Background: It is crucial to establish whether a stroke patient has type A acute aortic dissection (TAAAD), because administering recombinant tissue plasminogen activator (rt-PA) to these patients could prove fatal. We investigated the characteristics of stroke patients with TAAAD to determine the useful factors for differential diagnosis. Methods: We retrospectively analyzed the backgrounds of 13 cases of stroke patients with TAAAD (TAAAD group) and compared them with 57 cases of ischemic stroke patients, who had been administered rt-PA (rt-PA group). Results: The TAAAD group statistically exhibited more instances of pain (p<0.01). All 13 cases in the TAAAD group had transient or persistent alteration of consciousness level. The average systolic blood pressure of the TAAAD group was significantly lower than that of the rt-PA group (p<0.01) and systolic blood pressure less than 130 mmHg was statistically useful for the ROC analysis. Especially, systolic blood pressure less than 110 mmHg was statistically specific for the diagnosis of TAAAD (Likelihood ratio 35.1). The TAAAD group also tended to have bradycardia. Conclusion: It is important to confirm whether stroke patients are experiencing pain or not for the differential diagnosis of TAAAD. We may suspect TAAAD when we examine ischemic stroke patients who have low systolic blood pressure, less than 130 mmHg. We should strongly suspect ischemic stroke due to TAAAD if the systolic blood pressure of the patients is less than 110 mmHg and the heart rate is less than 60 beats per minute.

Background and Purpose: We investigated whether the background factors of patients with bilateral “Champagne bottle neck sign (CBNS)” are different from those with unilateral CBNS. Methods: The subjects were 26 patients with CBNS demonstrated by carotid ultrasonography, who received brain MRI. The CBNS was noted unilaterally in 13 patients and bilaterally in the other 13. We compared between the two groups of incidence of atherosclerotic risk factors, carotid plaque on ultrasonography, and brain MRA findings that were characteristic of moyamoya disease or atherosclerosis. Results: The prevalence rates of hypertension, dyslipidemia, and plaque at the carotid arteries were higher in the unilateral group than in the bilateral group significantly (p=0.03). The MRA findings of moyamoya disease were more frequently seen in the bilateral group than in the unilateral group (46% vs. 100%, p<0.001) and that of atherosclerotic disease was seen in 46% of the unilateral group but not in the bilateral group (p<0.001). Conclusions: It seems that bilateral CBNS is strongly related to moyamoya disease and the unilateral CBNS is not only to moyamoya disease but also to atherosclerosis.

The authors investigated that the impact of a Stroke Code and cooperation between doctors and nurses on door-to-needle (DTN) time in acute ischemic stroke patients anticipated to receive intravenous recombinant tissue-type plasminogen activator therapy (IV tPA). Nurses held study meetings about acute stroke care with the nurses in the emergency department, radiological technologists and stroke neurologists and neurosurgeons. After the meetings, we implemented the Stroke Code with the aim of achieving rapid stroke assessment and treatment. We included patients treated with IV tPA during pre-Stroke Code period, retrospectively and during the Stroke Code period, prospectively. The patients underwent magnetic resonance imaging (MRI) unless they had contraindications for MRI. The IV tPA time intervals, laboratory findings, imaging findings and outcomes (modified Rankin Scale [mRS] score 0 to 1 and mortality) at discharge and 90 days were corrected. Of the 153 patients, 82 patients received IV tPA under the Stroke Code. After the implementation of the Stroke Code, Completion of imaging-to-needle time and DTN time were significantly reduced by 6 and 8 minutes. However, there were no difference in the outcomes at discharge (mRS score 0 to 1; p=0.232 and mortality; p=0.251) and 90 days (mRS score 0 to 1; p=1.000 and mortality; p=0.548). We demonstrated that the implementation of Stroke Code and cooperation between doctors and nurses could improve the DTN time.

On this occasion, we report a rare case of cardiac embolization caused by a free-floating thrombus (FFT) at the kinking of internal carotid artery (ICA). A 79-year-old male patient was admitted in our department, with JCS I-3, total aphasia, NIHSS 14, and atrial fibrillation. Initially performed MRI showed multiple cerebral infarction in the region of left ICA. Ultrasonography showed FFT and severe stenosis at the origin of ICA. Angiography showed bilateral ICA kinking, FFT, and little anterograde blood flow. In order to prevent the progression of infarction, we performed endovascular thrombectomy under proximal balloon occlusion. On the first postoperative day, we found no signs of infarction progression, and the patient was discharged 8 days later. ICA kinking is not rare, but the situation antero-grade blood flow coexist with trapped FFT at ICA is very rare. We suspect the pathogenic mechanism that kinking artery reduced the lumen diameter.

A 65-year-old woman was admitted to our hospital with a diagnosis of a crescendo transient ischemic attack. She had a history of two cerebral infarctions with stenosis and occlusion of intracranial artery and was receiving treatment with clopidogrel. Before admission to our hospital, JAK2V617F mutation was detected at the time of investigating polycythemia, but did not lead to a diagnosis of polycythemia vera (PV) because the increase in blood cells was mild. After hospitalization, PV was diagnosed based on a marked increase in blood cell count, and was treated with phlebotomy and addition of cilostazol and hydroxyurea. She was discharged from the hospital on hospital day 19 without sequelae. PV is a clonal hematopoietic stem cell disorder. JAK2V617F mutation is detected in more than 95% of patients with PV, and is associated with a significantly increased risk of thrombosis. However, the exact mechanisms underlying the association between the JAK2V617F mutation and intracranial ischemic disease remain unclear. Our report suggests that the JAK2V617F mutation itself may affect endothelial cells as a pathogenic mechanism for intracranial ischemic disease in patients with PV.

We here report a very rare case of a 54-year-old man presenting with pseudoxanthoma elasticum (PXE), a primary metabolic disorder characterized by the progressive accumulation of calcium phosphate and proteoglycan deposits in the connective tissues and fragmentation of the elastic fibers, along with concomitant carotid rete mirabile (CRM), an abnormal collateral anastomosis between the internal and external carotid arteries, generally located around the cavernous sinus. At the 19-month follow-up, angiography revealed that the flow of the CRM in the left internal carotid artery was decreased; accordingly, cerebral revascularization surgery with superficial temporal artery and middle cerebral artery anastomosis was successfully performed. There is currently no report on superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis for PXE with CRM, and this is the first report of the midterm outcomes of revascularization surgery for such a patient. The STA may be used as the donor artery for revascularization surgery; however, the effects of harvest and anastomosis on the disease process of PXE in the STA and long-term patency of the STA graft remain unknown. The histological findings in the STA of the present case revealed no apparent impairment and accumulation of calcium phosphate, indicating that the STA is a suitable donor artery.

A 31-year-old man was admitted to our hospital with vertigo, staggering while walking, and vomiting. He had a history of hemophilia A and HIV infection. At admission, neurological examination showed horizontal nystagmus toward the left side. Factor VIII activity was reduced to 30% (control 50–200%). Brain computed tomography (CT) and magnetic resonance imaging (MRI) revealed hemorrhage in the left cerebellar nuclei. Cerebral angiography showed cerebral developmental venous anomalies (DVA) in the left cerebellum. The patient was diagnosed with intracerebral hemorrhage (ICH) with DVA, and hemophilia A might be related to ICH in this case. In general, ICH is a rare complication of DVA. However, the hemorrhagic tendency can increase the risk of ICH with DVA, even in young patients.

A 50-year-old woman with progressive ocular palsy, ataxia and sensory impairment who was diagnosed with anti-GQ1b antibody-positive Fisher syndrome (FS) was initially treated with intravenous immunoglobulin (IVIg). However, weakness in the left upper limb developed due to arterial hypertension 3 days later. Magnetic resonance imaging showed an intracranial hemorrhage in the right frontal and parietal lobes, and the areas of hyperintensity in the left frontal and the bilateral occipital white matter, cortex and bilateral cerebellum. The IVIg was discontinued because the arterial hypertension caused posterior reversible encephalopathy syndrome (PRES). The symptoms further progressed to include facial palsy, dysarthria and dysphagia, indicating advanced FS. Immunoadsorption plasmapheresis ameliorated the course of the disease, hastened recovery and improved functional outcomes to the point where the patient was transferred to a rehabilitation hospital. This experience supports the notion that IVIg can lead to complicating PRES that can cause the progression of extant FS.

A 50-year-old woman developed sudden right hearing loss, vertigo with the right cervical pain for 6 days. Neurological examinations revealed rotatory nystagmus to the left direction and right hearing loss. Diffusion-weighted image (DWI) on MRI showed the cerebellar infarction in the right anterior inferior cerebellar artery (AICA) and posterior inferior cerebellar artery (PICA) supply. On day 22, cerebral angiography showed that the right AICA and PICA diverged from the right vertebral artery (VA). We diagnosed that the cause of the right hearing loss was the AICA occlusion caused by the right VA dissection. Now, her right hearing loss was not recovered.