Symptomatic Developmental Venous Anomaly with an Increased β2-microglobulin Level in Cerebrospinal Fluid: A Case Report

Background: Gadolinium-enhanced magnetic resonance imaging (MRI) can be used to observe the progression of cerebral infarction, which sometimes mimics malignant brain tumors. While the β2-microglobulin (β2MG) level in blood plasma or cerebrospinal fluid (CSF) is useful for the diagnosis of malignant tumors or degenerative diseases, these results may create confusion regarding a definitive diagnosis, because it is not a specific marker. We present a rare case of symptomatic developmental venous anomaly (DVA), accompanied by transient, irregular, enhanced cerebral lesions and elevated β2MG in the CSF. Case Description: A 56-year-old woman developed dysarthria and underwent MRI, which revealed a right frontal hyperintense area around a previous lesion on diffusion-weighted imaging (DWI). She was treated based on the tentative diagnosis of an ischemic cerebrovascular event, and symptoms subsided in 3 days. MRI on day 7 revealed an enlargement of the hyperintense area on DWI. Post-gadolinium MRI showed multiple, enhanced patchy areas in the right frontal lobe and an abnormally large vein connected to dilated medullary venules, indicating DVA. Magnetic resonance angiography showed no stenosis or arterial occlusion. The β2MG level in the CSF was elevated at 2,061 μg/l, and a differential diagnosis from malignant tumor was required. However, MRI on day 23 revealed total disappearance of the enhanced lesions and a decrease in the high intensity area on DWI. Considering the clinical course, the DVA was symptomatic because of the perfusion disturbance. Conclusion: Careful evaluation is necessary when considering the associated pathologies and potential complications of DVA if detected near a gadolinium-enhanced lesion

Solitary Cranial Langerhans Cell Histiocytosis : Two case reports

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as a unisystem (unifocal or multifocal) or multisystem disease. We describe the clinical and histologic spectrum of LCH of the orbit and skull in our two cases. Both cases had unifocal erosive skull lesions with a history of trauma. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. LCH has an excellent prognosis when treated with surgical resection, steroids and radiotherapy or chemotherapy. One of our patients is disease free at 7 year follow-up and one patient had regression of lesion on follow-up

Zygomatic Osteotomy for Resection of Medial Temporal Cavernous Angioma in Dominant Hemisphere after Subdural Grid Electroencephalographic Study

We report the treatment of a patient suffering from intractable epilepsy caused by a large cavernous angioma in the hippocampus of his dominant hemisphere. Surgical removal of a lesion and epileptic focus located in the surrounding brain tissue significantly improves seizure conditions. However, total removal of a large cavernous angioma in the medial temporal structure of dominant hemisphere is occasionally difficult because of the depth of the lesion and its contiguity with eloquent brain structures. Furthermore, when an intracranial electrocorticogram with grid electrodes is adopted, swelling of soft tissue caused by the initial operation for the installation of the grid electrodes narrows the operative view obtained by conventional frontotemporal craniotomy. We added a zygomatic osteotomy to the operative procedure for the present patient, and this enabled us to push soft tissue down and away from the operative field to provide a wider operative corridor through which total removal of the lesion and epileptic focus was accomplished. The patient has had a favorable postoperative course without suffering any seizure for 4 years

Treatment of Meningioma in a Patient with Congenital Immunodeficiency Disorder

We treated a patient with meningioma suffering from a congenital immunodeficiency syndrome. The patient was diagnosed with common variable immunodeficiency (CVID) in youth, and had suffered from repeated infections, especially in the respiratory tract, requiring continuous treatment with antibiotics. Imaging revealed a tumor located in the left frontal region with a broad attachment suggesting meningioma in the convexity dura mater. Gammaglobulin was infused intravenously preceding the operation and serum gammaglobulin was controlled at over 400 mg/dl and then a Simpson grade 1 operation was performed. The patient did not show any complications in infectious susceptibility, and there was no recurrence of the tumor in the 5 years following the operation. The surgical risk for CVID patients in the neurosurgical field is still not clear, but we could maintain the condition of the patient by controlling the serum gammaglobulin level in the perioperative period

: Cerebral metabolism in STOP KO mice

International audienceIn mice, deletion of the STOP protein leads to subtle anatomic changes and induces depleted synaptic vesicle pools, impaired synaptic plasticity, hyperdopaminergy, and major behavioral disorders alleviated by neuroleptics, hence leading to a schizophrenic-like phenotype. In this study, we applied the quantitative autoradiographic [(14)C]2-deoxyglucose technique to study to what extent the basal rate of cerebral glucose utilization in STOP-knockout (STOP-KO) mice occurs in regions where metabolic changes have been reported in schizophrenic patients. Studies were performed on wild-type, heterozygous, and homozygous STOP-KO mice (7-8 per group). Mice were implanted with femoral artery and vein catheters, and cerebral glucose utilization was quantified over 45 min. Compared with that in wild-type mice, glucose utilization in STOP-KO mice was significantly increased in the olfactory cortex, ventromedial and anterolateral hypothalamus, ventral tegmental area, and substantia nigra pars compacta. Nonsignificant increases, ranging between 9% and 19%, were recorded in the whole auditory system, CA1 pyramidal cell layer, and dorsal raphe. Glucose utilization was also significantly increased in heterozygous mice compared with that in wild-type mice in olfactory cortex. These data might reflect hyperdopaminergic activity, olfactory deficits, and sleep disturbances in STOP-KO mice that have also been reported in schizophrenic patients

Voltage-dependent calcium channel abnormalities in Hippocampal CA3 neurons of spontaneously epileptic rats

Purpose: Hippocampal CA3 neurons of spontaneously epileptic rats (SER; zi/zi, tm/tm), which show both absence-like seizures and tonic convulsions, exhibit a long-lasting depolarization shift with repetitive firing by a single stimulation of mossy fibers. Therefore, a whole cell patch clamp study using acutely dissociated hippocampal CA3 neurons from SER was performed to elucidate whether or not such abnormal excitability was due to abnormalities in voltage-dependent Ca2+ channels (VDCCs). Methods: Hippocampal CA3 neurons were acutely dissociated with enzymatic and mechanical treatments. In a voltage-clamp mode with whole-cell recording, depolarizing step pulses were applied to induce Ca2+ currents in the presence of tetrodotoxin and tetraethylammonium. Results: The threshold level of the Ca2+ current induced by depolarizing pulses was found to be lower in hippocampal CA3 neurons of SER compared with that of control Wistar rats. In addition, the Ca2+ current peak amplitude was greater, and decay of the current was weaker in CA3 neurons of SER than those of normal Wistar rats. Conclusion: These findings suggest that enhancements of Ca2+ influx into hippocampal CA3 neurons due to the easier activation properties of VDCCs, as well as a decrease in decay, are involved in SER epileptic seizures

Secondary anaplastic oligodendroglioma after cranial irradiation: a case report

Secondary brain tumors rarely arise after cranial irradiation; among them, meningiomas and glioblastomas are the most common and secondary oligodendroglial tumors the most rare. We present a 48-year-old man who developed an oligodendroglial tumor 38 years after receiving 50 Gy of cranial irradiation to a pineal tumor. He underwent gross total removal of a calcified, ring-enhanced mass in the right temporal lobe. The tumor was histologically diagnosed as anaplastic oligodendroglioma. Our review of previously reported secondary oligodendroglial tumors that developed after cranial irradiation revealed that these rare tumors arose after low-dose cranial irradiation or at the margin of a field irradiated with a high dose. We suggest that secondary oligodendroglial tumors arising after cranial irradiation are more aggressive than primary oligodendrogliomas