Notes

Anatomical along with well-designed studies identify one particular pathological NFATC1 mutation in the Chinese tricuspid atresia family.
Intraabdominal hemorrhage is a rare complication of subarachnoid hemorrhage(SAH). Recently, there have been a number of reports about the coexistence of intracranial and Intraabdominal aneurysms caused by segmental arterial mediolysis(SAM). The etiology of SAM is still unclear, but catecholamine is known to be one of the causes of SAM. The authors report a rare case in which an increase of catecholamine by SAH affected the Intraabdominal hemorrhage during the perioperative period of SAH. A 67-year-old woman was admitted to our hospital with SAH. Cerebral angiography revealed a right internal carotid-posterior communicating artery aneurysm, and an emergent neck clipping was performed. The intraoperative finding was a saccular aneurysm with a partial red wall of the internal carotid artery. Thirteen days after SAH, the patient complained of abdominal pain, but there were no particular findings on abdominal CT. The next day, anemia progressed suddenly because of Intraabdominal hemorrhage caused by rupture of the splenic artery aneurysm. She developed shock and consciousness disturbance, and left hemiparesis due to vasospasm appeared. She recovered from shock after receiving transfusion and coil embolization of the splenic artery aneurysm. Her consciousness and left hemiparesis improved after two months of rehabilitation. The radiological findings of the splenic artery aneurysm are compatible with SAM. We believe that an increase in catecholamine by SAH affected the development and rupture of the splenic artery aneurysm. In cases of SAH with abdominal pain, a detailed examination of the abdomen might be needed, and the use of catecholamine for symptomatic vasospasm should be carefully performed.We encountered a case of cerebral amyloid angiopathy(CAA)-related cerebral hemorrhage that recurred 20 times within 9 months. The patient was a 68-year-old woman with a history of hypertension, and whose blood pressure was controlled with antihypertensive medicine. She was hospitalized due to cerebral hemorrhage of the right temporal lobe and underwent conservative treatment;however, cerebral hemorrhage of the right frontal lobe recurred on day 60, and 18 cerebral hemorrhages occurred in the following 7 months. Most of the bleeding occurred in the cerebral subcortex, and CAA-related inflammation(CAA-I)had appeared to have developed since subacute progressive cerebral white matter lesions and meningeal contrasting findings were found with MRI. Although cerebral hemorrhage recurrence was not observed for 1 month after steroid pulse therapy, cerebral hemorrhage was subsequently repeated. Thus, in rare cases, CAA-I results in the recurrence of cerebral hemorrhage within a short period of time, and steroids may be effective for preventing this recurrence.Ependymoma is the most common primary intramedullary tumor of the spinal cord, accounting for a quarter of these tumors. We experienced a case of 'ependymoma of the spinal cord with a cystic lesion that presented as an intradural extramedullary tumor extending from the thoracic to lumbar vertebrae. In review of past literature on reports of spinal intradural extramedullary ependymoma, lesions spanning three or more vertebrae with cystic lesions were frequent, and about half were World Health Organization grade II or higher. Dissemination or recurrence of these tumors may occur after surgery. There is no consensus on postoperative adjuvant therapy for intradural extramedullary spinal ependymoma, but careful consideration should be given to the intraoperative findings and the characteristics of the tumor.
Erdheim-Chester disease(ECD)is a rare type of non-Langerhans histiocytosis. We report a surgical case of ECD with multiple lesions at the falx cerebri, tentorium cerebelli, and in the suprasellar region, with a literature review.

A 70-year-old woman presented with frequent falling and difficulty in standing. Her past medical history revealed ovarian cystectomy, transient thrombocytopenia, hypertension, left lower leg pain, and overactive bladder. Her head CT and MRI findings revealed well-defined mass lesions, suspected of meningioma, at the falx cerebri and tentorium cerebelli. Craniotomy and near total resection of the tumor at the falx cerebri was performed, leaving a hard portion of the tumor on the right falx. Intraoperative findings showed a solid and hard tumor, which was extremely difficult to decompress. Although the histopathological diagnosis was originally a metaplastic meningioma, considering her complaints of lower leg pain, we suspected ECD and performed a right tibial biopsy. The right tibial biopsy revealed ECD. Twenty-two months after the operation, the patient exhibited a marked enlargement of the tentorium lesion and a new lesion in the suprasellar region. Resection of the tentorial lesion was performed. The second intraoperative findings were similar to those of the first. The histopathological diagnosis of the tentorial lesion was ECD. After the surgeries, steroid therapy and radiation therapy were performed, but only with temporary improvement.

ECD is a rare disease; therefore, accumulation of clinical data to establish its treatment is necessary.
ECD is a rare disease; therefore, accumulation of clinical data to establish its treatment is necessary.Diffuse leptomeningeal glioneuronal tumor(DLGNT)is a rare primary neoplasm of the central nervous system, and is a condition that is newly listed in the 2016 World Health Organization(WHO)classification of tumors of the central nervous system. We report an adult case of DLGNT that was characteristically merged with subarachnoid hemorrhage. A 46-year-old woman reported persistent dizziness upon walking. MRI of the brain revealed a diffuse, infiltrating lesion with high intensity on FLAIR around the cerebellopontine angle to the lateral ventricle and in the leptomeninges of the spinal cord. The lesion on the cerebellopontine angle showed high intensity on T1 weighted images with contrast enhancement. Since diffuse glioma and meningeal carcinomatosis were suspected, we performed an endoscopic biopsy for the lesion in the right lateral ventricle. Although the tumor was tentatively diagnosed as WHO grade II diffuse astrocytoma, a definitive diagnosis could not be obtained. Selleck TGFbeta inhibitor One month after surgery, the patient presented with acute headache and dizziness.
My Website: https://www.selleckchem.com/TGF-beta.html