[Ruptured Spinal Arteriovenous Malformation Mimicking Bacterial Meningitis:A Case Report].

A 23-year-old woman was referred to our hospital with progressive headache and back pain. Cerebrospinal fluid(CSF)analysis revealed hypoglycorrhachia and pleocytosis. However, imaging studies did not reveal evidence of subarachnoid hemorrhage(SAH), and she was diagnosed with bacterial meningitis. CSF analysis repeated after antibiotic treatment continued to show evidence of blood. Spinal SAH was suspected, and MRI revealed a spinal arteriovenous malformation(AVM). Spinal digital subtraction angiography revealed the nidus and a feeder aneurysm, which was implicated as the source of bleeding;therefore, she was diagnosed with spinal SAH secondary to spinal intramedullary AVM(SAVM). She underwent endovascular treatment for the spinal aneurysm and was discharged without worsening neurological symptoms. SAVMs presenting without neurological findings and/or evidence of bleeding on plain CT images are diagnostically challenging;notably, findings on CSF analysis could mimic those observed in cases of infectious disease. We should cite vascular diseases such as SAVM.

[A Case of Pediatric C1 Dysplasia with Ruptured Aneurysm in Collateral Plexiform Arterial Network].

A 3-year-old girl was admitted to our hospital with symptoms including headache, nausea, and vomiting. Head CT scan showed subarachnoid hemorrhage in the right carotid cistern. Digital subtraction angiography revealed right internal caortid artery(ICA)malformation at the C1 segment with collateral plexiform arterial network. The right ICA branched into posterior communicating artery and anterior choroidal artery(AChoA)and the ICA was decreased in caliber. The distal portion of the C1 segment of the ICA continued to the collateral plexiform arterial network, forming a saccular aneurysm. The plexiform arterial network connected to the right AChoA and the anterior communicating artery and continued to the distal portion of the right M1 segment. Right cervical carotid artery was normal. There was no transdural collateral flow from the right external carotid artery. Genetic analysis of a variant of RING finger protein 213 was negative. We diagnosed this patient with C1 dysplasia. We performed coil embolization for the aneurysm. The patient was discharged without any neurological deficit. Four months after the surgery, recurrence of the aneurysm was observed. We suspected that the aneurysm was formed due to hemodynamic mechanism and vulnerability of the collateral plexiform arterial network.

A case report of CIC-rearranged undifferentiated small round cell sarcoma in the cerebrum.

CIC-rearranged undifferentiated small round cell sarcoma (CIC-rearranged USRCS) is a recently established type of Ewing-like small round cell sarcomas, characterized by CIC gene rearrangement, most commonly CIC-DUX4 fusion. This report presents the second case of CIC-rearranged USRCS arising primarily in the cerebrum. A 64-year-old otherwise healthy woman presented with a 1 × 1 cm sized hemorrhagic subcortical tumor in the left temporo-parietal lobe. The tumor repeatedly recurred, and the patient underwent three surgeries, chemotherapy with doxorubicin and ifosfamide, and radiotherapy, as well as gamma knife surgery. Systemic examination revealed no other extracranial masses. Imprint cytology revealed small to moderate-sized round-to-ovoid tumor cells with mild pleomorphism and variations in size and shape. The nuclei contained finely granular chromatin, and some had easily-recognizable nucleoli. The tumor exhibited a mainly cytoplasmic pattern of CD99 immunostaining, rather than a diffuse membranous pattern. The tumor also exhibited diffuse positivity for calretinin and p16, as well as partial positivity for WT1 (nuclear and cytoplasmic staining pattern) and D2-40. FISH assessment showed CIC split signals. In conclusion, CIC-rearranged USRCSs can occur primarily in the cerebrum. It would be impossible to diagnose them through cytology alone, but cytology would be useful to rule out other small round cell brain tumors including gliomas, lymphomas, carcinomas, and germinoma. Immunohistochemical analysis including tests for CD99, calretinin, and WT1 would help to suggest CIC-rearranged USRCSs and distinguish them from Ewing sarcomas. Additionally, immunohistochemistry for p16 might be useful in the diagnosis. Diagn. Cytopathol. 2016;44:828-832. © 2016 Wiley Periodicals, Inc.

[Usefulness and Problems of Intraoperative Monitoring for Unruptured Aneurysm Surgery with the Motor Evoked Potential].

It has been pointed out that the motor evoked potential(MEP)with a subdural electrode is useful in the intraoperative monitoring for unruptured aneurysm surgery. However, in some cases, we experienced postoperative ischemic complications despite evaluating the motor function via MEP monitoring. Herein, we have reported the usefulness and problems of intraoperative monitoring with MEP to evaluate brain dysfunction caused by insufficiency of cerebral blood flow. Out of 279 aneurysm surgery procedures, we performed MEP monitoring in 142 cases and successfully recorded in 126 cases. We compared the ischemic complication rate of the group for which MEP was monitored with that of the group for which MEP was not monitored. The whole ischemic complication rate was decreased in the group that underwent MEP monitoring. Thus, it was suggested that MEP monitoring was useful for avoiding ischemic complications. In internal carotid artery aneurysms, the amplitude of MEP changed and recovered in 2 cases and disappeared in one case. In anterior cerebral artery aneurysms, the amplitude of MEP changed and recovered in 2 cases. In middle cerebral artery aneurysms, the amplitude of MEP changed and recovered in 5 cases. We could avoid ischemic complications by intraoperative MEP monitoring in many cases. However, in some cases, we found ischemic complications that were not detected by MEP monitoring with a subdural electrode. In these cases, transcranial stimulation in combination with subdural electrode might be effective in avoiding ischemic complications that might occur after dural closure.

CIC-rearranged Sarcomas: A Study of 20 Cases and Comparisons With Ewing Sarcomas.

The CIC gene rearrangement exists in a subset of small round cell sarcomas. As the nosologic relationship of these sarcomas to Ewing sarcomas remains undetermined, we examined 20 CIC-rearranged sarcomas to compare their clinicopathologic features with those of Ewing sarcomas. The CIC-rearranged sarcomas were from a group of 14 men and 6 women with a median age of 24.5 years. The primary tumor sites included the limbs, trunk wall, internal trunk, lung, cerebrum, and pharynx. A comparison of the demographic and clinical characteristics of the 20 patients with CIC-rearranged sarcomas with those of the 53 near-consecutive patients with EWSR1-rarranged Ewing sarcomas showed that there were no differences with respect to their ages and sexes. Although none of the CIC-rearranged sarcomas arose in the bone, 40% of the Ewing sarcomas primarily affected the skeleton. The overall survival of patients with Ewing sarcomas was significantly better than that for patients with CIC-rearranged sarcomas. A histologic comparison of the CIC-rearranged sarcomas with 20 EWSR1-rearranged Ewing sarcomas showed significantly higher degrees of lobulation, nuclear pleomorphism, the prominence of the nucleoli, spindle cell elements, and myxoid changes in the CIC-rearranged sarcomas. Distinguishing immunohistochemical features included heterogenous CD99 reactivity, nuclear WT1 expression, and calretinin expression in the CIC-rearranged sarcomas and NKX2.2 expression in the Ewing sarcomas. CIC-rearranged sarcomas are distinct from Ewing sarcomas clinically, morphologically, and immunohistochemically, and they should be considered a separate entity rather than being grouped within the same family of tumors.

Carotid angioplasty with stenting for chronic internal carotid artery occlusion: technical note.

INTRODUCTION: Carotid angioplasty with stenting (CAS) is becoming accepted as an effective and reliable treatment option for severe carotid artery stenosis. However, it is rarely applied for carotid occlusion, especially in its chronic stage. We report our experience of CAS for chronic internal carotid artery occlusion representing compromised cerebral blood flow using various protection methods. METHODS: A 77-year-old woman, who was already diagnosed with severe left internal carotid artery stenosis, suddenly had right hemiparesis and aphasia. At that time, she was treated conservatively because her neurological status was quite good, in spite of left carotid artery occlusion. Her symptoms improved in the short term, except slight aphasia, but deteriorated again 18 days from the onset, and magnetic resonance imaging (MRI) showed new ischemic lesions. CAS was then performed for the occluded carotid artery on the 23rd day from the first onset. RESULTS: Using the proximal protection technique, the occluded lesion was crossed carefully with a microguidewire. Stents were also placed successfully with the distal protection technique. The occluded carotid artery was completely recanalized without any unfavorable events or neurological deterioration. CONCLUSION: In this patient, CAS was successfully to treat chronic carotid artery occlusion. These procedures and techniques are reviewed and discussed.

Suppression of secondary generalization of limbic seizures by stimulation of subthalamic nucleus in rats.

OBJECT: Deep brain stimulation (DBS) of subcortical nuclei such as the subthalamic nucleus (STN) or the substantia nigra pars reticulata (SNR) may provide an alternative therapy for intractable epilepsy. The authors attempted to evaluate the antiepileptic effects of DBS to these structures in an experimental seizure model. METHODS: Three groups of rats were prepared. In the first two groups, the rats underwent unilateral implantation of stimulation electrodes in the STN (six rats) or the SNR (six rats). A control group received no electrodes (six rats). Kainic acid (KA) was systemically administered to induce limbic seizures, which started with focal seizures and became generalized secondarily. High-frequency electrical stimulation of the STN or SNR was begun immediately after KA administration, and changes on electroencephalography (EEG) and the magnitude of clinical seizures were evaluated. Results showed that STN stimulation significantly reduced the duration of generalized seizures on EEG, although the total duration of seizures (generalized plus focal seizures) was unchanged. The duration of focal seizures on EEG was prolonged by STN DBS, a result possibly due to the suppression of secondary generalization. In addition, STN DBS reduced the severity of clinical seizures. On the other hand, stimulation of the SNR demonstrated no effect. CONCLUSIONS: Unilateral STN DBS showed significant suppression of the secondary generalization of limbic seizures. Note, however, that SNR DBS was less effective, which implies that in addition to the nigral control of the epilepsy system, another antiepileptic mechanism such as antidromic stimulation of the corticosubthalamic pathway should be considered.

Pilomotor seizures in mesial temporal lobe epilepsy: a case confirmed by intracranial EEG.

We report the first case in which intracranial EEG was used to confirm pilomotor seizures of mesial temporal origin. A 41-year-old man who had pilomotor seizures in the left arm in addition to complex partial seizures was admitted for presurgical evaluation. Intracranial EEG revealed ictal discharges in the left mesial temporal area during left pilomotor seizures. Moreover, electrical stimulation of the left mesial temporal area induced a habitual pilomotor seizure. After anteromesial temporal lobectomy, the patient became seizure-free. The localizing and lateralizing value of pilomotor seizures are discussed.