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译168 | 文献综述 | 低估了的脊髓后动脉梗死 [自发性]

 昵称42715024 2018-10-15

脊髓梗死(SCIs)主要发生在脊髓前动脉(ASA)供血区域,表现为急性无力和痛温觉障碍。相比之下,可能是由于成对的脊髓后动脉(PSAs)有旁系动脉供血,故PSA梗死更为罕见。导致低估了PSA梗死的诊断。关于PSA梗死的临床和影像学特征及预后的报道很少。本文记录了我们在梅奥诊所(1997 - 2017)所见到的所有PSA梗死,以提高对这种罕见脊髓病的理解和认识。所有患者都同意使用他们的病历进行研究。


133例在我院诊断的自发性SCIs (排除围手术期梗死),有15例(11%)为PSA梗死。表中总结了15例患者的临床和诊断细节。14例(93%)有血管病危险因素:高脂血症,11例;吸烟,6例;高血压,6例;糖尿病,1例;心房颤动,1例。对其他病因的辅助检查没有阳性发现,包括维生素B12 (n = 14),风湿病/血管炎标志物(n = 13),感染(n = 12),AQP-4 IgG(n = 11),高凝(n = 8),铜(n = 8),肌电图/神经传导检查(n = 4)。


 脊髓后动脉梗死的临床和诊断特征


患者数量 (%)

中位数 (范围)

患者

15


   年龄 >50 y 

14 (93)

64 (36–75)

   女性

9 (60)


   血管病危险因素

14 (93)


临床特征



    症状达到高峰时间



    ≤4 h

12 (80)


    ≤6 h

1 (7)


    ≥24 h (缓慢进展)

2 (13)


  感觉障碍(后柱)

15 (100)


  无力

11 (73)


  起病时疼痛

9 (60)


  尿/便障碍

8 (53)


  感觉性共济失调

7 (47)


CSF



  蛋白升高(>35 mg/dL)

7 (47)

59 (43–116)

  WBCs升高

0 (0)


  OCBs 升高

0 (0)


MRI脊髓损害

15 (100)

1 (0–38)

 初始MRI正常(从起病算

5 (33)

0 (0–1)

 非连续性T2高信号

9 (60)


 长节段病灶a

9 (60)


 钆增强

6 (40)

14 (6–21)

 囊性脊髓软化 (≥1)

6 (40)


 椎体梗死

3 (20)

4 (1–19)

 弥散受限

2 of 3 (67)

2 (1–3)

 定位于颈髓

8 (53)


 定位于胸髓

7 (47)


缩写:OCB=寡克隆带;WBC=白细胞;

a≥3 椎体节段.


脊柱MRI的中位时间为1天(范围为0-38天)。5例患者初始MRI正常(第0 - 1天);所有患者在第二次MRI检查(1天后)时,脊髓后部可见T2高信号()。中总结了MRI的其他表现。10例患者行血管造影(MR/CT/常规血管造影),都是正常的。


 脊髓后动脉梗死的MRI表现

 (A)患者,超急性起病,表现为左侧感觉障碍及共济失调步态。MRI显示颈髓背外侧T2高信号病变(A.a, A.d),弥散加权成像和表观弥散系数显示弥散限制(A.b, A.c)。(B)患者在24小时内多次突然出现腿部麻木和无力,矢状位显示脊髓背侧T2高信号(B.a,箭头),在轴位中更容易看到(B.b,箭头),并伴有椎体缺血(B.a,箭头)。(C)患者突发感觉性共济失调步态。矢状位可见脊髓后部长且不连续的T2高信号(C.a,箭头)伴囊性脊髓软化,轴位上信号与脑脊液相似(C.b,箭头)。(D)患者突然出现手臂和躯干感觉障碍,并迅速出现下肢无力,矢状位(D.a,箭头)和轴位(D.b,箭头)显示局灶性线型T2高信号,提示脊髓软化。


11例(73%)疑似脊髓炎或格林-巴利综合征的患者接受了免疫治疗(皮质类固醇10例;IV免疫球蛋白[IVIg] 4例);无并发症。1例患者在发病4小时后接受了组织型纤溶酶原激活剂治疗,无明显疗效,但2个多月后恢复,可以用踝足矫形器行走。患者在诊断SCI后,进行血管性危险因素干预和抗血小板治疗。13例患者有效,2例无效(感觉性脊髓病,1例;<>


Discussion    讨    论


这是迄今为止最大的PSA梗死系列。15例患者均突发出现感觉障碍,常伴有疼痛和感觉性共济失调。许多患者脊髓损伤超过后三分之一(如:无力),可能来自于重叠的/变异的脊髓动脉供血区,最初合并有ASA缺血,水肿。大多数人怀疑是炎症性病因,尤其是4例患者接受了IVIg治疗,IVIg在血栓早期有潜在危害。


PSA和ASA梗死的重叠特征包括数小时内病情达到高峰、起病时疼痛、运动和感觉障碍、年龄较大以及存在血管病危险因素。与ASA缺血相比,PSA梗死有明显的后柱功能障碍和更好的预后(93%可行走)。颈髓PSA比文献报道的更常见,排除以胸髓多见的围手术期SCI(如主动脉修复) 后,颈髓PSA比文献报道的更常见。


最初的脊柱MRI可能是正常的,但最终会表现为脊髓背部T2高信号病变。弥散序列和椎体梗死()可以帮助诊断;特别是在颈髓时应考虑行血管成像除外夹层或闭塞。随访(≥1个月)MRI显示脊髓囊性软化倾向于梗死而非炎症。在数小时内出现严重功能障碍,非炎症性脑脊液,MRI特征符合SCI,排除其他病因后,即支持所有患者的SCI诊断;当怀疑SCI时,应该寻找这些特征和辅助检查的特征表现(如扩散限制)。


尽管我们的病例进行了许多的检查,但没有发现明确的缺血机制,大多数病例都有常见的血管病危险因素。自发性SCI的治疗是支持治疗,应该避免潜在的有害疗法。康复很重要,因为预后可能还不错。以前的报告显示可能的机制是动脉粥样硬化或夹层,建议酌情给予适当的抗血小板治疗和危险因素干预。

●★●★●★●★●★●★●中英对照●★●★●★●★●★



Spinal cord infarctions (SCIs) predominantly affect the anterior spinal artery (ASA) territory and manifest with acute weakness and loss of pain and temperature sensation. In comparison, posterior spinal artery (PSA) infarctions are more rare, possibly due to paired PSAs with increased collateral arterial supply; the diagnosis may also be under recognized. 1–5 Reports on clinical and radiologic features and outcomes of PSA infarcts are scant. We sought to describe all PSA infarcts seen at Mayo Clinic (1997–2017) to improve the understanding and awareness of this uncommon myelopathy. All patients consented to use of their medical records for research.


脊髓梗死(SCIs)主要发生在脊髓前动脉(ASA)供血区域,表现为急性无力和痛温觉障碍。相比之下,可能是由于成对的脊髓后动脉(PSAs)有旁系动脉供血,故PSA梗死更为罕见。导致低估了PSA梗死的诊断。关于PSA梗死的临床和影像学特征及预后的报道很少。本文记录了我们在梅奥诊所(1997 - 2017)所见到的所有PSA梗死,以提高对这种罕见脊髓病的理解和认识。所有患者都同意使用他们的病历进行研究。


Of 133 spontaneous SCIs (i.e., excluding periprocedural infarcts) diagnosed at our facility, 15(11%) were PSA infarctions. Clinical and diagnostic details of the 15 included patients are summarized in the table. Fourteen patients (93%) had vascular risk factors: hyperlipidemia, 11; tobacco use, 6; hypertension, 6; diabetes mellitus, 1; and atrial fibrillation, 1. Diagnostic evaluations for alternative etiologies were unrevealing, including vitamin B12 (n = 14), rheumatologic/vasculitic markers (n = 13), infection (n = 12), aquaporin-4 immunoglobulinG (n = 11), hypercoagulability (n = 8), copper (n = 8), and EMG/nerve conduction studies(n = 4).


133例在我院诊断的自发性SCIs (排除围手术期梗死),有15例(11%)为PSA梗死。表中总结了15例患者的临床和诊断细节。14例(93%)有血管病危险因素:高脂血症,11例;吸烟,6例;高血压,6例;糖尿病,1例;心房颤动,1例。对其他病因的辅助检查没有阳性发现,包括维生素B12 (n = 14),风湿病/血管炎标志物(n = 13),感染(n = 12),AQP-4 IgG(n = 11),高凝(n = 8),铜(n = 8),肌电图/神经传导检查(n = 4)。



脊髓后动脉梗死的临床和诊断特征


患者数量 (%)

中位数 (范围)

患者

15


   年龄 >50 y 

14 (93)

64 (36–75)

   女性

9 (60)


   血管病危险因素

14 (93)


临床特征



    症状达到高峰时间



    ≤4 h

12 (80)


    ≤6 h

1 (7)


    ≥24 h (缓慢进展)

2 (13)


  感觉障碍(后柱)

15 (100)


  无力

11 (73)


  起病时疼痛

9 (60)


  尿/便障碍

8 (53)


  感觉性共济失调

7 (47)


CSF



  蛋白升高(>35 mg/dL)

7 (47)

59 (43–116)

  WBCs升高

0 (0)


  OCBs 升高

0 (0)


MRI脊髓损害,

15 (100)

1 (0–38)

 初始MRI正常(从起病算)

5 (33)

0 (0–1)

 非连续性T2高信号

9 (60)


 长节段病灶a

9 (60)


 钆增强,

6 (40)

14 (6–21)

 囊性脊髓软化 (≥1)

6 (40)


 椎体梗死,

3 (20)

4 (1–19)

 弥散受限,

2 of 3 (67)

2 (1–3)

 定位于颈髓

8 (53)


 定位于胸髓

7 (47)


缩写:OCB=寡克隆带;WBC=白细胞;

a≥3 椎体节段.


Median time to spine MRI was1day (range0–38days).Five patients had an initial normal MRI(day 0–1);all subsequently showed posterior spinal cord T2 hyperintensity (figure) on the second MRI (≥1 day later). Other MRI findings are summarized in the table. Ten patients had vascular imaging performed (MR/CT/conventional angiography); all were normal.


脊柱MRI的中位时间为1天(范围为0-38天)。5例患者初始MRI正常(第0 - 1天);所有患者在第二次MRI检查(1天后)时,脊髓后部可见T2高信号()。中总结了MRI的其他表现。10例患者行血管造影(MR/CT/常规血管造影),都是正常的。


脊髓后动脉梗死的MRI表现

 (A)患者,超急性起病,表现为左侧感觉障碍及共济失调步态。MRI显示颈髓背外侧T2高信号病变(A.a, A.d),弥散加权成像和表观弥散系数显示弥散限制(A.b, A.c)。(B)患者在24小时内多次突然出现腿部麻木和无力,矢状位显示脊髓背侧T2高信号(B.a,箭头),在轴位中更容易看到(B.b,箭头),并伴有椎体缺血(B.a,箭头)。(C)患者突发感觉性共济失调步态。矢状位可见脊髓后部长且不连续的T2高信号(C.a,箭头)伴囊性脊髓软化,轴位上信号与脑脊液相似(C.b,箭头)。(D)患者突然出现手臂和躯干感觉障碍,并迅速出现下肢无力,矢状位(D.a,箭头)和轴位(D.b,箭头)显示局灶性线型T2高信号,提示脊髓软化。


Eleven patients (73%) received immunotherapy for suspected myelitis or Guillain-Barré syndrome (corticosteroids, 10; IV immunoglobulin [IVIg], 4); no complications were reported. One patient received IV tissue plasminogen activator 4 hours after onset with nobenefit acutely but delayed recovery over 2 months allowed ambulation with an ankle-foot orthosis. Once SCI was recognized, vascular risk factor modification and antiplatelet treatment was undertaken. Thirteen patients reported improvement and 2 did not (sensory-predominant myelopathy without improvement, 1; <1 month="" follow-up,="" 1).="" at="" last="" follow-up="" (median="" 8="" months;="" range="" 1–71="" months),="" the="" ambulatory="" status="" was="" ambulatory="" without="" assistive="" device,="" 8;="" gait-aid="" dependent,="" 6;="" and="" nonambulatory,="" 1;="" none="" had="" developed="" new="" neurologic="">


11例(73%)疑似脊髓炎或格林-巴利综合征的患者接受了免疫治疗(皮质类固醇10例;IV免疫球蛋白[IVIg] 4例);无并发症。1例患者在发病4小时后接受了组织型纤溶酶原激活剂治疗,无明显疗效,但2个多月后恢复,可以用踝足矫形器行走。患者在诊断SCI后,进行血管性危险因素干预和抗血小板治疗。13例患者有效,2例无效(感觉性脊髓病,1例;<>


Discussion    讨    论


This is the largest series of PSA infarctions to date. All 15 patients presented with an abrupt sensory disturbance, often accompanied by pain and sensory ataxia. Many patients had impairment beyond the posterior 1/3 of the spinal cord (e.g., weakness)1,3–5, possibly from overlapping/heterogeneous spinal arterial territories, initial coexisting ASA ischemia, or edema. An inflammatory etiology was suspected in most and notably 4 received IVIg, which is potentially harmful with prothrombotic properties6.


这是迄今为止最大的PSA梗死系列。15例患者均突发出现感觉障碍,常伴有疼痛和感觉性共济失调。许多患者脊髓损伤超过后三分之一(如:无力),可能来自于重叠的/变异的脊髓动脉供血区,最初合并有ASA缺血,水肿。大多数人怀疑是炎症性病因,尤其是4例患者接受了IVIg治疗,IVIg在血栓早期有潜在危害。


Overlapping features of PSA and ASA infarcts include deficit nadir within hours, pain at onset, motor and sensory deficits, older age, and presence of vascular risk factors. 1 In contrast to ASA ischemia, PSA infarcts have predominantly posterior column dysfunction and better outcomes(93% ambulatory) 7. Cervical location was more frequent than reported in the literature, which may partially reflect exclusion of periprocedural SCI (e.g., aortic repair) that is thoracic predominant.


PSA和ASA梗死的重叠特征包括数小时内病情达到高峰、起病时疼痛、运动和感觉障碍、年龄较大以及存在血管病危险因素。与ASA缺血相比,PSA梗死有明显的后柱功能障碍和更好的预后(93%可行走)。颈髓PSA比文献报道的更常见,排除以胸髓多见的围手术期SCI(如主动脉修复) 后,颈髓PSA比文献报道的更常见。


Initial MRI spine maybe normal, but ultimately a dorsal T2 hyperintense cord lesion is demonstrated. Diffusion sequences and vertebral body infarctions (figure) can be diagnostic; vessel imaging for dissection or occlusion should be considered, especially in the cervical spine. 2 Follow-up (≥1 month) MRI showing cystic myelomalacia favors infarction over inflammation. Development of severe deficits within hours, noninflammatory CSF, MRI features consistent with SCI, and exclusion of alternative etiologies supported SCI diagnosis in all our patients; these features and evaluation for specific findings (e.g.,diffusion restriction) should be sought when suspecting SCI.


最初的脊柱MRI可能是正常的,但最终会表现为脊髓背部T2高信号病变。弥散序列和椎体梗死()可以帮助诊断;特别是在颈髓时应考虑行血管成像除外夹层或闭塞。随访(≥1个月)MRI显示脊髓囊性软化倾向于梗死而非炎症。在数小时内出现严重功能障碍,非炎症性脑脊液,MRI特征符合SCI,排除其他病因后,即支持所有患者的SCI诊断;当怀疑SCI时,应该寻找这些特征和辅助检查的特征表现(如扩散限制)。


No clear ischemic mechanism was found in our cases despite extensive evaluations but most had traditional vascular risk factors. Treatment for spontaneous SCI is supportive; potentially harmful therapies should be avoided6. Rehabilitation is important, as good outcomes can occur7. Previous reports showed atherosclerosis or dissection as likely mechanisms, suggesting antiplatelet treatment and risk factor modification may be appropriate.


尽管我们的病例进行了许多的检查,但没有发现明确的缺血机制,大多数病例都有常见的血管病危险因素。自发性SCI的治疗是支持治疗,应该避免潜在的有害疗法。康复很重要,因为预后可能还不错。以前的报告显示可能的机制是动脉粥样硬化或夹层,建议酌情给予适当的抗血小板治疗和危险因素干预。

(全文终)

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