Treatment of cryptogenic new-onset refractory status epilepticus (C-NORSE) with tocilizumab: A case report.

A 35-year-old woman with no prior history of epilepsy developed status epilepticus (SE), which was highly resistant to multiple antiseizure medications and sedatives. The etiology of SE was not identified despite extensive investigation, and the patient was diagnosed with cryptogenic new-onset refractory status epilepticus (C-NORSE). Although first-line immunotherapies such as high-dose corticosteroids and plasma exchange were ineffective, the patient manifested a resolution of SE after the administration of tocilizumab, which inhibits interleukin-6. Non-antibody-mediated inflammation has been hypothesized to be a probable pathophysiology of C-NORSE in recent studies, and tocilizumab may be a plausible second-line treatment.

Copper Deficiency in Wilson's Disease with a Normal Zinc Value.

Copper deficiency (CD) is a rare complication of long-term treatment of Wilson's disease (WD) and is usually accompanied by high serum zinc levels. A 57-year-old woman with WD presented with limb weakness and sensory disturbance due to myeloneuropathy and macrocytic anemia after 36 years of treatment. Markedly reduced serum free copper values confirmed CD, which was considered to be caused by progressive dysphagia and severe diarrhea rather than zinc overdose because of the normal serum zinc levels. Discontinuing copper-reducing therapy and increasing copper intake improved her symptoms. Physicians should be alert for the risk of CD in WD patients, especially those with dysphagia.

Neuromyelitis Optica Complicated by Ornithine Transcarbamylase Deficiency Treated Safely with Pulse Steroid Therapy.

Steroid administration to patients with urea cycle disorders can cause hyperammonemia. We encountered a 36-year-old woman with neuromyelitis optica (NMO) complicated by ornithine transcarbamylase (OTC) deficiency. By reducing the doses of steroids and adequate infusion management, we were able to administer pulse steroid therapy without any severe complications. This case indicates the safety of steroid treatment in patients with urea cycle disorders.

Embolic Stroke Due to a Mural Thrombus in the Ascending Aorta Following Cisplatin-based Chemotherapy.

A 59-year-old woman with small-cell lung carcinoma achieved tumor disappearance after cisplatin-based chemotherapy (CBC) and radiation treatment but subsequently experienced right hemiparesis and aphasia. Brain magnetic resonance imaging revealed a left middle cerebral artery territory acute infarction and left internal carotid artery occlusion. Ultrasonography revealed a mobile thrombus in the left common and internal carotid arteries, and contrast computed tomography revealed a mural thrombus in the ascending aorta. Based on these findings, embolic stroke due to aortic mural thrombus following CBC was diagnosed. Aortic mural thrombus is a rare complication of CBC but carries a risk of embolic stroke.

Carotid Endarterectomy for Intractable Repetitive Stenosis Following Carotid Artery Stenting.

BACKGROUND: Restenosis after carotid artery stenting has raised concerns regarding the long-term durability of carotid stenting. Recurrent restenosis after multiple endovascular interventions may pose a challenge for clinicians. CASE DESCRIPTION: We encountered 2 cases of intractable restenosis after redo-carotid artery stenting and performed carotid endarterectomy. We removed the embedded stent and plaque simultaneously, used the internal shunting system, and performed patch angioplasty with no further recurrence. CONCLUSION: Carotid endarterectomy could be considered as a first-line treatment for recurrent stenosis that proves refractory to multiple endovascular interventions.

Delayed Hemiparkinsonism Associated with Kernohan's Notch in a Patient with a Ruptured Arteriovenous Malformation.

A 24-year-old female patient was admitted for a right frontal intracranial hematoma with an uncal herniation due to a ruptured arteriovenous malformation and therefore underwent emergency surgery. Neuroimaging revealed left-sided midbrain notching against the tentorium, indicating Kernohan's notch phenomenon. She denied experiencing any short-term neurological deficits but right-sided delayed hemiparkinsonism developed 18 months later. Dopamine transporter tracer uptake was severely reduced in the left striatum, suggesting nigrostriatal degeneration secondary to Kernohan's notch. Uncal herniations are potentially fatal, but surgery can save the patient's life and improve the functional outcomes. Clinicians should therefore be aware of delayed hemiparkinsonism as a rare complication of Kernohan's notch phenomenon.

Intraventricular Silicone Oil Migration Resembling Hemorrhagic Complication in a Patient with Ischemic Stroke Receiving Antithrombotic Therapy.

We report herein a case of intraventricular silicone oil migration, a rare complication of intraocular silicone oil tamponade, mimicking a hemorrhage during antithrombotic therapy for ischemic stroke. A 62-year-old male patient with a history of diabetic retinopathy was admitted for right hemiparesis and dysarthria. Brain magnetic resonance imaging on admission showed an acute left-sided ventral medullary infarction, and antithrombotic therapy was started. Head computed tomography done on the next day after admission showed an area of high-density resembling a hematoma in the lateral ventricle. Additional magnetic resonance imaging in the supine and lateral recumbent positions confirmed migration of the lesion within the ventricles by position, indicating intraventricular silicone oil migration. Several facilities in Japan perform magnetic resonance imaging instead of computed tomography as the first step in assessing stroke in the emergency clinical setting. While the silicone oil used in internal tamponade appears high-density on computed tomography, it does not register as an abnormality on diffusion-weighted imaging, thus creating a pitfall to diagnosis based on this modality.

Risk factors, etiology, and outcome of ischemic stroke in young adults: A Japanese multicenter prospective study.

PURPOSE: This study aimed to evaluate the risk factors, etiology, and outcomes of ischemic stroke (IS) in Japanese young adults. METHODS: This was a prospective multicenter study. We enrolled patients aged 16 to 55 years with IS within seven days of the onset of symptoms. We assessed the demographic data, risk factors, stroke etiology, and outcome at discharge. The clinical characteristics were compared between sexes and among age groups. RESULTS: We prospectively enrolled 519 patients (median age, 48 years: 139 females). The mean National Institute of Health Stroke Scale score was 3.6 ± 0.2. The most common risk factors were hypertension (HT) (55%), dyslipidemia (DL) (47%), and current smoking (42%). Body mass index, incidence of current smoking, and heavy alcohol consumption were higher in males. The prevalence of current smoking, HT, DL, and diabetes mellitus increased with aging. The most common etiologic subgroup of IS was small vessel disease (145/510, 28%). Intracranial arterial dissection (IAD) was the most common among the other determined causes (56/115, 49%). The outcome at discharge was relatively good (mRS 0-1, 71.7%); however, poor outcome (mRS ≥ 4) was observed at an incidence of 9.5%. CONCLUSIONS: Most young adults with IS had modifiable risk factors, of which prevalence increased with age. This emphasizes lifestyle improvement to prevent IS in the young population. Furthermore, we indicated that the incidence rate of IAD was high among the other determined causes.

Involvement of interleukins-17 and -34 in exacerbated orthodontic root resorption by jiggling force during rat experimental tooth movement.

BACKGROUND: Orthodontically induced root resorption (OIRR) is considered as an undesirable and unpredictable sequel of orthodontic treatment. Recent reports demonstrated that interleukin (IL)-17/IL-34, and T cells secrete inflammatory/osteoclastogenic cytokines, which might stimulate osteoclastogenesis/bone resorption. However, little is known about the role played by IL-17/IL-34 in OIRR. The present study was aimed at investigating the odontoclastic expression pattern of IL-17 and IL-34 in resorbed cementum during different experimental tooth movements in vivo. METHODS: Twenty-four 8-week-old male Wistar rats were divided into four groups: control group, optimal force group (10 g), heavy force group (50 g), and jiggling force group (compression and tension, repetition; 10 g). After 7, 14, and 21 days, the expression levels of IL-17 and IL-34 protein in the resorbed cementum were analyzed using immunohistochemical methods. RESULTS: On day 21, the immunoreactivity for IL-17 and IL-34 in resorbed roots in the jiggling force group was stronger than that in the heavy force and optimal force groups. Moreover, the number of IL-17-positive and IL-34-positive odontoclasts was significantly increased in the jiggling force group compared with those in the other groups on day 21. CONCLUSIONS: These results suggest that jiggling forces might exacerbate OIRR compared with heavy forces, as evidenced by the increased expression of IL-17 and IL-34 in odontoclasts obtained from resorbed roots.

Predictors of intracranial hemorrhage in acute ischemic stroke after endovascular thrombectomy.

BACKGROUND: Limited data are available regarding the predictors, clinical relevance, and bleeding rate by surgical devices of intracranial hemorrhage after endovascular thrombectomy. This is partially explained by the difference in the classification and definition of hemorrhage among studies. The purpose of this study was to identify the predictors of hemorrhagic transformation and isolated subarachnoid hemorrhage after endovascular thrombectomy. METHODS: This was a retrospective, multicenter observational cohort study of consecutive patients who underwent endovascular thrombectomy between January 2015 and December 2018. Univariate and logistic regression analyses were performed to determine the predictors, the impact on clinical outcomes, and the bleeding rate by surgical devices of hemorrhagic transformation and isolated subarachnoid hemorrhage. RESULTS: Among 610 eligible patients, hemorrhagic transformations occurred in 93 (15.2%). Fourteen patients (2.3%) were classified as having symptomatic intracranial hemorrhage. Isolated subarachnoid hemorrhage was found in 60 (9.8%) patients. In the logistic regression analyses, diabetes mellitus (odds ratio: 1.92; 95% confidence interval: 1.06-3.49) was associated with hemorrhagic transformation, and the number of device passes (odds ratio: 1.33; 95% confidence interval: 1.11-1.59) was associated with isolated subarachnoid hemorrhage. Both hemorrhagic transformation and isolated subarachnoid hemorrhage were associated with poor 90-day functional outcomes. There was a significant correlation between treatment with stent retrievers and isolated subarachnoid hemorrhage. CONCLUSIONS: Patients with diabetes mellitus were vulnerable to hemorrhagic transformation, whereas those who underwent several attempts of thrombectomy were susceptible to isolated subarachnoid hemorrhage. Both hemorrhage types worsened the functional outcome. Treatment with the stent retriever was significantly associated with postprocedural isolated subarachnoid hemorrhage.

A Rare Anomalous Origin of the Right Vertebral Artery from the Internal Carotid Artery with an Aberrant Right Subclavian Artery.

BACKGROUND: An anomalous origin of the right vertebral artery (VA) from the right internal carotid artery (ICA) occurs only rarely. CASE DESCRIPTION: A 70-year-old man underwent carotid artery stenting for right internal carotid artery stenosis. In this patient, the right VA arose from the right ICA associated with an aberrant right subclavian artery. CONCLUSION: Embryologically, failure of involution of 1 of the first 6 intersegmental arteries causes various abnormal origins of the VA. The embryonic development of this anomaly is also reviewed.

Tokyo Metropolitan Stroke Emergency Medical Services for Interventional Stroke Treatment: The Tama-REgistry of Acute Thrombectomy (TREAT) Study.

OBJECTIVE: It is not clear how patients with large vessel occlusion (LVO) who have undergone mechanical thrombectomy (MT) were transported to hospitals by emergency medical services. Here, we describe the current status of the stroke delivery system in a large city. METHODS: We investigated data from 328 patients (male, n = 199; average age, 74.8 ± 12.9 years) who underwent MT at 12 facilities in the Tama area of Tokyo, between January 2015 and December 2017. The patients were classified according to the destination institution as Stroke A eligible (group A, n = 266 [8.2%]), Tertiary critical care center (group T; n = 35 [10.7%]), and other destinations such as emergency rooms (group O; n = 27 [8.2%]), and then reasons for using Emergency Medical Service (EMS) services and outcomes were compared among the groups. RESULTS: Rates of milder stroke, and middle cerebral artery occlusion were significantly higher in group A than T, whereas that of vertebral-basilar artery occlusion was significantly lower in group A than in groups T and O. The amount of elapsed time from door to picture (DTP) was significantly lower in group A. The time from onset to recanalization, as well as rates of successful recanalization and favorable outcomes (90-day modified Rankin scale 0-2) did not significantly differ regardless of destination. CONCLUSIONS: Most patients with LVO in the Tama area were categorized into group A. DTP was significantly lower in group A.

Efficacy and Safety of Mechanical Thrombectomy for Occlusion of the Second Segment of the Middle Cerebral Artery : Retrospective Analysis of the Tama-REgistry of Acute endovascular Thrombectomy (TREAT).

BACKGROUND: The efficacy of mechanical thrombectomy in the treatment of occlusions of the second segment of the middle cerebral artery (M2) has not been firmly established. METHODS: This study analyzed data from patients who had undergone mechanical thrombectomy for the first segment of the middle cerebral artery (M1) and M2 occlusion from the Tama-REgistry of Acute endovascular Thrombectomy (TREAT) between January 2015 and March 2017, which is a multicenter database in the Tama area of Tokyo, Japan. The M1 and M2 occlusions were compared in order to evaluate the safety and efficacy of M2 thrombectomy. RESULTS: A total of 515 patients were registered, whereby 160 patients with M1 occlusion and 51 patients with M2 occlusion were included. While the puncture-to-reperfusion time was longer in the M2 occlusions (median 43 min, range 30-61 min vs. median 60 min, range 38-79 min, p = 0.01), no significant differences were seen in the proportion of patients with successful reperfusion, postoperative hemorrhagic complications and good outcome (modified Rankin scale ≤2 at 90 days). Younger age was the only independent factor associated with good outcome in patients with M2 occlusions as determined by the multivariate analysis (p = 0.033, odds ratio 0.91, 95% confidence interval 0.83-0.99). CONCLUSION: The outcome and the safety profile of mechanical thrombectomy for M2 occlusions are favorable and comparable to those of the M1 occlusion thrombectomy.

Multicenter Prospective Analysis of Stroke Patients Taking Oral Anticoagulants: The PASTA Registry - Study Design and Characteristics.

OBJECTIVES: The management of atrial fibrillation and deep venous thrombosis has evolved with the development of direct oral anticoagulants (DOAC), and oral anticoagulant (OAC) might influence the development or clinical course in both ischemic and hemorrhagic stroke. However, detailed data on the differences between the effects of the prior prescription of warfarin and DOAC on the clinical characteristics, neuroradiologic findings, and outcome of stroke are limited. DESIGN: The prospective analysis of stroke patients taking anticoagulants (PASTA) registry study is an observational, multicenter, prospective registry of stroke (ischemic stroke, transient ischemic attack, and intracerebral hemorrhage) patients receiving OAC in Japan. This study is designed to collect data on clinical background characteristics, drug adherence, drug dosage, neurological severity at admission and discharge, infarct or hematoma size, acute therapy including recanalization therapy or reverse drug therapy, and timing of OAC re-initiation. Patient enrollment started in April 2016 and the target patient number is 1000 patients. CONCLUSIONS: The PASTA prospective registry should identify the status of stroke patients taking OAC in the current clinical practice in Japan.

Successful endovascular recanalization of massive cerebral venous sinus thrombosis in a patient with tuberous sclerosis and protein S deficiency: a case report.

Here, we report the case of a 27-year-old woman with tuberous sclerosis complex who underwent successful endovascular intervention for cerebral venous thrombosis at the superior sagittal sinus. She had protein S deficiency and a long-term history of anemia caused by menorrhagia from uterine fibroids, possibly leading to a hypercoagulable state. Cerebral venous sinus thrombosis accounts for ~0.5-1% of all strokes. Several cases of venous thrombosis in patients with tuberous sclerosis complex and protein S or protein C deficiency have been reported, but further studies are needed to identify whether an association of this rare combination may be explained.

Outcomes of Endovascular Thrombectomy Performed 6-24 h after Acute Stroke from Extracranial Internal Carotid Artery Occlusion.

Thrombectomy has demonstrated clinical efficacy against acute ischemic stroke caused by intracranial occlusion of the internal carotid artery (ICA), even if performed 6-24 h after onset. This study investigated the outcomes of thrombectomy performed 6-24 h after stroke onset caused by extracranial ICA occlusion. Of 586 stroke patients receiving thrombectomy during the past 3 years and registered in the Tama Registry of Acute Endovascular Thrombectomy database, 24 were identified with ICA occlusion (14 extracranial and 10 intracranial), known to be well 6-24 h before presentation, and with pre-stroke modified Rankin Scale (mRS) score of 0 or 1. Clinical outcomes measured were the rate of functional independence at 90 days according to mRS score of 0-2 and 90 day mortality rate. Of patients with extracranial ICA occlusion, two received additional carotid stenting with thrombectomy. The median interval between the last time the patient was known to be well and hospital arrival was 601 (interquartile range, 476-729 min). Both the rate of functional independence at 90 days and 90 day mortality were comparable between patients with extracranial or intracranial ICA occlusion (36% vs. 40% and 7% vs. 10%, respectively). No symptomatic intracranial hemorrhages occurred within 24 h following treatment of extracranial ICA occlusion. Thrombectomy performed 6-24 h after extracranial ICA results in acceptable functional outcome. Further clinical study is warranted to better define the temporal window of thrombectomy for acceptable functional outcome in patients with extracranial ICA occlusion.

Endovascular treatment of acute basilar artery occlusion: Tama-REgistry of Acute Thrombectomy (TREAT) study.

OBJECTIVE: The effectiveness of mechanical thrombectomy (MT) for acute basilar artery occlusion (ABAO) remains unknown. We evaluated the feasibility, safety, and efficacy of endovascular treatment for ABAO. METHODS: We retrospectively investigated patients with ABAO who underwent MT, using modern stent retrievers and an aspiration device, between January 2015 and December 2017 at 12 comprehensive stroke centers. Functional outcomes and 90-day mortality were analyzed as primary outcomes. RESULTS: Forty-eight patients were included. Good outcome (modified Rankin Scale mRS 0-2) was achieved in 20/48 patients and the all-cause 90-day mortality rate was 25%. Successful recanalization (modified Thrombolysis In Cerebral Infarction [mTICI] grade 2b and 3) was achieved in 47/48 patients. National Institutes of Health Stroke Scale, posterior circulation Alberta Stroke Program Early CT Score (pc-ASPECTS), DWI Brain Stem Score, mTICI (3 > 2b), and intracranial hemorrhage were significantly different between good and poor functional outcome groups. The occlusion site of BA was significantly different between patients with moderate outcome (mRS 0-3) versus others. We found that age, pc-ASPECTS and mTICI were significantly associated with functional outcomes in the logistic regression model. CONCLUSION: MT with stent retrievers and an aspiration device for ABAO results in high successful recanalization and good outcomes. Further studies are required to confirm our results.

Widening Time Disparities between Two Paradigms: Tama-REgistry of Acute Endovascular Thrombectomy.

BACKGROUND: The Tama-REgistry of Acute endovascular Thrombectomy (TREAT) is a multicenter registry of endovascular thrombectomy in the Tama area of Tokyo. The objective of this study was to confirm the real-world status of 2 paradigms of transportation. METHODS: This was a retrospective analysis of data from TREAT. Patients were divided into 2 groups and 2 periods: directly admitted to an endovascular thrombectomy-capable center (ECC; group D)/secondary transfer from a non-ECC (group S), and the first period/the second period. Transfer distance, workflow metrics, and clinical outcomes were analyzed. RESULTS: A total of 326 patients, including 264 in group D and 62 in group S, were analyzed. The median distance from the onset-to-ECC was 3.62km for group D and 7.87km for group S (P < .001). The median onset-to-needle (OTN) time was longer for group S (168 minutes) than group D (138 minutes; P = .006). The median onset-to-reperfusion (OTR) time was significantly shorter for group D (247 minutes) than for group S (304 minutes; P = .029). With respect to the 2 periods, there was no significant difference in onset-to-puncture time between the 2 groups in the first period (207 minutes versus 243.5 minutes, respectively, P = .50), while there was one in the second period (164 minutes versus 246.5 minutes, respectively, P = .02). CONCLUSIONS: This region-wide registry study showed longer OTN and OTR times, with no improvement of the time course over time in patients transported via non-ECCs. These results should be used to create a regional medical policy for the management of acute ischemic stroke.

Thrombectomy for Stroke at 6-24 hours without Perfusion CT Software for Patient Selection.

BACKGROUND: Although, thrombectomy for stroke more than 6 hours after onset supported by automated perfusion computed tomography (CT) software (RAPID, iSchemaView) is effective, this software is not available in Japan. This study aimed to elucidate the efficacy of thrombectomy 6-24 hours after onset in our patient cohort using conventional imaging mismatch. METHODS: Of 586 ischemic stroke patients who underwent thrombectomy registered from January 2015 to December 2017, patients with occlusion of the intracranial internal carotid artery or middle cerebral artery, who had last been known to be well 6-24 hours earlier and who had a prestroke modified Rankin scale (mRS) score 0 or 1 were enrolled. Clinical outcomes were the scores of the utility-weighted (UW) mRS, which ranges from 0 (death) to 10 (no symptom or disability), and the rate of functional independence (mRS score of 0-2) at 90 days. RESULTS: This study sample included 31 patients. The median baseline National Institutes of Health Stroke Scale score was 17 (interquartile range [IQR], 13-20), and the median Diffusion-Weighted Imaging-Alberta Stroke Program Early CT Score was 7 (IQR, 5-8). The median interval between the time that the patient was last known well and revascularization was 741 (IQR, 641-818) minutes. The mean UW mRS score at 90 days was 5.3, the rate of functional independence was 32%, and the 90-day mortality rate was 13%. CONCLUSIONS: Thrombectomy 6-24 hours after onset which can be performed with conventional imaging mismatch might be secured for improving functional independence in stroke patients.