多發(fā)性周圍神經(jīng)病

多發(fā)性周圍神經(jīng)病病例特點(diǎn)右側(cè)上肢無(wú)力，伴記憶力、理解力障礙，反應(yīng)遲鈍一月，查體記憶力減退，計(jì)算力差，不能識(shí)別手指，左右側(cè)認(rèn)識(shí)不能，書寫不能。右側(cè)鼻唇溝淺，右側(cè)肢體肌力級(jí)Ⅴ－，右上肢腱反射活躍，雙側(cè)病理征（－)。頭MRI：左頂枕可見片狀長(zhǎng)T2混雜T1異常信號(hào)，左顳可見片狀長(zhǎng)T2短T1異常信號(hào)，

左頂枕、左顳可見不規(guī)則強(qiáng)化。2021/4/272病例特點(diǎn)全血增多，紅細(xì)胞系統(tǒng)尤為顯著，凝血機(jī)制輕度障礙，骨髓檢查呈增生改變；血氧飽和度95.9%；腦脊液蛋白115mg/dl；2021/4/273定位診斷周圍神經(jīng)(感覺神經(jīng)為主)：肢體麻木，長(zhǎng)手襪套樣痛覺減退，提示小纖維受損；雙肘、髖以下深感覺減退乃至消失，提示大髓纖維受損；雙下肢活動(dòng)無(wú)力，腱反射低下，提示運(yùn)動(dòng)纖維受損。2021/4/274定位診斷左大腦中動(dòng)脈系統(tǒng)Gerstmann綜合癥：主側(cè)角回；記憶力、理解力障礙，反應(yīng)遲鈍：顳葉右上肢活動(dòng)無(wú)力，腱反射活躍，右中樞性面癱：中央前回頭CT、MRI左額顳頂病灶。2021/4/275定性診斷

多發(fā)性周圍神經(jīng)?。簭闹w遠(yuǎn)端開始的對(duì)稱性深及淺感覺減退或消失，雙下肢活動(dòng)無(wú)力，腱反射減低，四肢遠(yuǎn)端皮膚營(yíng)養(yǎng)差，下肢誘發(fā)電位檢查發(fā)現(xiàn)傳入障礙。腦梗塞（出血性轉(zhuǎn)換)：存在血液學(xué)異常的證據(jù)，突發(fā)性局灶性神經(jīng)功能障礙，早期頭CT呈低密度改變,一周后頭MRI表現(xiàn)梗塞后出血改變2021/4/276病因診斷真性紅細(xì)胞增多癥患者顏面、手足皮膚呈暗紅色，結(jié)膜充血；全血增多，紅細(xì)胞系統(tǒng)尤其突出；骨髓呈增殖改變；出現(xiàn)腦血栓形成；血氧飽和度正常；脾腫大能排除相對(duì)性RBC增多及繼發(fā)性RBC增多癥。2021/4/277文獻(xiàn)復(fù)習(xí)Vaquez(1892)和Osler(1903)首先描述了真性紅細(xì)胞增多癥；為骨髓增殖性疾病(myeloproliferativedisorder)的一種，本綜合癥還包括：急性粒細(xì)胞性白血病慢性粒細(xì)胞白血病骨髓纖維化原發(fā)性血小板增多癥這些疾病各具特征，其臨床表現(xiàn)可有重疊，常常可從一種類型轉(zhuǎn)化為另一種。2021/4/278文獻(xiàn)復(fù)習(xí)發(fā)病率大約為1/100,000Annalsofinternalmedicine,1995,123(9)報(bào)道20年1213例：年齡跨度10－90歲，高發(fā)年齡41－80歲。男>女，為1.3:1，或2：1。2021/4/279文獻(xiàn)復(fù)習(xí)病因至今不明，1951年Dameskek提出了骨髓增殖癥的概念，認(rèn)為本病為其中一種。骨髓由多種多潛能干細(xì)胞克隆家族構(gòu)成，PV可能為起源于單一多潛能干細(xì)胞的克隆病。10％PV可轉(zhuǎn)化為粒細(xì)胞白血病。2021/4/2710臨床表現(xiàn)隱襲發(fā)病皮膚紫紅，頭痛，頭暈，視覺障礙，注意力不能集中，感覺異常高血壓，高心輸出狀態(tài)血栓或出血文獻(xiàn)復(fù)習(xí)2021/4/2711血栓：動(dòng)脈(冠狀動(dòng)脈，腦動(dòng)脈，周圍動(dòng)脈)靜脈(周圍靜脈,肝或門靜脈)小血管供血不足：紫紺，紅斑肢痛，甚至指趾壞疽。出血：Mild：鼻出血，牙齦出血，皮膚易于青紫Severe：消化道出血，月經(jīng)過多，咯血，腦出血文獻(xiàn)復(fù)習(xí)2021/4/2712神經(jīng)系統(tǒng)癥狀常為PV的主要臨床表現(xiàn)，百分比高達(dá)56－78％，據(jù)Millikan(1960)報(bào)道頸動(dòng)脈或椎基底動(dòng)脈間歇性供血不全發(fā)作是各類型紅細(xì)胞增多癥的重要合并癥。腦梗塞是PV最嚴(yán)重的合并癥，Lawrence(1953)報(bào)道207例中腦動(dòng)脈梗塞占10％，Silverstein(1962)報(bào)道511例PV首發(fā)癥狀中，頭痛41％，眩暈或頭暈30％，肢體麻木13％，中心暗點(diǎn)、視力模糊11％，絕大多數(shù)為閉塞性腦血管病，少數(shù)為腦出血文獻(xiàn)復(fù)習(xí)2021/4/2713Poza等（1996)分析了28例PV患者，發(fā)現(xiàn)13例有明確的多發(fā)性周圍神經(jīng)病，20例神經(jīng)傳導(dǎo)指數(shù)異常，屬于以感覺為主的軸索性神經(jīng)病；Yiannikas等(1983)分析了26例PV患者,其中部分行腓腸神經(jīng)活檢。符合慢性軸索變性神經(jīng)病。周圍神經(jīng)受損可能與微血管缺血有關(guān)(血粘度升高及血小板功能不全)。文獻(xiàn)復(fù)習(xí)2021/4/2714Thevascularcomplicationsinpatientswithpolycythemiaveraaremicrovascularcirculatorydisturbancestypicalofthrombocythemiaincludingerythromelalgia,peripheralischemia,atypicalcerebralischemicattacks,andmajorarterialandvenousthromboticevents.文獻(xiàn)復(fù)習(xí)2021/4/2715Polycythaemiaveraisaclonalmyeloproliferativedisordermainlyinvolvingproliferationoftheerythropoiesis.Themostseriouscomplicationsofthediseaseareathrombotictendencyandmyeloidmetaplasia.Thrombosesmainly(2/3)occurinthearterialandlessoften(1/3)inthevenoussystemandarethemostcommoncauseofdeath.文獻(xiàn)復(fù)習(xí)2021/4/2716HaematologicalDisordersAssociatedWithischaemicStroke

Cellulardisorders:Myeloproliferative:

Polycythaemiarubravera

Essentialthrombocythaemia(b)Sicklecelldisease(c)Paroxysmalnocturnalhaemoglobinuria(d)Thrombocytopenia(e)Leukaemia(f)Intravascularlymphoma

2021/4/2717Disordersofcoagulation/fibrin:Congenital:

Naturalanticoagulantdisorders:

ProteinCdeficiency,ProteinSdeficiencyActivatedproteinCresistanceAntithrombinIIIdeficiencyFibrinolyticsystemdisorders:

PlasminogendeficiencyAcquired:

HaematologicalDisordersAssociatedWithischaemicStroke

2021/4/2718(b)Acquired:Disseminatedintravascularcoagulation

Lupusanticoagulant/anticardiolipinsyndromePregnancyandthepuerperium

OralcontraceptivepillParaproteinaemias

HaematologicalDisordersAssociatedWithischaemicStroke(Continue)2021/4/2719Thegoaloftreatmentisthepreventionofthromboemboliccomplicationsandofmyeloidmetaplasia.Theinitialtreatmentalwaysconsistsofphlebotomiesuntilahematocrit<45%isachieved.文獻(xiàn)復(fù)習(xí)2021/4/2720Thedecisionregardingmaintenancetherapyisdifficult.Whilehydroxyureaorradiophosphorusisthetreatmentofchoiceinolderpatients(>70yearsofage),itismoredifficulttoselecttheappropriatetherapyforyoungerpatients(<60years),sincerecentstudieshaveindicatedthathydroxyureatreatmentmightincreasetheriskofleukemia.文獻(xiàn)復(fù)習(xí)2021/4/2721Interferoniseffectivebutitsroleinthetreatmentofpolycythaemiaverahasnotbeenestabl