Cerebral swelling caused by deep venous thrombosis immediately after cranioplasty.

Recently, different groups have reported a rare, unexplained complication of sudden death with massive cerebral oedema immediately after cranioplasty.Case presentation: A 34-year-old woman underwent decompressive hemicraniectomy after traumatic brain injury. She was reportedly on oral contraceptives. She developed massive cerebral swelling immediately after an uneventful cranioplasty. After surgery, her pupils became fixed and dilated, and brain MRI revealed massive brain oedema. Magnetic resonance venography displayed occlusion in the deep venous sinus. The oedema was believed to be caused by venous sinus thrombosis. The patient's family declined to avail any surgical and medical treatment, and the patient died on the second postoperative day.Conclusions: Fatal massive cerebral oedema is an uncommon complication after cranioplasty in patients who had previously undergone decompressive craniectomy. Our patient was using oral contraceptives and was thus susceptible to the development of venous sinus thrombosis. Neurosurgeons must be aware of the complications associated with venous sinus thrombosis and discuss it with the patients and their families.

Spinal cord subependymoma mimicking syringomyelia in a child: a case report.

Spinal cord subependymomas (SCSEs) in children are extremely rare, and no reports distinguishing SCSEs from syringomyelia have been published. We report a case of a 10-year-old boy who presented with torticollis, scoliosis, as well as pain that had begun in the posterior portion of the neck and progressed to the right shoulder and upper arm. Magnetic resonance imaging showed an intramedullary cyst-like lesion with the same signal intensity as that of cerebrospinal fluid. Idiopathic syringomyelia with scoliosis was first suspected, and a syrinx-subarachnoid space shunt was performed. After surgery, the lesion was slightly smaller; however, 2 years after surgery, it had re-grown, causing excruciating pain but no other symptoms. A second surgery was performed, and gross total resection was achieved. Pathological evaluation revealed SCSE. SCSE needs to be considered as a differential diagnosis for spinal centric cyst-like lesions in children.

Intracerebral steal phenomenon induced focal reversible vasogenic edema and decrease in cerebral blood flow after carotid endarterectomy.

BACKGROUND: Intracerebral steal phenomenon (ISP) is a rare complication following surgical treatment of carotid stenosis. However, the factors responsible remain unknown. We described the rear case of the ISP who had vasogenic edema and cerebral blood flow (CBF) decline and presented with hemiparesis after carotid endarterectomy (CEA). CASE DESCRIPTION: A 72-year-old male with stenosis of the bilateral carotid artery (NASCET right 90% and left 70%) presented with cerebral hypoperfusion manifesting as right hemiparesis, after left CEA. Fluid-attenuated inversion recovery images showed edema of the motor area around an old infarction and a decrease in CBF. This lesion was an area of vasogenic edema caused by ISP and focal cerebral hypoperfusion. CBF of the contralateral cerebral hemispheres had increased. The treatment with an intravenous infusion of a free radical scavenger and glycerol improved the patient's symptoms and brain edema. Magnetic resonance imaging showed a gradual decline in the brain edema, which completely disappeared 2 weeks after CEA. He was discharged with no neurological deficit. CONCLUSION: In this report, we described the case of a patient with ISP who had vasogenic edema induced by CBF decline and presented with hemiparesis following CEA. This is the first report of progressing focal vasogenic edema caused by ISP after endarterectomy.

Radiation-Induced Spinal Cord Cavernous Malformations Associated with Medulloblastoma: Case Report and Review of the Literature.

BACKGROUND: Radiation-induced spinal cord cavernous malformations (CMs) are rare pathologies compared with radiation-induced cerebral CMs. We present a case of a radiation-induced spinal cord CM developed 31 years after radiation therapy for medulloblastoma. CASE DESCRIPTION: A 37-year-old man developed a symptomatic spinal hemorrhagic lesion 31 years after radiation therapy for medulloblastoma. Magnetic resonance imaging revealed an intramedullary cystic lesion with a fluid-fluid level in the C7 area. Surgery was performed leading to an unclear diagnosis. Two years later, the patient had a relapse and underwent a second operation, allowing a definitive diagnosis of radiation-induced spinal cord CM. This is believed to be the second case of de novo intramedullary CM formation following spinal radiation therapy for medulloblastoma. CONCLUSIONS: Radiation-induced spinal cord CMs should be recognized as a possible late adverse effect in patients treated with radiation therapy for medulloblastoma.

Reversible cerebral vasoconstriction syndrome concomitant with cerebral venous sinus thrombosis following ovarian tumor resection: A report of two cases.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) presents with characteristic clinical, brain imaging, and angiographic findings. The most common clinical feature of RCVS is a severe acute headache, which is often referred to as a thunderclap headache due to the nature of its presentation. It may occur spontaneously or may be provoked by various precipitating factors. We present a rare case of RCVS concomitant with cerebral venous sinus thrombosis (CVST) in a woman who underwent resection of an ovarian tumor. CASE DESCRIPTION: Case 1 - A 42-year-old woman was admitted to our hospital with severe headache radiating to the neck, with associated vomiting. She revealed a medical history of ovarian cancer and underwent an operation for the resection of the tumor, a month before presentation. After resection, her estradiol (E2) levels were reduced from 288 pg/ml to 31 pg/ml (normal range, 0-49 pg/ml). Initial imaging on admission to our hospital revealed the left posterior convexity subarachnoid hemorrhage. Magnetic resonance angiography (MRA) showed findings consistent with RCVS affecting the left posterior cerebral artery. Magnetic resonance venography (MRV) showed CVST of the left transverse and sigmoid sinuses. Single-photon emission computed tomography (SPECT) showed a left posterior ischemic lesion. These findings improved following treatment with nimodipine and anticoagulant. Case 2 - A 39-year-old woman presented with holocranial headache associated with vomiting. She was diagnosed with an ovarian tumor. She underwent an operation 3 months before presentation. After tumor resection, her E2 level decrease from 193 pg/ml to 19 pg/ml (normal range, 0-49 pg/ml). Magnetic resonance angiography (MRA) confirmed the presence of a vasospasm involving the right anterior cerebral artery. Magnetic resonance venography (MRV) confirmed the presence of thrombosis involving the superior sagittal sinus. She was discharged on postpartum day 31 without neurological deficits after treatment with anticoagulants. At her 3-month follow-up, both MRA and MRV were within the normal limits. CONCLUSION: This is the first report of two women diagnosed with RCVS with concomitant CVST following ovarian tumor resection. Marked reductions in postoperative E2 levels could have contributed to the development of CVST and RCVS.

Risk factors of diffuse alveolar hemorrhage after acute ischemic stroke treated with tissue-type plasminogen activator. The effectiveness of activated recombinant factor VII treatment.

BACKGROUND: Diffuse alveolar hemorrhage (DAH) is a rare and frequently life-threatening complication of a variety of conditions. DAH may result from coagulation disorders, inhaled toxins, or infections. We report a series of patients who developed DAH after receiving a tissue-type plasminogen activator (tPA) for acute cerebral infarction. We aimed to find risk factors of DAH in patients receiving tPA and the effectiveness of activated recombinant factor VII (rFVIIa) treatment for the same. CASE DESCRIPTION: A total of 1023 acute ischemic stroke (AIS) patients who received tPA in our department from January 2006 to December 2018 were enrolled in this study. Four of the 1023 patients (0.39%) developed DAH. The modified Rankin scale was used to assess clinical severity. Infarction volume was assessed upon follow-up using DWI (diffusion-weighted imaging). Atherothrombotic brain infarction cases were excluded from the study. The age, sex, occlusion site, area of infarction, emphysema, intracranial hemorrhage, and neurological outcomes were analyzed. Patients who developed DAH were more likely to have a history of emphysema. We administered rFVIIa to three DAH patients with good prognosis. CONCLUSION: The inclusion/exclusion criteria of tPA were based on the AHA/ASA Guidelines for the early management of patients with AIS.These patients had no evidence of infections, bronchoscopy, autoimmune diseases, HIV, and transplantations. Our study suggests that systemic administration of rFVIIa for DAH is effective. Emphysema may be a risk factor for the development of DAH following tPA. When we use tPA for emphysema patients, we must be careful about DAH enough.

The Importance of STAT6 in a Schwannoma-like Grade III Solitary Fibrous Tumor/Hemangiopericytoma Located in the Cerebellopontine Angle and Meckel's Cave.

BACKGROUND: Hemangiopericytoma (HPC) is a rare mesenchymal tumor of the brain that occurs in the central nervous system as a solitary fibrous tumor. Surgical treatment of HPC is known to be beneficial, but results on the role of complete resection and adjuvant radiation are conflicting. However, it is often difficult to detect a central nervous system solitary fibrous tumor/HPC before surgery. We describe a presumed left trigeminal schwannoma that was histopathologically shown to be an HPC. CASE DESCRIPTION: A 65-year-old man presented with paresthesia of the second and third branches of the left trigeminal nerve. Magnetic resonance imaging showed a tumoral lesion in the left cerebellopontine angle and Meckel's cave. We suspected a schwannoma and performed surgery, which resulted in only a partial resection because of tumor bleeding and unexpected intraoperative histologic findings. We could not achieve a definitive diagnosis. However, we observed local recurrence and disseminated lesions 5 and 6.5 years later, respectively. We performed a second surgery and were able to definitively diagnose the lesion as a grade III HPC based on the positive expression of STAT6 during immunostaining. CONCLUSIONS: As solitary fibrous tumor/HPC requires gross total resection for effective resolution, it is imperative to consider it in the differential diagnoses of similar tumors. If an unusual pathologic image is found, we recommend rapid immunostaining for STAT6 to reach a definitive conclusion regarding the tumor type. This case highlights the importance of considering HPC when screening or diagnosing central nervous system lesions, especially in this rare location.

Intracranial Low-Grade Fibromyxoid Sarcoma: Findings on Electron Microscopy and Histologic Analysis.

BACKGROUND: Low-grade fibromyxoid sarcoma is a rare tumor that manifests as a deep soft tissue mass characterized by a benign histology, but with potentially aggressive clinical behavior and a high rate of recurrence; primary intracranial sarcomas are even rarer. We present a case of primary intracranial low-grade fibromyxoid sarcoma, emphasizing its clinical, radiologic, and histologic features. CASE DESCRIPTION: A 39-year-old woman presented with a recent history of headache and seizures. A right parietal mass was detected on computed tomography and magnetic resonance imaging, and she was subsequently operated on to remove the intracranial mass. Histologic examination of the resected tumor revealed mildly atypical fibroblastic cells embedded within a myxoid matrix. The diagnosis was confirmed by electron microscopy and cytogenetic analyses. CONCLUSIONS: This report describes electron microscopic evaluation of intracranial low-grade fibromyxoid sarcoma, which has an extremely rare occurrence.

Transient ischemic attack in elderly patient with PHACE syndrome.

BACKGROUND: Posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities (PHACE) is a rare congenital anomaly with a broad spectrum of clinical manifestations. CASE DESCRIPTION: We describe a 75-year-old male with PHACE anomaly, aortic anomaly, malformation of brain, aplastic right carotid artery, and cervical vasculopathy. He presented with a transient ischemic attack with the left hemiparesis, a rare clinical presentation of the PHACE syndrome. He had an uneventful recovery and recently completed a 2-year follow-up after the superficial temporal artery to middle cerebral artery anastomosis. CONCLUSION: PHACE syndrome should be kept in mind, even in individuals of advanced age, in the instance of a TIA, especially in situations which may involve induced hypoperfusion.

Nocardia paucivorans cerebellar abscess: Surgical and pharmacotherapy.

BACKGROUND: Nocardia species are ubiquitous in nature and mainly cause pulmonary disease in humans; however, they can also infect the central nervous system and skin. The management of cerebellar nocardiosis is troublesome and requires multiple considerations of the severity of the underlying systemic disease, difficulties in identifying the bacterium, and frequent delay in initiating adequate therapy. CASE DESCRIPTION: We report a 52-year-old diabetic female patient with Nocardia paucivorans cerebellar abscesses. Brain magnetic resonance imaging (MRI) revealed innumerable small ring-enhancing lesions of posterior fossa. In this report, we present a case of primary single cerebellar abscesses due to N. paucivorans. Early diagnosis and surgical interventions were significant for the patient. The diagnosis was confirmed by DNA sequencing and the organism was susceptible to trimethoprim-sulfamethoxazole (TMP/SMX). The patient was successfully treated with drugs and surgical excision. CONCLUSION: According to the literature, surgical excision or aspiration of cerebellar abscess seems to provide favorable outcomes. In our experience, a successful outcome was achieved with subtotal resection and prolonged adequate antibiotic therapy.

Deep Venous Thrombosis with Decreased Cerebral Blood Flow to the Thalamus was Completely Restored by Factor Xa Inhibitor.

BACKGROUND: Cerebral venous thrombosis is rare and an uncommon cause of stroke and has diverse etiologies and varied clinical presentations. Here, we report 2 cases of deep cerebral venous thrombosis. CASE DESCRIPTION: A 64-year-old woman presented with cerebral venous thrombosis due to a hypercoagulable state associated with ovarian tumor. On initial fluid-attenuated inversion recovery and diffusion-weighted imaging, there was a diffuse high-intensity lesion in the bilateral thalamus. Computed tomography angiography showed occlusion of the straight sinus, vein of Galen, and internal cerebral vein. Single-photon emission computed tomography showed decreased cerebral blood flow in the bilateral thalamus. After 3 weeks of factor Xa inhibitor therapy, the patient's consciousness gradually improved and eventually became clear enough to leave the hospital. She had no neurological deficit. Another patient was a 47-year-old man who presented with splitting headache and drowsiness. Magnetic resonance venography confirmed deep thrombosis of the vein of Galen. He completely recovered after 4 weeks of factor Xa inhibitor therapy. CONCLUSIONS: This study reports on 2 rare cases of decreased cerebral blood flow in the bilateral thalamus on single-photon emission computed tomography, which improved following the administration of factor Xa inhibitor.

Endoscopic Surgery for Thalamic Hemorrhage with Intraventricular Hemorrhage: Effects of Combining Evacuation of a Thalamic Hematoma to External Ventricular Drainage.

OBJECTIVES: Intraventricular hemorrhage (IVH) caused by thalamic hemorrhage leads to hydrocephalus, increased intracranial pressure, and reduced levels of consciousness. The aim of this study was to investigate the efficacy and compare the results of endoscopic surgery for the evacuation of a thalamic and intraventricular hematoma against those of external ventricular drainage (EVD) surgery. MATERIALS AND METHODS: From January 2010 to December 2018, 68 patients with IVH caused by thalamic hemorrhage were treated in our department. Our study was approved by the Institutional Ethics Committee. The included patients were randomly divided into an EVD group and an endoscopic surgery group. The outcome was measured after 3 months using a 30-day mortality rate, pneumonia onset rate, ventriculoperitoneal (VP) shunt dependency rate, and Glasgow Outcome Scale (GOS) score. RESULTS: Thirty-eight of the 68 patients were randomly assigned to the endoscopic surgery group and 30 were assigned to the EVD group. Patients treated with endoscopic surgery had significantly less drainage dependency on day 30 (P = 0.00014 < 0.00005) in comparison to those treated with EVD. The difference in the functional outcomes between the two groups of patients was mainly dependent on the onset of pneumonia and the consciousness level at the time of admission. The onset rate of aspiration-related pneumonia until day 30 was 11% in the endoscopic surgery group and 45% in the EVD group. The VP shunt rates were 27.8% in the endoscopic surgery group and 60% in the EVD group. The endoscopic surgery group had a significantly lower VP shunt rate compared with the EVD group. Intracerebral hemorrhage evacuation late was found to be associated with shunt-dependent rate and hospitalization. CONCLUSIONS: Endoscopic surgery was found to be associated with a lower GOS score and lower onset rates for shunt-dependent hydrocephalus and aspiration-related pneumonia in comparison to EVD. High evacuation rate was associated with lower shunt-dependent rate and short hospitalization.

Primate neurons show different vulnerability to transient ischemia and response to cathepsin inhibition.

Previously, we reported "calpain-induced leakage of lysosomal enzyme cathepsin" as a mechanism of ischemic neuronal death specific for primates. Cathepsin inhibitors such as CA-074 and E-64c were demonstrated to significantly inhibit hippocampal neuronal death. Pyramidal neurons of the hippocampus, Purkinje cells in the cerebellum, and neurons in the caudate nucleus, outer putamen and cortical III, V layers, are known to be vulnerable to ischemia. However, regional differences of the vulnerability and response to neuroprotectants, have not been studied in detail. Here, the monkey brains undergoing transient ischemia were studied to clarify such regional differences by the microscopic counting of surviving neurons. The dead neurons were characterized by eosinophilic coagulation necrosis without apoptotic bodies. The control postischemic brain without treatment showed surviving neurons in caudate nucleus (55.8%), outer putamen (44.4%), cortical III layer (37.8%), CA4 (35.3%), cortical V layer (34.1%), cerebellum (28.2%), CA3 (24.3%), CA2 (16.2%), and CA1 (2.0%). Only the CA1 showed an almost total neuronal loss. In contrast, a single postictal injection of CA-074 or E-64c led to significant inhibition of postischemic neuronal death in all brain regions studied. Overall, more surviving neurons were seen after E-64c treatment than with CA-074: cerebellum, 91.6% vs 85.6%; CA4, 88.6% vs 77.3%; caudate nucleus, 86.1% vs 89.8%; CA2, 83.6% vs 53.0%; outer putamen, 81.3% vs 87.7%; CA1, 80.1% vs 47.4%; CA3, 79.6% vs 60.3%; cortical layer III, 75.5% vs 67.7%; and cortical layer V, 75.0% vs 65.9%, for E-64c and CA-074, respectively. Cathepsin plays a critical role in ischemic neuronal death, and its inhibitors may protect neurons throughout the brain.