Cranial base chordoma--long term outcome and review of the literature.

BACKGROUND: The purpose of this study is to clarify the latest long-term therapeutic result for cranial base chordomas. We are seeking an improvement of long term therapeutic outcome through a review of cranial base chordomas treated in our institute and of the published literature in the era of multimodality therapy. MATERIALS AND METHODS: We retrospectively reviewed 13 consecutive patients with cranial base chordoma, including ten males and three females with mean age of 39.5 years (range 5-76 years). RESULTS: The method of initial treatment included surgery and post-operative conventional local irradiation (IR) in 9 patients, surgery and IR followed by post-operative stereotactic radiosurgery (SRS) in 2 patients, surgery as well as SRS in one patients, and surgery as well as SRS followed by IR in one patient. Subtotal removal (over 95%) was accomplished in eight patients. The mean follow-up period after completion of surgery and initial radiotherapy was 122 months (median 108 months). According to the Kaplan-Meier estimate method, the 5-year survival rate was 82.5%: 11 out of 13 patients survived longer than 5 years and five patients survived longer than 10 years. With a longer follow-up period than the previous reports, our series has provided a 5-year survival rate comparable to that of proton beam therapy. Although our series indicates a favourable outcome, surgical resection followed by IR or SRS failed to control tumour growth in five patients. CONCLUSIONS: IR and/or SRS provided results comparable with proton beam or heavy particle therapy in our series of cranial base chordomas probably because the radiation field must have covered the target of the tumour volume sufficiently, and reduction of gross tumour volume reduced the target size for radiotherapy. Pursuit of further effective combinations of IR and stereotactic radiotherapy (SRS, proton beam, heavy particle) after tangible resection, especially for residual and recurrent lesions, will be an acceptable framework to achieve a better therapeutic outcome for cranial base chordomas than at present.

Evaluation of post-procedure changes in aneurysmal lumen following detachable coil-placement using multi-planar reconstruction of high-field (3.0T) magnetic resonance angiography.

BACKGROUND: Placement of detachable coil(s) for intracranial aneurysms has become one of the standard methods of management. Although detailed analysis of post-procedure changes in aneurysmal lumen is essential, technical difficulties often limit such evaluation. Development of higher magnetic field systems is steadily widening clinical usage of magnetic resonance imaging (MRI) primarily due to its significantly higher signal to noise ratio. OBJECTIVE: In this study, we evaluated a multi-planar reconstruction (MPR) technique of magnetic resonance angiography (MRA) on a 3.0T system in an attempt to develop a routine method of post-procedure evaluation following detachable coil placement. METHODS: Eleven patients with an intracranial aneurysm following placement of a Guglielmi detachable coil (GDC) participated in the study. Time of flight (TOF) magnetic resonance angiography (MRA) was obtained immediately after, and up to two years after coil embolisation utilising a GE 3.0T system. Data was analysed using standard maximum intensity projection (MIP) as well as the MPR technique and the results were compared to conventional catheter angiography. RESULTS: The study demonstrated that, compared to MIP, MPR can provide further information of alteration in aneurysm lumen, especially in analysis of: 1) jet of blood flow, 2) thrombus formation, 3) neck remnant or re-filling of blood, 4) location and shape of coils including compaction, and 5) coil protrusion into the parent artery. CONCLUSIONS: Combined MPR/MIP analysis of high-field MRA appears to be a powerful non-invasive method for evaluating GDC-treatment that can potentially replace conventional catheter angiography in many clinical situations.

Human Kluver-Bucy syndrome following acute subdural haematoma.

We present a rare case of complete human Kluver-Bucy syndrome (KBS) following recovery from transtentorial herniation caused by acute subdural haematoma (ASDH). A 17-year-old right-handed high school boy got into stupor within five minutes after 3-rounds of sparing at boxing. Emergency computed tomographic (CT) scan showed right cerebral hemispheric ASDH, which was evacuated following intentional decompressive craniectomy. After recovery of consciousness, he developed emotional changes (placidity with loss of normal fear and anger), psychic blindness, aberrant sexual behaviour, excessive oral tendencies, increased appetite, and hypermetamorphosis in order of mention, which were observed with waxing and waning from 17th to 28th hospital day. Peri-operative CT scaning and magnetic resonance imaging showed lesions of the right temporal lobe and right-dominant orbitofrontal regions including bilateral rectal and medial orbital gyri, and the intact left temporal lobe. Two pathogeneses can be thought of and the whole picture of KBS following ASDH can arise even though one (left in this case) temporal lobe is preserved, 1) in which associated orbitofrontal lesions of the frontal lobes may correlate with occurrence of KBS, or 2) cerebral blood hypoperfusion of both temporal lobes due to increased intracranial pressure and/or compression of both posterior cerebral arteries at the edge of the tentorium cerebelli occurs.

Ruptured de novo intracranial aneurysms.

OBJECTIVE: We describe the actual state of ruptured de novo intracranial aneurysms to contribute to a guideline of follow-up for the patients with treated intracranial aneurysm. METHODS: The authors retrospectively investigated 12 cases drawn from 483 consecutive cases of aneurysmal subarachnoid hemorrhage at our institute over a period of 22 years, in which a previously undemonstrated (hence "de novo") intracranial saccular aneurysm formed and ruptured after successful treatment of a prior aneurysm. FINDINGS: The 12 cases constitute 2.5% of the 483 patients who left our hospital alive. Eleven cases were females and one was a male with a mean age of 55.7 years (range 29-75) at the first subarachnoid haemorrhage (SAH) and an interval between the first and the second rupture of 10.7 years (range: 2.6-23.8, standard deviation: 6.86, 95% confidence interval: 6.39-15.1). Four cases did not have risk factors such as hypertension, family history, smoking, multiple aneurysms, and moyamoya disease. None of these ruptured de novo aneurysms was at the same location as the original lesion. One-third (4 cases) of the de novo lesions in our series were found on the opposite side to each prior lesion. INTERPRETATION: For not only young but also elder patients with a treated aneurysm (from the fifth decade to the sixth), especially for women, late angiography or alternative modalities of less-invasive examination should be considered. To detect de novo intracranial aneurysms before rupture, the search for a de novo aneurysm should be performed within 6.39 years after a previous examination that shows an aneurysm to be nonexistent, in view of the 95% confidence interval of the mean time to de novo aneurysmal rupture (6.39-15.1 years). If applied this survey, 75% (8 cases of 12 cases) of our de novo aneurysms would be detected before rupture.

Intra-arterial Chemotherapy for Malignant Tumors of Head and Neck Region Using Three Types of Modified Injection Method.

SUMMARY: Relatively higher infusion rate in the intra-arterial chemotherapy (IA chemotherapy) could induce the higher concentration and the more sufficient distribution of chemotherapeutic agents on tumors. To get the relatively higher infusion rate in IA chemotherapy, we used three types of injection method: high-flow injection, high-dose injection with detoxification and flowcontrolled injection method for the treatment of malignant brain tumors, skull base tumors and head and neck tumors. Between January 1997 and October 2001, twenty-seven patients (mean age 61 y.o.) with supratentorial glioblastoma (4 cases), supratentorial anaplastic astrocytoma (1), CNS lymphoma (2), matastatic skull base tumors (3), and neck tumors (15 squamous cell carcinoma, 1 malignant melanoma and 1 neuroblastoma) received our three types of IA chemotherapy. Sixty- five consecutive procedures were performed. Conventional radiation therapy and/or surgical removal were performed in some of these patients. The median follow-up period was 10 months ranging 2 to 56 months. Fifteen (55.6%) and 6 (22.2%) of 27 patients achieved complete response (CR) and partial response (PR) respectively after initial treatment [CR+PR: 21 (77.8%)]. All responded patients showed clinical improvement. The response rate declined to 55.6% at the end of follow-up period. Eighteen patients are still alive and 15 of them show no evidence of local recurrence. The median post treatment survival was 12 months. There was no serious complication except transient nausea in 4 of 27 (14%) patients, vertigo and granulocytopenia in 1 each (3%) of 27 patients. Our modified IA chemotherapy has provided favorable clinical and radiological results without technical difficulties and serious complications.

Pre-operative histopathological evaluation of meningiomas by 3 0T T2R MRI.

BACKGROUND: To assess the potential of T2 reversed (T2R) magnetic resonance imaging (MRI) for the pre-operative histopathological assessment of meningiomas. METHOD: Twenty patients scheduled for meningioma resection were prospectively assessed using T2R MRI on a 3 tesla system. Image characteristics were compared with intra-operative findings and post-operative histopathological examination of excised meningioma tissues. FINDINGS: The averaged T2 characteristics expressed as averaged gray scale levels of the tumour correlated highly with tumour consistency, in agreement with previous reports. Furthermore, detailed evaluation of the structural appearance of tumour on T2R images revealed brightness of tumour (shorter T2) was dependent on the degree of histopathological heterogeneity. Significantly shorter T2 value correlated with collagen-rich fibrous tissue. INTERPRETATION: The study demonstrated the advantage of T2R imaging in pre-operative determination of histopathological characteristics of meningiomas. As a step towards MR microscopy and improved pre-operative treatment planning, T2R imaging on a 3.0 T system appears to play an important role in the non-invasive pre-operative structural assessment of a tissue of interest.

Planum temporale: where spoken and written language meet.

Functional magnetic resonance imaging studies on spoken versus written language processing were performed in 20 right-handed normal volunteers on a high-field (3.0-tesla) system. The areas activated in common by both auditory (listening) and visual (reading) language comprehension paradigms were mapped onto the planum temporale (20/20), primary auditory region (2/20), superior temporal sulcus area (2/20) and planum parietale (3/20). The study indicates that the planum temporale represents a common traffic area for cortical processing which needs to access the system of language comprehension. The destruction of this area can result in comprehension deficits in both spoken and written language, i.e. a classical case of Wernicke's aphasia.

MRA demonstration of "periarteritis" in Tolosa-Hunt syndrome.

BACKGROUND: Modern magnetic resonance imaging (MRI) diagnosis of Tolosa-Hunt syndrome rests upon demonstration of cavernous sinus abnormalities. We present a case of Tolosa-Hunt syndrome who has no abnormal mass lesion in the cavernous sinuses on MRI but with a diagnostic lesion on magnetic resonance angiography (MRA). CLINICAL PRESENTATION: A 48-year-old woman developed acute periorbital pain and abducens palsy of the right side at the first episode, and subacute peri-orbital pain and rapidly deteriorating visual acuity on the left side at the second episode with a four months interval. MRI showed no soft-tissue abnormality in the cavernous sinuses. FINDINGS: MRA demonstrated a narrowing of the right cavernous carotid artery at the first episode, and narrowings of the left clinoid carotid and ophthalmic arteries at the second episode. Based on these findings, the patient underwent urgent steroid therapy and the symptoms resolved dramatically in each episode. Follow-up MRA confirmed resolution of arterial narrowings. INTERPRETATION: MRA may help prompt the noninvasive diagnosis in certain cases of Tolosa-Hunt syndrome with little inflammatory reaction in the cavernous sinus but with predominant intra- and juxta-cavernous periarteritis.

High-field magnetic resonance imaging in patients with moyamoya disease.

OBJECT: The purpose of this study was to assess the utility of high-field magnetic resonance (MR) imaging as a quantitative tool for estimating cerebral circulation in patients with moyamoya disease. METHODS: Eighteen patients with moyamoya disease who were scheduled to undergo revascularization surgery and 100 healthy volunteers were examined using T2-reversed MR imaging performed using a 3-tesla system. Ten of the 18 patients underwent a second study between 1 year and 3 years after revascularization. Magnetic resonance images obtained in the patients with moyamoya disease were statistically analyzed and compared with those obtained in healthy volunteers. The MR imaging findings were also correlated with results of single-photon emission computerized tomography and conventional cerebral angiography studies. Transverse lines in the white matter (medullary streaks) were observed in almost all persons. In healthy volunteers, the diameter sizes of the medullary streaks increased significantly with age (p < 0.001). Multiple logistic regression analysis revealed that age-adjusted medullary streak diameters were significantly larger in patients with moyamoya disease (p < 0.001). Diameter sizes also increased significantly with the increased severity of cerebral hypoperfusion (p < 0.001) and a higher angiographically determined stage of the disease (p < 0.001). Diameter sizes decreased significantly after surgery (p < 0.001). CONCLUSIONS: The increases in medullary streak diameters observed in patients with moyamoya disease appear to represent vessels dilated due to cerebral hypoperfusion. High-field T2-reversed MR imaging is useful in estimating cerebral circulation in patients with moyamoya disease.

Central tinnitus: a case report.

We report a case of acute-onset unilateral tinnitus in a 25-year-old woman. Analysis of imaging studies indicated that the tinnitus was likely caused by an acute hemorrhage of a small cavernous angioma that was located adjacent to the contralateral primary auditory cortex. This case provides substantial support for the concept that central tinnitus might indeed represent a pathologic activation of neural networks of nonspecific auditory perception.

Intrauterine methylmercury intoxication. Consequence of the inherent brain lesions and cognitive dysfunction in maturity.

We studied the effects of intrauterine neurotoxicity by methylmercury (MeHg) on the postnatal developing and adult stages of rats. We used offspring delivered from dams that had been given 1 mg/kg/day methylmercury chloride for 5 pregestational days and throughout pregnancy. Histopathological examination of the brains of a proportion of the offspring on postnatal days 1 (P1) and P3 revealed degenerative neurons in the brain stem and the limbic system, including the hippocampus and the amygdala. At P7 and P14, degenerative neurons were indiscernible, but reactive astrocytosis remained in the brain stem. At P70 and P180, the brains seemed to have developed well. However, in behavioral analyses performed at 6 months of age, MeHg-exposed rats showed a significant learning disability in the passive avoidance response compared with controls, but no differences in water maze performance. Furthermore, morphometric analysis of the amygdala and hippocampus revealed significantly fewer neurons in both areas in the MeHg-exposed rats. Thus, chronic intrauterine exposure to low-dose MeHg induces a decrease in neuron population in the limbic system, and the offspring have impaired higher brain function.

Growth of incidental meningiomas.

The aim of this study was to assess the growth of incidental meningiomas, to establish a strategy for dealing with these tumours. The cases of 37 patients with a meningioma revealed incidentally by computerized tomography or magnetic resonance imaging, who were followed at least once by an additional imaging study, were reviewed. The tumour volume was calculated, to estimate the annual growth rate of the incidental meningiomas. Nine of the 37 patients (24.3%) showed a considerable increase (the annual growth rate > 1 cu cm/year) in their tumour volume (tumour growth). There was no significant difference in the follow-up period, age, or the volume of tumour between the patients with and without tumour growth. However, a multivariate analysis revealed that the likelihood of tumour growth independently and significantly increased according to a decrease in the age of the patients (Odds ratio 0.18 for one-standard-deviation change (ISD) 12.6 years, p = 0.042) and according to an increase in the volume of the tumour (Odds ratio 3.64 for ISD 4.46 cu cm, p = 0.042). The majority of patients with incidental meningioma can be apparently observed without any surgical intervention, because their annual growth rates are generally less than 1 cu cm/year. However, clinical and radiological observations would be advisable for these patients (especially young patients and patients with a large tumour), in view of the presence of rapidly growing tumours in some of the patients.

Administration of prosaposin ameliorates spatial learning disturbance and reduces cavity formation following stab wounds in rat brain.

The effectiveness of prosaposin as a neurotrophic factor was investigated using rats with bilateral stab wounds, injecting 240 ng per day of prosaposin for 3 days. In Morris water maze task, after 3 weeks postoperation, the stab-wounds rats show significant impairment in acquisition compared with the sham-operated rats. In the transfer test the mean number of crossings of the platform place in stab-wounds was significantly lower than that in sham-operated rats (P < 0.01). The stab-wounds rats treated with prosaposin showed significant improvement (P < 0.05). The cavities following stab wounds in the rats treated with prosaposin were significantly smaller than those in the rats treated with (P < 0.05). Our data support that prosaposin is likely to be a new agent for brain injury.

An experimental study of radiation-induced cognitive dysfunction in an adult rat model.

The objectives of this study were to establish an adult rat model for the late onset of radiation-induced cognitive dysfunction and to compare behavioural dysfunction with histopathological changes. While under anaesthesia, 30 rats (experimental group) were irradiated with a total dose of 40 Gy, given as eight fractions in 24 days. Another 30 rats (control group) underwent sham irradiation. The cognitive functions of all rats were evaluated at 6, 9 and 12 months after irradiation using the Morris water maze and passive avoidance tasks. Histopathological examination of these rats was carried out after the evaluation of cognitive functions was complete. At 6 and 9 months after irradiation there were no significant differences between the control and irradiated groups in passive avoidance and water maze tests. At 12 months after irradiation, the passive avoidance task revealed a deterioration of cognitive function in the experimental group. Histopathological observations revealed no abnormal findings in the irradiated brains at the light microscope level. Late onset cognitive dysfunction following cranial irradiation was observed in an adult rat model. Pathological investigations showed no abnormalities in the irradiated brains. These findings indicate that radiation-induced cognitive dysfunction can precede morphological changes in the brain or that they arise without them. The present model seems useful for elucidating the pathogenesis of radiation-induced cognitive dysfunction and for developing methods for therapy and prophylaxis.

Ruptured distal anterior choroidal artery aneurysm presenting with casting intraventricular haemorrhage.

This report describes a rare case of a distal anterior choroidal artery aneurysm which developed intraventricular haemorrhage without subarachnoid haemorrhage as shown on computerized tomographic (CT) scan. A 69-year-old hypertensive man suddenly became unconscious. An emergency CT scan showed a severe intraventricular haemorrhage and a small round low-dense lesion within the haematoma at the right trigone. The haematoma with obstructive hydrocephalus made the lateral ventricles larger on the right than on the left. CT scan could not detect any subarachnoid haemorrhage. Right interal carotid angiography revealed a saccular aneurysm at the plexal point of the right anterior choroidal artery. We approached the aneurysm and the small round lesion through the trigone via a right temporo-occipital corticotomy. We could clip the aneurysmal neck and remove the intraventricular haematoma and the papillary cystic mass (corresponding to the small round lesion on CT scan) totally in one sitting. Histological examination revealed the aneurysm to be a true one and the papillary cystic mass to be a choroid plexus cyst.

Frequent TIA in the territory fed by the anastomosed STA after combined therapeutic ICA occlusion and extracranial-intracranial bypass: case report.

Frequent transient ischaemic attacks (TIAs) in the territory fed by the anastomosed superficial temporal artery (STA) after combined therapeutic internal carotid artery (ICA) occlusion and extracranial-intracranial bypass is described in a 52-year-old woman with a giant aneurysm in the supraclinoid portion of the left ICA showing impairment of visual acuity in the left eye and right upper quadrantanopia. After the balloon test occlusion of the left ICA which was tolerated, the left STA-middle cerebral artery anastomosis was performed and occlusion of the left ICA using detachable balloons was carried out a day later. TIAs corresponding to the territory fed by the anastomosed STA occurred nine times two to four days and five times eight to nine days after the ICA occlusion without new infarction on computed tomography (CT) scan. Single-photon emission computed tomography showed no hypoperfusion immediately after the initial TIA. CT scan revealed thrombosis of half of the aneurysm a day after the ICA occlusion. The patient developed the same TIA as previously by compression of the left anastomosed STA at the time of follow-up angiography which was carried out eight days after the occlusion. Although heparin was continuously administered after the ICA occlusion for two days, the initial TIA occurred during heparinization. Anticoagulation seemed to be inadequate judging from activated coagulation time and incomplete thrombosis of the aneurysm occurred during heparinization. It is likely that the TIAs are caused by embolism via the STA, which is a rare ischaemic complication.