Endoscopic Management for Recurrent Hydrocephalus Associated with Neurosarcoidosis.

BACKGROUND: Recurrent hydrocephalus may occur as a complication of neurosarcoidosis with chronic inflammation. We present a case that required a combination of multistage endoscopic diversion of the cerebrospinal fluid pathway and shunt surgery. CASE DESCRIPTION: A 34-year-old man presented with progressive nausea and vomiting. Magnetic resonance imaging revealed hydrocephalus with leptomeningeal enhancement along the base of the fourth ventricle and the bilateral foramina of Luschka. Concurrent endoscopic third ventriculostomy and biopsy were performed. The diagnosis was neurosarcoidosis. Immediately after the procedure, the endoscopic third ventriculostomy stoma was occluded, and a right ventriculoperitoneal shunt was urgently performed. However, left unilateral hydrocephalus developed during the late phase of immunosuppressive therapy for neurosarcoidosis. Endoscopic septostomy with repositioning of the ventricular catheter was indicated. Intraoperative findings included a white pasty tissue with nodules that covered the ventricular wall close to the foramen of Monro and sealed the side holes of the catheter. Chemotherapy with a tumor necrosis factor-α inhibitor was initiated after the surgical procedure. The patient had an uneventful course without recurrence of hydrocephalus for >6 months. CONCLUSIONS: Endoscopic diversion of the cerebrospinal fluid pathway should be actively considered for treating hydrocephalus without a shunt and performing biopsy simultaneously. Even if a subsequent shunt is needed, complex hydrocephalus can be avoided with a combination of endoscopic techniques.

[Repeated Rupture of Bilateral Internal Carotid Artery Aneurysms in a Short Period in Association with Polyarteritis Nodosa:A Case Report].

Cerebral involvement is rare in polyarteritis nodosa(PAN);furthermore, secondary intracranial hemorrhage due to cerebral aneurysm is extremely rare. We describe an unusual case of repeated subarachnoid hemorrhage(SAH)in a 64-year-old woman with a history of PAN. Initially, she developed severe headache(probable first SAH, day 0), and presented at our hospital with second severe headache with disturbed consciousness on day 6. Computed tomography(CT)revealed that SAH was mainly distributed in the right basal cistern and sylvian fissure(second SAH). Three aneurysms were detected using CT angiography on the bilateral internal carotid arteries. An intentionally delayed surgery was planned because of the high risk period of cerebral vasospasm and takotsubo cardiomyopathy. On day 15, she complained of headache and had a convulsion. CT revealed a third SAH in the left sylvian fissure;cerebral angiography revealed enlargement of the left internal carotid-posterior communicating artery(IC-PC)aneurysm. Coil embolization of the aneurysm was performed on day 16, and she was treated using prednisolone(20mg/day)for PAN. However, on day 20, the patient became comatose, and CT revealed a fourth SAH in the right sylvian fissure. Cerebral angiography revealed enlargement of the right IC-PC aneurysm. Clipping of the aneurysm was successfully performed in spite of ventricular dysfunction, and the dose of prednisolone was increased to 40mg/day. After treatment, the ventricular dysfunction gradually resolved. Cerebral aneurysms with PAN are candidates for intervention because of their strong tendency to rupture. In our case, takotsubo cardiomyopathy might have been associated with impairment of the coronary microcirculation due to PAN. We suggest that aggressive immunosuppressive treatment for PAN and curative treatments for cerebral aneurysms should be considered with careful radiological examination and follow-up monitoring.

Onyx removal after embolization of a superior sagittal sinus dural arteriovenous fistula involving scalp artery.

BACKGROUND: Most dural arteriovenous fistula (DAVF) in superior sagittal sinus (SSS) requires multimodal treatment. Onyx embolization is useful for DAVF; however, scalp artery embolization has cast extrusion risk. CASE DESCRIPTION: A 59-year-old male presented with involuntary movements of both legs and progressive dementia. Cerebral angiography demonstrated the DAVF in the SSS fed by bilateral superficial temporal, occipital, and middle meningeal arteries. The posterior SSS was thrombosed, and the main drainers were cortical veins. Combined treatment with transarterial embolization using Onyx and transvenous embolization using coils was performed. Although symptoms were improved, a small DAVF remained. Two months later, Onyx cast extrusion through the scalp was observed, requiring removal and debridement because of infection at the extrusion sites. Surgery for the residual DAVF would be difficult because of scalp condition; therefore, an additional endovascular treatment was conducted, completely occluding DAVF. CONCLUSION: Onyx embolization is useful for DAVF; however, scalp artery embolization has cast extrusion risk. Therefore, scalp infection should be considered because it may preclude additional surgical procedures.

Transient disappearance of microbleeds in the subacute period based on T2*-weighted gradient echo imaging in traumatic brain injury.

We report three cases of traumatic microbleeds evaluated by sequential observation. Hypo-intensities on T2* gradient echo imaging (T2*GEI) appeared just 2-3 h after the injury (the hyper-acute period). However, these hypo-intensities on T2*GEI disappeared or became obscure 2-6 days after the injury (the subacute period). A follow-up MRI again revealed clear hypo-intensities on T2*GEI 1-3 months after the injury (the chronic period). Our cases indicate that hypo-intensities on T2*GEI might change dynamically from the hyper-acute to the chronic period. The differences of susceptibility effects by hematoma age might be the cause of this dynamic change.

Postprandial Hypotension and Coma Following Subarachnoid Hemorrhage in a Patient with Parkinson's Disease.

A 79-year-old woman with a history of Parkinson's disease was admitted to our hospital because of a subarachnoid hemorrhage. She underwent clipping the next day. On postoperative days 7-9, she exhibited hypotension and disturbance of consciousness after each meal. The administration of midodrine relieved the hypotension, and postprandial coma was no longer observed. In this case, the autonomic dysfunction in Parkinson's disease and impairment of cerebral autoregulation during cerebral vasospasm may have been involved in the postprandial hypotension (PPH) and coma. PPH occurs not only in patients with Parkinson's disease but also in elderly patients, particularly those with diabetes or hypertension. Therefore, PPH must be considered in the management of cerebral vasospasm following subarachnoid hemorrhage.

[Progressive Intracranial Hypertension due to Superior Sagittal Sinus Thrombosis Following Mild Head Trauma: A Case Report].

Cerebral venous sinus thrombosis after mild head trauma without skull fracture or intracranial hematoma is exceptionally rare. We describe an unusual case of progressive intracranial hypertension due to superior sagittal sinus thrombosis following mild head trauma. A 17-year-old boy presented with nape pain a day after a head blow during a gymnastics competition (backward double somersault). On admission, he showed no neurological deficit. CT scans revealed no skull fractures, and there were no abnormalities in the brain parenchyma. However, his headache worsened day-by-day and he had begun to vomit. Lumbar puncture was performed on Day 6, and the opening pressure was 40 cm of water. After tapping 20 mL, he felt better and the headache diminished for a few hours. MR venography performed on Day 8 revealed severe flow disturbance in the posterior third of the superior sagittal sinus with multiple venous collaterals. Because of the beneficial effects of lumbar puncture, we decided to manage his symptoms of intracranial hypertension conservatively with repeated lumbar puncture and administration of glycerol. After 7 days of conservative treatment, his symptoms resolved completely, and he was discharged from the hospital. Follow-up MR venography performed on Day 55 showed complete recanalization of the superior sagittal sinus. The exact mechanism of sinus thrombosis in this case is not clear, but we speculate that endothelial damage caused by shearing stress because of strong rotational acceleration or direct impact to the superior sagittal sinus wall may have initiated thrombus formation.

Surgical Treatment of a Dural Arteriovenous Fistula in the Sphenoid Wing with a Unique Drainage Pattern through the Basal Vein of Rosenthal.

A 69-year-old man consulted our department regarding further examination because abnormal venous ectasia of the basal vein of Rosenthal (BVR) was accidentally found on magnetic resonance imaging. Angiography revealed a dural arteriovenous fistula (DAVF) in the left sphenoid wing; the fistula was supplied by the ophthalmic artery and the middle meningeal artery. Venous drainage from the fistula consisted of a large collector vein located in the temporal tip, which drained into the BVR. We selected open surgery because this lesion may be difficult to access and is associated with significant risks through an endovascular approach. After temporal clip ligation of the drainer, the superficial middle cerebral vein became the main drainage route directly in connection with the fistula. Therefore, both were ligated, after coagulation of feeding arterial networks on the dura around the sphenoid wing. The patient experienced no complications from the surgical procedure, and postoperative angiography demonstrated obliteration of the fistula. DAVF in the sphenoid wing with deep drainage is believed to carry a high risk of hemorrhage or venous infarction because of the presence of Galenic drainage, varix, and cortical venous reflux. Treatment is strongly recommended even if the symptoms are minimal. Open surgery appears to be safe and often the best therapeutic option.

Contralateral hearing loss after acoustic neuroma surgery.

A 74-year-old man suffered contralateral hearing loss after left acoustic neuroma surgery. Steroid therapy was administered, but no improvement was observed. Contralateral hearing loss is an extremely rare and distressing complication that can occur following acoustic neuroma surgery. Although the mechanism of this rare phenomenon remains unclear, we speculate that in this patient the loss of cerebrospinal fluid or internal auditory artery thrombosis may be involved.

[Pure motor monoparesis of a lower limb due to head injury: a case report].

A 70-year-old woman sustained a head injury after a motor vehicle accident. Physical examination conducted on admission revealed pure motor monoparesis (PMM) and pathological reflexes in the right lower extremity. Her left lower extremity and upper extremities were intact. Computed tomography showed a spotty high-density lesion in the left precentral gyrus and a subgaleal hematoma in the left occipital region. Magnetic resonance imaging was performed on the next day. Fluid-attenuated inversion recovery (FLAIR) imaging demonstrated a high-intensity lesion in the left precentral gyrus, and T2 imaging revealed a low-intensity lesion suggesting a small hemorrhage in the same area. The small hemorrhage and perifocal edema were identified on diffusion-weighted images in which low- and high-intensity lesions were observed in the anterior and posterior left precentral gyrus, respectively. Subsequent neurological examinations over 2 weeks showed improvement. We discuss the clinical presentation, diagnosis, and treatment of PMM due to head injury. We concluded that FLAIR and T2 and diffusion-weighted imaging may be useful techniques for diagnosing PMM due to head injury.

Hyperperfusion syndrome after neck clipping of a ruptured aneurysm on a dolichoectatic middle cerebral artery.

A 40-year-old female presented with sudden onset of severe headache and vomiting due to subarachnoid hemorrhage. Angiography demonstrated a saccular aneurysm on a dolichoectatic left middle cerebral artery (MCA) and delayed filling of the MCA. Magnetic resonance imaging showed a partially thrombosed giant aneurysm on the dolichoectatic MCA. An intentionally delayed operation was performed, during which the neck of the aneurysm was successfully clipped. The patient exhibited aphasia 48 hours after surgery. Single-photon emission computed tomography revealed hyperperfusion in the territory of the left MCA. The patient's blood pressure was maintained normotensively, and her symptoms gradually improved. She returned to work 1 month after surgery. The saccular aneurysm was formed on the dolichoectatic MCA, presumably due to an abnormal arterial wall and hemodynamic stress. The preoperative hypoperfusion might have been caused not only by the giant aneurysm, but also to some degree by the dolichoectatic MCA. After neck clipping, the increase in blood flow might have caused hyperperfusion.

[Case of infected subdural hematoma diagnosed by diffusion-weighted imaging].

An 80-year-old man who had undergone total gastrectomy and splenectomy for gastric cancer 13 years ago presented with headache, drowsiness, and high fever 1 month after a traffic accident. Brain CT scans revealed bilateral subdural fluid collections. Diffusion-weighted imaging (DWI) showed mixed high and low signal intensities in the left subdural fluid, and contrast-enhanced MR imaging revealed capsule enhancement of the left subdural fluid collection. The patient was diagnosed with left subdural empyema, and 2 burr-holes were drilled for drainage and irrigation. Operative findings revealed a neomembrane underneath the dura mater. Old hematoma and yellowish-white purulent fluid were present within the neomembrane. This confirmed the diagnosis of infected subdural hematoma (ISH). Abscess culture results were positive for Escherichia coli. The patient's symptoms resolved postoperatively with subsequent antibiotic therapy. However, 4 months after the operation, he suddenly died of severe sepsis and disseminated intravascular coagulation following cholecystitis, which was possibly associated with splenectomy. The clinical presentation, diagnosis, and treatment of an unusual case of ISH have been discussed. We emphasize that DWI and enhanced MR imaging may be useful for diagnosing ISH, and serial DWI evaluations may help in monitoring the therapeutic response in ISH.

Primary cystic germinoma originating from the midbrain.

A primary intracranial germinoma that involves the midbrain is rare. We describe an unusual case of primary cystic germinoma originating from the midbrain. A 29-year-old man presented with diplopia, and his MRI showed a cystic, ring-like enhanced lesion in the thalamo-mesencephalic junction. Open biopsy was performed and the diagnosis of germinoma was based upon the histopathological findings. Following chemotherapy and radiotherapy, the symptoms improved and the tumor disappeared. We propose that primary intracranial germinoma should be included in the differential diagnosis of midbrain tumors, because early diagnosis and appropriate treatment for midbrain germinoma improves clinical outcome.

Prolonged somatic survival of clinically brain-dead adult patient.

A 43-year-old woman suffered clinical brain death after severe head injury. The patient met the criteria for the diagnosis of clinical brain death on Day 3. Aggressive hemodynamic and respiratory managements coupled with triple hormone therapy were performed at the family's request, resulting in continued cardiac activity for a prolonged period. Spinal reflexes and automatisms were observed until cardiac arrest. Ventilatory support was discontinued on Day 168, when cardiac death was confirmed, and her kidneys and eyeballs were removed for transplantation. The patient survived for 165 days after the diagnosis of clinical brain death, which is an extremely prolonged period of somatic support for an adult patient after brain death. An extensive and informed discussion on the end-of-life treatment of clinically brain-dead patients is urgently required in Japan to establish treatment guidelines for such patients.

Meningeal hemangiopericytoma manifesting as massive intracranial hemorrhage--two case reports.

Meningeal hemangiopericytoma is rare, and only seven cases have manifested as intracranial hemorrhage. We treated two patients with meningeal hemangiopericytoma manifesting as life-threatening massive intracerebral hemorrhage. Case 1: A 66-year-old woman presented with consciousness disturbance and left hemiparesis. Computed tomography showed a mass lesion in the right frontal parasagittal region and massive hematoma in the brain tissue at the medial border of the lesion. Immediate evacuation of the hematoma was performed. Postoperative magnetic resonance imaging revealed a well-enhanced mass lesion with small intratumoral hemorrhage. The tumor was removed totally through a bilateral frontal craniotomy. The postoperative course was uneventful. The histological diagnosis was meningeal hemangiopericytoma. Radiotherapy was not performed. Case 2: A 59-year-old man presented with consciousness disturbance. Computed tomography showed a mass lesion in the right frontotemporal convexity region and massive hematoma in the brain tissue at the medial border of the lesion. His neurological condition was refractory to any treatment and the clinical diagnosis of brain death was confirmed. Autopsy was performed and the histological diagnosis was meningeal hemangiopericytoma. Meningeal hemangiopericytoma manifesting as intracranial hemorrhage is quite rare, but carries the risk of life-threatening massive bleeding from the tumor.

[A case of cystic cavernous angioma accompanied by a fluid-fluid level on magnetic resonance imaging].

We describe the case of a patient with cavernous angioma (CA). A 44-year-old woman complained of numbness on the left side of the body as an initial symptom of the disease. The initial magnetic resonance (MR) imaging revealed a cystic mass with a fluid-fluid level without perifocal edema in the right thalamus on the T 2-weighted image (T 2WI) and T2*-weighted image (T2*WI). Her symptoms were self-controllable; therefore we decided to observe her natural course only with serial MR imaging. The cystic mass was not enhanced by gadolinium on T1-weighted images, although, we suspected the tumor was complicated by vascular malformation. Therefore, we performed cranial angiography to eliminate the possibility of bleeding from the vascular malformation. Angiography did not demonstrate tumor staining nor vascular malformation. Longitudinally, the tumor demonstrated mosaic signal intensities on each sequence with perifocal edema. Moreover, the tumor exhibited hypointensities on T2* WIs without perifocal edema. The natural history of the tumor on MR imaging exhibited a typical case of CA. Some previous reports described cystic CA with perifocal edema and vascular malformation. In our present case, we clinically diagnosed CA on the basis of the final MR imaging together with previous reports. An intra-axial fluid-fluid level is a very rare finding of MR imaging. Here, we report the case of a patient with cystic CA accompanied by a fluid-fluid level. This finding is not a pathognomonic sign of CA; although, we consider that it is very important to follow up carefully the natural history of such cases.