Isolated cerebellar infarction as a presenting symptom of polycythemia vera.

Although polycythemia vera is one of the reported causes for cerebral infarction, isolated cerebellar infarction, a rare disorder, was never reported in combination with polycythemia vera. This is a report of a 72-year-old woman in whom isolated cerebellar infarction was the presenting manifestation of polycythemia vera. The patient was treated with recurrent phlebotomies until the hematocrit decreased to < 45%. This treatment was followed by marked neurological improvement. A better awareness of the possibility of cerebellar infarction in polycythemia vera may disclose additional cases.     

Cerebral Infarction and Myalgia in a 75-year-old Man with Eosinophilic Granulomatosis with Polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare condition of systemic vasculitis of small to medium-sized blood vessels. We herein report the case of a 75-year-old man who presented with hemiplegia on his right side due to cerebral infarction following myalgia and a high fever. He had no history of asthma or allergic rhinitis. He was diagnosed with EGPA based on the presence of eosinophilia, sinusitis suggested by magnetic resonance imaging, and muscle pathology. His hemiplegia improved rapidly after corticosteroid therapy. This case suggests that EGPA should be a differential diagnosis of cerebral infarction with myalgia and eosinophilia.     

Paraneoplastic eosinophilic fasciitis: a case report

A 60-year-old white woman with polycythemia rubra vera post splenectomy in November 2001 was found to have peripheral white blood cell counts increasing over 3 months. Cytogenetics revealed trisomy of chromosomes 8 and 9, and bone marrow biopsy showed hypercellular, fibrotic bone marrow consistent with myelofibrosis of polycythemia rubra vera. Two months later, the patient developed acute swelling and pain in her lower extremities. The clinical symptoms along with confirmatory histology supported the diagnosis of eosinophilic fasciitis. This is the first reported case in the English literature of an association between polycythemia vera and eosinophilic fasciitis.     

Persistent Hemichorea as a Preceding Symptom of Cerebral Infarction Due to Middle Cerebral Artery Stenosis

We herein report an 84-year-old woman with right middle cerebral artery (MCA) stenosis who presented with persistent left hemichorea preceding cerebral infarction. She visited our hospital on day 9 after the hemichorea onset. Magnetic resonance imaging (MRI) showed no acute cerebral infarction. Magnetic resonance angiography revealed right MCA stenosis. Her hemichorea persisted for 19 days and subsequently disappeared. On day 21, she developed left hemiplegia. Repeat MRI revealed a cerebral infarction in the right putamen. MCA stenosis can present with persistent hemichorea, even in the absence of cerebral infarction. Persistent hemichorea with MCA stenosis may presage cerebral infarction.     

Mirtazapine in progressive multifocal leukoencephalopathy associated with polycythemia vera

Progressive multifocal leukoencephalopathy (PML) is a usually fatal cerebral white matter disease found in patients with human immunodeficiency virus infection and other immunocompromised states. We present the case of a 63-year-old woman with polycythemia vera who developed a progressive focal neurological deficit with white matter abnormalities on magnetic resonance images of the brain that was proved on biopsy to be PML. She was treated with the serotonin reuptake inhibitor mirtazapine and remains neurologically stable, with resolution of cerebral lesions, >2 years after diagnosis. We propose that mirtazapine should be investigated further for use in PML.     

Acquired von Willebrand disease in patients with polycythemia rubra vera

Platelet function and factor VIII complex were evaluated in ten patients with polycythemia rubra vera. Seven patients showed abnormal epinephrine-induced aggregation. The intracellular concentrations of adenosine diphosphate (ADP) were below normal, and the ratio of adenosine triphosphate (ATP)/ADP was greater than normal. In four of eight cases, there was a decrease in ristocetin cofactor activity and a reduction in the slowly migrating forms of vWF:Ag on crossed immunoelectrophoresis. Defect of large multimers of vWF:Ag was also observed. The ratio of vWF:Ag to ristocetin cofactor was elevated in these patients. Plasma from the patients had no effect on normal plasma except in one case, in which isolated IgG appeared to cause inactivation of ristocetin cofactor. Treatment with 1-deamino-8-arginine vasopressin caused correction of the vWF abnormalities with rapid return of ristocetin cofactor to baseline in some patients. The present study shows that the alterations of multimeric structure of vWF occur in more than 50% of patients with polycythemia rubra vera and are in some part due to the inhibitor specific for vWF.     

Phase II open label trial of imatinib in polycythemia rubra vera

Polycythemia rubra vera is a chronic myeloproliferative disorder characterized by panmyelosis with the resultant potential for thrombosis, myelofibrosis, and acute leukemia. Treatment has rested on phlebotomy and hydroxyurea. In 2002, we reported two patients who were unable to tolerate hydroxyurea but responded to imatinib mesylate (Gleevec). These patients have remained in complete hematologic remission on imatinib since 1999. As a result we began a phase II, open label trial of imatinib in patients with polycythemia vera. Patients meeting the Polycythemia Vera Study group criteria for the diagnosis of polycythemia vera, either naïve or intolerant to prior treatment were allowed to enroll. Initial therapy was begun with imatinib mesylate at 400 mg a day and two dose escalations, one to 600 and second to 800 mg a day, were allowed for patients not achieving a target hematocrit of 44 or less; or a platelet count of less than 600,000/mm³. Twenty patients were enrolled, 15 achieved complete hematologic remission within 12 weeks and ten remain on study. Six patients remain in remission on 400 mg a day and four on 500 mg a day. Gastrointestinal or cutaneous toxicities were primarily grade I or II. All patients were negative for bcr/abl. Imatinib mesylate is capable of producing hematologic remission in the majority of patients with polycythemia vera and provides another option for patient management, particularly in those intolerant to hydroxyurea.     

十例Percheron动脉梗死患者的临床特征分析

目的分析10例Percheron动脉梗死患者的临床特征,以期提高临床对该病的认识。方法选取2015年9月—2018年10月六安市人民医院收治的Percheron动脉梗死患者10例,回顾性分析其首发症状、临床表现、危险因素、发病机制、影像学特征、治疗策略、预后及后遗症等。结果(1)首发症状及临床表现:4例以意识障碍为首发症状,其中1例伴有应激性溃疡及反复呕吐,1例发病前出现黑蒙,1例伴有头晕、复视;6例表现为肢体偏瘫,2例表现为反应下降,2例表现为人事不知,1例表现为眼睑下垂。(2)危险因素及发病机制:7例合并高血压,4例合并2型糖尿病,3例合并血脂异常,4例吸烟;5例为小动脉闭塞性病变,2例为大动脉粥样硬化性病变,1例为心源性栓塞(心房颤动),2例发病机制不明。(3)影像学特征:10例患者颅脑CT检查均未见丘脑病变,行颅脑磁共振成像(MRI)检查发现双侧丘脑旁正中区域呈对称或不对称蝶形长T1、长T2信号,弥散加权成像(DWI)示双侧丘脑旁正中区域、中脑高信号,1例见中脑“V”字征。(4)治疗策略、预后及后遗症:10例患者均给予神经内科专科治疗,其中1例采用阿替普酶(rt-PA)静脉溶栓治疗后意识障碍很快缓解;随访3个月,4例患者预后良好,6例患者预后不良,其中3例放弃治疗而死亡;5例患者遗留肢体偏瘫,2例患者遗留眼球运动障碍。结论Percheron动脉梗死“三联症”为意识障碍、记忆障碍、双侧垂直注视性麻痹,但临床表现为典型“三联症”的Percheron动脉梗死患者可能很少;Percheron动脉梗死的发病机制尚存在争议,而不同丘脑核受累在Percheron动脉梗死患者中的阶段性作用尚不完全清楚;Percheron动脉梗死患者病情进展较快,预后多不良,静脉溶栓及动脉介入治疗可能有利于改善Percheron动脉梗死患者预后。     

Polycythemia vera presenting as acute myocardial infarction: An unusual presentation

Acute myocardial infarction (AMI) is usually seen in the setting of atherosclerosis and its associated risk factors. Myocardial infarction in the young poses a particular challenge, as the disease is less likely, due to atherosclerosis. We report the case of a 37-year-old female patient who presented with ST segment elevation anterolateral AMI. The only abnormality on routine blood investigation was raised hemoglobin and hematocrit. After further testing, she was diagnosed according to the World Health Organization (WHO) criteria with polycythemia vera. This case illustrates the importance of recognizing polycythemia vera as an important cause of thrombosis, which can present initially as AMI, and to emphasize the early recognition of the disease in order to initiate appropriate management strategies.     

Another look at survival curves in chronic myelocytic leukemia

A patient with polycythemia rubra vera and myeloid metaplasia required splenectomy because of local symptoms attributable to a massively enlarged spleen. Preoperative embolization of the splenic circulation with inert materials was performed to reduce splenic size and vascularity in order to permit safe splenectomy. This case illustrates the utility of preoperative splenic embolization when routine splenectomy is contraindicated for technical reasons. The hazards of this technique and guidelines for its clinical use are outlined.     

57例老年人无肢体瘫痪脑梗塞临床与CT分析

目的：探讨老年人无肢体瘫痪脑梗塞临床与CT的关系。方法：对57例老年人无肢体瘫痪脑梗塞的发病诱因，临床表现及CT进行回顾性分析，结果：高血压，TIA，高血脂，冠心病仍为无肢体瘫痪脑梗塞的重要危险因素，结论：肢体瘫痪的发生与否是和传导通路，梗塞部位和病灶大小有头，而与梗塞数目无关，在无MRI条件时，早期头颅CT扫描是诊断的唯一依据。     

The effect of the JAK2 V617F mutation on PRV-1 expression

JAK2(V617F)an acquired mutation of JAK2, is present in a majority of patients with polycythemia vera and to a lesser extent among patients with the other myeloproliferative disorders. We analyzed the effect of JAK2(V617F) on the expression of polycythemia rubra vera 1(PRV-1), using an in vitro model. Compared to wild-type JAK2, the presence of JAK2(V617F) increased both PRV-1 protein and mRNA levels in murine myeloid cells. A JAK2 inhibitor eliminated the V617F-induced increase in PRV-1 expression.     

Aspirin-responsive painful red,blue, black toe,or finger syndrome in polycythemia vera associated with thrombocythemia

Five patients with red, purple blue, or black toes or fingers due to thrombocythemia associated with polycythemia vera (polycythemia and thrombocythemia vera) in four and essential thrombocythemia (thrombocythemia vera) in one are described. The microvascular erythromelalgic syndrome of thrombocythemia was overlooked and progressed to cold blue swollen and painful fingers or black toes in three patients with polycythemia and thrombocythemia vera due to arteriographically documented occlusions of digital or large peripheral arteries with no evidence of preexistent atherosclerotic vascular disease. Concomitant erythromelalgia of the hand palm could be confirmed by the histopathological findings of arteriolar thrombotic lesions in the reticular dermis in two patients with polycythemia and thrombocythemia vera. The increased hematocrit in the presented patients with polycythemia and thrombocythemia vera contributed to the progression of the microvascular syndrome of thrombocythemia to major occlusive ischemic events of the extremities. Standard therapy with oral anticoagulants and reduction of the hematocrit to normal by bloodletting did not affect the platelet-mediated microvascular erythromelalgic, ischemic symptoms in the patients with polycythemia vera because thrombocythemia vera persisted. Complete relief of pain and restoration of the ischemic acral circulation disturbances in patients with thrombocythemia vera or thrombocythemia associated with polycythemia vera in maintained remission by bloodletting could be obtained by long-term treatment with low-dose aspirin.     

Polycythemia as the first manifestation of Cushing's disease

A 39-yr-old man presented to our hospital with unexplained erythrocytosis and hypertension. His follow-up for erythrocytosis had begun 2 yr earlier in another hospital and he had been diagnosed with polycythemia rubra vera. On admission to our hospital he was hypertensive (165/95 mmHg) and, except for the presence of moon-like face and facial plethora, his physical examination was normal. His hemoglobin concentration was 19.2 g/dl, and hematocrit was 58.9% with an increased red blood cell mass of 58 ml/kg as measured by radioisotope (Cr51). Blood film, other hematological indices except for elevated leukocyte alkaline phosphatase score, arterial gas analysis, and examination of aspirated bone marrow were all normal. An abdominal ultrasonography showed no evidence of splenomegaly. A diagnosis of probable secondary erythrocytosis was made. Early-morning serum cortisol and 24-h urinary free cortisol concentration as well as serum ACTH were high. Serum cortisol was not suppressed by low-dose dexamethasone, but suppressed by high-dose dexamethasone. Pituitary magnetic resonance imaging showed no lesion. After inferior petrosal sinus sampling suggesting right-central ACTH secretion, the patient underwent transnasal-transsphenoidal pituitary adenomectomy. Both hypercortisolemia and erythrocytosis regressed completely after the adenomectomy. After the operation, the patient's hemoglobin concentration and hematocrit decreased steadily, and 1 month post-adenomectomy his hemoglobin is 14.9 g/dl and hematocrit 44.8%. Thus, Cushing's syndrome should be a routine part of evaluation of unexplained polycythemia.     

Expression of polycythemia rubra vera-1 decreases the dependency of cells on growth factors for proliferation

An increase in the level of polycythemia rubra vera -1 (PRV-1) mRNA has been reported in some myeloproliferative disorders. We have studied the effects of PRV-1 on cell proliferation and cell survival. In cell growth assays, the number of heterologous cells expressing PRV-1 increased faster than sham-transfected cells, a difference that was more pronounced in serum-free media. Even after 5 days of exposure to serum-free media, cells expressing PRV-1 continued to proliferate, whereas the control cells ceased to proliferate. We conclude that PRV-1 is a pro-proliferation molecule, and hypothesize that its overexpression may have a role in the pathogenesis of myeloproliferative disorders.     

脑小血管病引起的认知障碍磁共振波谱和弥散张量成像

脑小血管病(cerebral small vessel disease,SVD)的临床症状主要表现为认知障碍,病理学改变则表现为白质疏松和(或)腔隙性梗死.然而,目前尚不清楚SVD引起的认知障碍是否与白质损害程度相关.磁共振波谱分析能无创件榆测活体脑组织的代谢产物浓度,直接反映脑代谢;弥散张量成像是特异性观察脑白质的无创性新技术,通过平均弥散、各向异性等不同参数来定量评价脑白质的细微结构异常.磁共振波谱分析与弥散张量成像联合应用能更敏感地显示白质损害程度,为SVD引起的认知障碍提供研究工具.     

Acute brain injury in hypoglycaemia-induced hemiplegia.

BACKGROUND: The development of hemiplegia as a result of hypoglycaemia was first described in 1928. However, the mechanism remains unclear. CASE REPORT: We report a case of a 58-year-old male with diabetes, who developed left hemiplegia during a severe hypoglycaemic event. Results Diffusion-weighted magnetic resonance imaging detected an increased signal intensity in the pons, indicating that the patient's hemiplegia resulted from acute brain injury. CONCLUSIONS: This report provides evidence that acute brain injury may be a cause of the neurological deficit.     

Plaque rupture on the carotid artery observed by Doppler ultrasonography--a case report.

Plaque rupture may play an important role in acute cerebral events. Recent technological advances in ultrasonographic systems provide a feasible diagnostic modality of atherosclerotic lesion by clearly visualizing flow conditions and vessel morphology. A 58-year-old man with chronic renal failure underwent duplex Doppler ultrasound in which ruptured plaque on carotid arterial wall was imaged. Color Doppler imaging showed that the plaque shoulder at the proximal site was hit by forward flow to the brain and ruptured. Curling flow in the inner space of the plaque was observed. Carotid arteriography at the corresponding site of ruptured plaque revealed the space within the plaque similarly contrasted. A magnetic resonance imaging of brain performed after identifying plaque rupture revealed small cerebral infarcts at the cerebral cortex. Plaque rupture could be demonstrated by color Doppler imaging at the proximal edge of plaque and curling flow in the intraplaque space may have released small thrombi to vessel lumen. Plaque rupture of the carotid artery may play an important role in the development of small cerebral infarction.