A Spontaneous Extracranial Internal Carotid Artery Dissection with Autosomal Dominant Polycystic Kidney Disease: A Case Report and Literature Review.

Background and Objectives: Non-cystic manifestation of autosomal dominant polycystic kidney disease (ADPKD) is an important risk factor for cerebral aneurysms. In this report, we describe a rare spontaneous internal carotid artery (ICA) dissection in a patient with ADPKD. Observations: A 38-year-old woman with a history of ADPKD and acute myocardial infarction due to coronary artery dissection experienced severe spontaneous pain on the left side of her neck. Magnetic resonance imaging (MRI) revealed a severe left ICA stenosis localized at its origin. Carotid plaque MRI showed that the stenotic lesion was due to a subacute intramural hematoma. Close follow-up by an imaging study was performed under the diagnosis of spontaneous extracranial ICA dissection, and spontaneous regression of the intramural hematoma was observed uneventfully. Conclusions: When patients with a history of ADPKD present with severe neck pain, it is crucial to consider the possibility of a spontaneous ICA dissection. A carotid plaque MRI is beneficial in the differential diagnosis. Conservative management may benefit patients without ischemic symptoms.

Unusual course of the vagus nerve passing anterior to the internal carotid artery during carotid endarterectomy.

BACKGROUND: Carotid endarterectomy (CEA) is a conventional surgical technique to prevent ischemic stroke and the effectiveness for advanced lesions is established in many large studies. The vagus nerve is one of the cranial nerves that we usually encounter during CEA manipulation, which is identified as located posterior to the vessels in a position posterolateral to the carotid artery and posteromedial to the internal jugular vein. CASE DESCRIPTION: We experienced an extremely rare case of the vagus nerve passing anterior to the internal carotid artery during CEA. CONCLUSION: We should be careful not to accidentally cut off because the variation of the vagus nerve can be mistaken for an ansa cervicalis. A delicate and complete dissection to understand the variation of the vagus nerve is crucial to minimize the risk of cranial nerve injury during CEA.

[Asymptomatic Aplastic or Twig-Like Middle Cerebral Artery Associated with Unruptured Cerebral Aneurysms at the Origin (A1) of a Collateral Artery and the Anterior Communicating Artery: A Case Report with Multiple Intracranial Atherosclerotic Stenoses].

We report a rare case of asymptomatic aplastic or twig-like middle cerebral artery (Ap/T-MCA) with small unruptured aneurysms at the origin (A1) of the anomalous collateral artery containing plexiform (twig-like) networks and in the anterior communicating artery. In Ap/T-MCA, other cerebrovascular systems are usually considered to exhibit normal findings not associated with atherosclerosis. At first admission, atherosclerotic M1 occlusion was suspected because of the patient's risk factors and multiple stenoses in major intracranial arteries. Cerebral blood flow (CBF) studies revealed reduction of resting CBF and vascular reserve in the ipsilateral MCA territory. After 5 years, a diagnosis of Ap/T-MCA was reached based on detailed image examination and intraoperative findings during aneurysm clipping in combination with extracranial-intracranial (EC-IC) bypass. It should be noted that atherosclerosis can coexist with Ap/T-MCA, which is considered a congenital anomaly in which bleeding often occurs due to a ruptured aneurysm within the fragile collateral vessels. In previous reports, A1 aneurysms at the origins of the collateral arteries ruptured even when they were small. Aggressive radical surgery using EC-IC bypass can be considered for the treatment of unruptured aneurysms associated with Ap/T-MCA, but further research is needed. (Received September 14, 2020; Accepted November 4, 2020; Published 1 April, 2021).

A1 Segment Unruptured Aneurysm of Persistent Primitive Olfactory Artery Coexisted with Accessory Middle Cerebral Artery.

A persistent primitive olfactory artery (PPOA) is a rare anomaly of anterior cerebral artery (ACA), which generally arises from the internal carotid artery (ICA), runs along the olfactory tract, and makes a hairpin bend to supply the territory of the distal ACA. PPOA is also associated with cerebral aneurysms. An accessory MCA is a variant of the middle cerebral artery (MCA) that arises from either the proximal or distal portion of the A1 segment of the ACA, which runs parallel to the course of the MCA and supplies some of the MCA territory. We experienced a rare case of coexistence of PPOA with an unruptured aneurysm and accessory MCA. Three-dimensional computed tomographic angiography (3D-CTA) has an excellent picture of the spatial relationship of the surrounding bony and vascular structure.

Carotid Endarterectomy Using Lone Star Retractor System.

Cervical carotid disease is typical atherosclerosis, which is responsible for ischemic stroke. The effectiveness of carotid endarterectomy (CEA) for advanced carotid stenosis has been established in many large studies, and CEA is the gold standard in surgical treatment. On the other hand, endovascular carotid artery stenting (CAS) has become increasingly popular recently. It is very important to avoid any complications to maintain the effectiveness of CEA. The retractor device is important for the exposure of carotid arteries and for the safe surgical manipulation. We have started to use lone star retractor system (LSRS) to deploy the surgical field. LSRS provides the usability to handle and a shallower surgical field without the disturbance of surgical manipulation. And it can facilitate exposure of the distal internal carotid artery because surgeon can retract freely in whole circumference by towing with moderate strength. LSRS may bring the smoother and easier surgical manipulations in CEA.

Current status of a helicopter transportation system on remote islands for patients undergoing mechanical thrombectomy.

BACKGROUND: Mechanical thrombectomy (MT) is standard treatment for acute ischemic stroke (AIS) with large-vessel occlusion within 6 h of symptom onset to treatment initiation (OTP). Recent trials have extended the therapeutic time window for MT to within 24 h. However, MT treatment remains low in remote areas. Nagasaki Prefecture, Japan has many inhabited islands with no neurointerventionalists. Our hospital on the mainland is a regional hub for eight island hospitals. We evaluated clinical outcomes of MT for patients with AIS on these islands versus on the mainland. METHODS: During 2014-2019, we reviewed consecutive patients with AIS who received MT at our hospital. Patients comprised the Islands group and Mainland group. Patient characteristics and clinical outcomes were compared between groups. RESULTS: We included 91 patients (Islands group: 15 patients, Mainland group: 76 patients). Seven patients (46.7%) in the Islands group versus 43 (56.6%) in the Mainland group achieved favorable outcomes. Successful recanalization was obtained in 11 patients (73.3%) on the islands and 67 (88.2%) on the mainland. The median OTP time in the Islands was 365 min. In both the Islands and Mainland groups, the OTP time and successful recanalization were associated with functional outcome. The modified Rankin Scale (mRS) score at 90 days ≤2 was obtained in two patients and mRS = 3 in four patients among eight patients with OTP time >6 h. CONCLUSIONS: Few patients with AIS on remote islands have received MT. Although patients who underwent MT on the islands had longer OTP, the clinical outcomes were acceptable. OTP time on remote islands must be shortened, as this is related to functional outcome. In some cases with successful recanalization, a favorable outcome can still be obtained even after 6 h. Even if OTP exceeds 6 h, it is desirable to appropriately select patients and actively perform MT.

Vasospasm and Hydrocephalus Following Subarachnoid Hemorrhage Are Less Frequent in Coil Embolization Than in Clipping.

Objective: The effects of treatment methods for ruptured aneurysms on the incidence of vasospasm and normal pressure hydrocephalus (NPH) following subarachnoid hemorrhage (SAH) are controversial. We retrospectively examined the Nagasaki SAH registry data, and the complication rates of symptomatic vasospasm and NPH were analyzed based on the treatment methods. Methods: Between January 2015 and December 2017, 800 SAH patients were registered from 18 hospitals, and their age, sex, World Federation of Neurological Societies (WFNS) grade, Fisher group, size and location of cerebral aneurysms, treatment methods, incidence of symptomatic vasospasm and shunt-dependent hydrocephalus, and prognosis (discharge or 3 months later) were retrospectively analyzed. The effects of treatment methods for the ruptured aneurysm on the incidence of symptomatic vasospasm and shunt-dependent hydrocephalus were then statistically analyzed. Results: The mean age was 66.2 years old. There were 245 (30.6%) male patients and 555 (69.3%) female patients. Cerebral aneurysms were identified in 708 patients (87.5%) and surgical treatments were performed for 620. Neck clipping was employed in 416 patients (67.1%) and coil embolization was employed in 180 (29.0%). Symptomatic vasospasm developed in 118 (28.4%) in the clipping group and 30 (16.7%) in the coiling group (P = 0.0024). NPH developed in 148 (35.6%) in the clipping group and 42 (23.3%) in the coiling group (P = 0.0032). Vasospasm was listed as a major factor for an unfavorable outcome in 23 patients (8.9%) and as a minor factor in 33 (13.3%). NPH was listed as a major factor for an unfavorable outcome in 19 patients (3.5%) and as a minor factor in 46 (18.5%). Conclusions: The multicenter registry study demonstrated lower incidences of both symptomatic vasospasms and NPH in the coiling group than in the clipping group. This superiority may result in better outcomes in the coiling group.

[Orbital Compartment Syndrome Perhaps Secondary to Intra-Orbital Fluid Retention and Orbital/Palpebral Emphysema Following Frontotemporal Craniotomy for an Unruptured Cerebral Aneurysm:A Case Report].

A 58-year-old woman underwent left frontotemporal craniotomy for clipping of an unruptured cerebral aneurysm. A small defect was accidentally created in the orbital roof intraoperatively. The patient developed left eyelid edema and ocular pain after recovery from anesthesia. The following day, the eyelid edema worsened, and she had difficulty opening her eyes. On the 9th postoperative day, she noticed diminished visual acuity and diplopia in her left eye when she was able to spontaneously open her eyes. Ophthalmological evaluation revealed mild left visual loss, decreased light reflex, ophthalmoplegia, ptosis, and chemosis. Computed tomography(CT)/magnetic resonance imaging revealed left proptosis, optic nerve stretching, intra-orbital fluid retention, and orbital/palpebral emphysema. She was diagnosed with orbital compartment syndrome(OCS)and received conservative treatment;however, her visual acuity did not improve. OCS observed after cerebral aneurysm surgery is rare;to date, only 24 cases have been reported in the available literature. Although the mechanism of OCS after craniotomy is unclear, it may be attributed to ocular compression by a muscle flap or increased intra-orbital pressure secondary to venous congestion. In the present case, the left superior ophthalmic vein and cavernous sinus were not clearly visualized on CT angiography. Therefore, we concluded that the right superior ophthalmic vein and superficial facial veins underwent dilatation and served as collateral circulation of the left orbital venous system. We speculate that OCS occurred secondary to increased intra-orbital pressure, possibly caused by inflow of cerebrospinal fluid with air into the orbit through a small bone defect that was accidentally created during craniotomy in a setting of orbital venous congestion.

[Early Development of a Symptomatic Expanding Porencephalic Cyst in the Hematoma Cavity after Subcortical Hematoma Removal in an Adult:A Case Report].

A 69-year-old woman presented on an emergency basis, with headache and left hemiparesis. Initial head CT at the time of admission revealed a large subcortical hematoma with perihematomal edema extending from the right parietal to the occipital lobe. A small part of the hematoma extended toward the trigone of the right lateral ventricle. CT angiography revealed no vascular abnormalities. Emergency craniotomy was erformed, and the patient's initial postoperative course was unremarkable. However, the patient's neurological symptoms worsened 10 days postoperatively, and CT revealed a new low-density cystic lesion with perifocal edema at the site of hematoma removal, in addition to severe cerebral compression. We performed a reoperation, and intraoperatively we observed hematoma fluid mixed with cerebrospinal fluid without any abnormal blood vessels or neoplastic lesions in the hematoma cavity. We identified the choroid plexus deep within the surgical field, and slight leakage of cerebrospinal fluid was detected from the ventricular aspect, indicating the formation of a small passage between the hematoma cavity and the ventricle. After the second operation, her postoperative course was uneventful without recurrent cyst formation. An early symptomatic expanding porencephalic cyst in the hematoma cavity after removal of an intracerebral hematoma is rare, and only a few cases have been reported in the literature. Based on literature review and considering the likely mechanism of cyst development, we speculated that progressive cyst expansion could be attributed to a check valve mechanism between the ventricle and the cavity from which the hematoma was removed, as observed in the present case.

A Case of Internal Carotid Artery Restenosis Due to Neointimal Hyperplasia Following Balloon Angioplasty.

Objective: The causes of restenosis following balloon angioplasty are reported to be vasospasm, thrombosis, and recurrence of atherosclerosis. We report a case of internal carotid artery (ICA) occlusion treated by emergency endovascular renacalization and carotid endarterectomy (CEA) for the restenosis, which revealed that the cause of restenosis was neointimal hyperplasia. Case Presentation: A 70-year-old man was brought to our hospital because of sudden onset left hemiparesis. His National Institute of Health Stroke Scale (NIHSS) score was 13. Magnetic resonance imaging diffusion weighted imaging (MRI DWI) demonstrated hyper-intensity in the right basal ganglia, indicating acute ischemia. Neither the right ICA nor the MCA was visualized on MR angiography. Following intravenous tPA therapy, endovascular treatment was employed. First, the right ICA occlusion was treated by balloon angioplasty and the right M1 occlusion was recanalized by the stent-type thrombus retriever. Complete recanalization was achieved and the patient fully recovered. However, restenosis of the right ICA developed 5 months later and CEA was performed. The postoperative course was uneventful. Based on the pathological examination, the cause of restenosis was migration and proliferation of dedifferentiated smooth muscle (SM) cells, that is, neointimal hyperplasia. Conclusion: Neointimal hyperplasia can be a cause of restenosis following balloon angioplasty.

Effective Administration of Rituximab in Anti-MDA5 Antibody-Positive Dermatomyositis with Rapidly Progressive Interstitial Lung Disease and Refractory Cutaneous Involvement: A Case Report and Literature Review.

We describe the case of a 48-year-old man with dermatomyositis (DM) who demonstrated rapidly progressive interstitial lung disease (RP-ILD) and refractory cutaneous involvement together with high levels of anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab). Even after combination immunosuppressive therapy including a corticosteroid, cyclosporine A, and intravenous cyclophosphamide, his respiratory insufficiency and cutaneous involvement progressively worsened. However, the administration of rituximab (RTX) resulted in clinical remission as well as a visible reduction in anti-MDA5-Ab levels, suggesting that RTX could be a useful remedy in cases refractory to conventional immunosuppressive agents, especially those of RP-ILD related to anti-MDA5-Ab-positive DM.

A trial of semen collection by transrectal electroejaculation method from Amur leopard cat (Prionailurus bengalensis euptilurus).

We collected semen from a male Amur leopard cat using the transrectal electroejaculation method and investigated the semen qualities for about four years. In addition, the influence of the season on the spermatogenic function of the Amur leopard cat was investigated with regard to the semen qualities, testicular volume and serum testosterone level. As a result, we could collect semen with good sperm qualities that would be useable for artificial insemination. Some seasonality was noted in the testicular volume and serum testosterone level. We clarified that the semen qualities were favorable before and during the female breeding season compared with those after the breeding season.

Burst suppression electroencephalogram with mushroom poisoning, Amanita pantherina.

We report on a patient with Amanita pantherina poisoning who showed a burst suppression pattern on electroencephalography during a comatose state. The patient recovered without sequelae a week after ingestion. Burst suppression pattern is defined as alternating bursts and periods of electrical silence, and it is associated with comatose states of various causes. The major toxins contained in A. pantherina are ibotenic acid, an excitatory amino acid at the glutamate receptors, and muscimol, an agonist of the gamma-aminobutyric acid receptors. Alteration of the synaptic transmission in the central nervous system by these toxins may lead to a burst suppression pattern.

Expression of O(6)-methylguanine-DNA methyltransferase causes lomustine resistance in canine lymphoma cells.

The DNA repair protein O (6)-methylguanine-DNA methyltransferase (MGMT) causes resistance to nitrosoureas in various human cancers. In this study, we analyzed the correlation between canine lymphomas and MGMT in vitro. Two of five canine lymphoma cell lines required higher concentrations of lomustine to inhibit cell growth by 50%, but their sensitivity to the drug increased when they were cultured with an MGMT inhibitor. Fluorometric oligonucleotide assay and real-time polymerase chain reaction of these cell lines revealed MGMT activity and high MGMT mRNA expression, respectively. We analyzed the methylation status of the CpG islands of the canine MGMT gene by the bisulfite-sequencing method. Unlike human cells, the canine lymphoma cell lines did not show significant correlation between methylation status and MGMT suppression levels. Our results suggest that in canine lymphoma MGMT activity may influence sensitivity to nitrosoureas; thus, inhibition of MGMT activity would benefit nitrosourea-resistant patients. Additional studies are necessary to elucidate the mechanism of regulation of MGMT expression.

La protéine de réparation O6-méthylguanine-DNA méthyltransferase (MGMT) cause de la résistance aux produits nitroso-urée dans divers cancers humains. Dans la présente étude nous avons analysé in vitro la corrélation entre les lymphomes canins et le MGMT. Deux des cinq lignées cellulaires de lymphome canin ont nécessité des concentrations plus élevées de lomustine pour inhiber de 50 % la croissance cellulaire, mais leur sensibilité au médicament augmenta lorsqu'elles furent mises en culture avec un inhibiteur de MGMT. Une épreuve fluorométrique des oligonucléotides et une épreuve d'amplification en chaîne par la polymérase en temps réel sur ces lignées cellulaires ont révélé, respectivement, une activité MGMT et une expression élevée d'ARNm de MGMT. Nous avons analysé le statut de méthylation des ilots CpG du gène MGMT canin par la méthode de séquençage au bisulfite. Contrairement aux cellules humaines, les lignées cellulaires canines de lymphome n'ont pas montré de corrélation significative entre le statut de méthylation et les niveaux de suppression de MGMT. Nos résultats suggèrent que lors de lymphome canin l'activité de MGMT peut influencer la sensibilité aux produits nitroso-urée; ainsi, l'inhibition de l'activité MGMT bénéficierait les patients résistants au nitroso-urée. Des études additionnelles sont nécessaires pour élucider le mécanisme de régulation de l'expression de MGMT.(Traduit par Docteur Serge Messier).

Motor branch biopsy of the pronator teres muscle in a patient with painful forearm neuropathy.

Histological evaluation of a peripheral nerve is often the final diagnostic work-up for a neuropathy of unknown origin, and a distal sensory nerve is usually biopsied. Here, we report the case of a female patient with painful unilateral neuropathy in the upper arm. According to the histological evaluation of the pronator teres motor branch, vasculitis seemed to be the most probable cause of the condition, and steroid therapy improved the patients' symptoms. A biopsy of the motor branch of the pronator teres muscle nerve may be considered a valuable diagnostic option in selected cases with neuropathy affecting the upper limb, when performed in cooperation with neurologists and orthopedic surgeons.

An Abnormal MRI Signal in Both Lateral Geniculate Bodies Is a Diagnostic Indicator for Patients with Behçet's Disease.

A few case reports involving Behçet's disease have described visual field defects ascribed to the lateral geniculate body; however, no cases of lesions in both lateral geniculate bodies have been reported. A 50-year-old male who had fever and genital ulceration 3 months earlier noticed visual field defects. A T2-weighted MRI and FLAIR image showed high-intensity lesions in both lateral geniculate bodies. The posterior end of the optic tracts and lateral geniculate bodies were bilaterally enhanced with gadolinium. Because of the presence of three (aphthous stomatitis, genital ulceration and uveal inflammation) of the four major symptoms, the patient was diagnosed with an abortive type of Behçet's disease and started predonisolone at a dose of 0.5 mg/kg, after which he showed a rapid improvement of all symptoms. The lesions in the lateral geniculate bodies had disappeared on the MRI images taken 2 weeks after treatment onset. In addition to Behçet's disease, several case reports have observed lesions in both lateral geniculate bodies. Their visual field defects disappeared within half a year, in parallel with the healing process of the primary diseases. The pathogenesis of the lesions in both lateral geniculate bodies in these cases is believed to be related to the underlying disorders, especially vascular involvement from inflammation. The appearance of lesions in both lateral geniculate bodies on MRI scans may indicate the presence of systemic inflammatory diseases such as Behçet's disease, but further examinations are required.