Lacunar infarctions due to cholesterol emboli.

BACKGROUND AND PURPOSE: Hypertension is commonly considered the major cause of lacunar infarctions. However, in some cases, it has been suggested that lacunes could be caused by cerebral emboli from cardiac or carotid sources. Cholesterol cerebral emboli have been rarely reported as a cause of lacunes. CASE DESCRIPTION: We describe a 79-year-old patient with a progressive multi-infarct dementia who developed transient motor aphasia and paresis of the right arm. Computed tomography showed lacunar infarcts in the right caudate nucleus, left thalamus, and left putamen, as well as an old right frontal infarction. Neuropathological examination demonstrated no prominent vascular hyalinosis, but did show multiple cholesterol emboli occluding small arteries around lacunar infarcts and leptomeningeal arteries near cortical infarcts. The cholesterol material presumably originated in the extended atheromatous changes along the aortic arch. CONCLUSIONS: Our report confirms that lacunes can be caused by cholesterol emboli in some patients. Small cerebral emboli should not be overlooked as a cause of lacunes.     

Cardio-facio-cutaneous syndrome and moyamoya syndrome

We reported a patient with cardio-facio-cutaneous (CFC) syndrome associated with moyamoya syndrome. The patient was referred at 6 years 5 months with left hemiplegia and right-sided eye deviation. He had an apparently short stature, macrocephaly, left ptosis and atopic skin, and was odd looking. He exhibited an incomplete right bundle branch block on electrocardiogram and an atrial septal defect on ultrasound cardiography. He was diagnosed as having CFC syndrome. Head magnetic resonance imaging showed a flow void in the bilateral basal ganglia, but did not show any ischemic changes. Magnetic resonance angiography showed bilateral stenosis with an internal carotid artery at the Willis artery ring level and bilateral moyamoya. Contrast angiography demonstrated occlusion of both middle cerebral arteries. Cerebrovascular anomalies have not previously been reported in CFC syndrome. This is the first case of CFC syndrome associated with moyamoya syndrome.     

Large vessel occlusions requiring repeated mechanical thrombectomy caused by silent myocardial infarction in a young adult

ObjectivesSilent myocardial ischemia, defined as objective evidence of myocardial ischemia without symptoms, is associated with ischemic stroke. Nevertheless, silent myocardial infarction is a rare cause of ischemic stroke, especially in young adults with no medical history.Materials and methodsHerein, we report a young adult patient with acute ischemic stroke treated with repeated mechanical thrombectomy for recurrent large vessel occlusions caused by left ventricular thrombus following a silent myocardial infarction.ResultsA 40-year-old man was transferred by ambulance to our hospital because of a generalized seizure. He was diagnosed with cerebral infarction and left middle cerebral artery occlusion. We performed intravenous thrombolysis and mechanical thrombectomy. Recanalization was achieved and his symptoms gradually improved. However, the day after treatment he developed bilateral cerebellar infarction and basilar artery occlusion. We performed a second mechanical thrombectomy and recanalization was achieved. Transthoracic echocardiography revealed a mobile left ventricular thrombus. Although he had no previous chest symptomatic episodes, cardiac examination confirmed myocardial infarction of unknown onset. He was diagnosed with acute ischemic stroke with large vessel occlusions caused by left ventricular thrombus following a silent myocardial infarction. Anticoagulation therapy reduced the amount of thrombus. At 1-year follow-up, he had not experienced any recurrences or symptoms.ConclusionsSilent myocardial infarction should be considered a cause of ischemic stroke in young adults, even without any vascular risk factors. Recurrent large vessel occlusion may occur in patients with left ventricular thrombus, and repeated mechanical thrombectomy should be considered for treatment.     

Ischemic stroke related to pauci-symptomatic acute aortic dissection. Risks of intravenous thrombolysis

IntroductionAcute aortic dissection involving the cervical arteries often induces cerebral infarction. In this context, there is a high risk of hemorrhage and thrombolytics are contra-indicated.ObservationWe report two patients with a cerebral infarction which occurred after a pauci-symptomatic and undiagnosed aortic dissection treated with thrombolysis. The outcome was poor for both patients.DiscussionIschemic strokes related to acute aortic dissection often involve the right carotid artery territory and may manifest without pain. A systematic assessment of supra-aortic arteries might help to identify the underlying diagnosis.ConclusionAcute aortic dissection should be systematically mentioned in the management of the acute ischemic stroke.     

Spectrum of medial medullary infarction: clinical and magnetic resonance imaging findings

Among 4200 consecutive patients admitted to three hospitals with acute ischemic stroke, we found only 11 patients in whom magnetic resonance imaging (MRI) had proved that they had medial medullary infarction (MMI). In our centers, patients with MMI were less than 1 % of those with vertebrobasilar stroke. The infarcts documented by MRI were unilateral in 10 patients and bilateral in one. On clinico-topographical analysis there were four clinical patterns: (1) Classical Dejerine's syndrome was the most frequent, consisting of contralateral hemiparesis, lemniscal sensory loss and ipsilateral lingual palsy in 7 of the eleven patients. (2) Pure hemiparesis was present in 2 patients; (3) Sensorimotor stroke was present in 1 patient with contralateral hemiparesis, hypesthesia and mild decrease in pain sensation without lingual palsy; (4) Bilateral MMI syndrome in 1 patient, accompanied by tetraparesis, bilateral loss of deep sensation, dysphagia, dysphonia and anarthria. Presumed causes of MMI were intrinsic branch penetrator artery disease with concomitant vertebral artery stenosis in 6 of the 11 patients, vertebral artery occlusion in 2, dolichoectatic vertebrobasilar arteries in 2, a source of cardiac embolism in 1. Prognosis at 3 months was favorable in 8 patients, but the patient with bilateral MMI syndrome had persisting motor deficit causing limitation of daily activities, and 2 died from systemic causes. The classical triad of acute MMI facilitates the diagnosis, although the recognition of this syndrome in patients with incomplete manifestations can be difficult and occurs more frequently than commonly thought. Moreover, vertebral artery atherosclerosis and branch atheromatous disease of the penetrating arteries are the main causes of medullary infarction. Received: 21 February 2001, Received in revised form: 22 February 2001, Accepted: 17 May 2001     

分支动脉粥样硬化病附3例报告及文献复习

目的：探讨分支动脉粥样硬化病（BAD）的发病机制、临床特征和影像学诊断,并与腔隙性脑梗死相鉴别,提高对BAD的认识.方法：分析3例BAD患者的临床表现和影像学特征,并结合文献复习进行研究.结果：3例BAD患者临床分别表现为短暂脑缺血发作、纯运动型腔隙综合征和运动感觉型腔隙综合征; MRI水平位断层均显示侧脑室旁呈连续3~5个层面的斑点（片）状长T1、长T2信号,冠状位可见纵长形似＂逗号＂样的＂巨大腔隙梗死＂灶.结论：BAD是一种新型脑梗死,对临床疑似腔隙性脑梗死尤其是＂巨大腔隙性梗死＂而又不能完全符合诊断标准的病例,建议作头颅MRI冠状位检查以提高对BAD的诊断,有利于进一步治疗.     

La vasodilatation a-t-elle un rôle dans la migraine ? Non

IntroductionThe hypothesis that migraine pain is caused by vasodilation has been challenged by clinical and experimental evidence.State of artThe most convincing arguments against the vascular hypothesis come from neuroimaging data. Magnetic resonance imaging studies show that spontaneous migraine attacks are not accompanied by extracranial vasodilation, and by only slight dilation of the intracranial arteries. Pharmacologically-induced migraine attacks also provide further evidence against the role of vasodilation in migraine. Vasodilators such as sildenafil and nitroglycerine trigger attacks without dilation of the middle cerebral artery diameter, whereas VIP (vasoactive intestinal peptide) markedly dilates intra- and extracranial arteries but does not induce migraine attacks. Clinical studies also show a lack of correspondence between the subjective experience of throbbing headache and the arterial pulse. Moreover, many acute anti-migraine agents are not vasoconstrictors.PerspectivesFurther studies are necessary to clarify the mechanisms of migraine headache generation.ConclusionsContrary to a longstanding and widespread belief, vasodilatation is neither sufficient nor necessary to cause migraine headache and is probably an epiphenomenon.     

Mechanisms involved in large subcortical infarcts]

Large subcortical infarcts(maximum diameter of infarct > or = 20 mm) result from various stroke patterns, including striatocapsular infarcts (SCI), corona radiata infarcts, centrum semiovale infarcts, and internal borderzone infarcts. A systematic investigation of stroke pathogenesis involved in large subcortical infarcts, however, has not been performed. This study attempted to clarify the stroke mechanisms involved in large subcortical infarcts, by examining 50 patients with large subcortical infarcts out of 430 ischemic stroke patients consecutively registered in our department. The subjects were divided into two groups according to the vascular territories involved on the MRI: 1) the lenticulostriate arteries group for 39 patients whose infarcts were restricted to within the vicinity of the lenticulostriate arteries; 2) the internal borderzone group for 11 patients whose infarcts mainly involved the internal borderzone (the upper part of the corona radiata and the centrum semiovale) between the territories of the deep perforating branches from the basal cerebral arteries and the medullary branches from the superficial pial arteries. Stroke pathogenesis were classified into the following 6 categories: A) cardiogenic embolism, 9 patients; B) artery-to-artery embolism, 6 patients; C) cryptogenic embolism, 2 patients; D) thrombotic MCA (M1) occlusion, 9 patients; E) thrombotic ICA occlusion, 10 patients; F) undetermined cause, 14 patients. The lenticulostriate arteries group consisted of 9 patients with cardiogenic embolism, 6 with artery-to-artery embolism, 2 with cryptogenic embolism, 8 with thrombotic M1 occlusion, and 14 with undetermined cause. The internal borderzone group consisted of 10 patients with thrombotic ICA occlusion and 1 patient with thrombotic M1 occlusion. The stroke pathogenesis of the undetermined cause is considered to be thrombotic occlusion at the orifice of the lateral lenticulostriate artery, a so-called "branch atheromatous disease (BAD)". The patients in this group experienced a gradual onset, and did not have a cardiac source of the embolism or proximal large artery disease. Among the patients reported as having SCI, BAD may play a role in some cases, especially in those whose the cause was classified as "undetermined". In conclusion, the lenticulostriate arteries group exhibited a higher frequency of cerebral embolisms (cardiogenic embolism, artery-to-artery embolism, and cryptogenic embolism) and thrombotic M1 occlusion, whereas the internal borderzone group had a higher frequency of thrombotic ICA occlusion.     

Interventions in cerebrovascular emergencies among patients with Paroxysmal nocturnal haemoglobinuria - A word of caution

IntroductionParoxysmal nocturnal haemoglobinuria (PNH) is a clonal hematopoietic disorder, where there is deficiency of glycosylphosphatidylinositol (GPI) anchored proteins in the cell membrane, leading to increased complement sensitivity of red blood cells, intravascular hemolysis and vascular inflammation. Arterial and venous strokes in patients with PNH are a rarity posing significant diagnostic and therapeutic challenges. We report our experience with management of PNH patients with cerebrovascular emergencies.MethodsWe report 2 patients with PNH, one who was previously diagnosed with PNH and had arterial stroke, the other had an index presentation of cerebral venous sinus thrombosis (CVT) and was subsequently diagnosed with PNH. We also present the systematic review of literature reporting similar cases, highlighting the challenges in management.ResultsBoth patients presented to our centre with cerebrovascular emergency. The first patient was a diagnosed with PNH, and presented with left hemispheric infarction caused by thrombosis of middle cerebral artery. He was thrombolysed and underwent mechanical thrombectomy, which was unsuccessful in view of repeated re – thrombosis of the vessel. The patient survived with significant disability. The second patient had severe cerebral venous sinus thrombosis with large right hemispheric hemorrhagic venous infarction. She underwent emergency decompressive hemicraniectomy complicated by massive blood loss and disseminated intravascular coagulation. She subsequently had recurrent life threatening intracranial bleed secondary to platelet transfusions, thrombocytopenia, and use of contrast agents. She progressed to develop Budd Chiari syndrome and was initiated on Eculuzimab. She became transfusion independent, however remained in minimally conscious state and succumbed to sepsis.ConclusionsManagement of arterial and venous strokes is complex in patients with PNH. Invasive procedures and platelet transfusions are to be avoided in acute thrombosis, till robust evidence is available establishing the safety of the same in patients with PNH. Eculuzimab is a promising option, but far from reach for patients in developing countries.     

Fibrous dysplasia of the temporal bone and cerebral occlusive vasculopathy: report of a case]

Fibrous dysplasia is a benign disease with unknown etiology. Skull attempt may cause neurological disorders. The diagnostic can be made through radiological examination. A case of a patient with right temporal bone involvement with facial palsy, right parietal lobe infarctions and elevated anticardiolipin antibody titers is presented. Cerebral angiography showed occlusion of several cerebral arteries. Post mortem examination displayed recent myocardial infarction, ischemic cerebral softening, and generalized arteriosclerosis. The simultaneous occurrence of fibrous dysplasia and a probable anticardiolipin syndrome is commented.     

Toxocara canis cerebral vasculitis revealed by iterative strokes

IntroductionCerebral vasculitis is a rare and severe condition, posing problems for diagnosis and treatment. Toxocara canis cerebral vasculitis is exceptionally rare, with only 4 cases having been reported. We report an additional case revealed by iterative strokes.ObservationA 49-years-old Laotian man presented with right ACA infarction associated with contrast enhancement of cerebrospinal fluid, and multiple segmental stenoses in small and medium caliber encephalic arteries, in a context of hypereosinophilia and chronic headaches. Laboratory tests showed lymphocytic meningitis and T. canis antibody IgE in the blood and CSF. The diagnosis of T. canis cerebral vasculitis was retained. During follow-up, the patient presented again with left pontine hemorrhagic stroke. Conventional cerebral angiography confirmed progression of vasculitis despite treatment.ConclusionThis case-report illustrates the diagnostic and therapeutic difficulties associated with vasculitis.     

Simultaneously Occurring Ischemic Stroke,Leg Artery Occlusion,and Pulmonary Embolism Induced by a Uterine Myoma

ObjectivesParadoxical embolism from venous thrombosis through the patent foramen ovale is a rare but well-known cause of stroke in young adults. Here, we report a case of simultaneous middle cerebral artery infarction, multiple occlusions of the leg arteries, and pulmonary thromboembolism from the venous thrombus, all due to compression of the external iliac vein by a uterine leiomyoma.Materials and Methods (Case presentation)A 44-year-old woman presented with left hemiparesis and central-type left facial palsy. She denied a history of hypertension, diabetes mellitus, previous cerebral infarction, myocardial infarction, smoking, or oral contraceptive use. The patient recovered completely after injection of tissue plasminogen activator. Brain diffusion-weighted imaging showed an acute right middle cerebral artery infarction. Transcranial Doppler with saline agitation test revealed a right-to-left shunt, suggesting a patent foramen ovale. Chest computed tomography revealed multiple pulmonary thromboembolisms. Lower extremity sonography and lower extremity computed tomography revealed a multifocal thrombus in the major veins and arteries of the left leg. Moreover, a large uterine myoma compressing the left external iliac vein was noted on lower extremity computed tomography.ResultsAfter the treatment of pulmonary thromboembolism and venous thrombosis with rivaroxaban, surgical thrombectomy of the left popliteal artery, patent foramen ovale closure, and total hysterectomy were performed. Subsequently, she had no recurrent paradoxical embolism or pulmonary thromboembolism.ConclusionStructural abnormalities in the pelvic cavity are not commonly suspected as stroke etiology. However, examination of the pelvic cavity is advisable in young female stroke patients with pulmonary thromboembolism or other paradoxical embolisms.     

Arteriovenous Malformation with an Occlusive Feeding Artery Coexisting with Unilateral Moyamoya Disease

Background

Arteriovenous malformations (AVMs) with vascular abnormalities, including aneurysms, have been reported frequently. However, the coexistence of AVM and unilateral moyamoya disease is rare. We report herein an AVM patient who presented with acute ischemic stroke with unilateral moyamoya disease and occlusion of the feeding artery.

Case Report

A-41-year old man was admitted with sudden dysarthria and facial palsy. Brain computed tomography and magnetic resonance imaging revealed an acute infarction adjacent to a large AVM in the right frontal lobe. Cerebral angiography revealed occlusions of the proximal right middle cerebral and proximal anterior cerebral arteries, which were the main feeders of the AVM. Innumerable telangiectatic moyamoya-type vessels between branches of the anterior cerebral artery and dilated lenticulostriate arteries on the occluded middle cerebral artery were detected. However, a nidus of the AVM was still opacified through the distal right callosomarginal artery, which was supplied by the remaining anterior cerebral artery and leptomeningeal collaterals from the posterior cerebral artery.

Conclusions

While AVM accompanied by unilateral moyamoya disease is rare, our case suggests an association between these two dissimilar vascular diseases.     

脑梗死急性期梗死部位及白质病变与认知功能关系的临床分析

【摘要】
目的 研究急性脑梗死患者梗死部位及是否合并脑白质病变对患者早期认知功能的影响。
方法 采用临床痴呆评定量表、简易精神状态检查量表和中文版蒙特利尔认知评估量表对连续入组的急性脑梗死患者进行认知心理学评估,分析患者不同梗死部位包括皮层梗死、皮层下关键部位梗死和皮层下非关键部位梗死及是否合并白质病变对患者认知功能的影响。
结果 急性脑梗死患者血管性认知功能障碍组的平均年龄显著高于无认知功能障碍组（67.31 10.88 vs 57.09 9.91,P=0.015）,神经功能缺损评分显著高于无认知功能障碍组［3.0（2.0～4.0） vs 1.0（1.0～2.0）,P=0.012］。认知功能障碍组的日常生活能力评分显著低于无认知功能障碍组（81.67 23.55 vs 95.91 12.00,P=0.029）。两组患者梗死部位有显著差异（P=0.042）,皮层梗死更多见于血管性认知功能障碍组,皮层梗死患者的视空间与执行功能显著低于皮层下非关键位置梗死患者［1.5（0.0～3.0） vs 3.0（2.0～4.0）,P=0.016］。白质病变与认知障碍的发生无明显相关性。
结论 急性脑梗死患者早期认知障碍与年龄、严重的神经功能缺损、皮层梗死、日常生活能力下降密切相关。     

Clinical and neuroradiological features of internal watershed infarction and the occlusive diseases of carotid artery system

Abstract

Background: To describe the clinical and neuroradiological features of internal watershed (IWS) infarction and to analyze the relationship between IWS infarction and occlusive diseases of carotid artery system.

Methods: We identified 27 IWS infarction patients with diffusion-weighted magnetic resonance imaging by commonly used templates within 10 days after the onset. Patients with potential cardiac sources of embolism were excluded. Occlusive diseases of carotid arteries were assessed by magnetic resonance angiography or digital subtraction angiography. Baseline characteristics, clinical course, prognosis, neuroradiological features and symptomatic arteries were analyzed.

Results: Two patterns were identified: 14 patients had confluent IWS (C-IWS) infarction, and 13 patients had partial IWS (P-IWS) infarction. Twelve patients in C-IWS group and 10 patients in P-IWS group had severe stenosis (>70%) or occlusion in either the internal carotid artery (ICA) or middle cerebral artery (MCA). Occlusive diseases of ICA and non-lacunar syndrome were more prevalent in C-IWS group, while occlusive diseases of MCA and lacunar syndrome were more prevalent in P-IWS group. Concomitant small cortical lesions ipsilateral to IWS infarcts were more common in C-IWS group than in P-IWS group, and were significantly associated with ICA diseases. Clinical deterioration during the first seven days after admission and poor outcome assessed by the Modified Rankin Score 3 months after stroke were more prevalent in C-IWS group than in P-IWS group.

Conclusions: There is a relatively definite relationship between IWS infarction and occlusive diseases of carotid arteries. The distribution of symptomatic arteries (ICA or MCA) in C-IWS group is significantly different from that in P-IWS group. Both hypoperfusion and microembolism are probable mechanisms. There may be some differences in their roles in pathogenesis of C-IWS and P-IWS infarction. Microembolism may contribute C-IWS infarction in addition to hypoperfusion, which is the major pathogenesis of P-IWS infarction.     

脑穿支动脉病变梗死类型和神经影像学研究进展

大脑前动脉、大脑中动脉及后循环血管的穿支动脉,如豆纹动脉、脉络膜前动脉（anterior choroidal artery,AChA）和髓质动脉等,阻塞会导致脑深部缺血性病变。不同穿支动脉梗死类型不 同：豆纹动脉供血区域的梗死类型为腔隙性梗死、穿支动脉粥样硬化病型梗死和纹状体内囊梗死; AChA病变可导致AChA综合征;髓质分支梗死会导致半卵圆中心、放射冠部位的边界区梗死。7T二维 相位对比MRI、三维时间飞跃法MRA和三维快速自旋回波T1WI序列等神经影像学新技术有助于确定穿 支动脉早期病变,并可早期发现疾病的发生发展。     

Facial chorea and hemichorea due to cardiogenic cerebral embolism in the cortex and subcortical white matter]

A 62-year-old man was admitted to our hospital because of suddenly developed bilateral facial chorea and left-sided hemichorea. He had neither hemiparesis nor sensory disturbance. Diffusion-weighted magnetic resonance images of the brain showed acute cortical and subcortical infarctions at the right insula, frontal, temporal and parietal lobes. Tiapride hydrochloride was administered for his chorea. The chorea disappeared on the next day. We diagnosed him as cardiogenic cerebral embolism because he had a paroxysmal atrial fibrillation. We supposed that his chorea was induced by interruption of excitatory output from cerebral cortex to striatum and subthalamic nucleus. Contralateral cortical and subcortical infarction must be considered when a patient suddenly develops hemichorea.     

PRES and RCVS: Two Distinct Entities or a Spectrum of the Same Disease?

ObjectivesTo report a case of a patient with overlapping posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS), and review the existing literature emphasizing the pathophysiological overlap of these two entities.Materials and MethodsWe conducted a literature search in electronic database PubMed identifying studies reporting the overlap of PRES and RCVS.ResultsPRES and RCVS are two increasingly recognized entities that share similar clinical and imaging features. PRES is characterized by vasogenic edema predominantly in the parieto-occipital regions, associated with acute onset of neurological symptoms including encephalopathy, seizures, headaches, and visual disturbances. RCVS is characterized by reversible segmental and multifocal vasoconstriction of the cerebral arteries and classically presents with thunderclap headache, with or without associated focal neurological deficits and seizures. PRES is frequently associated with uncontrolled hypertension but can also be seen in the setting of renal failure, exposure to cytotoxic agents, or pre-eclampsia. RCVS is often triggered by exposure to vasoactive agents, postpartum state, or immunosuppression. We report a case of a patient presenting with vision changes and hemiparesis, and found to have extensive cytotoxic and vasogenic edema involving the cortex and subcortical white matter on brain imaging. These changes were primarily noted in the parieto-occipital and brainstem regions, along with features of reversible vasculopathy on vascular imaging suggestive of coexisting PRES and RCVS.ConclusionsPRES and RCVS share precipitating factors, clinical and radiological features, and frequently co-exist, suggesting a common pathophysiological mechanism related to reversible dysregulation of cerebral vasculature, endothelial dysfunction, and breakdown of the blood-brain barrier.