Sinus thrombosis in idiopathic hypereosinophilic syndrome causing fatal cerebral haemorrhage.

Idiopathic hypereosinophilic syndrome (HES) is a leukoproliferative disorder that is characterized by sustained overproduction of eosinophils and a trend towards damage to specific organs, usually the cardiovascular system. We report the case of a 76-year-old woman who was affected by idiopathic HES, which had an unusual and rapidly fatal course. Sinus thrombosis in the transverse and sigmoid sinuses was evident on cranial CT and CT angiography. In our review of the English-language literature we were unable to find any previously published direct images of sinus thrombosis in idiopathic HES.

Intracranial peripheral-type primitive neuroectodermal tumor.

A 15-year-old man presented with headache. Magnetic resonance (MR) imaging revealed a large extraaxial tumor with cyst at the right frontotemporal region. The solid part of the tumor was homogeneously enhanced on T(1)-weighted MR imaging after injection of gadolinium. Digital subtraction angiography of the external carotid artery revealed sunburst appearance corresponding to the tumor, which was fed by the right middle meningeal artery. His headache worsened and computed tomography revealed enlargement of the tumor and intracystic hemorrhage, so emergent operation was performed. At surgery, the tumor strongly adhered to the dural membrane, and was obviously extraaxial. The tumor and cyst were gross totally removed. The attachment site at the dura mater was resected. Histological examination showed solid growth of small round cells with uniform round nuclei and minimal cytoplasm. Immunohistochemical staining showed the cells were positive for MIC-2 (CD99). The MIB-1 labeling index was 53%. The histological diagnosis was peripheral-type primitive neuroectodermal tumor (pPNET). Following surgery, radiation therapy and chemotherapy were given. Ewing's sarcoma and pPNET form a family of small round cell tumors arising in the bone or soft tissue. MIC-2 is a useful marker in the differential diagnosis. Good prognosis may be attained if complete surgical excision of intracranial pPNET is achieved.

Ectopic recurrence of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease): a case report.

Lhermitte-Duclos disease is a rare clinical entity characterized by slow deformation of the cerebellar lesion. A 53-year-old woman presented with Lherimitte-Duclos disease manifesting as ataxic gait, occipital headache, and loss of consciousness. Magnetic resonance imaging demonstrated striated and laminar pattern lesions in the right cerebellar hemisphere and vermis. She underwent subtotal removal of the vermis compressing the brainstem, but the cerebellar hemisphere appeared normal and was preserved. Histological findings were consistent with Lherimitte-Duclos disease. Two years later, magnetic resonance imaging revealed enlargement of the right hemispheric lesion. Intraoperative appearance of the hemisphere demonstrated marked enlargement of the folia. Subtotal removal was performed. Histological examination showed no malignant findings. Lhermitte-Duclos disease may recur, and should be carefully followed up as a low-grade neoplasm, not as a hamartomatous disorder.

[Clinical investigation of cerebral infarction following head and neck injury].

The diagnosis of cerebral infarction following head and neck injury is difficult because of the lucid interval, which causes high mortality and morbidity. We report six patients with cerebral infarction following head and neck injury, whom we treated from 1993 to 2005. Only one patient suffered from cerebral infarction following direct neck injury. At the time of injury three patients had prodromal symptoms and the others had no symptoms. Three patients were diagnosed with artery-to-artery embolism based on the dissection of the internal carotid artery in the neck, two patients, from the dissection of the vertebral artery in the neck, and one patient was diagnosed with Wallenberg's syndrome. The overall patient outcomes at discharge as measured by using the Glasgow Outcome Scale were as follows: moderately disabled in one, severely disabled in three, vegetative survival in one, and death in two patients. No patient had been administered a screening test for neck vessel injury, indicating the difficulty in diagnosis. Prodromal symptoms, due to not only direct but also indirect neck injuries indicate the possibility of neck vessels injury, and hence, we should develop a method that enables the early detection of lesions caused by neck vessel injury.

Cavernous sinus dural arteriovenous fistula associated with the development of sphenoidal ridge meningioma--case report.

A 76-year-old man presented with a cavernous sinus (CS) dural arteriovenous fistula (AVF) associated with the development of a meningioma without venous sinus occlusion. Initial digital subtraction angiography did not reveal the CS dural AVF, which appeared simultaneously with the enlargement of the meningioma and lead to right oculomotor nerve paresis. In this case, the development of meningioma possibly increased the vascular tumor bed and affected the venous hemodynamic return, thus leading to the dural AVF.

Postpartum dissecting aneurysm of the posterior cerebral artery.

Intracranial dissecting aneurysm may cause subarachnoid hemorrhage (SAH) or infarction, and postpartum dissecting aneurysm is rare. A 30-year-old 6 days postpartum woman presented with posterior cerebral artery (PCA) dissection evolving dramatically over a short period. She had been well until 6 days after delivery when she suffered sudden onset of headache, vomiting, and unconsciousness. CT scan demonstrated SAH and digital subtraction angiography (DSA) revealed a fusiform dilatation of the left PCA (P3/P4 segment). The initial diagnosis was ruptured dissecting aneurysm, and conservative management was recommended in the acute period. DSA showed smoothening of the vascular wall 6 days after onset, and obliteration of the left P3/P4 segment was observed 13 days after onset. She was discharged without neurological deficits 26 days after onset. Postpartum SAH due to dissecting aneurysm of the PCA is rare, but should be considered in the differential diagnosis of postpartum headache.

[Intracranial osteolytic hemangiopericytoma manifesting as a rapidly enlarging extracranial soft tissue mass lesion].

Hemangiopericytoma is a highly vascular and rapidly growing tumor, which tends to recur at distant locations. Complete surgical resection is often difficult because of intraoperative excessive hemorrhage or brain swelling. A 31-year-old male presented with intracranial osteolytic hemangiopericytoma manifesting as a rapidly enlarging extracranial soft tissue mass lesion causing a subcutaneous mass in the forehead. Computed tomography (CT) and magnetic resonance (MR) imaging demonstrated a lesion in the frontal lobe. Careful follow up was performed because the patient suffered no neurological deficits. The subcutaneous mass rapidly enlarged for 6 months. CT and MR imaging showed an osteolytic intracalvarial lesion with an extradural soft tissue component. Angiography demonstrated obstruction of the superior sagittal sinus and dense tumor staining with slow circulation and venous drainage. Gross total surgical resection was performed following endovascular embolization of the feeding arteries. Histological examination demonstrated an hemangiopericytoma. Although rare, hemangiopericytoma should be included in the differential diagnosis of rapidly enlarging subcutaneous mass. Preoperative endovascular treatment may allow less invasive surgery.

[Retrospective analysis of effects and complications in cases treated with endoscopic evacuation of intracerebral hemorrhage].

We described our experience of 22 cases treated with endoscopic evacuation of intracerebral hematoma, including 16 putaminal, 3 thalamic, and 1 cerebellar hemorrhages. All endoscopic procedures were performed under local anesthesia. A rigid-rod endoscope and a suction device attached was introduced through a transparent sheath into the hematoma cavity. Putaminal hemorrhage was sufficiently evacuated (56-100%), but thalamic hemorrhage was insufficiently removed (less than 40%). In 3 cases, postoperative CT demonstrated enlargement of the hematoma after the endoscopic procedure. In one case, postoperative rebleeding was recognized on a postoperative follow-up CT scan. In 2 cases, uncontrollable arterial bleeding occurred during the endoscopic procedure, followed by craniotomy and removal of the hematoma under a microscope. Endoscopic hematoma evacuation should be an efficient procedure for intracerebral hemorrhage after a secure method of hemostasis during the endoscopic evacuation process has been developed.

[Intracranial granulocytic sarcoma in a patient with acute myeloid leukemia].

Granulocytic sarcoma (GS) is extramedullary tumor composed of immature leukemic cells. GS is presenting usually as a complication during the course of hematologic neoplasm, such as acute myeloblastic leukemia as well as myeloproliferative and myelodysplastic syndrome. The tumor was also called chroloma based on the green color of the tumorous mass. Central nervous system manifestations of GS are extremely rare. We report a 41-year-old man with acute leukemia type M7, who developed GS in the right occipital lobe after complete remission was achieved. Operative findings revealed the color of the hard tumor was greenish, which suggested the tumor was chroloma. Histological findings showed the tumor was GS. The majority of reported cases of GS in acute myeloid leukemia were M2 FAB classification and have chromosome translocation. Our patient was M7 FAB classification, not have specific chromosome translocation. GS occurrence in AML: M7 patient was extremely rare. This is the first case report of AML: M7 with GS in the central nervous system.

Cavernous sinus dural arteriovenous fistula complicated by pontine venous congestion. A case report.

BACKGROUND: Brainstem venous congestion is a rare but serious complication of the CS-dAVF (cavernous sinus dural arteriovenous fistual). CASE DESCRIPTION: A 71-year-old woman presented with right abducens nerve palsy. Brain MRI showed a solitary lesion in the right upper pons. Cerebral angiogram revealed the right CS-dAVF with retrograde venous drainage into the cerebellar cortical veins and the anterior pontomesencephalic vein. The patient was treated with stereotactic radiosurgery, resulting in complete resolution of the pontine lesion and the neurologic symptom. CONCLUSION: A solitary brainstem lesion can be caused by CS-dAVF as a rare complication. Careful diagnosis and treatment are important to avoid permanent neurologic deficits.

[A case of secondary anterior fossa dural arteriovenous fistula following posterior fossa craniotomy].

The etiology of dural arteriovenous fistula (AVF) remains uncertain. The authors present a case presenting with anterior fossa dural AVF, which developed after the resection of cerebellar arteriovenous malformation (AVM). The anterior fossa dural AVF, which was not observed on the preoperative angiography, was diagnosed on the follow-up angiography, after the resection of cerebellar AVM. A mechanism underlying the present case is not clearly understood. To the best of our knowledge, some cases of dural AVF secondary to craniotomy have been reported. It is postulated that intracranial vascular anomaly should be analyzed after the surgery.

[Nontraumatic arterial dissection of the anterior cerebral artery: six cases report].

Nontraumatic arterial dissection of the anterior cerebral artery (NAD-ACA) is a relatively rare disease entity, although case reports have recently been increased. We treated 6 patients suffering from NAD-ACA from January 1996 to December 2003, and the neuroradiological findings together with the clinical courses were reviewed. There were 3 males and 3 females with a mean age of 57.7-year-old, ranging from 41 to 65. Five patients had a past history of hypertension and one diabetes mellitus. At the onset, all patients presented with clinical manifestations of cerebral ischemia. Among them, all exhibited contralateral hemiparesis with greater weakness of the lower extremity, and two patients exhibited headache. Initial angiography revealed the pearl and string sign in four patients and string sign, tapered occlusion in each one. Follow-up angiographies revealed sequential changes in all patients; four improved and two progressed. Main anatomic site of the lesion was as follows; five in the A2 and one in the A1 portion, in addition, one patient was complicated by saccular aneurysm, one by PCA dissection, and two had with saccular aneurysm contralateral ACA & MCA and VA dissection each other. Four patients were treated conservatively by intravenous administration of argatroban, one by intravenous administration of Dextrane and one by anti-platelet agent in the acute stage. All patients were treated by anti-platelet agents in the chronic stage. Good recovery was achieved in five patients, but one who suffered from severe subarachnoid hemorrhage in the chronic stage died. Our experience suggests that hypertension and/or the succeeding abnormal structural changes in the arterial wall may contribute to the occurrence of this disease. NAD-ACA showing clinical manifestations of cerebral ischemia could result in a relatively good prognosis; however, attention should be paid to patients treated conservatively with a very closed follow-up angiography to prevent a possibility of severe hemorrhage.

Malignant intracranial epithelioid hemangioendothelioma presumably originating from the lung: case report.

OBJECTIVE AND IMPORTANCE: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that presents histological features and biological behavior of low-grade malignancy. The authors report a case of malignant intracranial EHE, in which surgical excisions and additional immuno-chemotherapy were ineffective. Emphasis is placed on the histological features of this rare tumor and its potential for malignancy. CLINICAL PRESENTATION: A 69-year old male presented with paresis of the right arm. Magnetic resonance imaging revealed intracranial multiple lesions in the right temporal and parietal and left frontal lobes. Chest radiography revealed a mass lesion occupying the lower lobe of the left lung that had been followed as old tuberculosis since it had not changed in size for as long as 5 years. INTERVENTION: We performed craniotomy and excised tumors. Histological examination demonstrated that the excised tumors had the features of EHE. Further intracranial recurrence after the excision of the tumors necessitated adjuvant treatment with interferon alpha-2b. However, the patient continued to deteriorate and died 3 months later. Postmortem study disclosed the tumor cells existing in the left lung, pleura, ribs, intercostal tissues and diaphragm. Other organs including liver, bone, and skin were free from tumor invasion, which indicated that the malignant EHE originated from the left lung and metastasized to the brain. CONCLUSION: To the authors knowledge, no case of malignant EHE that simultaneously involves the central nervous system and the lung has been previously reported. An extensive inspection for involvement of other organs is recommended after recognizing an intracranial EHE.

[A case of peripheral-type primitive neuroectodermal tumor arising in the dura mater at the frontal base].

A 7-year-old boy was admitted to our hospital because of headache and frequent vomiting. The patient was noted to have papilloedema and mild palsy of the right abducent nerve. Magnetic resonance image (MRI) revealed a large tumor in the frontal base with tumoral hemorrhage. Angiography showed the tumor was fed by anterior meningeal arteries. At surgery, the tumor was arising in the dura mater at the frontal base, and was removed totally. Histological examination showed the tumor to be composed of small cells with uniform round nuclei and minimal cytoplasm. Immunohistochemical studies were positive for MIC-2, NSE, C-KIT, vimentin, Class III-beta tublin and glycogen, but negative for NFP, synaptophysin, chromogranin A and GFAP. MIB-1 labeling index was 40-50%. The tumor was histologically confirmed to be peripheral-type primitive neuroectodermal tumor(pPNET). Following surgery, he underwent whole brain, whole spine and local radiation therapy(30 Gy in total respectively) and received two 5-day cycles of chemotherapy, consisting of intravenous administration of cisplatin 20 mg/m2/day, etoposide 60 mg/m2/day and IFOS 900 mg/m2/day. After these therapies, follow-up radiological examination showed there was no recurrence of the tumor for 24 months. Intracranial pPNET is rare. Ewing sarcoma and pPNET(ES/pPNET) is the designation given to a family of small round cell tumor arising in bone or soft tissues. Intracranial PNETs are devided into central nervous system PNET(cPNET) and pPNET. It is necessary that intracranial PNETs are divided into two types of PNETs because of different prognosis between these tumors. MIC-2 is a specific marker for pPNET/ES family and is useful in the differential diagnosis of these two types of tumors.

[Recurrent cerebellar abscess secondary to middle ear cholesteatoma: case report].

We report a case of recurrent cerebellar abscess secondary to middle ear cholesteatoma. A 57-year-old man was admitted to our hospital because of symptoms of headache and nausea in August, 1992. Brain CT scans revealed acute hydrocephalus complicated by a cerebellar abscess. The patient was discharged without any neurological deterioration after systemic antibiotics combined with intrathecal aminoglucoside administration via ventricular drainage. Mannitol was also administrated for 7 days immediately after the patient's admission. The clinical course was uneventful for 8 years afterwards. Follow-up MR images revealed no signs of recurrence. Unfortunately, the patient suffered a recurrence of cerebellar abscess in October, 2000. His condition continued to deteriorate in spite of being treated by systemic antibiototics. MR images and CT scans targeting a portion of his middle ear revealed extensive pus-coated mastoiditis and middle ear cholesteatoma. We thus performed radical mastoidectomy including removal of the middle ear cholesteatoma. After the operation, the cerebellar abscess was ameliorated. He has been free from recurrence for 2 years, so far. Early diagnosis and prompt intervention are necessary for reducing mortality and morbidity rates due to otogenic brain abscess. Recognizing middle ear cholesteatoma as one of the major causes of neurological entities in the cerebellopontine angle portion, accurate otological examination and prompt treatment can possibly bring about a better prognosis.