Early recurrence of Rosai-Doefman disease after total removal resection: a case report.

BACKGROUND: Rosai-Dorfman disease (RDD) involving the central nervous system (CNS) is rare and observed in 5% of all patients with extranodal RDD. According to a previous report, gross total resection is curative; however, we encountered a case of recurrence following gross total resection. We discuss our case and review previous reports on recurrent RDD. CASE DESCRIPTION: A 68-year-old woman came to the hospital complaining of left parietal mass. A tumor that had partially eroded the frontal bone was found. As the lesion was suspected to be malignant, we performed a total resection. Pathology results were indicative of an RDD. We did not prescribe adjuvant therapy because total resection was performed. However, after a year, abnormal accumulation in the left parietal bone was observed on FDG-PET. This was considered as recurrence, and re-excision was performed. Pathological assessments confirmed the recurrence of RDD. CONCLUSIONS: Our case demonstrated the recurrence of RDD following total resection. Future reports should assess these peculiarities. This will facilitate discussions on the risk factors and the effectiveness of treatment methods.

Primary neurolymphomatosis of the trigeminal nerve.

We report a case of a primary malignant lymphoma of the trigeminal nerve that was associated with facial pain. A 65-year-old man was examined at another hospital for unilateral facial pain. Carbamazepine was prescribed, but his symptoms did not improve. Magnetic resonance imaging (MRI) revealed swelling of the trigeminal nerve and a mass lesion in Meckel's cave. The patient was referred to our hospital at this point. Gadolinium-enhanced MRI and F18-Fluorodeoxyglucose-position emission tomography suggested a likely malignant tumour and a biopsy was performed. Histopathological examination showed diffuse a large B cell lymphoma. The patient was treated with high-dose methotrexate (HD-MTX) and radiotherapy. Despite responding well to initial treatment, the patient relapsed, with lymphoma observed throughout the body. He died of pneumonia 18 months after the initial diagnosis. Facial pain is a symptom that is commonly managed in general practice. If symptoms do not improve, repeated imaging studies, including contrast MRI, is warranted. This is the first reported case of primary neurolymphomatosis (NL) of the trigeminal nerve associated with facial pain alone. Furthermore, HD-MTX and radiotherapy may be considered for the management of primary NL of a cranial nerve.

Biopsy-proven case of Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system.

A 75-year-old woman was admitted to our hospital with rapidly deteriorating consciousness disturbance. She had a 7-year history of rheumatoid arthritis (RA), which had been treated with methotrexate (MTX) and prednisolone. Brain T2-weighted MRI showed diffuse high-intensity lesions in the cerebral subcortical and deep white matter, bilateral basal ganglia and thalamus. A cerebrospinal fluid examination revealed elevated protein levels and positive Epstein-Barr virus (EBV) DNA. Human immunodeficiency virus was negative. Brain biopsy showed perivascular lymphocytic infiltration in the parenchyma and meninx with EBV-encoded small RNA (EBER). Since this case did not fulfill the criteria for chronic active EBV infection (CAEBV), she was diagnosed with Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system. High-dose methylprednisolone, acyclovir, ganciclovir and foscarnet were not effective. Although EBV is a causative agent of infectious mononucleosis (IM), lymphomas and nasopharyngeal carcinomas, vasculitic pathology of the central nervous system with EBV reactivation in the elderly is rare. Immunosuppressive drugs such as steroids and MTX are widely used to treat autoimmune disorders, but may exacerbate the reactivation of EBV. This is the first case of biopsy-proven EBV-positive/HIV-negative vasculitis during the treatment of RA with MTX and steroids. This case indicates that EBV-associated vasculitis needs to be considered as a differential diagnosis of CNS vasculitis.

A case of pathology-proven neuromyelitis optica spectrum disorder with Sjögren syndrome manifesting aphasia and apraxia due to a localized cerebral white matter lesion.

A woman with Sjögren syndrome manifesting as aphasia with a left deep cerebral white matter lesion tested positive for anti-aquaporin 4 (AQP4) antibody. Open biopsy of the lesion revealed active demyelination with edematous changes and the preservation of most axons, indicating a non-necrotic demyelinating lesion. Immunostaining for AQP4 was diffusely lost, whereas the loss of glial fibrillary acidic protein immunostaining was limited but with highly degenerated astrocytic foot processes in perivascular areas. These results suggested neuromyelitis optica spectrum disorder (NMOSD) pathology rather than Sjögren-related vasculitis. Only cerebral cortical symptoms with a cerebral white matter lesion could be observed in NMOSDs.

Correlation of T1- to T2-weighted signal intensity ratio with T1- and T2-relaxation time and IDH mutation status in glioma.

The current study aimed to test whether the ratio of T1-weighted to T2-weighted signal intensity (T1W/T2W ratio: rT1/T2) derived from conventional MRI could act as a surrogate relaxation time predictive of IDH mutation status in histologically lower-grade gliomas. Strong exponential correlations were found between rT1/T2 and each of T1- and T2-relaxation times in eight subjects (rT1/T2 = 1.63exp-0.0005T1-relax + 0.30 and rT1/T2 = 1.27exp-0.0081T2-relax + 0.48; R2 = 0.64 and 0.59, respectively). In a test cohort of 25 patients, mean rT1/T2 (mrT1/T2) was significantly higher in IDHwt tumors than in IDHmt tumors (p < 0.05) and the optimal cut-off of mrT1/T2 for discriminating IDHmt was 0.666-0.677, (AUC = 0.75, p < 0.05), which was validated in an external domestic cohort of 29 patients (AUC = 0.75, p = 0.02). However, this result was not validated in an external international cohort derived from TCIA/TCGA (AUC = 0.63, p = 0.08). The t-Distributed Stochastic Neighbor Embedding analysis revealed a greater diversity in image characteristics within the TCIA/TCGA cohort than in the two domestic cohorts. The failure of external validation in the TCIA/TCGA cohort could be attributed to its wider variety of original imaging characteristics.

Multi-modal Mapping of the Face Selective Ventral Temporal Cortex-A Group Study With Clinical Implications for ECS, ECoG, and fMRI.

Face recognition is impaired in patients with prosopagnosia, which may occur as a side effect of neurosurgical procedures. Face selective regions on the ventral temporal cortex have been localized with electrical cortical stimulation (ECS), electrocorticography (ECoG), and functional magnetic resonance imagining (fMRI). This is the first group study using within-patient comparisons to validate face selective regions mapping, utilizing the aforementioned modalities. Five patients underwent surgical treatment of intractable epilepsy and joined the study. Subdural grid electrodes were implanted on their ventral temporal cortices to localize seizure foci and face selective regions as part of the functional mapping protocol. Face selective regions were identified in all patients with fMRI, four patients with ECoG, and two patients with ECS. From 177 tested electrode locations in the region of interest (ROI), which is defined by the fusiform gyrus and the inferior temporal gyrus, 54 face locations were identified by at least one modality in all patients. fMRI mapping showed the highest detection rate, revealing 70.4% for face selective locations, whereas ECoG and ECS identified 64.8 and 31.5%, respectively. Thus, 28 face locations were co-localized by at least two modalities, with detection rates of 89.3% for fMRI, 85.7% for ECoG and 53.6 % for ECS. All five patients had no face recognition deficits after surgery, even though five of the face selective locations, one obtained by ECoG and the other four by fMRI, were within 10 mm to the resected volumes. Moreover, fMRI included a quite large volume artifact on the ventral temporal cortex in the ROI from the anatomical structures of the temporal base. In conclusion, ECS was not sensitive in several patients, whereas ECoG and fMRI even showed activation within 10 mm to the resected volumes. Considering the potential signal drop-out in fMRI makes ECoG the most reliable tool to identify face selective locations in this study. A multimodal approach can improve the specificity of ECoG and fMRI, while simultaneously minimizing the number of required ECS sessions. Hence, all modalities should be considered in a clinical mapping protocol entailing combined results of co-localized face selective locations.

So-called bifocal tumors with diabetes insipidus and negative tumor markers: are they all germinoma?

BACKGROUND: The Delphi consensus statements on the management of germ cell tumors (GCTs) failed to reach agreements on the statement that the cases with (i) pineal and neurohypophyseal bifocal lesion, (ii) with diabetes insipidus, and (iii) with negative tumor markers can be diagnosed as germinoma without histological verification. To answer this, multicenter retrospective analysis was performed. METHODS: A questionnaire on clinical findings, histological diagnosis, and details of surgical procedures was sent to 86 neurosurgical and 35 pediatrics departments in Japan. RESULTS: Fifty-one institutes reported 132 cases that fulfilled the 3 criteria. Tissue sampling was performed in 91 cases from pineal (n = 44), neurohypophyseal (n = 32), both (n = 6), and distant (n = 9) lesions. Histological diagnosis was established in 89 cases: pure germinoma or germinoma with syncytiotrophoblastic giant cells in 82 (92.1%) cases, germinoma and mature teratoma in 2 cases, and granulomatous inflammation in 2 cases. Histological diagnosis was not established in 2 cases. Although no tumors other than GCTs were identified, 3 (3.4%) patients had non-germinomatous GCTs (NGGCTs). None of the patients developed permanent complications after endoscopic or stereotactic biopsy. Thirty-nine patients underwent simultaneous procedure for acute hydrocephalus without permanent complications, and hydrocephalus was controlled in 94.9% of them. CONCLUSION: All patients who fulfilled the 3 criteria had GCTs or granulomatous inflammation, but not other types of tumors. However, no fewer than 3.4% of the patients had NGGCTs. Considering the safety and the effects of simultaneous procedures for acute hydrocephalus, biopsy was recommended in such patients.

[A case of brain herniation due to delayed bone flap sinking].

Marked depression of the skin flap after external decompressive craniotomy, affecting the brain function, is known as sinking flap syndrome. However, to our knowledge, there have been no reports of delayed sinking of the entire bone flap after the procedure, inducing neurological symptoms. We encountered a patient with neurological symptoms due to sinking of the entire bone flap 15 years after the first operation. A 59-year-old male underwent clipping by craniotomy due to subarachnoid hemorrhage resulting from the rupture of a left internal carotid aneurysm 15 years earlier. He was discharged, but developed paresis in the right upper and lower limbs 6 months before symptom onset. CT showed sinking of the free bone flap, while MRI revealed left uncal herniation. After uncal resection and free flap fixation, the symptoms improved. This case confirmed the necessity of firm bone flap fixation at the time of cranial closure.

Treatment of Fourth Ventricle Arachnoid Cyst via Anterior Hone of Lateral Ventricle Using Flexible Endoscope.

BACKGROUND: Arachnoid cysts are common anomalies in the intracranial region. However, an intraventricular arachnoid cyst is rare, and occurrence within the fourth ventricle is especially uncommon; only 16 cases have been described in the literature. Arachnoid cysts in the fourth ventricle may cause obstructive hydrocephalus or cerebellar ataxia or cranial nerve palsy. Treatment of a fourth ventricular arachnoid cyst is complete or partial resection via a midline suboccipital approach. Recently, endoscopic fenestration has become the procedure of choice in the treatment of arachnoid cysts in supratentorial locations, but as yet there has been no report of treating a fourth ventricle arachnoid cyst using a flexible endoscope. CASE DESCRIPTION: We present the case of a 43-year-old man who suffered a recurrence of an arachnoid cyst in the fourth ventricle that had been partially excised 8 years previously using midline suboccipital craniectomy. Because of concerns of adhesions following the previous craniectomy, we decided to perform endoscopic treatment via the anterior horn of the lateral ventricle. CONCLUSIONS: As per our knowledge, this is the first case reporting the treatment of an arachnoid cyst of the fourth ventricle using a flexible endoscope via the anterior horn of the lateral ventricle. This method can be used to treat arachnoid cysts of the fourth ventricle.

Real-time spectrum quantification of tumor-related fluorescence during neurosurgery: A preliminary report.

OBJECTIVES: The fluorescent dye, 5-aminolevulinic acid (5-ALA), is currently applied for fluorescence-guided resections of high-grade gliomas. Present limitations of this technique are qualitative and subjective analyses, which show little of the background structures. This paper describes the intraoperative quantitative analysis of fluorescence intensity, hot-spot enhancement by frame averaging, and observation of surrounding structures by using 1000-nm lighting in real time. PATIENTS AND METHODS: A sample of diluted protoporphyrin IX (PpIX) in a bottle and 37 samples from nine patients with brain lesions were involved in this study. In this preliminary study, we determined appropriate conditions for image averaging and filters and selected the most sensitive spectrometer. In addition, we utilized a 1000-nm lighting system to visualize surrounding structures with no interference from PpIX fluorescence. RESULTS: The novel system permitted the real-time quantitative analysis of PpIX fluorescence in operative fields by illuminating structures with 1000-nm-lighting. The real-time quantification provided subjective evaluations for surgical decision-making. We found good correlations between the fluorescence and PpIX contents in brain tissue. Furthermore, 1000-nm lighting visualized the anatomical structures and PpIX fluorescence simultaneously. CONCLUSION: The combination of spectroscopy and a 1000-nm lighting system could enable surgeons to create a spectrogram of targets of interest while observing background structures. The spectrometer that we selected is highly sensitive to PpIX fluorescence and enables us to perform intraoperative real-time tissue mapping. By using a real-time system, we can perform quantitative and objective evaluations to achieve maximal tumor resection.

Clinical Impact and Implication of Real-Time Oscillation Analysis for Language Mapping.

BACKGROUND: We developed a functional brain analysis system that enabled us to perform real-time task-related electrocorticography (ECoG) and evaluated its potential in clinical practice. We hypothesized that high gamma activity (HGA) mapping would provide better spatial and temporal resolution with high signal-to-noise ratios. METHODS: Seven awake craniotomy patients were evaluated. ECoG was recorded during language tasks using subdural grids, and HGA (60-170 Hz) maps were obtained in real time. The patients also underwent electrocortical stimulation (ECS) mapping to validate the suspected functional locations on HGA mapping. The results were compared and calculated to assess the sensitivity and specificity of HGA mapping. For reference, bedside HGA-ECS mapping was performed in 5 epilepsy patients. RESULTS: HGA mapping demonstrated functional brain areas in real time and was comparable with ECS mapping. Sensitivity and specificity for the language area were 90.1% ± 11.2% and 90.0% ± 4.2%, respectively. Most HGA-positive areas were consistent with ECS-positive regions in both groups, and there were no statistical between-group differences. CONCLUSIONS: Although this study included a small number of subjects, it showed real-time HGA mapping with the same setting and tasks under different conditions. This study demonstrates the clinical feasibility of real-time HGA mapping. Real-time HGA mapping enabled simple and rapid detection of language functional areas in awake craniotomy. The mapping results were highly accurate, although the mapping environment was noisy. Further studies of HGA mapping may provide the potential to elaborate complex brain functions and networks.

Passive language mapping combining real-time oscillation analysis with cortico-cortical evoked potentials for awake craniotomy.

OBJECTIVE Electrocortical stimulation (ECS) is the gold standard for functional brain mapping; however, precise functional mapping is still difficult in patients with language deficits. High gamma activity (HGA) between 80 and 140 Hz on electrocorticography is assumed to reflect localized cortical processing, whereas the cortico-cortical evoked potential (CCEP) can reflect bidirectional responses evoked by monophasic pulse stimuli to the language cortices when there is no patient cooperation. The authors propose the use of "passive" mapping by combining HGA mapping and CCEP recording without active tasks during conscious resections of brain tumors. METHODS Five patients, each with an intraaxial tumor in their dominant hemisphere, underwent conscious resection of their lesion with passive mapping. The authors performed functional localization for the receptive language area, using real-time HGA mapping, by listening passively to linguistic sounds. Furthermore, single electrical pulses were delivered to the identified receptive temporal language area to detect CCEPs in the frontal lobe. All mapping results were validated by ECS, and the sensitivity and specificity were evaluated. RESULTS Linguistic HGA mapping quickly identified the language area in the temporal lobe. Electrical stimulation by linguistic HGA mapping to the identified temporal receptive language area evoked CCEPs on the frontal lobe. The combination of linguistic HGA and frontal CCEPs needed no patient cooperation or effort. In this small case series, the sensitivity and specificity were 93.8% and 89%, respectively. CONCLUSIONS The described technique allows for simple and quick functional brain mapping with higher sensitivity and specificity than ECS mapping. The authors believe that this could improve the reliability of functional brain mapping and facilitate rational and objective operations. Passive mapping also sheds light on the underlying physiological mechanisms of language in the human brain.

[Embolic complications associated with neurovascular intervention: prospective evaluation by use of diffusion-weighted MR imaging].

The most serious complication of neurovascular interventions is distal cerebral embolism. Diffusion-weighted MR imaging (DWI) appears to be the most sensitive technique for detecting early and small ischemic lesions. To evaluate the incidence and radiological features of embolic events associated with neuro-intervention, we applied DWI to screening for procedure-related ischemic lesions including silent embolisms. One hundred and thirty-seven patients who have received 154 neuro-interventional procedures were studied with DWI before and within 5 days after treatment. Imaging was performed, using single-shot echo-planar imaging with b value of 1000-1100 sec/mm2. DWI findings were classified into 5 groups by size and location of lesions: type 0 (n = 71), no lesions; I (n = 33), lesions in border-zone regions only; II (n = 9), lesions at perforator territories mainly; III (n = 29), small territorial lesions (< 5 mm); IV (n = 12), large territorial lesions (> or = 5 mm). DWI detected procedure-related lesions in 83 of 154 procedures (53.9%), 36 of which demonstrated new neurological symptoms during and/or after procedures. The parent artery occlusion for cerebral aneurysms had a higher incidence of symptomatic embolisms than other procedures. In 71 of 154 procedures (46.1%), DWI detected no lesions (type 0). Although type I was the most frequent pattern presented, it included few neurological symptoms. Type III often resulted in transient symptoms, and type II and IV tended to induce strokes. Because ischemic lesions detected by DWI were likely to arise in border-zone territories by parent artery occlusions, we considered that hypoperfusion as well as emboli were involved in the evolution of cerebral infarctions. Thus, DWI is a useful method to detect silent embolisms, and to determine the safety of neuro-intervention and the mechanism of embolic ischemia.

Functional localization of the supplementary motor area.

The supplementary motor area (SMA) is a key structure involved in behavioral planning and execution. Although many reports have indicated that SMA is organized somatotopically, its exact organization remains still unclear. This study aimed to functionally map SMA using functional magnetic resonance imaging (fMRI) and validate the fMRI-SMA by electrocortical stimulation (ECS) and postsurgical symptoms. Total 32 healthy volunteers and 24 patients participated in this study. Motor tasks were right and left finger tapping and language tasks included simple reading, lexical decision for presented words, and verb generating tasks. SPM8 was used to conduct individual and group analyses. In all subjects, the lexical decision task induced the greatest number of active fMRI pixels in SMA. fMRI during the language tasks showed anterior part of SMA compared to finger tapping tasks. We found an overlap spot with all different tasks in posterior part of SMA, which we termed SMA core. Six patients underwent awake craniotomy for ECS mapping for primary regions and SMA. During awake craniotomy, ECS to posterior part of SMA, which might involve the possible SMA core consistently, evoked both speech arrest and flaccid hemiparesis. The SMA mapping suggested posterior part of SMA might play more important roles in any executions, which might involve the SMA core.

Novel techniques of real-time blood flow and functional mapping: technical note.

There are two main approaches to intraoperative monitoring in neurosurgery. One approach is related to fluorescent phenomena and the other is related to oscillatory neuronal activity. We developed novel techniques to visualize blood flow (BF) conditions in real time, based on indocyanine green videography (ICG-VG) and the electrophysiological phenomenon of high gamma activity (HGA). We investigated the use of ICG-VG in four patients with moyamoya disease and two with arteriovenous malformation (AVM), and we investigated the use of real-time HGA mapping in four patients with brain tumors who underwent lesion resection with awake craniotomy. Real-time data processing of ICG-VG was based on perfusion imaging, which generated parameters including arrival time (AT), mean transit time (MTT), and BF of brain surface vessels. During awake craniotomy, we analyzed the frequency components of brain oscillation and performed real-time HGA mapping to identify functional areas. Processed results were projected on a wireless monitor linked to the operating microscope. After revascularization for moyamoya disease, AT and BF were significantly shortened and increased, respectively, suggesting hyperperfusion. Real-time fusion images on the wireless monitor provided anatomical, BF, and functional information simultaneously, and allowed the resection of AVMs under the microscope. Real-time HGA mapping during awake craniotomy rapidly indicated the eloquent areas of motor and language function and significantly shortened the operation time. These novel techniques, which we introduced might improve the reliability of intraoperative monitoring and enable the development of rational and objective surgical strategies.

Hydrocephalus due to diffuse villous hyperplasia of the choroid plexus.

An 8-month-old female presented with hydrocephalus caused by cerebrospinal fluid (CSF) overproduction due to bilateral choroid plexus enlargement, which was clinically diagnosed as diffuse villous hyperplasia of the choroid plexus, but differentiation from bilateral choroid plexus papilloma was difficult. She initially underwent ventriculoperitoneal shunt surgery, but developed marked retention of ascites. Therefore, the peritoneal end of the shunt was removed for external drainage, but excessive CSF (1,500 ml/day) was collected. Computed tomography and magnetic resonance imaging revealed marked symmetric enhancement of the choroid plexuses in the bilateral lateral ventricles. Thallium-201 chloride single-photon emission computed tomography showed pronounced uptake on both early and delayed images, and good washout. CSF examination revealed no abnormalities such as atypical cells, and a ventriculoatrial shunt was inserted, achieving good control of the hydrocephalus.

Effectiveness of brain hypothermia treatment in patients with severe subarachnoid hemorrhage: comparisons at a single facility.

The effectiveness of hypothermia treatment for severe subarachnoid hemorrhage (SAH) was evaluated at the same facility under the same director. A total of 187 patients with SAH, 67 admitted before the introduction of hypothermia treatment in May 1999 (early cases) and 120 treated thereafter (late cases), were transported to the National Cardiovascular Center and treated in the acute phase between November 1997 and September 2001. Brain hypothermia treatment was performed in 19 patients of the 120 late cases, 10 males and 9 females aged 33-72 years (mean 57. 6 years), treated by direct surgery in 15 and endovascular surgery in 4. The indications for hypothermia treatment were age of 75 years or younger, SAH due to rupture of a cerebral aneurysm, Japan Coma Scale score of 100 or higher, and initiation of treatment within 24 hours after the onset. The body core temperature was sustained at 34°C for 48 hours, rewarming was performed over 48 hours, and normothermia was maintained thereafter. The outcome, evaluated according to the modified Rankin scale (m-RS) on transfer to another hospital or after 3 months, was m-RS 3 in 1 patient, m-RS 4 in 4, m-RS 5 in 3, and death in 11. Before the introduction of hypothermia treatment (early period), 16 patients showed the indications for the treatment, and their outcomes were m-RS 3 in 2, m-RS 4 in 3, m-RS 5 in 2, and death in 9. Cerebral vasospasm was important as a prognostic factor, markedly deteriorating the outcome. Hyperthermia after therapeutic hypothermia induced brain swelling and markedly affecting the outcome. Brain hypothermia treatment did not improve the outcome of severe SAH compared with the period before its introduction. The emphasis in treating severe SAH should be placed on the maintenance of normothermia to prevent brain swelling and elimination of factors that may induce cerebral vasospasm, rather than interventional hypothermia for aggressive brain protection.