Among the various disorders that manifest with gait disturbance, cognitive impairment, and urinary incontinence in the elderly population, idiopathic normal pressure hydrocephalus (iNPH) is becoming ...of great importance. The first edition of these guidelines for management of iNPH was published in 2004, and the second edition in 2012, to provide a series of timely, evidence-based recommendations related to iNPH. Since the last edition, clinical awareness of iNPH has risen dramatically, and clinical and basic research efforts on iNPH have increased significantly. This third edition of the guidelines was made to share these ideas with the international community and to promote international research on iNPH. The revision of the guidelines was 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. This revision proposes a new classification for NPH. The category of iNPH is clearly distinguished from NPH with congenital/developmental and acquired etiologies. Additionally, the essential role of disproportionately enlarged subarachnoid-space hydrocephalus (DESH) in the imaging diagnosis and decision for further management of iNPH is discussed in this edition. We created an algorithm for diagnosis and decision for shunt management. Diagnosis by biomarkers that distinguish prognosis has been also initiated. Therefore, diagnosis and treatment of iNPH have entered a new phase. We hope that this third edition of the guidelines will help patients, their families, and healthcare professionals involved in treating iNPH.
Here we describe the procedures of endoscopic pituitary and skull base surgery in our institute. We also review the literature to reveal recent advances in this field. Endonasal approach via the ...sphenoid ostium was carried out for pituitary lesions without the nasal speculum. Postoperative nasal packing was basically not needed in such cases. For meningiomas, craniopharyngiomas, and giant pituitary adenomas, which required intra-dural procedures, nasal procedures such as middle nasal conchotomy and posterior ethmoidectomy, and skull base techniques such as optic canal decompression and removal of the planum sphenoidale were carried out to gain a wider operative field. Navigation and ultrasonic Doppler ultrasonography were essential. Angled endoscopes allowed more successful removal of tumors under direct visualization extending into the cavernous sinus and lower clivus. If cerebrospinal fluid (CSF) leakage occurred during operation, the dural opening was covered with a vascularized mucoseptal flap obtained from the nasal septum. Lumbar drainage system to prevent postoperative CSF rhinorrhea was frequently not required. Angled suction tips, single-shaft coagulation tools, and slim and longer holding forceps, all of which were newly designed for endoscopic surgery, were essential for smoother procedures. Endonasal endoscopic pituitary surgery allows less invasive transsphenoidal surgery since no postoperative nasal packing and less dependence on lumbar drainage are needed. Endoscopic pituitary surgery will be more common and become a standard procedure. Endoscopic skull base surgery has enabled more aggressive removal of extrasellar tumors with the aid of nasal and skull base techniques. Postoperative CSF leakage is now under control due to novel methods which have been proposed to close the dural defect in a water-tight manner. Endoscopic skull base surgery is more highly specialized, so needs special techniques and surgical training. Patient selection is also important, which needs collaboration with ear, nose, and throat specialists. As a safe and successful procedure in skull base surgery, this complex procedure should be carried out only in specialized hospitals, which deal with many patients with skull base lesions.
The endoscopic endonasal trans-sphenoidal approach can decompress the traumatized and swollen optic nerve without brain retraction. Three cases of traumatic optic neuropathy (TON) were treated by ...this endoscopic approach. We performed 58 endoscopic endonasal trans-sphenoidal approaches in 52 patients between April 2006 and December 2007. Three patients, 2 men and a woman aged 42-66 years, with TON were treated by endoscopic endonasal trans-sphenoidal optic nerve decompression. Adequate decompression of the optic canal was observed in all three patients. Two experienced improved visual function, but the third did not. No surgical complications were observed in any patient. We were able to reach the optic canal above the opticocarotid recess near the orbit. Endoscopic endonasal trans-sphenoidal optic canal decompression using a navigation system may be a safe and effective surgical strategy in carefully selected patients with TON.
The authors report their investigation on the current status of neuroendoscopic biopsy for ventricular and paraventricular tumors as well as treatment for associated hydrocephalus in Japan.
Patients ...who had undergone therapeutic neuroendoscopy between 2005 and 2009 were included in this study. The main items examined were age; sex; localization of tumor; pathological diagnosis using biopsy; the presence, treatment, and efficacy of treatment of associated hydrocephalus; perioperative complications; activities of daily living (ADL) before and after therapeutic neuroendoscopy; and the presence of dissemination during the postoperative course.
Seven hundred fourteen patients from 123 sites (462 male and 252 female patients, mean age 33.3 years) were enrolled. Localization of the tumor was mainly classified into the lateral ventricle in 91 patients, the third ventricle in 339, the fourth ventricle in 18, the suprasellar region in 75, and other paraventricular areas in 191 patients. The most commonly observed tumors were germ cell tumors in the third ventricle (177 cases 39%), cystic lesions in the suprasellar region (56 cases 75%), and astrocytic tumors in the thalamus-basal ganglia (71 cases 38%). Although 641 (92.8%) of 691 patients could receive neuroendoscopic diagnosis using biopsy, the diagnosis obtained with endoscopic biopsy differed from the final diagnosis based on subsequent craniotomy in 18 patients and clinical course in 3 patients. Of these 21 patients, 7 had astrocytic tumors, 4 had pineal tumors, 6 had germ cell tumors, and 4 had other tumors. The final diagnostic accuracy rate was 89.7%. Associated hydrocephalus was observed in 517 patients (72.4%), of whom 316 and 39 underwent third ventriculostomy and fenestration of the septum, respectively. The response rates were 96.2% and 89.7%, respectively. Third ventriculostomy was required for recurrence of hydrocephalus in 41 patients (13.0%), and the long-term response rate was therefore 83.2% (263 of 316 patients). Perioperative complications other than fever, such as new onset of or progressive hydrocephalus, infection due to CSF leakage, and bleeding in the ventricle or tumor, were found in 81 patients (11.3%). The median Karnofsky Performance Scale score before endoscopic surgery was 80, but it increased to 90 after surgery. The score was thus significantly increased after surgery (p < 0.0001, Mann-Whitney U-test). Activities of daily living after surgery decreased due to perioperative complications in 15 patients (2.1%). The incidence of new dissemination after endoscopic biopsy was 6.8% and not high compared with routine surgical treatment.
The authors concluded that neuroendoscopic diagnosis using biopsy for ventricular and paraventricular tumors is adequately accurate and safe. It was demonstrated that endoscopic procedures play important roles not only in the treatment of hydrocephalus associated with intra- and paraventricular tumors but also in significantly improving ADL. Furthermore, the long-term outcome of endoscopic third ventriculostomy was clearly favorable.
INTRODUCTION:
Primary intracranial germ cell tumors (iGCT) are rare neoplasms that occur in children and adolescents. This study examined both the pathogenesis and the origin of these tumors, as it ...has been hypothesized that they originate from a totipotent primordial germ cell. MATERIALS AND
METHODS:
We applied recent knowledge from gonadal germ cell tumors and analyzed expression of a wide panel of stem cell-related proteins(C-KIT, OCT-3/4 (POU5F1), NANOG, SOX2, CD30 and PLAP). Expression shown by immunohistochemistry was analyzed in 12 children and young adults with iGCT, contributing to a careful description of these unusual tumors and adding to the understanding of pathogenesis.
RESULTS:
Immunohistochemistry showed 5/5 positive in Oct3/4, 5/5 in NANOG, 9/9 in C-KIT and 1/5 in SOX2. All cases that showed positive staining in Oct3/4 or NANOG indicated that the tumors contained germinoma or embryonal carcinoma component. Immunohistochemistry revealed that stem cell related proteins were highly expressed in iGCT, and many similarities were detected with their gonadal equivalents, including a close similarity with primordial germ cells.
CONCLUSION:
The expression of genes associated with embryonic stem cell pluripotency in CNS germ cell tumors strongly suggests that these tumors are derived from cells that retain, at least partially, an embryonic stem cell-like phenotype, which is a hallmark of primordial germ cells. In addition, these data can be applied to stratify iGCT for treatment strategy in order to provide good survival rate and good functional outcome.
Abstract
Germ cell tumor (GCT) is a rare juvenile CNS tumor that is more frequent in eastern Asia. Most survivors require continuous medical care for hormone replacement, maintenance of shunting ...devices, and late radiation-induced effects. In the present study, we retrospectively analyzed medical records of long-term GCT survivors, and make the health and social issues clear. Ninety-two GCT patients were treated in our institute from 1982 to 2018, and 81 patients, of which medical records are available, are included. The median follow-up period is 12.2 years, and 47 patients (58.1%) are followed for more than ten years. The overall survival rate is gradually decreasing more than ten years follow-up, such as 10-, 15- and 25-years survival are 92.3, 87.7, and 73.3%, respectively. In the long-term follow-up, eight subsequent malignancy and seven cerebrovascular events are recorded. These events occurred 20 years or more after the treatments, and six CNS malignancies were observed in survivors irradiated with 50Gy or more. As social issues, forty-two of 50 adult survivors had been employed after the treatments, but only thirty-four (70.8%) are still working. Of note, only nine (18.8% of adults) survivors got married. All four married women require any hormone replacement, while only one of 4 men requires the replacement. Long-term follow-up of GCT survivors revealed subsequent malignancy and social problems. A recent attempt to decrease the dose of irradiation might overcome some issues. As a conclusion, GCT survivors require a supporting program for not only health but also social issues.
The proto-oncogene, BCL-2, has been suggested to participate in cell survival during development of, and after injury to, the CNS. Transgenic (TG) mice overexpressing human Bcl-2 (n = 21) and their ...wild-type (WT) littermates (n = 18) were subjected to lateral controlled cortical impact brain injury. Lateral controlled cortical impact brain injury resulted in the formation of a contusion in the injured cortex at 2 days, which developed into a well-defined cavity by 7 days in both WT and TG mice. At 7 days after injury, brain-injured TG mice had a significantly reduced cortical lesion (volume = 1.99 mm3) compared with that of the injured WT mice (volume = 5.1 mm3, P < 0.01). In contrast, overexpression of BCL-2 did not affect the extent of hippocampal cell death after lateral controlled cortical impact brain injury. Analysis of motor function revealed that both brain-injured WT and TG mice exhibited significant right-sided deficits at 2 and 7 days after injury (P < 0.05 compared with the uninjured controls). Although composite neuroscores (sum of scores from forelimb and hind limb flexion, lateral pulsion, and inclined plane tests) were not different between WT and TG brain-injured mice, TG mice had a slightly but significantly reduced deficit in the inclined plane test (P < 0.05 compared to the WT mice). These data suggest that the cell death regulatory gene, BCL-2, may play a protective role in the pathophysiology of traumatic brain injury.
Abstract
Germ cell tumor (GCT) is a juvenile tumor, completely cured by adequate treatments, including radiation therapy and chemotherapy. However, survivors require continuous medical treatment ...mostly due to hypopituitarism, and several issues could be observed during follow-up. In the present report, we analyzed medical records of long-term GCT survivors, retrospectively, and make the issues clear. Of ninety-three, 82 GCT patients with several medical and social information are included in the analysis. Median follow-up time is 11.95 years, and 47 patients followed more than ten years. Fifteen deaths are recorded, and 8 are tumor-progression, and 7 are treatment-related long-term events, including four subsequent gliomas, one cerebral vascular disease, one renal cancer, and one sudden death. Seven survivors developed subsequent malignancy, and all lived more than 19 years and received more than 50Gy of irradiation. The overall survival rate is gradually decreasing more than ten years follow-up, such as 10-, 15- and 25-years survival are 92.3, 87.7 and 73.3%, respectively. Long-term follow-up also clarified several social issues. Forty-two of 50 adult survivors had a job after the treatments, but only thirty-four (70.8%) are still working. Of note, only nine (18.8% of adults) survivors got married. All four women require any hormone replacement, while only one of 4 men requires the replacement. Long-term follow-up of GCT survivors revealed subsequent malignancy and social problems. Recent attempt to decrease radiation dose and to give adequate hormone replacement might overcome these problems. Clinicians still observed GCT patients closely.
The mossy fiber system in the hippocampus of amygdaloid-kindled rats was examined by using highly polysialylated neural cell adhesion molecule (PSA-NCAM) as a marker for immunohistochemical detection ...of immature dentate granule cells and mossy fibers in combination with bromodeoxyuridine (BrdU) labeling of newly generated granule cells. Statistically significant increases in BrdU-labeled cells and PSA-NCAM-positive cells occurred in the dentate gyrus following kindling. The increase in PSA-NCAM-immunoreactive neurites was confined to the entire stratum lucidum of CA3. Immunoelectron-microscopic examination also revealed that PSA-NCAM-positive immature synaptic terminals of the sprouting mossy fibers increased in the stratum lucidum of CA3 in the kindled rats. The increase in the numbers of PSA-NCAM-positive granule cells correlated well with the increase in the immunopositive neurites and synaptic terminals on the mossy fiber trajectory. The increase in these PSA-NCAM-immunopositive structures is thought to reflect the enhancement of sprouting and synaptogenesis of mossy fibers by a subset of granule cells newly generated during amygdaloid-kindling and suggests that the reorganization of the mossy fiber system on the normal trajectory at least in part contributes to the acquisition and maintenance of an epileptogenic state.
Our previous community-based study demonstrated that some individuals with AVIM asymptomatic ventriculomegaly with features of idiopathic normal pressure hydrocephalus (iNPH) on magnetic resonance ...imaging (MRI) progressed to iNPH in several years. In this hospital-based study, we investigated the progression rate from AVIM to iNPH and its possible predictors.
We conducted a prospective study of participants with AVIM from several medical institutions/hospitals in Japan. AVIM is defined as “asymptomatic ventriculomegaly with features of iNPH on MRI”; in the present study, asymptomatic was defined as “0 (no symptoms) or 1 (presence of only subjective, but not objective, symptoms) on the iNPH Grading Scale (iNPH-GS).” We also measured possible predicting factors for AVIM-to-iNPH progression, including age, sex, body weight, blood pressure, diabetes mellitus, dyslipidemia, history of mental disease/head injury/sinusitis/smoking/alcohol-intake, Evans index, and the presence of DESH (disproportionately enlarged subarachnoid-space hydrocephalus) findings on brain MRI, and analyzed these potential predictive values.
In 2012, 93 participants with AVIM were registered and enrolled in the study. Of these, 52 participants were able to be tracked for three years (until 2015). Of the 52 participants, 27 (52%) developed iNPH during the follow-up period (11 definite, 6 probable, and 10 possible iNPH), whereas 25 participants remained asymptomatic in 2015. Among the possible predictive factors examined, the baseline scores of iNPH-GS predicted the AVIM-to-iNPH progression.
The multicenter prospective study demonstrated that the progression rate from AVIM to iNPH was ~17% per year, and the baseline scores of iNPH-GS predicted the AVIM-to-iNPH progression.
•An annual progression rate from AVIM to iNPH is estimated at 17%.•DESH findings on brain MRI predict a risk for progression to iNPH.•iNPH-GS scores predict AVIM-to-iNPH progression.
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