Subthalamic nucleus deep brain stimulation restores normal rapid eye movement sleep in Parkinson's disease.

BACKGROUND: In Parkinson's disease, sleep disturbance is a common occurrence. METHODS: We evaluated sleep in 10 patients with Parkinson's disease (age, 57.5 ± 9.8 years; disease duration, 12.3 ± 2.7 years) before and after subthalamic nucleus deep brain stimulation using the Parkinson's disease sleep scale and polysomnography. RESULTS: Their total sleep scale scores and daytime sleepiness subscale scores significantly improved after subthalamic nucleus-deep brain stimulation. The novel findings from this study significantly increased normal rapid eye movement sleep, and decreased abnormal rapid eye movement sleep without atonia after deep brain stimulation in patients with Parkinson's disease. The improved total sleep scale score correlated with decreased wakefulness after sleep onset. Moreover, improved daytime sleepiness correlated with increased normal rapid eye movement sleep time. Sleep improvement did not significantly correlate with resolution of motor complication or reduced dopaminergic dosages. CONCLUSIONS: Subthalamic nucleus-deep brain stimulation may have beneficial effects on sleep disturbance in advanced Parkinson's disease by restoring sleep architecture and normal rapid eye movement sleep.

Hypofractionated stereotactic radiotherapy for acoustic neuromas: safety and effectiveness over 8 years of experience.

BACKGROUND: Little information is available about long-term outcomes of hypofractionated stereotactic radiotherapy (hypo-FSRT) for acoustic neuromas. In this study, the safety and effectiveness of hypo-FSRT for unilateral acoustic neuroma were reviewed over 8 years of experience at our institution. METHODS: Between May 1998 and October 2006, 27 patients were consecutively treated by linear accelerator-based hypo-FSRT. Two patients were excluded from this study because they were lost to follow-up within 12 months. The median follow-up period for the rest was 59 (range 24-133) months. Two types of treatment schedules were adopted. Thirteen patients received 30-39 Gy, given in 10-13 fractions (regimen A), whereas after July 2003, 12 patients received 20-24 Gy, given in 5-6 fractions at the tumor periphery (regimen B). These treatments were scheduled to be delivered in three fractions per week (Monday, Wednesday, Friday). The median planning target volume was 2.0, with 1.7 ml (range 0.7-10.6) in regimen A and 5.2 ml (range 0.9-9.3) in regimen B. In the pretreatment audiogram, seven patients (two in regimen A and five in regimen B) had serviceable hearing (Gardner-Robertson Class I-II). RESULTS: Local control rates were 100% with regimen A and 92% with regimen B. Serviceable hearing was preserved in four of five patients in regimen B but no patients in regimen A at the last follow-up. No permanent facial or trigeminal nerve morbidity was observed following treatment, and no salvage surgery was needed. CONCLUSIONS: Hypo-FSRT for acoustic neuromas achieved a high local control rate with minimal facial and trigeminal nerve morbidity.

Ependymoma-like tumor with mesenchymal differentiation harboring C11orf95-NCOA1/2 or -RELA fusion: A hitherto unclassified tumor related to ependymoma.

Recurrent fusion genes involving C11orf95, C11orf95-RELA, have been identified only in supratentorial ependymomas among primary CNS tumors. Here, we report hitherto histopathologically unclassifiable high-grade tumors, under the tentative label of "ependymoma-like tumors with mesenchymal differentiation (ELTMDs)," harboring C11orf95-NCOA1/2 or -RELA fusion. We examined the clinicopathological and molecular features in five cases of ELTMDs. Except for one adult case (50 years old), all cases were in children ranging from 1 to 2.5 years old. All patients presented with a mass lesion in the cerebral hemisphere. Histologically, all cases demonstrated a similar histology with a mixture of components. The major components were embryonal-appearing components forming well-delineated tumor cell nests composed of small uniform cells with high proliferative activity, and spindle-cell mesenchymal components with a low- to high-grade sarcoma-like appearance. The embryonal-appearing components exhibited minimal ependymal differentiation including a characteristic EMA positivity and tubular structures, but histologically did not fit with ependymoma because they lacked perivascular pseudorosettes, a histological hallmark of ependymoma, formed well-delineated nests, and had diffuse and strong staining for CAM5.2. Molecular analysis identified C11orf95-NCOA1, -NCOA2, and -RELA in two, one, and two cases, respectively. t-distributed stochastic neighbor embedding analysis of DNA methylation data from two cases with C11orf95-NCOA1 or -NCOA2 and a reference set of 380 CNS tumors revealed that these two cases were clustered together and were distinct from all subgroups of ependymomas. In conclusion, although ELTMDs exhibited morphological and genetic associations with supratentorial ependymoma with C11orf95-RELA, they cannot be regarded as ependymoma. Further analyses of more cases are needed to clarify their differences and similarities.

Suppressed levels of growth hormone and insulin-like growth factor-1 during successful pregnancy in persistent acromegaly.

Pregnancy is a rather rare event in acromegaly because fertility is often reduced during active disease. Previous reports of pregnancy in acromegalic patients showed that the pituitary growth hormone (GH) level was unaffected and the insulin-like growth factor (IGF)-1 level was elevated during the second and third trimesters. We describe here a case of persistent acromegaly that showed suppressed levels of GH and IGF-1 during pregnancy. The suppression of GH secretion and IGF-1 may be due to increased estrogen or other factors circulating in mid- to late pregnancy.

Endogenous tenascin-C enhances glioblastoma invasion with reactive change of surrounding brain tissue.

Tenascin-C is an extracellular matrix glycoprotein implicated in embryogenesis, wound healing and tumor progression. We previously revealed that tenascin-C expression is correlated with the prognosis of patients with glioblastoma. However, the exact role of endogenous tenascin-C in regulation of glioblastoma proliferation and invasion remains to be established. We show here that endogenous tenascin-C facilitates glioblastoma invasion, followed by reactive change of the surrounding brain tissue. Although shRNA-mediated knockdown of endogenous tenascin-C does not affect proliferation of glioblastoma cells, it abolishes cell migration on a two-dimensional substrate and tumor invasion with brain tissue changes in a xenograft model. The tyrosine phosphorylation of focal adhesion kinase, a cytoplasmic tyrosine kinase that associates with integrins, was decreased in tenascin-C-knockdown cells. In the analysis of clinical samples, tenascin-C expression correlates with the volume of peritumoral reactive change detected by magnetic resonance imaging. Interestingly, glioblastoma cells with high tenascin-C expression infiltrate brain tissue in an autocrine manner. Our results suggest that endogenous tenascin-C contributes the invasive nature of glioblastoma and the compositional change of brain tissue, which renders tenascin-C as a prime candidate for anti-invasion therapy for glioblastoma.

Intracerebral schwannoma in a child with infiltration along perivascular spaces resembling meningioangiomatosis.

Schwannoma arising within brain parenchyma is a rare lesion, usually found in children. Reported herein is a case of intracerebral schwannoma in a 5-year-old boy, with a review of the English-language literature on the subject, in which 47 cases were found. Few detailed histological reviews of intracerebral schwannoma exist. The tumor had a distinctive plexiform growth pattern, and small aggregates of Schwann cells spread extensively into the surrounding brain tissue along perivascular spaces adjacent to the tumor nodule. Histological differential diagnoses included perivascular schwannosis and meningioangiomatosis. A few intratumoral axons, seen on immunostaining for neurofilament protein, were trapped at the periphery of the main lesion, but there was no evidence of intralesional axons in the multiple nodules of Schwann cell proliferations that extended into the perivascular spaces, suggesting that the lesions are neoplastic. Because Schwann cells are not a natural component of the central nervous system, the origin of intracerebral schwannomas remains unknown. The histology suggests that Schwann cells of the perivascular nerve plexus are a likely site of origin.

[An advantage of T2*-weighted MRI for early detection of straight sinus thrombosis: a case report].

A 44-year-old man presented with a 12-day history of severe non-throbbing headache. He showed no physical abnormality but obesity. On day 12, ring-shaped low intensity lesions inside straight sinus were revealed on T2*-weighted MRI image (T2*WI). On the following day (day 13), he was found unresponsive at home, and ambulated with disturbed consciousness. FLAIR and diffusion-weighted MRI image disclosed high intensity signals in bilateral thalamus which were postulated as vasogenic edema. MR venography and conventional cerebral angiography showed an absence of flow in inferior sagittal sinus, vein of Galen, and straight sinus. These findings confirmed the diagnosis of cerebral venous thrombosis (CVT). Anticoagulant treatment was introduced and his consciousness level was gradually improved. On day 43, he was discharged with no neurological sequelae. A delay of correct diagnosis and treatment with CVT can lead to devastating disability or even to death. An early diagnosis of CVT is often dismissed owing to the nonspecific symptoms such as headache and nausea. Recent reports described high sensitivity of T2*WI for detecting CVT. Alterations in blood flow and oxyhemoglobin reduced products, deoxyhemoglobin, in thrombosed veins often produce the magnetic susceptibility on T2*WI. A detection of ring-shaped low intensity lesions within venous sinus on T2*WI were quite rare, and the signal changes of these sinus lesions were successfully visualized by chronological T2*WI. Taken together, our case implies that T2*WI is the powerful tool for the early detection of CVT, even before the critical symptoms might happen.

Gene expression-based molecular diagnostic system for malignant gliomas is superior to histological diagnosis.

PURPOSE: Current morphology-based glioma classification methods do not adequately reflect the complex biology of gliomas, thus limiting their prognostic ability. In this study, we focused on anaplastic oligodendroglioma and glioblastoma, which typically follow distinct clinical courses. Our goal was to construct a clinically useful molecular diagnostic system based on gene expression profiling. EXPERIMENTAL DESIGN: The expression of 3,456 genes in 32 patients, 12 and 20 of whom had prognostically distinct anaplastic oligodendroglioma and glioblastoma, respectively, was measured by PCR array. Next to unsupervised methods, we did supervised analysis using a weighted voting algorithm to construct a diagnostic system discriminating anaplastic oligodendroglioma from glioblastoma. The diagnostic accuracy of this system was evaluated by leave-one-out cross-validation. The clinical utility was tested on a microarray-based data set of 50 malignant gliomas from a previous study. RESULTS: Unsupervised analysis showed divergent global gene expression patterns between the two tumor classes. A supervised binary classification model showed 100% (95% confidence interval, 89.4-100%) diagnostic accuracy by leave-one-out cross-validation using 168 diagnostic genes. Applied to a gene expression data set from a previous study, our model correlated better with outcome than histologic diagnosis, and also displayed 96.6% (28 of 29) consistency with the molecular classification scheme used for these histologically controversial gliomas in the original article. Furthermore, we observed that histologically diagnosed glioblastoma samples that shared anaplastic oligodendroglioma molecular characteristics tended to be associated with longer survival. CONCLUSIONS: Our molecular diagnostic system showed reproducible clinical utility and prognostic ability superior to traditional histopathologic diagnosis for malignant glioma.

Clinical significance of preoperative fibre-tracking to preserve the affected pyramidal tracts during resection of brain tumours in patients with preoperative motor weakness.

OBJECTIVE: To clarify the clinical usefulness of preoperative fibre-tracking in affected pyramidal tracts for intraoperative monitoring during the removal of brain tumours from patients with motor weakness. METHODS: We operated on 10 patients with mild to moderate motor weakness caused by brain tumours located near the pyramidal tracts under local anaesthesia. Before surgery, we performed fibre-tracking imaging of the pyramidal tracts and then transferred this information to the neuronavigation system. During removal of the tumour, motor function was evaluated with motor evoked potentials elicited by cortical/subcortical electrical stimulation and with voluntary movement. RESULTS: In eight patients, the locations of the pyramidal tracts were estimated preoperatively by fibre-tracking; motor evoked potentials were elicited on the motor cortex and subcortex close to the predicted pyramidal tracts. In the remaining two patients, in which fibre-tracking of the pyramidal tracts revealed their disruption surrounding the tumour, cortical/subcortical electrical stimulation did not elicit responses clinically sufficient to monitor motor function. In all cases, voluntary movement with mild to moderate motor weakness was extensively evaluated during surgery and was successfully preserved postoperatively with appropriate tumour resection. CONCLUSIONS: Preoperative fibre-tracking could predict the clinical usefulness of intraoperative electrical stimulation of the motor cortex and subcortical fibres (ie, pyramidal tracts) to preserve affected motor function during removal of brain tumours. In patients for whom fibre-tracking failed preoperatively, awake surgery is more appropriate to evaluate and preserve moderately impaired muscle strength.

Clinical impact of integrated functional neuronavigation and subcortical electrical stimulation to preserve motor function during resection of brain tumors.

OBJECT: The authors evaluated the clinical impact of combining functional neuronavigation with subcortical electrical stimulation to preserve motor function following the removal of brain tumors. METHODS: Forty patients underwent surgery for treatment of brain tumors located near pyramidal tracts that had been identified by fiber tracking. The distances between the electrically stimulated white matter and the pyramidal tracts were measured intraoperatively with tractography-integrated functional neuronavigation, and correlated with subcortical motor evoked potentials (MEPs) and clinical symptoms during and after resection of the tumors. Motor function was preserved after appropriate tumor resection in all cases. In 18 of 20 patients, MEPs were elicited from the subcortex within 1 cm of the pyramidal tracts as measured using intraoperative neuronavigation. During resection, improvement of motor weakness was observed in two patients, whereas transient mild motor weakness occurred in two other patients. In 20 patients, the distances between the stimulated subcortex and the estimated pyramidal tracts were more than I cm, and MEPs were detected in only three of these patients following stimulation. CONCLUSIONS: Intraoperative functional neuronavigation and subcortical electrical stimulation are complementary techniques that may facilitate the preservation of pyramidal tracts around 1 cm of resected tumors.

Hypophysitis caused by Rathke's cleft cyst. Case report.

A 62-year-old woman presented with general malaise persisting for 2 months and narrowing of her visual field. T1-weighted magnetic resonance (MR) imaging showed swelling of the pituitary gland and stalk, and a homogeneous isointense intra- and suprasellar mass enhanced by gadolinium. During outpatient follow up, her condition deteriorated rapidly and she developed diabetes insipidus and panhypopituitarism. T1-weighted MR imaging demonstrated shrinkage of the lesion and disappearance of the central hypointense area indicating the cyst cavity, especially in the pituitary stalk. She underwent surgical exploration via the transsphenoidal approach under a provisional diagnosis of lymphocytic hypophysitis. Histological examination revealed ciliated columnar cells and squamous metaplasia on the inner surface of the cyst wall, with many foamy cells, infiltration by many lymphoid cells and some eosinophils, and fibrosis in the adenohypophysitis and inflammatory hypophysitis in the anterior and posterior pituitary gland. The present neuroimaging findings of cyst shrinkage associated with rapid clinical deterioration strongly support the suggestion that rupture of Rathke's cleft cyst causes inflammatory hypophysitis.

Multiple repair pathways mediate tolerance to chemotherapeutic cross-linking agents in vertebrate cells.

Cross-linking agents that induce DNA interstrand cross-links (ICL) are widely used in anticancer chemotherapy. Yeast genetic studies show that nucleotide excision repair (NER), Rad6/Rad18-dependent postreplication repair, homologous recombination, and cell cycle checkpoint pathway are involved in ICL repair. To study the contribution of DNA damage response pathways in tolerance to cross-linking agents in vertebrates, we made a panel of gene-disrupted clones from chicken DT40 cells, each defective in a particular DNA repair or checkpoint pathway, and measured the sensitivities to cross-linking agents, including cis-diamminedichloroplatinum (II) (cisplatin), mitomycin C, and melphalan. We found that cells harboring defects in translesion DNA synthesis (TLS), Fanconi anemia complementation groups (FANC), or homologous recombination displayed marked hypersensitivity to all the cross-linking agents, whereas NER seemed to play only a minor role. This effect of replication-dependent repair pathways is distinctively different from the situation in yeast, where NER seems to play a major role in dealing with ICL. Cells deficient in Rev3, the catalytic subunit of TLS polymerase Polzeta, showed the highest sensitivity to cisplatin followed by fanc-c. Furthermore, epistasis analysis revealed that these two mutants work in the same pathway. Our genetic comprehensive study reveals a critical role for DNA repair pathways that release DNA replication block at ICLs in cellular tolerance to cross-linking agents and could be directly exploited in designing an effective chemotherapy.

Phase II study of nimustine, carboplatin, vincristine, and interferon-beta with radiotherapy for glioblastoma multiforme: experience of the Kyoto Neuro-Oncology Group.

OBJECT: This Phase II study was performed to determine the safety, tolerability, and efficacy of combining nimustine (ACNU)-carboplatin-vincristine-Interferon-beta (IFNbeta) chemotherapy. METHODS: Ninety-seven patients with Karnofsky Performance Scale scores of 50 or greater were enrolled in the study. Nimustine (60 mg/m2), carboplatin (110 mg/m2), vincristine (0.6 mg/m2), and IFNbeta (10 microg) were administered on Day 1 concomitant with radiotherapy (63 Gy); vincristine (0.6 mg/m2) and IFNbeta (10 microg) on Days 8 and 15; and IFNbeta alone (10 microg) three times per week throughout the course of radiotherapy. Fifty-six days after radiotherapy ended, the time schedule for chemotherapy was reset and ACNU, carboplatin, vincristine, and IFNbeta were again administered on the new Day 1 and vincristine and IFNbeta on the new Days 8 and 15. This course was repeated every 56 days. Instances of nonhematological toxicity were rare and mild. During the course of radiotherapy, the percentages of patients who experienced Grade 3 toxicity were 14% with neurocytopenia and 7% with thrombocytopenia. Seven percent of all adjuvant chemotherapy cycles following radiotherapy were associated with Grade 3 toxicity, as manifested in neurocytopenia or thrombocytopenia. No instance of Grade 4 toxicity was observed. The median duration of progression-free survival was 10 months (95% confidence interval [CI] 8-12 months) and the median duration of overall survival was 16 months (95% CI 13-20 months). CONCLUSIONS: The combination of ACNU-carboplatin-vincristine-IFNbeta chemotherapy and radiotherapy is safe and well tolerated, and may prolong survival in patients with glioblastoma multiforme.

MR imaging of Liliequist's membrane.

Liliequist's membrane is an arachnoid structure well-known to neurosurgeons. However, the importance of this membrane had been lost until the development of endoscopic third ventriculostomy (ETV). ETV is superior in its minimal invasiveness, but in some subgroups of hydrocephalus, the effectiveness of ETV may be reduced. Liliequist's membrane may block the cerebrospinal fluid (CSF) flow from the defect of the third ventricle floor, which may cause failure of ETV. Liliequist's membrane can be visualized on magnetic resonance (MR) imaging in normal healthy individuals, however, its visibility is different among individuals. CSF artifacts exist to varying degrees, but do not impede visualization of Liliequist's membrane in most subjects. Since Liliequist's membrane is a cisternal structure, the three-dimensional (3D) constructive interference in steady state (CISS) sequence is useful. The outcome of ETV could be predicted with MR imaging findings of Liliequist's membrane in a patient with obstructive hydrocephalus. High-field (> or =3 Tesla) MR imaging of Liliequist's membrane also offers superior resolution and is expected to provide additional information about Liliequist's membrane.

Evaluation of primary brain tumors with FLT-PET: usefulness and limitations.

PURPOSE OF THE REPORT: The purpose of this report was to investigate the potential of positron emission tomography using F-18 fluorodeoxythymidine (FLT-PET) in evaluating primary brain tumors. MATERIALS AND METHODS: FLT-PET was performed in 25 patients with primary brain tumors. FLT uptake in the lesion was semiquantitatively evaluated by measuring the maximal standardized uptake value (SUVmax) and the tumor-to-normal tissue ratio (TNR). SUVmax and TNR were compared with the histologic grade and the expression of the proliferation marker (Ki-67). RESULTS: FLT uptake in normal brain parenchyma was very low, resulting in the visualization of brain tumors with high contrast. Both SUVmax and TNR significantly correlated with the malignant grade of brain gliomas, in which high SUVmax/TNR was obtained for high-grade gliomas. Patients with primary lymphoma also showed SUVmax/TNR equivalent to glioblastoma. There was a positive correlation between SUVmax/TNR and the Ki-67 index. In contrast, spuriously high SUVmax and TNR were obtained in 3 of 6 patients with suspected recurrent tumors (2 patients with recurrent grade 2 glioma and one patient with postoperative granuloma), all of which showed lesion enhancement on MRI after Gd administration. CONCLUSIONS: FLT-PET can be used to evaluate the malignant grade and proliferation activity of primary brain tumors, especially malignant brain tumors. However, the presence of benign lesions showing blood-brain barrier disruption cannot be distinguished from malignant tumors and needs to be carefully evaluated.

Long-term outcome in patients harboring intracranial ependymoma.

OBJECT: The prognostic significance of tumor grade and resection and the efficacy of prophylactic radiation remain controversial in the management of intracranial ependymoma. The outcomes in patients with intracranial ependymoma treated at the Kyoto University Hospital were reviewed retrospectively, and prognostic significance was analyzed. METHODS: Between 1972 and 2002, 29 patients were seen at the authors' institution. Eighteen cases involved a Grade II lesion according to the World Health Organization classification of ependymoma and 11 involved a Grade III lesion. Postoperative radiation was applied in 24 cases and chemotherapy was administered in nine. Overall survival and progression-free survival rates were significantly higher in patients with Grade II ependymoma (p = 0.006 and 0.004, respectively) and in patients who had undergone gross-total resection of the tumor (p = 0.002 and 0.04, respectively). Fourteen patients relapsed from 10 to 120 months (median 39 months) after diagnosis. In nine patients the ependymoma recurred only at the original tumor site. Three patients experienced both local and distant relapse, whereas two others had only a distant relapse. All relapses of the Grade II ependymoma initially occurred at the primary tumor site. Histological grade and extent of resection were significantly associated with tumor dissemination (p = 0.0034 and 0.0011, respectively). The field of postoperative radiation had no impact on patient survival or lesion recurrence. CONCLUSIONS: Tumor grade and resection are the two important prognostic factors with respect to patient survival, tumor recurrence, and tumor dissemination. Considering that relapses were predominantly local and that there was no apparent benefit from prophylactic radiation, the authors concluded that postoperative radiation should be focused on local control, especially for Grade II ependymomas.

Ectopic posterior pituitary high signal in preoperative and postoperative macroadenomas: dynamic MR imaging.

BACKGROUND AND PURPOSE: In patients with macroadenoma, posterior pituitary high signal (PPHS) on T1-weighted magnetic resonance (MR) imaging is sometimes observed in an ectopic location. The present study compared incidences of ectopic PPHS before and after macroadenoma surgery using MR imaging, including dynamic MR imaging to ascertain whether this ectopic change is irreversible. MATERIALS AND METHODS: MR imaging was performed preoperatively in 111 cases of macroadenoma, and then repeated more than 1-year postoperatively in 47 patients. Enhancement of PPHS was assessed using dynamic MR imaging. Areas of enhanced hyperintensity were considered true PPHS, and the relationship between presence and location of true PPHS and adenoma volume was analyzed. Moreover, changes in the presence and location of true PPHS were ascertained among the patients who underwent postoperative follow-up MR imaging. RESULTS: Preoperatively, PPHS was seen only in the normal location in 29 patients (Group A: 26.1%). High signal was detected only in an ectopic location in 58 patients, and early enhancement of this ectopic high signal was confirmed by dynamic MR imaging in 56 patients (Group B: 50.5%). No PPHS was observed in 24 patients (Group C: 21.6%). Adenoma volume was significantly greater for Group B than for Group A (p<0.001). Among the Group B patients who underwent MR imaging postoperatively (n=31), the location of PPHS was not changed, except for two patients in whom PPHS was absent. Postoperatively, PPHS was not observed in the normal location in any patient in the Group B. CONCLUSIONS: Greater volume of adenoma is associated with a higher incidence of ectopic PPHS, and the ectopic change is irreversible.

Postoperative evaluation of microsurgical resection for cavernous malformations of the brainstem.

OBJECT: The aim of this study was to propose criteria to determine whether complete resection of cavernous malformations in the brainstem had been achieved. METHODS: The authors retrospectively analyzed data in 10 patients harboring a single cavernous malformation who had presented with hemorrhagic symptoms and had been followed up for longer than 2 years postsurgery. The study population consisted of five male and five female patients ranging in age from 13 to 57 years (mean 36.8 years). When preoperative magnetic resonance (MR) images demonstrated the lesion as a homogeneous hyperintense mass, the surgery was defined as complete or incomplete based on intraoperative findings. When preoperative MR images revealed other findings, complete resection was determined according to whether postoperative MR imaging results demonstrated lesions distinct from the peripheral hemosiderin rim. Among the 13 operations in this series, nine resulted in complete resection and were associated with no postoperative clinical relapse of hemorrhage, whereas four operations resulted in incomplete resection and were correlated with postoperative recurrent hemorrhage. The seven patients in whom the outcome of the initial operation was complete demonstrated good neurological recovery in the long-term follow-up period, whereas the three patients in whom the outcome of the initial surgery was judged to be incomplete showed inadequate neurological recovery due to recurrent hemorrhage. CONCLUSIONS: The criteria proposed in this study to evaluate surgical treatment may be a reliable means of predicting the recurrence of hemorrhage postoperatively.

MRI and CT findings of neurohypophyseal germinoma.

OBJECTIVE: Magnetic resonance (MR) imaging and computed tomography (CT) findings of neurohypophyseal germinoma have not previously been described in detail. The purpose of the present study was to establish the spectrum of MR imaging and CT findings in neurohypophyseal germinomas. MATERIALS AND METHODS: MR and CT images of 13 consecutive patients (seven males, six females; mean age: 15 years; range: 6-31 years) with neurohypophyseal germinoma were retrospectively analyzed. The diagnosis had been made either histologically (n=8) or clinically according to established criteria (n=5). All patients had been examined using MR imaging and CT before treatment. RESULTS: On MR imaging, infundibular thickening (up to 16 mm) was observed in all 13 cases. Hyperintensity of the posterior pituitary on T1-weighted image was absent in all 13 cases (100%) and 12 of the 13 displayed central diabetes insipidus. Ten germinomas (77%) were isointense to cerebral cortex on T1-weighted image, but variable intensities were exhibited on T2-weighted image. MR images revealed intratumoral cysts in six cases (46%), most of which demonstrated intra-third ventricular extension. Eleven of the 13 cases (85%) revealed hyperdense solid components on unenhanced CT. Calcification was absent in all cases (100%). CONCLUSION: Infundibular thickening, absence of the posterior pituitary high signal on T1-weighted image, lack of calcification and hyperdensity on unenhanced CT are common imaging features of neurohypophyseal germinoma.

Rendu-Osler-Weber disease with a giant intracerebral varix secondary to a high-flow pial AVF: case report.

BACKGROUND: Intracranial varices are rare and most are associated with vein of Galen arteriovenous malformations (AVM) or fistulas (AVF). DESCRIPTION: A 43-year-old left-handed man presented with right hemihypesthesia and spastic gait. Neuroradiological examination revealed a spinal AVF and a giant intracerebral varix associated with a high-flow pial AVF. He had recurrent episodes of nasal bleeding, which were also confirmed in his mother's medical history, and telangiectases in the tip of his tongue and fingers. He was diagnosed with Rendu-Osler-Weber disease. After resection of the spinal AVF that produced his symptoms, we surgically exposed and obliterated the giant varix and AVF under intra- and postoperative hypotension and mild barbiturate therapy. The arteriovenous shunt was completely obliterated without hyperperfusion of the surrounding brain. CONCLUSION: This is an extremely rare case of Rendu-Osler-Weber disease with a giant intracerebral varix secondary to a high-flow pial AVF that did not involve the vein of Galen.