[Autoimmune polyglandular syndrome type 3 diagnosed with cerebral venous sinus thrombosis: a case report].

A 67-year-old woman with a history of diabetes mellitus was admitted to our hospital with convulsions due to bilateral frontal subcortical hemorrhages. MR venography showed a defect in the superior sagittal sinus, and thrombi were demonstrated in the same lesion with head MRI three-dimensional turbo spin echo T1-weighted imaging. She was diagnosed with cerebral venous sinus thrombosis. As precipitating factors, we found high levels of free T3 and T4, low levels of thyroid stimulating hormone, anti-thyroid stimulating hormone receptor antibody, and anti-glutamic acid decarboxylase antibody with her. We diagnosed her with autoimmune polyglandular syndrome type 3 with Graves' disease and slowly progressive type 1 diabetes mellitus. Since she also had nonvalvular atrial fibrillation, she was treated with apixaban subsequently to intravenous unfractionated heparin in the acute phase, resulting in partial regression of the thrombi. Autoimmune polyglandular syndrome should be considered when multiple endocrine disorders are identified as precipitating factors for cerebral venous sinus thrombosis.

Fatal Cerebral Venous Thrombosis in a Pregnant Woman with Inherited Antithrombin Deficiency after BNT162b2 mRNA COVID-19 Vaccination.

Antithrombin deficiency is a high-risk factor for venous thromboembolism during pregnancy, whereas cerebral venous thrombosis is rare. Cerebral venous thrombosis related to coronavirus disease 2019 (COVID-19) vaccines has been reported; however, there are a few reports of cerebral venous thrombosis after a messenger RNA (mRNA) vaccination. A 25-year-old female in her sixth week of pregnancy presented with headache 24 days after BNT162b2 mRNA COVID-19 vaccination. The following day, she presented with altered sensorium and was diagnosed with severe cerebral venous thrombosis. She demonstrated heparin resistance and was found to have an inherited antithrombin deficiency. A heterozygous missense variant in SERPINC1 (c.379T>C, p.Cys127Arg, 'AT Morioka') was detected by DNA analysis. Despite intensive care with unfractionated heparin, antithrombin concentrate, and repeated endovascular treatments, she died on the sixth day of hospitalization. Cerebral venous thrombosis in pregnant women with an antithrombin deficiency can follow a rapid and fatal course. Treatment with unfractionated heparin and antithrombin concentrate may be ineffective in severe cerebral venous thrombosis cases with antithrombin deficiency. Early recognition of antithrombin deficiency and an immediate switch to other anticoagulants may be required. Although the association between cerebral venous thrombosis and the vaccine is uncertain, COVID-19 vaccinations may require careful evaluation for patients with prothrombic factors.

[Detection of secondary atrial fibrillation following percutaneous foramen ovale closure for cryptogenic stroke using an insertable cardiac monitor: a case report].

A 61-year-old man presented with transient dysarthria and left upper extremity numbness. Head MRI showed an acute infarct in the left temporal lobe and multiple old infarcts in the bilateral cortices. A transesophageal echocardiogram revealed a patent foramen ovale with a large shunt. No deep vein thrombosis was found. He suffered a recurrent cerebral infarction while taking antiplatelet therapy. An insertable cardiac monitor was implanted on the 41st day, and the antiplatelet treatment was changed to warfarin. The insertable cardiac monitor did not detect atrial fibrillation, even when the patient had a recurrent transient ischemic attack on the 57th day under warfarin therapy. The patient underwent percutaneous foramen ovale closure on the 63rd day. On postoperative days 18-25, an insertable cardiac monitor detected brief atrial fibrillation, and he took rivaroxaban for three months. Atrial fibrillation may occur secondary to percutaneous patent foramen ovale closure for cryptogenic stroke. The insertable cardiac monitor may help diagnose the pathogenesis of secondary atrial fibrillation and determine the optimal antithrombotic therapy.

A low score on the National Institutes of Health Stroke Scale with eye movement disorder may indicate a good candidate for acute mechanical thrombectomy for posterior circulation large vessel occlusion: illustrative cases.

BACKGROUND: Basilar artery occlusion (BAO) accounts for 1% of all strokes, and its natural prognosis is extremely poor. There is no consensus on the treatment strategy for mild BAO. OBSERVATIONS: Between August 2015 and May 2021, 429 patients received mechanical thrombectomy (MT) in the authors' hospital. Three patients had a BAO with a National Institutes of Health Stroke Scale (NIHSS) score of ≤6 and showed eye movement disorder as the main symptom. MT immediately improved ocular symptoms in all three cases, and the patients were discharged with a modified Rankin Scale ≤2. LESSONS: Lesions responsible for the eye movement disorder are distributed from the midbrain to the pontine tegmentum. These lesions are supplied by the arteries of the interpeduncular fossa, which is impaired by BAO. Symptoms due to problems with the arteries of the interpeduncular fossa can be rapidly improved by MT, and it is useful for preventing neurological deterioration in mild cases. BAO with a low NIHSS score in the presence of eye movement disorder as the main symptom may be a good indication for MT.

Different aspects of early and late development of atrial fibrillation during hospitalization in cryptogenic stroke.

The detection of underlying atrial fibrillation (AF) has become increasingly possible by insertable cardiac monitoring (ICM). During hospitalization for cryptogenic stroke, factors related to the early and late development of AF have not been studied. CHALLENGE ESUS/CS is a multicenter registry of cryptogenic stroke patients undergoing transesophageal echocardiography. Twelve-lead electrocardiogram, continuous cardiac monitoring, and 24-h Holter electrocardiogram were all used for the detection of AF. Early and late detection of AF was determined with an allocation ratio of 1:1 among patients with AF. A total of 677 patients (68.7 ± 12.8 years; 455 men) were enrolled, and 64 patients developed AF during hospitalization. Four days after admission was identified as the approximate median day to classify early and late phases to detect AF: ≤ 4 days, 37 patients; > 4 days, 27 patients. Multiple logistic regression analysis showed that spontaneous echo contrast (SEC) (OR 5.91; 95% CI 2.19-15.97; p < 0.001) was associated with AF ≤ 4 days, whereas a large infarction > 3 cm in diameter (OR 3.28; 95% CI 1.35-7.97; p = 0.009) was associated with AF > 4 days. SEC and large infarctions were important predictors of in-hospital AF detection, particularly in the early and late stages, respectively; thus, they could serve as indications for recommending ICM.

[Spontaneous intracranial hypotension complicated by atlantoaxial subluxation: a case report].

A 54-year-old woman presented at the hospital with headache and posterior neck pain, which worsened when standing or in the sitting position and improved when in the supine position. A diagnosis of rheumatoid arthritis was made at the age ofin 33 years, and the patient has been taking methotrexate and methylprednisolone. Cervical MRI and magnetic resonance myelography showed the appearance of CSF leakage, resulting in a diagnosis of spontaneous intracranial hypotension. A diagnosis of atlantoaxial subluxation was also made based on the abnormal anterior position of the atlas (C1) in the cervical X-ray image. The CSF leakage corresponded with the atlantoaxial subluxation region, which indicated that spontaneous intracranial hypotension was caused by the compression of the dura mater. These symptoms were improved following treatment with the intravenous drip of the extracellular fluids, and she was discharged from the hospital on day 25. The disruption of the dura matter induced by atlantoaxial subluxation is a rare complication but is worth considering when determining the etiology of spontaneous intracranial hypotension.

Effect of Preoperative Administration of Proprotein Convertase Subtilisin/Kexin Type 9 Inhibitor on Carotid Artery Stenting.

BACKGROUND: Perioperative embolic stroke is one of the most serious complications during carotid artery stenting (CAS). Proprotein convertase subtilisin/kexin type 9 inhibitor (PCSK9i) is a low-density lipoprotein-lowering drug that inhibits proprotein convertase subtilisin/kexin type 9, which normally binds to the low-density lipoprotein cholesterol (LDL-C) receptor. Its combination with statin significantly decreases LDL-C levels. PCSK9i is expected to achieve lower LDL-C levels than single use of statin. This study aimed to investigate whether perioperative PCSK9i administration stabilizes carotid artery plaque and reduces perioperative complications of CAS. METHODS: Nine patients with symptomatic stenosis (North American Symptomatic Carotid Endarterectomy Trial [NASCET] 50%) or asymptomatic stenosis (NASCET ≥ 80%) were included. PCSK9i was administered at least twice (once in 2 weeks) in the outpatient clinic before CAS. Perioperative complications; results from blood tests, magnetic resonance imaging (MRI), magnetic resonance angiography, and carotid ultrasonography (US); and modified Rankin scale score at discharge were assessed. RESULTS: High intensity on diffusion-weighted imaging was not observed in 8 patients. Changes in carotid plaque characteristics were found with MRI and/or carotid US in 7 patients. The plaque to muscle ratio decreased in 3 patients. The carotid plaque became hyperechoic in 2 patients, and the fibrous cap was seen more clearly on carotid US. Two patients had findings of stabilized plaque on MRI and carotid US, which indicates that plaque transformation was more stable. CONCLUSIONS: Lowering LDL-C level could reduce ischemic complications, and low LDL-C level affects plaque stability and antithrombus formation. PCSK9i can possibly stabilize carotid plaque and reduce perioperative complications of CAS.