centers across Japan to survey their experiences with delivery associated with MMD within the preceding 5 years (Survey I); another questionnaire was sent to 554 adult female patients with MMD regarding their experience with childbirth (Survey II). Survey I included 59 deliveries among patients with previously diagnosed MMD. The incidence of perinatal neurological events and morbidity was 5.1% and 1.7%, respectively. In another five cases, newly diagnosed after perinatal attacks, disability was noted in three cases, including one death from intracranial hemorrhage. Survey II included 278 deliveries. The perinatal attack rate was 6.6% in 76 previously diagnosed cases and 2.0% in 202 cases undiagnosed at pregnancy, but neither group reported permanent morbidity. Caesarean section in previously diagnosed cases accounted for 76.3% of deliveries in Survey I and 69.7% in Survey II, but no significant difference in event rate was found between caesarean section and vaginal delivery in either survey. Although the incidence of perinatal neurological events is low when MMD has been diagnosed, careful monitoring is required in light of the potential for stroke. Serious events, especially intracranial hemorrhage, can occur if MMD has not been diagnosed at pregnancy. Further efforts to establish management guidelines are required to ensure safer childbirth in patients with MMD.
TL;DR: A 68-year-old male presented with a very rare case of spindle cell oncocytoma (SCO), a recently identified very rare neoplasm of the anterior pituitary, manifesting as panhypopituitarism and visual field defect, and received postoperative conventional radiation therapy of 50 Gy.
Abstract: A 68-year-old male presented with a very rare case of spindle cell oncocytoma (SCO), a recently identified very rare neoplasm of the anterior pituitary, manifesting as panhypopituitarism and visual field defect. The pituitary tumor with suprasellar extension was only partially resected via transsphenoidal surgery because of the tumor consistency and bleeding. Histological diagnosis was consistent with schwannoma. The tumor regrew and angiography revealed hypervascularity, so a transcranial approach was employed for the re-operation which only achieved partial resection because of intraoperative extensive bleeding. The tumor cells showed similar histological and immunohistochemical profiles to the previous specimen, but electron microscopy demonstrated that cytoplasm abundantly filled with mitochondria. The final diagnosis of SCO was established and the patient received postoperative conventional radiation therapy of 50 Gy. Only 15 cases of SCO have been reported, and the diagnosis was mistaken in many cases as schwannoma, oncocytic pituitary adenoma, or craniopharyngioma, and multiple surgeries followed by radiation therapy were required.
TL;DR: PELD seemed to be a promising minimally invasive surgery for HNP in the lumbar spine with minimum damage to the musculoskeletal structure based on early clinical outcomes.
Abstract: We report our early clinical experience with percutaneous endoscopic lumbar discectomy (PELD) for herniated nucleus pulposus (HNP) in the lumbar spine. We introduced PELD to our clinical practice in June 2009. A total of 311 patients with degenerative lumbar spine disease were treated in our hospital up to August 2011. Thirty-seven patients with lumbar HNP were treated by PELD. PELD was carried out under local anesthesia, and the endoscope was continuously irrigated with saline. Twenty-eight patients were treated through the transforaminal approach, 5 were treated through the interlaminar approach, and 4 were treated through the extraforaminal approach. Surgery was discontinued due to uncontrollable intraoperative pain or anatomical inaccessibility in one case of the interlaminar approach and 2 cases of the extraforaminal approach. In the other 34 patients, the elapsed time of surgery was 34 to 103 minutes (mean 62.4 minutes). Extracorporeal blood loss was insignificant. Immediate symptom relief was achieved in all patients, and postoperative magnetic resonance imaging revealed sufficient removal of the HNP. The length of the postoperative hospital stay was 1 or 2 days in all patients. The surgical method of PELD is completely different from percutaneous nucleotomy, and the aim is to directly remove the HNP with minimum damage to the musculoskeletal structure. Although this study is based on our early clinical outcomes, PELD seemed to be a promising minimally invasive surgery for HNP in the lumbar spine.
TL;DR: It is indicated that neuropsychological test scores reflect the subjective assessment of postoperative change in cognition, and can detect subjective improvement and impairment in cognition after CEA using the optimal cut-off points for the test scores.
Abstract: Objective and subjective assessments of postoperative improvement and impairment in cognition were prospectively compared in patients who underwent carotid endarterectomy (CEA). Each patient underwent subjective cognitive assessment by a neurosurgeon and the patient's next of kin, and neuropsychological testing consisting of five test scores within 7 days before surgery and between 1 and 2 months after surgery. Of 213 patients studied, 24 (11%), 166 (78%), and 23 (11%) patients were defined as having subjectively improved, unchanged, and impaired cognition, respectively, following surgery. In all neuropsychological tests, differences in test scores between the two tests (postoperative test score - preoperative test score) significantly differentiated patients with subjectively improved, unchanged, and impaired cognition after surgery. Receiver operating characteristic analysis showed that the cut-off point for the differences in neuropsychological test scores in detecting subjective improvement and impairment in cognition after surgery was identical to mean +2 standard deviations (SDs) and mean -2 SDs, respectively, of the control value obtained from normal subjects. Of 27 patients with differences in neuropsychological test scores more than the upper cut-off point and 26 patients with differences in neuropsychological test scores less than the lower cut-off point in one or more neuropsychological tests, 24 (89%) and 23 (88%) exhibited subjectively improved and impaired cognition, respectively, after surgery. The present study indicates that neuropsychological test scores reflect the subjective assessment of postoperative change in cognition, and can detect subjective improvement and impairment in cognition after CEA using the optimal cut-off points for the test scores.
TL;DR: It is necessary to recognize syringomyelia and arachnoid cysts due to adhesive arACHnoiditis as a late complication of SAH as well as microlysis of the adhesions focusing on the lesion thought to be the cause of the symptoms.
Abstract: 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.
Journal Article•
TL;DR: CBF reduction and MTT prolongation before the onset of delayed CVS might influence the clinical outcome of SAH and discriminant analysis of these parameters could predict patient outcome with a probability of 74.5%.
Abstract: Subarachnoid hemorrhage (SAH) causes dynamic changes in cerebral blood flow (CBF), and results in delayed ischemia due to vasospasm, and early perfusion deficits before delayed cerebral vasospasm (CVS). The present study examined the severity of cerebral circulatory disturbance during the early phase before delayed CVS and whether it can be used to predict patient outcome. A total of 94 patients with SAH underwent simultaneous xenon computed tomography (CT) and perfusion CT to evaluate cerebral circulation on Days 1-3. Cerebral blood flow (CBF) was measured using xenon CT and the mean transit time (MTT) using perfusion CT and calculated cerebral blood volume (CBV). Outcome was evaluated with the Glasgow Outcome Scale (good recovery [GR], moderate disability [MD], severe disability [SD], vegetative state [VS], or death [D]). Hunt and Hess (HH) grade II patients displayed significantly higher CBF and lower MTT than HH grade IV and V patients. HH grade III patients displayed significantly higher CBF and lower MTT than HH grade IV and V patients. Patients with favorable outcome (GR or MD) had significantly higher CBF and lower MTT than those with unfavorable outcome (SD, VS, or D). Discriminant analysis of these parameters could predict patient outcome with a probability of 74.5%. Higher HH grade on admission was associated with decreased CBF and CBV and prolonged MTT. CBF reduction and MTT prolongation before the onset of delayed CVS might influence the clinical outcome of SAH. These parameters are helpful for evaluating the severity of SAH and predicting the outcomes of SAH patients.
TL;DR: A threshold of 2% for the MIB-1 labeling index predicts higher risk of progression of residual adenomas after surgery, so shorter interval of follow-up neuroimaging, and early initiation of adjuvant therapy might be required.
Abstract: Reliable prognostic parameters indicating progression in residual pituitary adenoma after surgery are necessary. The World Health Organization classification of tumors of endocrine organs defines atypical pituitary adenomas as tumor with Ki-67 labeling index higher than 3%, excessive p53 immunoreactivity, and increased pleomorphism. The real value of Ki-67 labeling index correlating with tumor progression is controversial. We investigated the relationship between positive labeling for MIB-1 and clinical features of 39 patients with pituitary adenomas with and without rapid re-growth after initial surgery. Ki-67 expression revealed pituitary adenomas with progression (re-growth within 5 years after initial surgery) had a mean proliferation index of 3.66 ± 3.00% (mean ± standard deviation [SD], n = 12), which was significantly higher than in tumors without progression of 1.89 ± 1.25% (mean ± SD, n = 27) (p < 0.05, Mann-Whitney test). Receiver operating characteristic analysis showed a threshold level of Ki-67 expression greater than 2.0% predicts progression with high specificity. Younger patients had higher MIB-1 index and more progression (p < 0.05). Adenomas with cavernous sinus invasion, functioning adenomas, and giant adenomas had higher MIB-1 index (p < 0.05). There was no significant correlation between tumor size or cavernous sinus invasion and progression. More completely removed tumors were less progressive. A threshold of 2% for the MIB-1 labeling index predicts higher risk of progression of residual adenomas after surgery, so shorter interval of follow-up neuroimaging, and early initiation of adjuvant therapy might be required.
TL;DR: The present findings suggest that the types of CSDH associated with high TM levels tend to have higher recurrence rate.
Abstract: Inflammation and the coagulation system may influence the genesis of chronic subdural hematoma (CSDH). The appearance of CSDH on computed tomography (CT) varies with the stage of the hematoma. This study investigated the pathogenesis and the recurrence of CSDH by comparing cytokine levels with the CT features of CSDH in 26 patients with 34 CSDHs who underwent single burr-hole surgery at our hospital between October 2004 and November 2006. The hematoma components removed during the procedure were examined, and the hematoma serum levels of cytokines measured such as thrombomodulin (TM), interleukin-6 (IL-6), tumor necrosis factor-α (TNFα), and interleukin-10 (IL-10). Using CT, mixed density hematomas were distinguished from other homogeneous hematomas, and found that the TM level was significantly higher in mixed density hematomas than in homogeneous hematomas (p = 0.043). Mixed density hematomas were classified into three subtypes (laminar, separated, and trabecular hematomas). The TM level was significantly higher in laminar and separated hematomas than in other hematomas (p = 0.01). The levels of IL-6, TNFα, and IL-10 were extremely high, but showed no significant differences in relation to the CT features. Mixed density hematomas had high recurrence rate, as reported previously, and TM level was high in mixed density hematomas such as laminar and separated mixed density hematomas. The present findings suggest that the types of CSDH associated with high TM levels tend to have higher recurrence rate.
TL;DR: Cervical SCS increased cerebral blood flow diffusely in the brain, and CBF increased by 22.2% during the stimulation period compared with CBF before stimulation in MCS patients, and five-Hz cervical SCS could increase CBF and induce muscle twitches of the upper extremities.
Abstract: Minimally conscious state (MCS) is characterized by inconsistent but clearly discernible behavioral evidence of consciousness, and can be distinguished from coma and the vegetative state (VS). Ten MCS patients were evaluated neurologically and electrophysiologically over 3 months after the onset of brain injury, and were treated by spinal cord stimulation (SCS). A flexible four-contact, cylinder electrode was inserted into the epidural space of the cervical vertebrae, and placed at the C2-C4 levels. Stimulation was applied for 5 minutes every 30 minutes during the daytime at an intensity that produced motor twitches of the upper extremities. We used 5 Hz for SCS, considering that the induced muscle twitches can be a useful functional neurorehabilitation for MCS patients. Eight of the 10 MCS patients satisfied the electrophysiological inclusion criteria, which we proposed on the basis of the results of deep brain stimulation for the treatment of patients in the VS. Seven patients recovered from MCS following SCS therapy, and were able to carry out functional interactive communication and/or demonstrate the functional use of two different objects. Cervical SCS increased cerebral blood flow (CBF) diffusely in the brain, and CBF increased by 22.2% during the stimulation period compared with CBF before stimulation in MCS patients (p < 0.0001, paired t-test). Five-Hz cervical SCS could increase CBF and induce muscle twitches of the upper extremities. This SCS therapy method may be suitable for treating MCS.
TL;DR: Simple hematoma evacuation is adequate as a first operation for CSDH associated with arachnoid cyst with good clinical courses and without recurrence more than 1.5 years after surgery.
Abstract: Arachnoid cysts are well known to induce chronic subdural hematoma (CSDH) after head injury. However, histological observations of the arachnoid cyst and hematoma membrane have only been rarely described. An 8-year-old boy and a 3-year-old boy presented with CSDH associated with arachnoid cyst. Surgical removal of the hematoma and biopsy of the hematoma membrane and cyst wall were performed. Clinical courses were good and without recurrence more than 1.5 years after surgery. Histological examination suggested that the cysts did not contribute to hematoma development. Pediatric hematoma membranes, similar to adult hematoma membranes, are key in the growth of CSDH. Therefore, simple hematoma evacuation is adequate as a first operation for CSDH associated with arachnoid cyst.
TL;DR: Adult MMD is not a syndrome, but a distinct disease entity with significant progression of unilateral MMD to bilateral MMD and ASD, which should be different from pediatric cases when considering adult MMD.
Abstract: Fifteen years of experience with adult moyamoya disease (MMD) in a single department were analyzed to describe the clinical features and to clarify the existence of the disease entity of adult MMD with special reference to its diagnosis. This study included 348 patients treated between 1996 and 2010. Male to female ratio was 128 to 220 with mean age of 39 years. The presence of neurological symptoms and signs during childhood and mean Suzuki stage according to the age were evaluated. Adult MMD was differentiated from other diseases, such as vasculitis, by comparing the laboratory data including common markers and specific markers for such diseases between 22 adult MMD and 21 atherosclerotic disease (ASD) patients. Conventional angiographic data were analyzed to detect progression in 52 unilateral adult MMD patients over a mean follow-up duration of 4.8 years. Only one of 128 patients (0.8%) aged over 40 years showed childhood symptoms and signs. The distribution of the mean Suzuki stage between patients aged 40 years and under and over 40 years was similar. Laboratory tests differentiating the other diseases with the same phenotype with MMD revealed no difference between adult MMD and ASD. The progression of unilateral MMD to bilateral MMD occurred in 6 of 52 patients (11.5%). Adult MMD is not a syndrome, but a distinct disease entity with significant progression of unilateral MMD to bilateral MMD. Diagnostic and therapeutic strategies should be different from pediatric cases when considering adult MMD.
TL;DR: The QOL and medical costs of chronic-phase stroke patients, and many cost-utility analyses will be based on the data in the future in Japan, are clarified.
Abstract: Stroke leaves serious neurological sequelae, which require long-term medical and social care, imposing financial and mental burdens on the patients and their families, and causing enormous losses to society. It is currently required that medical resources be used efficiently and the cost-effectiveness of treatment be analyzed carefully. We conducted a follow-up survey of stroke patients admitted to the hospital attached to our university in order to build a picture of the current status of chronic-phase stroke medical treatment. In total, 330 patients were analyzed in this study. We evaluated utility and medical cost at one year after onset. To investigate the relationship between activities of daily living (ADL) classified according to the modified Rankin scale (mRS) and quality of life (QOL), utility was calculated for each ADL level. Utility at 1 year post-onset for each mRS level was: ADL0, 0.89; ADL1, 0.79; ADL2, 0.65; ADL3, 0.58; ADL4, 0.36; and ADL5, 0.09. A significant correlation was seen between utility and mRS. Direct monthly medical costs at 1 year post-onset were 61,536 yen in the ADL0 group and 383,444 yen in the ADL5 group, indicating that a worse ADL score required higher medical costs. Direct monthly costs were significantly different between ADL levels. This present study has clarified the QOL and medical costs of chronic-phase stroke patients, and many cost-utility analyses will be based on our data in the future in Japan.
TL;DR: OA-AICA anastomosis with aneurysm trapping could be the optimal surgical management of the AICA aneurYSm located exclusively inside the internal auditory canal, especially if the parent artery supplies a wide vascular territory.
Abstract: A 77-year-old woman presented with an extremely rare exclusively intra-meatal anterior inferior cerebellar artery (AICA) aneurysm manifesting as subarachnoid hemorrhage. The aneurysm was located at a non-branching site of its meatal loop, deeply inside the internal auditory canal. The ipsilateral posterior inferior cerebellar artery was hypoplastic and the affected AICA supplied a wide vascular territory in the right cerebellum. The patient underwent microsurgical trapping of the distal AICA aneurysm in the acute stage. Collateral back flow to the parent artery was poor, so right occipital artery (OA)-AICA anastomosis was performed prior to aneurysm trapping. The postoperative course was uneventful, and magnetic resonance imaging after surgery did not demonstrate any ischemic change. Postoperative angiography showed complete disappearance of the AICA aneurysm and the apparently patent OA-AICA bypass. She did not suffer neurological deficit except for right incomplete hearing disturbance, and postoperative single photon emission computed tomography demonstrated absence of hemodynamic compromise in the cerebellum. OA-AICA anastomosis with aneurysm trapping could be the optimal surgical management of the AICA aneurysm located exclusively inside the internal auditory canal, especially if the parent artery supplies a wide vascular territory.
TL;DR: GKRS forMTLE causes adverse effects of delayed seizure remission and symptomatic radiation-induced complications, therefore, GKRS cannot be considered as an ideal alternative to surgery for MTLE.
Abstract: Gamma knife radiosurgery (GKRS) for mesial temporal lobe epilepsy (MTLE) has been proposed as an alternative to surgical resection. We report serious adverse effects of the treatment after follow-up periods over 9 years in 11 patients treated with GKRS between 1997 and 2000. The target volume of the entorhinoamygdalohippocampectomy area was 4.8-17.1 ml. Marginal dose of 20-25 Gy to the 50% isodose was delivered. One patient was drowned after suffering seizure 7 months after GKRS. Two patients did not show any reduction in seizure frequency over 9 and 18 months. Both patients requested open surgery and became seizure-free postoperatively. Four of the other eight patients were classified as Engel's class I within 4 years after GKRS. One of the four patients experienced symptomatic radiation-induced cerebral edema transiently, one developed radiation necrosis and required surgery 5 years after GKRS, and one developed cognitive impairment with hemiparesis 10 years after GKRS. Magnetic resonance (MR) imaging showed a large cyst in the irradiated temporal lobe. This patient recovered fully after the cyst excision. Only one patient became seizure-free and antiepileptic drug-free without symptomatic radiation-induced complications. However, MR imaging revealed abnormal enhancement, cyst formation, and diffuse white matter change in the irradiated temporal lobe 9 years after GKRS. GKRS for MTLE causes adverse effects of delayed seizure remission and symptomatic radiation-induced complications. Therefore, GKRS cannot be considered as an ideal alternative to surgery for MTLE. Long-term follow-up studies including MR imaging with contrast medium are required for the patients even after successful control of seizures.