格林巴利綜合征

1、格林巴利綜合征 (Guillain-Barre syndrome)MRI病例圖片影像診斷分析時間：2011-01-10來源：醫(yī)影志ACR, November, 2009作者：freemanpyw編譯 【復(fù)制分享】【討論-糾錯】【舉報】【臨床病史】：患者，10歲男孩，近期病毒感染，出現(xiàn)下肢麻痹和反射減弱。CSF檢查提示蛋白含量增高，不伴有腦脊液細胞增多。10-year-old male with recent viral infection is admitted with lower extremity paralysis and hyporeflexia. CSF findings reve

2、al elevated protein level without pleocytosis.ZPn影像園XCTMR.com【影像圖片】MRI圖像 ZPn影像園XCTMR.com ZPn影像園XCTMR.comZPn影像園XCTMR.com【影像表現(xiàn)】：Figure 1,Figure 2,Figure 3and Figure 4: When compared with pre-contrast T1 weighted sagittal images (Figure 3), post-gadolinium sagittal T1-weighted images demonstrate enhanc

3、ement and thickening of the cauda equina (Figure 4).對比平掃T1和增強矢狀位T1顯示馬尾增厚強化。ZPn影像園XCTMR.com【影像診斷】：Guillain-Barre syndrome 格林巴利綜合征ZPn影像園XCTMR.comDifferential diagnosis of abnormal intrathecal nerve root enhancement includes: 異常的鞘內(nèi)神經(jīng)根強化鑒別診斷包括：1.AIDS-related polyradiculopathy AIDS相關(guān)的多發(fā)性神經(jīng)根??；2.Arachnoidi

4、tis 蛛網(wǎng)膜炎；3.Sarcoidosis 結(jié)節(jié)??；4.Metastatic disease 轉(zhuǎn)移性病變。ZPn影像園XCTMR.com【診斷要點】：ZPn影像園XCTMR.comGuillain Barre syndrome is a heterogeneous grouping of immune-mediated processes generally characterized by motor, sensory, and autonomic dysfunction. Classically, GBS is an acute inflammatory demyelinating po

5、lyneuropathy characterized by progressive symmetric ascending muscle weakness, paralysis, and hyporeflexia with or without sensory or autonomic symptoms; however, variants involving the cranial nerves or pure motor involvement are not uncommon. 格林巴利綜合征是一種免疫介導(dǎo)的多類型病變，一般特征為運動、感覺及自主功能障礙。典型的情況下，GBS是一種急性炎

6、癥性脫髓鞘性多發(fā)性神經(jīng)病，表現(xiàn)為急性進行性對稱的逐步向上延伸的肌無力、癱瘓和反射減弱，伴有或不伴有感覺或自主功能障礙。然而其他類型-顱神經(jīng)或單純的運動神經(jīng)受累并非罕見。ZPn影像園XCTMR.com MRI of the thoracolumbar spine is performed in cases of equivocal CSF and EEG findings or atypical clinical presentation. MRI findings include abnormal thickening and enhancement of the intrathecal ne

7、rve roots and cauda equina. The enhancement of only anterior spinal nerve roots is strongly suggestive of Guillain Barre syndrome. MRI is a sensitive but non-specific examination. 當CSF和EEG檢查疑診或臨床癥狀不典型時需要行胸腰椎MR掃描。MRI表現(xiàn)包括異常增粗強化的鞘內(nèi)神經(jīng)根和馬尾。僅僅是前方的神經(jīng)根強化強烈的提示GBS。MRI是一種敏感但并不特異的檢查。 【討論】：Guillain Barre syndrom

8、e (GBS) is an acute inflammatory demyelinating polyneuropathy (AIDP). It is an autoimmune disease affecting the peripheral nervous system, usually triggered by an acute infectious process. It is frequently severe and usually presents as an ascending paralysis beginning as weakness in the legs that s

9、preads to the upper limbs and the face along with complete loss of deep tendon reflexes. Hypo- or areflexia, autonomic dysfunction and cranial nerve involvement is commonly associated. It is generally preceded by an antecedent bacterial or viral infection. Nearly 40% of patients are seropositive for

10、 Campylobacter jejuni. Diagnosis is based on clincial signs and symptoms, lumbar puncture and electrophysiologic crtieria. CSF findings include elevated protein level without pleocytosis. Nerve conduction findings suggestive of GBS include nerve conduction slowing, conduction block, prolonged distal

11、 latency, and prolonged or absent F waves. Therapeutic options include supportive measures, plasmapharesis, and immunoglobulin.ZPn影像園XCTMR.com GBS是一種急性炎癥性脫髓鞘性多發(fā)性神經(jīng)病（AIDP）。這是一種影響外周神經(jīng)系統(tǒng)的自身免疫性病變，通常由急性感染性病變所誘發(fā)。經(jīng)常表現(xiàn)嚴重并且通常表現(xiàn)為上升性癱瘓，最初表現(xiàn)為腿部無力并擴展至上肢和面部，伴隨著深感覺的完全喪失。反射減低或減低，自主功能障礙和顱神經(jīng)受累是常見的并發(fā)癥。通常之前會有細菌或病毒感染，將近

12、40的病人空腸彎曲桿菌陽性。診斷基于臨床征像和癥狀、腰穿、電生理學標準。CSF改變包括蛋白水平升高不伴有腦脊液細胞增多。提示GBS的神經(jīng)學傳導(dǎo)異常包括神經(jīng)傳遞減慢、傳導(dǎo)阻滯、持續(xù)很長時間的潛伏期，以及F波延長或缺如。治理包括支持性治療、血漿置換術(shù)以及免疫球蛋白。ZPn影像園XCTMR.comZPn影像園XCTMR.com Although ascending paralysis is the most common form of spread in GBS, other variants also exist. Miller Fisher syndrome (MFS) is a rare v

13、ariant of GBS and manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS. It usually affects the ocular muscles first and presents as opthalmoplegia, ataxia and areflexia. Anti-GQ1b antibodies are found in 90% of cases. Acute motor axonal neuropathy (AMAN

14、), also known as Chinese Paralytic Syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico. The disease can be seasonal and recovery can be rapid. Anti-GD1a antibodies are often present. Acute motor sensory neuropathy (AMSAN) is similar to AMAN but also affects sensory nerves w

15、ith severe axonal damage. Recovery can be incomplete.ZPn影像園XCTMR.com 雖然上行性麻痹是GBS最常見的發(fā)展方式，其他的類型也存在。米勒費希爾綜合征是一種少見的GBS亞型，表現(xiàn)為下行性麻痹，與常見的GBS類型相反。通常首先影響眼外肌，表現(xiàn)為眼外肌麻痹、共濟失調(diào)和反射消失。90的病例可以發(fā)現(xiàn)Anti-GQ1b抗體。急性運動軸突神經(jīng)病（AMAN），又被稱為中式麻痹綜合癥，流行于中國和墨西哥。病變可以是季節(jié)性的，并可以很快的復(fù)原。Anti-CQ1b抗體常常出現(xiàn)，急性運動感覺神經(jīng)?。ˋMSAN）類似于AMAN，但是也可以影響感覺神經(jīng)及嚴重

16、的軸突損傷。復(fù)原可能會不完全ZPn影像園XCTMR.comZPn影像園XCTMR.com Radiologic studies are typically performed in order to exclude other causes. MR of the spine is performed to define infiltrative etiologies such as tranverse myelitis and compressive causes of polyradiculopathy. In cases of equivocal CSF or nerve conducti

17、on findings or atypical clinical findings, MRI of the spine can confirm or exclude the diagnosis. Findings usually include thickening and enhancement of the nerve roots that surround the medullary cone and extend along the length of the cauda equina. In GBS, there is lymphocytic and macrophagic infi

18、ltration around endoneural vessels with associated demyelination of the affected nerves. The abnormal enhancement of the intrathecal nerve roots is suggestive of blood brain barrier breakdown. Differential diagnosis of abnormal intrathecal nerve root enhancement includes AIDS-related polyradiculopathy in patients affected by CMV, arachnoiditis, sarcoidosis, and