The importance of considering competing risks in recurrence analysis of intracranial meningioma.

BACKGROUND: The risk of recurrence is overestimated by the Kaplan-Meier method when competing events, such as death without recurrence, are present. Such overestimation can be avoided by using the Aalen-Johansen method, which is a direct extension of Kaplan-Meier that accounts for competing events. Meningiomas commonly occur in older individuals and have slow-growing properties, thereby warranting competing risk analysis. The extent to which competing events are considered in meningioma literature is unknown, and the consequences of using incorrect methodologies in meningioma recurrence risk analysis have not been investigated. METHODS: We surveyed articles indexed on PubMed since 2020 to assess the usage of competing risk analysis in recent meningioma literature. To compare recurrence risk estimates obtained through Kaplan-Meier and Aalen-Johansen methods, we applied our international database comprising ~ 8,000 patients with a primary meningioma collected from 42 institutions. RESULTS: Of 513 articles, 169 were eligible for full-text screening. There were 6,537 eligible cases from our PERNS database. The discrepancy between the results obtained by Kaplan-Meier and Aalen-Johansen was negligible among low-grade lesions and younger individuals. The discrepancy increased substantially in the patient groups associated with higher rates of competing events (older patients with high-grade lesions). CONCLUSION: The importance of considering competing events in recurrence risk analysis is poorly recognized as only 6% of the studies we surveyed employed Aalen-Johansen analyses. Consequently, most of the previous literature has overestimated the risk of recurrence. The overestimation was negligible for studies involving low-grade lesions in younger individuals; however, overestimation might have been substantial for studies on high-grade lesions.

Clinicopathologic analysis of pineal parenchymal tumors of intermediate differentiation: a multi-institutional cohort study by the Kyushu Neuro-Oncology Study Group.

PURPOSE: Pineal parenchymal tumors of intermediate differentiation (PPTIDs), which were recognized in the 2007 World Health Organization (WHO) classification, are rare, accounting for less than 1% of all central nervous system tumors. This rarity and novelty complicate the diagnosis and treatments of PPTID. We therefore aimed to evaluate the clinicopathological significance of this tumor. METHODS: At 11 institutions participating in the Kyushu Neuro-Oncology Study Group, data for patients diagnosed with PPTID were collected. Central pathology review and KBTBD4 mutation analysis were applied to attain the diagnostically accurate cohort. RESULTS: PPTID was officially diagnosed in 28 patients: 11 (39%) with WHO grade 2 and 17 (61%) with WHO grade 3 tumors. Median age was 49 years, and the male:female ratio was 1:2.1. Surgery was attempted in all 28 patients, and gross total resection (GTR) was achieved in 46% (13/28). Adjuvant radiotherapy and chemotherapy were administered to, respectively, 82% (23/28) and 46% (13/28). The 5-year progression-free survival (PFS) and overall survival rates were 64.9% and 70.4% respectively. Female sex (p = 0.018) and GTR (p < 0.01) were found to be independent prognostic factors for PFS and female sex (p = 0.019) was that for OS. Initial and second recurrences were most often leptomeningeal (67% and 100% respectively). 80% (20/25) of patients harbored a KBTBD4 mutation. CONCLUSIONS: Female sex and GTR were independent prognostic factors in our patients with PPTID. Leptomeningeal recurrence was observed to be particularly characteristic of this tumor. The rate of KBTBD4 mutation observed in our cohort was acceptable and this could prove the accuracy of our PPTID cohort.

[A Case Of An Elderly Patient With Blake's Pouch Cyst].

Blake's pouch cyst is a congenital disease in which the ventricle enlargement is a non-communicating hydrocephalus. Some elderly patients have a process of the idiopathic normal pressure hydrocephalus (communicating hydrocephalus). We report an elderly patient with Blake's pouch cyst. A 68-year-old man visited our hospital with a gait disturbance that had begun 2 years previously but had become aggravated. He did not have dementia or urinary disturbance. CT scan and MRI showed the hydrocephalus included an IV ventricle, and the cerebrocerebellar fluid space in the posterior fossa was large. A Tap test was positive, so we attempted lumboperitoneal shunt, and his gait disturbance improved.

[A Surgical Case of Arachnoid Cyst With Rheumatoid Meningitis].

Rheumatoid meningitis is a rare neurological manifestation that is difficult to diagnose. Here we report a 77-year-old woman who developed a left hemiparesis because of an arachnoid cyst with rheumatoid meningitis. The patient had a 13 year history of rheumatoid arthritis, which had been successfully treated. She developed left hemiparesis and visited our hospital. MRI showed a high signal intensity lesion in the right frontal subarachnoid space and an arachnoid cyst on Fluid attenuated inversion recovery (FLAIR). A gadolinium enhanced T1 weighted image revealed associated abnormal enhancement. We removed the arachnoid cyst and diagnosed the patient as having rheumatoid meningitis. Her symptoms and MRI findings improved rapidly by methylprednisolone pulse therapy. Rheumatoid meningitis is rare, but a case of arachnoid cyst associated with rheumatoid meningitis was extremely rare. This is the first report of such a case.

[A Case of the Internal Carotid Artery Occlusion Immediately After Intravenous Recombinant Tissue Plasminogen Activator Treatment for Contralateral Middle Cerebral Artery Occlusion].

Early recurrent ischemic stroke (ERIS), as well as symptomatic intracranial hemorrhage (SICH) and progressive stroke (PS), causes early neurological deterioration. Here we report a case of a patient with right internal carotid artery (ICA) occlusion immediately after intravenous recombinant tissue plasminogen activator (rt-PA) treatment for left middle cerebral artery (MCA) occlusion. A 79-year-old woman with drowsiness, aphasia and right hemiparesis was brought to our hospital. MRI showed acute infarction in the left internal capsule and occlusion of the left middle cerebral artery. rt-PA was administered intravenously to the patient 2 hours after the onset of the event. Her consciousness disturbance and aphasia improved, but the right hemiparesis did not. We performed emergent endovascular thrombectomy, but the right ICA (cervical portion) was occluded during the surgery. Finally, the endovascular thrombectomy achieved the recanalization of the left MCA and right ICA. When performing intravenous thrombolysis, we should beware the possibility of re-occlusion and prepare for interventional treatment.

[A Surgical Case of Hemifacial Spasm Due to Tentorial Meningioma -peritorcular type- in Posterior Fossa].

Hemifacial spasm is commonly caused by compression of the facial nerve due to overlying vessels, and also due to various types of tumor or aneurysm, and other factors. It occurs, although rarely, as a secondary effect of cerebellar or brainstem shift resulting from a tumor. In such a case, the presence of a large tumor often leads to additional neurological deficits. We present a case of hemifacial spasm caused by a peritorcular type of large tentorial meningioma in the posterior fossa. A 68-year-old woman presented with right facial numbness 4 months ago and right hemifacial spasm 2 weeks ago. Upon visiting our hospital, she displayed no neurological deficits other than the right hemifacial spasm. MRI revealed a peritorcular type of large tentorial meningioma in the posterior fossa with perifocal edema. The right cerebellopontine cistern was narrowed, and the cerebellar tonsil was herniated. The right facial nerve ran adjacent to the anterior inferior cerebellar artery (AICA). Angiography showed that only the left posterior meningeal artery (PMA) flowed to the tumor. The loop of the right AICA extended into the right cerebellopontine cistern. After embolization of the PMA, the tumor was surgically removed, leading to an improvement in the patient's hemifacial spasm. Postoperative MRI confirmed complete removal of the tentorial meningioma without any contact with the right facial nerve. The hemifacial spasm was caused secondarily by the cerebellar or brainstem shift due to the large tentorial meningioma in the posterior fossa. This large tumor had not produced any other neurological deficits before the hemifacial spasm appeared. We report this case because it is extremely rare.

[A Case of Bilateral Medial Medullary Infarction With Heart Appearance Sign].

We report a bilateral medial medullary infarction in which diffusion-weighted images revealed a unique configuration: a heart appearance sign. If it is early diagnosed, it might predict a poor outcome. An 85-year-old man developed dysarthria and numbness in his four limbs and was transferred to our hospital. Brain MR diffusion-weighted images revealed a high-intensity lesion in the bilateral medial medulla oblongata -- a heart appearance sign -- and we diagnosed a bilateral medial medullary infarction. Although his symptom changed aggressively for the worse, it finally changed for the better without bulbar paralysis, and he was transferred to another hospital for rehabilitation. When the medial medulla oblongata is supplied by the unilateral control of the anterior spinal artery, its occlusion can cause a bilateral medial medullary infarction.

[A Case of Hearing Loss Preceding Anterior Inferior Cerebellar Artery Infarction Syndrome With a Progressive Course].

Anterior inferior cerebellar artery (AICA) syndrome is a well-known symptomatic disorder. AICA syndrome is one of the causes of sudden hearing loss seen in recent years, but most of these cases were diagnosed as hearing loss preceded by other symptoms. Due to variations in AICA, we consider that many cases are actually not recognized as AICA syndrome. In this report, we describe a case of AICA syndrome preceded by hearing loss and describe its course of progression.

Longest survival by the combination of radiation-therapy and resection in patient with metastatic spinal paragangliomas from primary-neck lesion with succinate dehydrogenase subunit B (SDHB) mutation.

Metastatic paraganglioma (MPG) of the spine is a rare condition, with no established management. Herein, we report the longest survival case of a primary neck tumor that caused spinal MPG with a succinate dehydrogenase subunit B (SDHB) mutation (c.470delT, p.L157X) which could have promoted its malignancy. This male patient initially presented with a left neck PG which was diagnosed by a biopsy when he was 54 years-old. Simultaneously performed additional examinations revealed the spinal metastatic tumors on the T5-7 vertebrae and L3 vertebra-sacrum. These primary neck and metastatic spinal tumors' growths were once suppressed under the radiation therapy. Nineteen years later, he developed acute progressive paraparesis due to a mass located at the T2-3 level, tightly compressing the spinal cord, and protruding into the left thoracic cavity. We resected the maximum possible area of tumor in the spinal canal, confirmed MPG by histological examination, and then, we administered radiation therapy of 40 Gy in 20 fractions. Eventually, the patient was able to walk unaided with no evidential tumor recurrence for 3 years after treatment. Generally, clinical feature of MPG with SDHB mutation from abdominal lesion is thought to be poor prognosis. However, our case suggests the possibility of long-term control of spinal MPG with the adequate combination of radiation therapy and resection if metastatic lesions from primary-neck lesion with an SDHB mutation are remained to spine.

Posterior fossa subdural hematoma in a neonate with cleidocranial dysostosis after a spontaneous vaginal delivery: a case report.

BACKGROUND: Cleidocranial dysostosis (CCD) is an anomaly characterized by delayed closure of the cranial sutures, midface hypoplasia, moderately short stature, hypoplastic or aplastic clavicles, dental abnormalities, and other complications. CASE PRESENTATION: We report a case of posterior fossa subdural hematoma (PFSDH) after vaginal delivery in a neonate with CCD, which presented with several clinical symptoms such as apnea, vomiting, and bradycardia. Our patient, who had a family history of CCD, developed apnea and vomiting shortly after birth; PFSDH was detected by head computed tomography, and the patient recovered well following standard medical treatment. CONCLUSION: The prognosis of intracranial hemorrhage in neonates with CCD is generally poor. In neonates, PFSDH occurs by the following mechanism: the distortion of the infant's cranium during delivery, by the strong force, causes elongation of the falx and angulation of the tentorium that leads to tears in the posterior fossa venous structures, which then cause bleeding into the subdural space. In CCD, the forces occurring during vaginal delivery may causeexcessive distortion of the fragile skull. An awareness of CCD is hence important to avoid vaginal delivery in prenatally diagnosed CCD cases with a family history of CCD.

In Vivo Study of the Efficacy and Safety of 5-Aminolevulinic Radiodynamic Therapy for Glioblastoma Fractionated Radiotherapy.

To treat malignant glioma, standard fractionated radiotherapy (RT; 60 Gy/30 fractions over 6 weeks) was performed post-surgery in combination with temozolomide to improve overall survival. Malignant glioblastoma recurrence rate is extremely high, and most recurrent tumors originate from the excision cavity in the high-dose irradiation region. In our previous study, protoporphyrin IX physicochemically enhanced reactive oxygen species generation by ionizing radiation and combined treatment with 5-aminolevulinic acid (5-ALA) and ionizing radiation, while radiodynamic therapy (RDT) improved tumor growth suppression in vivo in a melanoma mouse model. We examined the effect of 5-ALA RDT on the standard fractionated RT protocol using U251MG- or U87MG-bearing mice. 5-ALA was orally administered at 60 or 120 mg/kg, 4 h prior to irradiation. In both models, combined treatment with 5-ALA slowed tumor progression and promoted regression compared to treatment with ionizing radiation alone. The standard fractionated RT protocol of 60 Gy in 30 fractions with oral administration of 120 and 240 mg/kg 5-ALA, the human equivalent dose of photodynamic diagnosis, revealed no significant increase in toxicity to normal skin or brain tissue compared to ionizing radiation alone. Thus, RDT is expected to enhance RT treatment of glioblastoma without severe toxicity under clinically feasible conditions.

Atypical and Fibrous Meningioma with Differential Cerebral Blood Volume on Magnetic Resonance Imaging: A Case Report.

Magnetic resonance imaging (MRI) features of meningiomas typically reveal a well-circumscribed and contrast-enhancing dural mass. Here we discuss the differences in MRI findings between typically benign and atypical meningioma, and their clinical implications. An MRI of a 67-year-old man revealed a substantial homogeneous enhancing tumor nodule. The MRI also showed two components in the tumor, and the regional cerebral blood volume (rCBV) was higher in the lateral than in the medial mass. A pathological examination also revealed features of both benign and atypical meningiomas. The Ki-67 labeling index was 1% on the medial side and 5% on the lateral side. There were clearly two components within the tumor mass, and the MRI revealed differential rCBV in the tumor. The results indicated a nontypical MRI of meningiomas, allowing for predictability of atypical meningiomas using MRI before surgical resection.

[Duplication of Basilar Artery - A Case Report].

We report a duplication of basilar artery, it is a rare variation of vessel and arisen from unfusing of a posterior longitudinal neural arteries. 71-year-old man with hypertension, was referred to our hospital for a visual disturbance. The duplication of basilar artery was incidentally discovered on a magnetic resonance imaging (MRI). MRI showed an artery lined up side by side a basilar artery in front of brain stem, it was connected from vertebral artery to anterior inferior cerebellar artery. So it was diagnosed a duplication of basilar artery. A duplication of basilar artery is not a rare variation of vessel but a comparatively few variation of vessel, because some cases of the fenestration of basilar artery were included the duplication of basilar artery on review of the literature. And it occurred to be stabilized its back flow.

Clinical outcomes of cerebral infarction in nonagenarians compared among four age groups.

BACKGROUND: Average female life expectancy in Japan is approximately 90 years. Occasionally, we encounter stroke patients older than 90 years. AIMS: To determine the clinical features and outcomes associated with cerebral infarction in patients aged ≥ 90 years. METHODS: We examined 289 consecutive patients (163 males, 129 females; mean age 77.5 years) diagnosed with cerebral infarction. We divided them into four groups according to age in years: middle (< 65), pre-old (65-74), old (75-89), and super old (≥ 90). We divided the super old group into mild symptoms (NIHSS ≤ 5) and severe symptoms (NIHSS > 5) and examined outcomes. RESULTS: Statistically significant associations were observed between female sex, cardiogenic infarction, and high complication rates and super old age. NIHSS and mRS scores at 30-day post-stroke were higher in the super old group. In some cases, complications led to poor prognoses. Eighty-seven percent of patients with mild symptoms (NIHSS ≤ 5) recovered to mRS 0-2 similar to the younger age group. None of the patients with severe symptoms (NIHSS > 5) recovered to mRS 0-2. DISCUSSION: We investigated the clinical outcomes following cerebral infarction in patients aged 90 years or older and found that mild symptoms were consistently associated with good prognoses, regardless of patients' age. CONCLUSIONS: Patients in the super old group had more severe symptoms and poorer outcomes than younger age groups. However, patients with mild symptoms tended to have better prognoses and returned to daily life similar to the younger age group.

Removal of Double Cavernous Angioma of the Frontal Lobe using a Three-Dimensional Printed Model: A Case Report.

Cerebral cavernous angiomas are vascular anomalies with dilated spaces. We report the case of rare double cavernous angiomas causing higher brain dysfunction. A 74-year-old man exhibited cognitive dysfunction. Magnetic resonance imaging showed two tumors with hemorrhage in the left frontal lobe. Preoperative diagnosis was hemorrhage caused by cavernous angiomas. A 3D model of the double cavernous angioma was made to confirm their association with cortical veins and tumors. Tumors were removed using a single small corticotomy. This is the first report of a rare double cavernous angioma and the 3D printed model facilitated removal of the tumors.

5-Aminolevulinic Acid: Pitfalls of Fluorescence-guided Resection for Malignant Gliomas and Application for Malignant Glioma Therapy.

5-Aminolevulinic acid (ALA) has been widely used as an intravital fluorescence marker in the fluorescence-guided resection of malignant gliomas. Although not a photosensitizer itself, 5-ALA is a prodrug that accumulates protoporphyrin IX (PpIX) in the mitochondria of glioma cells; PpIX acts as a photosensitizer. Fluorescence-guided resection for malignant gliomas has some pitfalls. Moreover, 5-ALA is not merely a fluorescence marker but has potential as a mitochondria-targeting drug for malignant glioma therapy. In this article, we review the literature related to 5-ALA, discuss the pitfalls of fluorescence-guided resection using 5-ALA for malignant gliomas, and describe the application of 5-ALA for malignant glioma therapy with personal opinions.

Recurrent Shoulder Tip Pain After Ventriculoperitoneal Shunt Placement Associated with Infectious Peritonitis with Propionibacterium acnes; A Case Report and Review of the Literature.

Ventriculoperitoneal (VP) shunt placement is commonly performed for the treatment of hydrocephalus, and several complications of this procedure are well known. Radiating shoulder tip pain after VP shunt placement has been reported as an unusual complication in a few cases, associated with dislocation of the peritoneal catheter. We described the case of a 9-year-old girl who presented with recurrent radiating shoulder tip pain after VP shunt placement. The pain recurred after peritoneal catheter repositioning because of peritoneal inflammation and adhesion due to peritonitis with Propionibacterium acnes (P. acnes). This bacterium was isolated using 16S ribosomal RNA gene polymerase chain reaction (16S rRNA gene PCR), and anaerobic and prolonged culture tests. After antibacterial treatment, ventriculoarterial (VA) shunt placement was successfully performed. Hemidiaphragm irritation by the peritoneal catheter leads to radiating shoulder tip pain, and peritoneal inflammation and adhesion caused by infectious peritonitis may cause recurrence of this despite catheter repositioning. Clinicians should be aware of shoulder pain as a complication of VP shunt placement, and should consider VA shunt placement as an alternative treatment if this symptom recurs after catheter repositioning. Furthermore, 16S rRNA gene PCR and anaerobic and prolonged culture tests should be considered to detect P. acnes infection.

Importance of Finding Embolic Sources for Patients with Embolic Stroke of Undetermined Source.

OBJECTIVE: The concept of embolic stroke of undetermined source refers to cryptogenic strokes caused by either major or minor risks. Although antiplatelet treatments are most often used for secondary prevention of embolic stroke of undetermined source, optimal strategies remain unclear. To determine the ideal treatment strategy for secondary prevention, we investigated embolic sources among patients with embolic stroke of undetermined source. METHODS: The study included 292 consecutive patients (135 men, 157 women; mean age: 74.3 ± 11.6 years) diagnosed with cerebral infarction, 27 of whom were diagnosed with embolic stroke of undetermined source (9.2%; 14 men, 13 women; mean age: 70.7 ± 11.5 years). These 27 patients were examined using contrast-enhanced whole-body computed tomography, transesophageal echocardiography, and Holter electrocardiography. We evaluated whether antiplatelet or anticoagulant treatment was preferred based on the embolic source. RESULTS: Embolic sources among patients with embolic stroke of undetermined source included valve calcification (11.1%), left ventricle diastolic dysfunction (18.5%), cancer-associated stroke (25.9%), covert atrial fibrillation (7.4%), aortic arch atherosclerotic plaques (11.1%), paradoxical embolism (3.7%), and sick sinus syndrome (3.7%). Embolic sources remained unidentified in 5 patients (18.5%). Our analysis revealed that 21 of the 27 patients (77.8%) with embolic stroke of undetermined source required anticoagulant therapy for secondary prevention. CONCLUSION: Although aspirin is the most commonly used antithrombotic drug for embolic stroke of undetermined source, our results suggest that some patients require anticoagulant therapy. Determining embolic sources is important for selecting the appropriate treatment options for this patient population.

Characteristics of Bone Destruction in Cranial Vault Lymphoma Compared with Other Skull Tumors.

Cranial vault lymphomas are rare and challenging to diagnose. We present herein two cases of cranial vault lymphoma. The first patient was a 72-year-old woman who presented with a large mass in the parietal bone found incidentally following a head injury. The second patient was a 63-year-old man who presented with an occipital subcutaneous mass associated with visual disturbance and occipital headaches. The diagnosis of a malignant tumor in the second patient was straightforward due to his symptoms and considerable bone destruction, but the first patient was more difficult to diagnose due to a lack of symptoms and only slight bone destruction detected by computed tomography (CT). Both were histophathologically diagnosed with diffuse large B cell lymphoma (DLBCL) in the cranial vault. We also investigated the clinical features, including initial symptoms and patterns of bone destruction, in 23 patients with other types of skull tumors. This comparison showed that cranial vault lymphomas cause large masses on the scalp and lead to characteristic incomplete bone destruction, indicating that cranial bone is destroyed very slowly despite the expanding subcutaneous mass. This feature is unique compared with other benign and malignant skull tumors. In addition, cranial vault lymphoma can be confirmed via bone window CT.

[Takotsubo Cardiomyopathy and Neurogenic Pulmonary Edema Following Fibrinolytic Therapy for Embolic Stroke:A Case Report].

A 79-year-old man presented with left hemiparesis and disturbance of consciousness. Brain magnetic resonance(MR)imaging revealed an infarction in the right insular cortex. MR angiography showed a defect in the inferior trunk of the right middle cerebral artery. The patient was treated with alteplase about 2.5 h after onset. Immediately after the intravenous alteplase administration, the hemiparesis improved. However, his respiratory condition unexpectedly worsened 10 h after onset. Chest radiography demonstrated an infiltrative shadow in both lung fields. Transthoracic echocardiogram showed a dysfunction in the left ventricle and no contraction at the apex of the heart, consistent with a type of cardiomyopathy, known as takotsubo cardiomyopathy(TCM). Gradually, the patient's respiratory and cardiac function improved. Here, we describe a very rare case of TCM and neurogenic pulmonary edema(NPE)following an acute cerebral infarction, which was treated with alteplase intravenous administration. TCM and NPE have a poor prognosis, therefore diagnosis, management, and treatment in the acute phase is required.