A case of subarachnoid hemorrhage caused by multiple cerebral aneurysms due to segmental arterial mediolysis.

Background: Segmental arterial mediolysis (SAM) is a condition in which an aneurysm is formed by causing lysis of the media and remodeling of blood vessels. Short-term recurrence has been reported in abdominal aortic aneurysms. Cerebral aneurysms have been suggested to form in a short period not only in the abdominal cavity but also in the intracranial arteries in SAM. Case Description: A 36-year-old pregnant woman at 35 weeks' gestation developed sudden headache and disorientation. Head magnetic resonance imaging showed a small amount of subarachnoid hemorrhage in the right ambient cistern. A fusiform cerebral aneurysm was found in the periphery of the right superior cerebellar artery, and small saccular aneurysms were found in the periphery of the right posterior cerebral artery and left posterior inferior cerebral artery. After delivery of the fetus, endovascular embolization of the ruptured aneurysm was performed. However, 10-week postoperatively, she developed sudden headache. Hemorrhage was found in the fourth ventricle, and enlargement of the left posterior inferior cerebellar artery (PICA) peripheral aneurysm and disappearance of the right posterior cerebral artery peripheral aneurysm were confirmed. A ruptured aneurysm in the peripheral left PICA was removed after trapping. Intraoperatively, an unruptured thrombosed aneurysm that was not visualized by imaging was also removed. Histopathological examination showed no calcification or inflammation, rupture of the internal elastic lamina, and lack of segmentation, and SAM was diagnosed. Conclusion: In atypical dissecting aneurysms, SAM should be considered as a differential diagnosis. Systemic examination and short-term follow-up are also necessary.

Neurosurgical Pathology and Management of Limited Dorsal Myeloschisis Associated with Congenital Dermal Sinus in Infancy.

BACKGROUND: Because of the shared origin of limited dorsal myeloschisis (LDM) and congenital dermal sinus (CDS), CDS elements may be found within the fibroneural LDM stalk. When part of the CDS invested in the intradural stalk is left during untethering surgery, inclusion tumors such as dermoid cysts may develop. However, the most appropriate surgical strategy for LDM with CDS is still under debate. METHODS: Of 19 patients with LDM, 3 (15.8%) had histologically verified CDS elements. We retrospectively analyzed the clinicopathological findings of these patients. RESULTS: In patient 1, the entire stalk including a tiny dermoid cyst at the intradural stalk could be resected through two-level laminectomy during untethering at 6 months of age. In patients 2 and 3, the stalk appeared to be a typical LDM stalk during the initial surgery at 18 and 7 days, respectively; however, CDS was histologically diagnosed in the proximal severed end of the stalk. Postoperative three-dimensional heavily T2-weighted imaging demonstrated spherical enlargement of the remnant stalk, and the entire length of the remnant stalk including newly developed dermoid was resected during the second surgery at 3 years 11 months and 11 months, respectively. Histopathologically, glial fibrillary acidic protein-immunopositive neuroglial tissues and CDS elements were mainly located at the proximal and distal sites of the stalk, respectively, supporting the "dragging down and pulling up" theory. In patients 2 and 3, however, the proximal head of the dermoid cyst passed the distal head of the neuroglial tissues and located at the stalk-cord attachment. CONCLUSION: Surgeons should be aware of the approximately 10% possibility of the coexistence of CDS when managing infant LDM. However, the recommendation for excision of the entire length of the LDM stalk in all patients should be more carefully made because such a strategy may result in an unnecessary extent of laminotomy/laminectomy for most patients with pure LDM. However, once the postoperative histological examination reveals coexistence of CDS in the resected proximal part of the stalk, the entire length of the remnant stalk should be excised as soon as possible.

Palliative lumboperitoneal shunt for leptomeningeal metastasis-related hydrocephalus: A case series.

BACKGROUND: Leptomeningeal metastasis-related hydrocephalus causes distress to patients with end-stage cancer through headache and other symptoms by elevating intracranial pressure, thus reducing quality of life. Ventriculoperitoneal shunt has been used as a treatment option in palliative care. We review four cases of patients who underwent lumboperitoneal shunt for leptomeningeal metastasis-related hydrocephalus. CASES: All patients suffered from severe headache and nausea. The primary lesion was histologically diagnosed as lung adenocarcinoma in each case. The duration from diagnosis to onset of hydrocephalus symptoms ranged from 0 to 52 (mean 26) months. Cerebrospinal fluid pressure in every case was above the normal range due to high intracranial pressure. Case management: Conventional procedures for lumboperitoneal shunt were employed for all patients. Adjustable pressure valves were retrofitted into the shunt system. Case outcome: Three patients demonstrated significant improvement of clinical symptoms and quality of life after placement of lumboperitoneal shunts. In two cases, not only did performance status improve to independent daily activity but also comparatively long-term survival was achieved due to subsequent chemotherapies after surgery. No symptoms of peritoneal dissemination by floating cancer cells in cerebrospinal fluid were seen in any patients. CONCLUSION: Lumboperitoneal shunt appears to improve quality of life if the patient is suffering from symptoms of leptomeningeal metastasis-related hydrocephalus. Compared to ventriculoperitoneal shunt, lumboperitoneal shunt is less invasive and simpler, providing a suitable option for frail patients with end-stage cancer. Adjustable pressure shunt valves can cope with varying symptoms and ventricle sizes.

Intratumoral hemorrhage in a patient with malignant meningioma under anticoagulant therapy.

We report the case of an elderly woman with malignant meningioma and atrial fibrillation who started taking anticoagulants after an ischemic stroke and subsequently developed intratumoral hemorrhage. Further studies are required to confirm whether a particular anticoagulant agent is suitable for patients with brain tumor and atrial fibrillation.

[A case of lumboperitoneal shunt as an effective palliative tool in a patient with leptomeningeal metastasis].

A 64 year-old woman with a diagnosis of lung adenocarcinoma was admitted to our neurosurgical division in February, 2007, suffering from severe headache and dizziness. Systemic chemotherapy had been repeated for multiple metastases to the bone and cerebral cortex since 7 months before. Reexamination with MRI revealed mild hydrocephalus without cortical metastasis. Cytological analysis of the cerebrospinal fluid (CSF) provided the diagnosis of leptomeningeal metastasis. Removal of 8 ml of CSF dramatically alleviated the patient's symptoms. To improve the quality of her remaining life, she underwent lumboperitoneal (L-P) shunt using a Strata adjustable pressure valve. Severe headache disappeared and other symptoms gradually improved after the operation. She survived for 10 months after the shunt placement, perticipating in family life for 6 months. Pressure level of the Strata valve was changed twice according to the degree of hydrocephalus and functioned well while her life lasted. The present case showed that L-P shunt is one of the effective palliative procedures and an adjustable pressure valve is available for this kind of CSF shunt in patients with leptomeningeal metastasis.

CHOP is involved in neuronal apoptosis induced by neurotrophic factor deprivation.

Neurotrophic factors are essential for the survival of neurons. We found that the endoplasmic reticulum (ER) stress-C/EBP homologues protein (CHOP) pathway to be activated during neurotrophic factor deprivation-induced apoptosis in PC12 neuronal cells and in primary cultured neurons, and this apoptosis was suppressed in the neurons from chop(-/-) mice. In addition, we found that CHOP is expressed in the subventricular zone (SVZ) and striatum of the young adult mouse brain. The number of apoptotic cells in the SVZ decreased in chop(-/-) mice. These results indicate that the ER stress-CHOP pathway plays a role in neuronal apoptosis during the development of the brain.