Summary Objective This retrospective study was designed to assess the impact of corpus callosotomy (CC) in patients with intractable West syndrome (WS) without lesions on magnetic resonance imaging (MRI). Methods This study involved 56 patients with WS who underwent CC between January 2000 and December 2014. Seizure outcomes and changes in psychomotor development were analyzed. Results Mean age at the onset of epilepsy and at the time of CC was 5.1 and 22.6 months, respectively. Mean duration of epilepsy before CC was 17.6 months. Video‐electroencephalography (EEG) monitoring showed bilateral ictal and interictal abnormalities before CC. Mean follow‐up duration was 36.6 months. At final follow‐up, seizure outcomes after CC were seizure‐free in 18 patients (32.1%), excellent (E: >80% reduction in seizure frequency) in 15 (26.8%), good (G: >50% reduction) in 10 (17.9%), and poor (P: <50% reduction) in 13 (23.2%). Epileptic spasms (ES) were eliminated in 24 patients (42.9%). However, tonic seizure (TS) outcomes were poor (P < 0.05). Of preoperative predictive factors related to seizure outcome, developmental delay before epilepsy onset correlated with poor outcome (P < 0.05). One year post‐CC, 6 patients (10.7%) had no epileptic abnormality on EEG, 19 (33.9%) had lateralized epileptic abnormalities, and 31 (55.4%) had bilateral asynchronous epileptic abnormalities. All patients without epileptic discharge achieved seizure freedom. Fifteen of 19 (78.9%) patients in the lateralized group and 12 of 31 (38.7%) in the bilateral asynchronous group had worthwhile outcomes (F + E). The patterns of EEG changes after CC correlated with seizure outcome (P < 0.01). Progressive declines in developmental quotient were prevented in patients with worthwhile outcomes. Significance CC represents an important therapeutic option for patients with WS without resectable MRI lesions. Transcallosal seizure bilateralization is critical for bilateral ES generation. Early identification of potential CC candidates and surgical intervention are important for better seizure control and cognitive capacity preservation before severe developmental delay development.
We report a case of brain surface angiocentric glioma in a 6-year-old Japanese boy with medically intractable partial epilepsy. MRI showed somewhat ill-defined high-signal lesions on fluid-attenuated inversion recovery and T2-weighted imagings of the right occipitoparietal cortex. At surgery, a yellowish tumor was localized in the superficial cortex. Histologically, the tumor was predominantly composed of elongated astrocytic cells forming rings around blood vessels. Tumor cells circumferential to vessels predominanted in low cellurarity areas, whereas radial alignment with perivascular pseudorosettes was observed in more cellular regions. These perivascular pseudorosettes closely resembled those of ependymoma. The tumor cells showed variable cytoplasmic immunoreactivity with GFAP. These findings were more likely monomorphous angiocentric glioma, which was first described by Wang et al. in 2005. However, in our case the tumor had a small foci of polymorphous appearance and a comparatively high MIB-1 labeling index (8%). Therefore, the present case may be an atypical form of monomorphous angiocentric glioma. However, no de novo anaplastic monomorphous angiocentric glioma similar to our case has yet been reported in the literature. It remains to be determined whether the behavior of monomorphous angiocentric glioma is an example of benign biological characteristics or whether it more closely resembles a low-grade malignant tumor.
Characterization and isolation of a large population of cells are indispensable procedures in biological sciences. Flow cytometry is one of the standards that offers a method to characterize and isolate cells at high throughput. When performing flow cytometry, cells are molecularly stained with fluorescent labels to adopt biomolecular specificity which is essential for characterizing cells. However, molecular staining is costly and its chemical toxicity can cause side effects to the cells which becomes a critical issue when the cells are used downstream as medical products or for further analysis. Here, we introduce a high-throughput stain-free flow cytometry called in silico-labeled ghost cytometry which characterizes and sorts cells using machine-predicted labels. Instead of detecting molecular stains, we use machine learning to derive the molecular labels from compressive data obtained with diffractive and scattering imaging methods. By directly using the compressive ‘imaging’ data, our system can accurately assign the designated label to each cell in real time and perform sorting based on this judgment. With this method, we were able to distinguish different cell states, cell types derived from human induced pluripotent stem (iPS) cells, and subtypes of peripheral white blood cells using only stain-free modalities. Our method will find applications in cell manufacturing for regenerative medicine as well as in cell-based medical diagnostic assays in which fluorescence labeling of the cells is undesirable.
Patients with rheumatoid arthritis (RA), especially those treated with methotrexate (MTX), might have an increased risk of lymphoproliferative disorders that are associated with Epstein-Barr virus (EBV). We describe a case of EBV-associated central nervous system (CNS) lymphoma (diffuse large B-cell lymphoma) in a patient with RA on a short course of MTX treatment. The neoplastic cells express the B-cell surface markers (CD20, Pax-5 and CD30), and EBV-encoded RNA was demonstrated by in situ hybridization. The patient's lymphoma did not recur for the 8-year follow-up period after the tumor resection and cessation of MTX. MTX may promote EBV-positive CNS lymphoma in RA patient due to its immunosuppressive properties as well as reactivating latent EBV infection.
Objective We analyzed the features of fast oscillations (FOs) and connectivity in hypsarrhythmia to identify biomarkers for predicting seizure outcomes after total corpus callosotomy (TCC) in children with pharmacoresistant infantile spasms (IS). We hypothesize that the power of FOs and connectivity of slow waves in hypsarrhythmia would indicate the prognosis of IS. Method We retrospectively identified 42 children with pharmacoresistant IS who underwent TCC from 2009 to 2014 at Nagasaki Medical Center. We collected preoperative hypsarrhythmia for 200 seconds from each child. Children were categorized into three groups with interictal epileptic discharges on EEG at 6 months after TCC: group A, no epileptic discharge; group B, lateralized epileptic discharges; and group C; bilateral epileptic discharges. We analyzed spectral power and phase synchronization in preoperative hypsarrhythmia among the three groups. Results We found 10 children in group A, 10 children in group B, and 22 children in group C. All group A and 1 in group B achieved seizure freedom after TCC. Six (67%) of 9 group B children who underwent further surgeries achieved seizure freedom. Ten (45%) of group C children had seizure reduction >50% after TCC, and 13 (87%) of 15 children who underwent further surgeries had residual seizures. The clinical profiles of the three groups did not differ significantly. The power of FOs (≥45 Hz) in hypsarrhythmia was significantly stronger in group C at the midline and temporal regions than in groups B and A (P = .014). The connectivity of theta (4‐9 Hz) and FOs (29‐70 Hz) tended to increase in group C, compared with the increased connectivity of 1‐2 Hz in group A (P = .08). Significance The increased power and connectivity of FOs in hypsarrhythmia may correlate with pharmacoresistant and surgically resistant seizures in IS. The existence and connectivity of FOs are associated with unilateral/bilateral cortical epileptogenicity in hypsarrhythmia. Prominent slow waves and connectivity without FOs might correlate with seizure freedom after TCC. Modulation of the callosal system with subcortical/cortical epileptic discharges might play a role in generating hypsarrhythmia and IS.
As a cause of acute clinical deterioration after aneurysmal clipping, CSF hypovolemia is likely under-recognized, and may actually be misdiagnosed as vasospasm or brain swelling. We should always take the etiology of CSF hypovolemia into consideration, and especially pay attention in patients with pneumocephalus and subdural fluid collection alongside brain sag on computed tomography. These patients are at higher risk developing of pressure gradients between their cranial and spinal compartments, and therefore, brain sagging after LD, than after ventricular drainage. We should be vigilant to strictly manage LD so as not to produce high pressure gradients.
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