A cerebral neuroblastoma with extracranial metastases.

A rare case of cerebral neuroblastoma with extracranial metastases is reported. This patient was followed for over ten years. The biopsy specimen of the brain tumor taken at the first operation revealed the architecture of a poorly differentiated ependymoma having perivascular rosettes. The histological pattern of the second biopsy taken eight years after the first operation was highly cellular and vascular with abundant mitoses, showing Homer-Wright rosettes. The histological diagnosis of this second specimen was a cerebral neuroblastoma. The problem of the multipotentiality of the brain tumor is discussed.

Spontaneous subperiosteal hematoma of the orbit--case report.

A 62-year-old female was admitted with complaints of sudden proptosis of the left eye and severe left orbital pain without history of trauma or previous illness. Computed tomography and magnetic resonance imaging demonstrated a biconvex extraconal hematoma in the upper part of the left orbit. Surgical exploration of the orbit revealed the presence of a subperiosteal hematoma without a causative lesion. Such spontaneous subperiosteal hematoma of the orbit is extremely rare, but may result from an occult vascular disorder.

Study on cases with posterior fossa epidural hematoma--clinical features and indications for operation.

In this retrospective study, 11 cases of posterior fossa epidural hematoma were analyzed in terms of clinical and radiological features, treatment, and outcome. Posterior fossa epidural hematomas accounted for 11.8% of all epidural hematomas encountered during the 7-year period studied. There were eight males and three females ranging in age from 2 to 53 years (mean, 20.7 years). Glasgow Coma Scale scores on admission were relatively good in many cases. Headache and/or vomiting were common symptoms on admission, whereas cerebellar signs were rare. As all 11 cases involved trauma, occipital fractures were present in eight (72.7%). Six patients underwent surgery. The indications for surgery, in terms of computed tomography findings, were: 1) the maximum thickness of the epidural hematoma was more than 15 mm; 2) the posterior fossa cisterns (e.g., the quadrigeminal and ambient cisterns) were poorly visualized; 3) there was marked deformity and/or displacement of the fourth ventricle; and 4) the hematoma extended to the supratentorial region and severely compressed the brain. At discharge, eight patients showed good recovery and one was moderately disabled. Two patients died. The prognosis for posterior fossa epidural hematoma appears relatively good, if it is not accompanied by severe primary brainstem injury and is diagnosed early, and appropriately and promptly treated.

Monostotic fibrous dysplasia of the left parietal bone--case report.

A 14-year-old female presented with a hard, painless mass, 5 x 5 cm, in the left parietal region. Skull x-rays showed a radiolucent skull tumor with a sclerotic margin in the parietal region. Computed tomography revealed an intradiploic multilocular mass separated by bony trabeculae. The outer table had thinned and protruded outward. The inner table was also thin and protruded inward slightly. External carotid angiography revealed a faint tumor stain and feeding from the middle meningeal artery. Bone scintigraphy revealed abnormal uptake in the lesion. Total removal of the skull tumor and cranioplasty were performed. The histological diagnosis was fibrous dysplasia. Fibrous dysplasia within the cranial vault is often expressed as painless bulging without neurological symptoms. Surgery is recommended when neurological symptoms and/or cosmetic problems are present. Histological confirmation of the diagnosis is also important.

[Peritumoral hemorrhage after radiosurgery for metastatic brain tumor: a case report].

An unusual case of peritumoral hemorrhage after radiosurgery for the treatment of metastatic brain tumor is reported. This 64-year-old woman had a history of breast cancer and underwent right mastectomy in 1989. She remained well until January 1993, when she started to have headache, nausea and speech disturbance, and was hospitalized on February 25, 1993. Neurological examination disclosed right hemiparesis and bilateral papilledema. CT scan and MR imaging showed a solitary round mass lesion in the left basal ganglia region. It was a well-demarcated, highly enhanced mass, 37mm in diameter. Cerebral angiography confirmed a highly vascular mass lesion in the same location. She was treated with radiosurgery on March 8 (maximum dose was 20Gy in the center and 10Gy in the peripheral part of the tumor). After radiosurgery, she had an uneventful course and clinical and radiosurgical improvement could be detected. Her neurological symptoms and signs gradually improved and reduction of the tumor size and perifocal edema could be seen one month after radiosurgery. However, 6 weeks after radiosurgery, she suddenly developed semicoma and right hemiplegia. CT scan disclosed a massive peritumoral hemorrhage. Then, emergency craniotomy, evacuation of the hematoma and total removal of the tumor were performed on April 24. Histopathological diagnosis was adenocarcinoma. It was the same finding as that of the previous breast cancer. Histopathological examination revealed necrosis without tumor cells in the center and residual tumor cells in the peripheral part of the tumor.(ABSTRACT TRUNCATED AT 250 WORDS)

[A case of cervical subpial lipoma].

The authors report the rare case of a 48-year-old woman with a cervical subpial lipoma unassociated with spinal dysraphism. Her symptoms were progressive weakness and numbness in the four extremities and the neurological symptom of mild cervical myelopathy. Plain radiographs of the cervical spine showed diffuse widening of the spinal canal, which was occupied by a large low-density mass revealed by axial CT scan. MR imaging identified the mass as being dorsolateral to the cord in the intradural region. It was hyperintense on both T1- and T2-weighted images. Axial images with fat suppression sequence clearly showed dorsal nerve roots traveling through the lesions, but the interface between the spinal cord and lesion was not well demarcated. A cervical laminectomy between C3-6 revealed a yellowish subpial mass after the dura was opened. The mass was only partially resected and intraoperative SEP monitoring data remained normal including N20 latency. Histological examination found mature adipose tissue covered by thickened pia mater, which was compatible with a diagnosis of lipoma. After surgery, no complications occurred and the patient's myelopathy subsequently improved. Subpial spinal lipomas unassociated with dysraphism are uncommon and they are reported in only 1% of all spinal cord tumors. They are thought to arise from premature dysjunction of the cutaneous ectoderm during neural tube formation. Without any invasive procedure, MR imaging is indispensable to show their longitudinal dimension as well as their infiltrative extension into the spinal cord. The main purpose of surgery is to decompress the lipoma from the adjacent neural structures, because it is benign in nature and there is no cleavage plane identifiable between the lipoma and the spinal cord.

[Dilated pericerebellar fluid space in patients with chronic subdural hematoma].

It is not uncommon to observe the dilation of the pericerebellar fluid space (PCFS) on CT in patients with chronic subdural hematoma (CSDH). CT scans of 92 patients with CSDH proven by surgery were reviewed with respect to the dilatation of PCFS and we evaluated the incidence of dilated PCFS and the relationship between PCFS and other factors. There were 68 males and 24 females. Patients ranged in age from 20 to 90 years (mean 65.2 years). Another 50 patients without CSDH were also reviewed as a control group. A new PCFS grading based on the CT findings was proposed, divided into 3 grades as follows. In grade 0, no PCFS could be seen on CT scans. In grade 1, PCFS could be detected along the posterior aspect of the petrous pyramid, and in grade 2, PCFS could be seen not only along the posterior aspect of the petrous pyramid but also under the tentorium cerebelli. The dilation of PCFS was seen in 78 patients (84.8%) out of the 92 cases. In 50 patients without CSDH (control group), the dilatation of PCFS was noted only in 6 (12%). The dilatation of PCFS was almost always seen on the same side as the CSDH. Among many factors, the significant factor was the degree of the midline shift, the bigger the midline shift caused by CSDH, the larger was the dilated PCFS. Although the mechanism of the dilated PCFS in patients with CSDH is not clear, it is postulated that the mechanism is caused by CSF flow disturbance, compression or adhesion of the subarachnoid space due to CSDH.(ABSTRACT TRUNCATED AT 250 WORDS)

[Preoperative embolization with gelfoam powder for intracranial meningioma causing unusual peritumoral hemorrhage--with reference to the mechanism of hemorrhage].

Preoperative embolization for intracranial meningioma has been performed at many institutions and its effectiveness has been well recognized. The complications of embolization such as facial pain, fever and facial nerve palsy, et al, were mild and temporary except embolus migration into intracranial vessels, but a peritumoral hemorrhage due to preoperative embolization was extremely rare. Recently we have experienced such an unusual complication, then we describe this complication here and discuss the mechanism of hemorrhage briefly. A 73-year-old female who had the left falx meningioma underwent preoperative embolization with gelfoam powder through the transfemoral route. About 10 hours later, she developed disturbance of consciousness and right hemiplegia. At that time, CT scan showed peritumoral hemorrhage and an increase in midline shift. An emergency craniotomy was performed and total removal of the falx meningioma (Simpson grade II) and evacuation of the hematoma were done. The postoperative course was uneventful. We conclude that gelfoam powder (average particle size 40-60 mu) is a useful material for preoperative embolization but may at times cause peritumoral hemorrhage.

[Unusual case of depressed fracture of the posterior cranial fossa associated with the syndrome of acute central cervical spinal cord injury].

Linear fractures through the occipital bone are common, whereas depressed fractures in the posterior cranial fossa are rare because the occipital bone is protected by the surrounding thick muscles. The authors describe an unusual case of depressed fracture localized in the posterior cranial fossa associated with the syndrome of acute central cervical spinal cord injury. A 50-year-old female struck her face against the table and fell backward, while drunk, resulting in the bruise over the occipital region. Three days after injury she was transferred to our hospital because of progressive disturbance of consciousness, brain stem dysfunction and tetraplegia. Neurological examination on admission showed that she was drowsy, had tetraplegia and could not speak. However, she could obey commands only by moving her eyes and the ocular movements were normal in all directions except for horizontal nystagmus. Plain skull x-ray revealed a conspicuously depressed fracture in the posterior cranial fossa, but cervical spine x-ray showed neither fracture nor dislocation. Immediately suboccipital craniectomy was done and there were a thin subdural hematoma on the cerebellar surface, cerebellar contusion, and subarachnoid hemorrhage around the cisterna magna. Soon after operation impaired consciousness and paraplegia were improved, but recovery of both arms was delayed. Five months after injury, she still had left IX, X and XI nerve paresis, bilateral arm weakness, dysarthria, swallowing disturbance and bilateral sensory disturbance below C4 level. These findings indicated that she had sustained brain stem and cerebellar compression by the depressed fracture and also had suffered an acute central cervical spinal cord injury.

[Fractionated intracavitary radiation for glioblastoma with remote afterloading system].

Three patients harboring glioblastoma were treated by fractionated intracavitary radiation using remote afterloading system. For this therapy, a special intracranial applicator was designed and used. Radiation schedule was planned to deliver 3000 rads to the margin of the tumor in 3 fractions over 10 days. The advantages of this treatment are that complete elimination of all unnecessary radiation exposure is possible, and that placing a radioactive source in the tumor provides maximal delivery of radiation to the tumor with relative sparing of surrounding normal brain. The method of treatment is presented and discussed with the reports of the clinical cases.

[A case of cerebral polyuric hyponatremia (author's transl)].

A case of cerebral polyuric hyponatremia was reported. A 64-year-old lady had complained of visual disturbance for 4 years. She was operated upon under the diagnosis of tuberculum sellae meningioma. Sever days following operation tremendous polyuria was senn, i. e. polyuria over 40 l/day, massive sodium wasting in the urine, hyponatremia and severe thirst feeling. These situations could not easily controlled by pitressin, and 2 days after the onset of polyuria the level of sodium in the serum decreased up to 112 mEq/l, the amount of excretion of sodium into urine increased up to 186 mEq/l, and the patient had finally a convulsive seizure followed by loss of consciousness. This hyponatremia was easily controlled by infusion of hypertonic NaCl. The pathogenesis of this condition could not be explained by applying the classical concept of "Diabetes Insipidus" nor "SIADH". This hyponatremia might be the result of massive sodium wasting in the urine. And from the results obtained in our patient, these conditions should be classified in the syndrome of "Cerebral Polyuric Hyponatremia" as reported by Oi et al. It should be emphasized that the measurement of osmorality and level of electrolytes in the serum and urine had to be performed frequently when the postoperative polyuric situation was found, so that the clinical diagnosis could be established in the earlier stage. The clinical diagnosis and differentiation of postoperative disorders in water and electrolytes of central origin were discussed.

[Three cases of carotid anterior cerebral anastomosis].

Three cases are presented with rare carotid-anterior cerebral anastomosis (anomalous ACA) originating from the internal carotid artery at the level of the origin of the ophthalmic artery, running first underneath and then between both optic nerves, and finally joining the anterior communicating artery. Case 1 (47 year-old woman) and case 2 (56 year-old man) were both admitted with subarachnoid hemorrhage from a ruptured aneurysm, which was located at the anomalous ACA (carotid-ACA anastomosis). Case (61 year-old man) was diagnosed as having left occipital intracerebral hematoma on CT, and bilateral anomalous ACA was found on angiograms. This anomalous ACA is very rare; only 21 cases have been reported in the literature. Several synonyms have been proposed as follows: 1) interoptic course of the anterior cerebral artery (ACA), 2) interoptic course of ACA, 3) an anomalous branch of the internal carotid artery (ICA) and 4) an anastomosis between the ICA and the ACA (carotid-ACA anastomosis). In cases with this carotid-ACA anastomosis, coexisting intracranial aneurysm has been reported. However, this is the first report of ruptured aneurysm located at the anomalous ACA itself. Case 1 and 2 were operated on through the ipsilateral pterional approach and the aneurysm was successfully clipped. Postoperative course was uneventful in each case. Case 3 was treated conservatively. From the angiographical and operative findings, we prefer to use "carotid-anterior cerebral artery anastomosis" as the medical terminology best suited to describe this condition.

[von Hippel-Lindau disease--hemangioblastoma associated with pheochromocytoma].

The association of a pheochromocytoma with von Hippel-Lindau disease is uncommon. We had a family with eight patients affected by von Hippel-Lindau disease, of whom five had hemangioblastoma of the central nervous system and seven had pheochromocytoma. As other lesions, retinal angiomatosis, spinal A-V malformation and spinal hemangioma were included in this family. In this paper five hemangioblastomas, four of whom had the association of hemangioblastoma and pheochromocytoma, are presented and clinical features of hemangioblastoma associated with pheochromocytoma are discussed, comparing to the 16 reported cases that had the association of hemangioblastoma and pheochromocytoma. The mean age of our own five cases at the onset of clinical symptoms was 32.2 years (ranging from 24 to 41 years) and that of reported 16 cases was 33.9 years (ranging from 18 to 55 years). These ages are slightly younger than that of sporadic hemangioblastoma. On the other hand, the mean age at the onset of pheochromocytoma was 28.3 years in our cases and 31.0 years in reported cases. This may suggest that hemangioblastoma when it is associated with pheochromocytoma presents its symptoms several years after the signs and symptoms of pheochromocytoma are manifested. In our cases a male to female ratio was 4:1 and in reported ones it was 9:7, showing that hemangioblastoma associated with pheochromocytoma as well as sporadic hemangioblastoma is likely to occur more in male.(ABSTRACT TRUNCATED AT 250 WORDS)

Emergence and progress of white matter lesion in brain check-up.

White matter lesion (WML) is thought to emerge and progress with increasing age but has not yet been well investigated. In this study of WML, risk factors and prevalence added to emergence rate (%/person year) and progress speed [increase of leukoencephalopathy score (LES)/person year] were investigated in 1674 brain check-up subjects from August 1993 to August 2001. Significant (P < 0.01) risk factors were aging and hypertension. Prevalence rate (%) was 0 in 20-29 years, 1.4 in 30-39 years, 4.2 in 40-49 years, 20.9 in 50-59 years, 42.6 in 60-69 years, and 73.9 in 70-years. Emergence rate was 0 in 20-29 years, 0.7 in 30-39 years, 1.5 in 40-49 years, 3.4 in 50-59 years, 6.0 in 60-69 years, and 20 in 70-years. And progress speed was 0.12 in 40-49 years, 0.15 in 50-59 years, 0.21 in 60-69 years, and 0.21 in 70-years. WML begins to emerge early in middle age and both prevalence and severity increase with age.