Fisher综合征合并Bickerstaff脑干脑炎1例

Fisher综合征（Miller-Fisher syndrome,FS）是一种表现为眼肌麻痹、共济失调、腱反射消失的三联征;Bickerstaff脑干脑炎（Bickerstaff brainstem ence-phalitis,BBE）是一组急性起病的以眼肌麻痹、共济失调、意识障碍、腱反射亢进或病理反射阳性为特征的临床综合征。两者在临床均少见,本文报道1例Fs合并BBE患者。     

软组织黏液纤维肉瘤的临床及超声特征分析

目的 探讨软组织黏液纤维肉瘤(MFS)的超声表现、临床特征及相关的病理学特征，提高对MFS超声图像的认识，帮助诊断及鉴别诊断。方法 收集经穿刺或手术病理证实的MFS患者27例，回顾性分析患者的性别、年龄、病史及超声特征，并对不同组织病理学级别病灶的超声特征进行比较。结果 27例MFS患者(男∶女=2∶1),17例高级别MFS,10例低-中级别MFS,18例(66.7%)单发，16例(33.3%)肿块发生于下肢。超声表现：所有肿块均为实性低回声，18例(66.7%)肿块形态不规则；16例(59.3%)肿块边界不清晰；23例(85.1%)内部回声不均匀，5例(18.5%)肿块内可见囊变、坏死区，4例(11.8%)可见“筋膜尾征”;肿块内血流0～Ⅰ级6例(22.2%),Ⅱ～Ⅲ级21例(77.8%)。低-中级别组和高级别组MFS肿块边界的差异有统计学意义(P<0.05)。所有病例均未发现远处转移。结论 MFS超声表现有一定的特征，肿块为不均质实性低回声，多数边界不清晰，形态不规则，少数可出现液化、坏死，少数可见“筋膜尾征”,肿块内血流信号较丰富，有助于MFS与其他软组织肿瘤的鉴别。     

超声心动图诊断儿童马凡综合征

目的 观察儿童马凡综合征（MFS）患者的超声心动图表现。方法 回顾性分析临床疑诊MFS的59例患儿的影像学及临床检查资料,观察其超声心动图表现。结果 12例诊断为MFS,6例诊断为潜在MFS,其中11例存在家族史,7例为散发病例。上述18例中,4例存在主动脉窦部增宽,11例存在主动脉窦部扩张,1例合并主动脉瓣轻度反流,2例合并肺动脉窦部扩张;8例二尖瓣受累,5例二尖瓣脱垂并中—重度反流,5例三尖瓣受累;1例同时存在二尖瓣及三尖瓣脱垂而接受二尖瓣成形术;1例合并室间隔缺损,接受室间隔缺损修补术。结论 儿童MFS中,以主动脉窦部增宽或扩张最为常见,可合并二尖瓣脱垂;超声心动图检查有助于早期诊断和及时干预儿童MFS。     

Bickerstaff’s脑干脑炎8例并文献复习

目的:分析Bickerstaff脑干脑炎(BBE)的临床特点,以提高临床诊疗水平。方法:对8例BBE患者的临床资料进行回顾性分析,并结合相关文献,总结其临床特点、发病机制、治疗方法及预后。结果:8例患者均为急性起病,发病前有上呼吸道感染者2例,腹泻2例,水痘-带状疱疹病毒感染1例。首发症状为肢体麻木无力伴言语含混3例;意识不清、双下肢无力、声音嘶哑伴吞咽困难、面部和手指麻木、头晕伴行走不稳的各1例。临床主要表现为急性眼肌麻痹(眼外肌麻痹7例,眼内肌麻痹1例),共济失调4例,意识障碍4例,腱反射减弱或消失5例,病理征阳性8例,累及面神经4例,累及舌咽和迷走神经6例,同时累及脑神经(面神经、舌咽、迷走神经、舌下神经)3例。2例患者脑脊液检查正常,4例头颅MRI检查无特征性表现,2例在脑干内相应部位有异常病灶,1例硬脑膜广泛强化。5例行血清抗GQ1b抗体检查者均为阴性。8例患者均使用免疫球蛋白或联合糖皮质激素治疗,7例患者预后较好,1例患者死亡。结论:BBE多呈单相良性病程,确诊主要依据临床表现、血清抗体、脑脊液病原基因组、影像学及神经电生理作为辅助诊断,免疫球蛋白或联合糖皮质激素治疗有效。     

眶内电针治疗Fisher综合征所致眼肌麻痹临床观察

目的:回顾分析眶内电针(IEA)治疗Fisher综合征(MFS)眼肌麻痹疗效及MFS眼肌麻痹患者的临床特点。方法:收集接受IEA治疗的MFS患者27例的资料,对一般资料、受累颅神经、感染情况、治疗前后最大复视角度、治疗及恢复情况等进行回顾分析。结果:患者从发病到开始接受IEA治疗的平均时间为(31.04±46.23)d,接受治疗后达到临床痊愈的平均天数为(33.67±21.96)d。临床痊愈(无复视致残)24例(88.9%)。治疗后,左眼和右眼的最大复视角度均较治疗前显著降低(P0.01)。MFS眼肌麻痹特点:100%患者有外展神经病变,其中11.1%为单侧病变,88.9%为双侧受累;37.0%合并III、VI对颅神经麻痹,无孤立的III或IV对颅神经麻痹患者。70.3%有感染史;11.1%伴发高血压、7.4%伴发高脂血症、3.7%伴发2型糖尿病。22.2%伴有Bell征,双侧受累4例,单侧受累2例。25.9%眼睑下垂,双侧受累5例,单侧受累2例。3.7%伴有双侧瞳孔散大。结论:IEA治疗可加快MFS眼肌麻痹症状恢复,MFS眼肌麻痹的临床特点可提示临床诊断。     

中枢神经系统受累的儿童噬血细胞性淋巴组织增生症临床分析

目的分析儿童噬血细胞性淋巴组织增生症(hemophagocytic lymphohistiocytosis, HLH)患儿中枢神经系统受累的临床特征和预后。方法回顾性分析13例出现中枢神经系统受累的HLH患儿的临床及影像学资料,并应用Kaplan-Meier法分析5年生存率。结果 13例HLH患儿中癫痫发作7例,意识障碍3例,面神经麻痹1例,烦躁1例,吞咽困难和构音障碍1例;1例有恶性血液病家族史;3例MUNC13-4基因突变,1例编码perforin-1基因突变;4例感染EB病毒;13例患儿均行脑CT和MRI检查,7例影像学表现正常,6例异常;7例癫痫发作患者中2例服用抗癫痫药物治疗,5例未服用抗癫痫药物情况下癫痫停止发作;13例患儿死亡4例,5年生存率为69%。结论中枢神经系统受累的HLH患者死亡风险较高,脑CT/MRI影像检查可表现异常,对提高HLH诊断有一定价值。     

血嗜酸性粒细胞联合高分辨率CT对单纯性肺结节良恶性鉴别诊断的价值分析

目的 分析血嗜酸性粒细胞联合高分辨率CT对单纯性肺结节良恶性的鉴别诊断价值,为临床提供参考。方法 回顾性分析2021年1月至2023年2月宁波开发区医院收治的70例单纯性肺结节患者的临床资料。根据穿刺活检或术后病理结果将患者分为恶性组（43例）和良性组（27例）。两组患者均接受高分辨率CT检查,并测定其血嗜酸性粒细胞水平,以穿刺活检或术后病理检查结果为金标准,分析血嗜酸性粒细胞联合高分辨率CT对单纯性肺结节良恶性的鉴别诊断价值。结果 穿刺活检或术后病理检查结果显示,70例患者中43例（61.43%）为恶性病变,27例（38.57%）为良性病变。恶性组患者的胸膜凹陷征、血管集束征、分叶征及毛刺征的高分辨率CT征象检出率和血嗜酸性粒细胞水平升高占比均高于良性组（均P<0.05）,而两组患者空泡征、卫星灶及钙化灶等高分辨率CT征象检出率比较,差异均无统计学意义（均P>0.05）。高分辨率CT和血嗜酸性粒细胞水平升高判断肺结节恶性病变的敏感度分别为86.05%和83.72%,特异度分别为92.59%和77.78%,两者联合检查判断肺结节恶性病变的敏感度、特异度分别为95.35%和9...     

肝内胆管乳头状瘤的CT及MR表现

目的：探讨肝内胆管乳头状瘤（Intrahepatic biliary papillomatosis,IHBP）的临床病理特征、影像学表现。方法 ：回顾性分析5例经手术病理证实的IHBP患者的临床病理特征及影像学表现,并结合文献进行分析和讨论。结果：5例IHBP患者中2例表现为间歇性黄疸、1例腹痛伴发热、1例左腰背部酸痛,1例体检时发现。影像学特点：5例均发现全胆管树系统扩张,胆总管下段无明确占位或结石,受累肝内胆管相对比另一肝叶胆管或胆总管不成比例的扩张,受累肝内胆管呈＂瘤样＂扩张,扩张的肝内胆管出现乳头状或长条状肿块影,MRCP示扩张胆管内多发乳头状及条状充盈缺损,3例位于右肝内胆管、2例位于左肝内胆管,肿块直径约0.3~2.5 cm,于CT平扫呈等或稍低密度,MR T1WI表现为等或稍低信号,T2WI上多呈等高信号,DWI上呈乳头状及条状高信号,增强扫描肿块呈轻度强化。结论：IHBP的影像学表现有一定的特征性,尤其MRCP、DWI及动态增强检查,熟悉该病的特征性影像学表现有助于对IHBP诊断及鉴别诊断。     

肺原发性淋巴瘤的CT影像学特点及文献复习

目的：分析肺原发性淋巴瘤患者的影像学及临床表现特征,提高对于该疾病的诊断和鉴别诊断能力。方法：回顾性分析经病理明确诊断的肺原发淋巴瘤患者的CT影像学表现,结合相关文献,总结肺部原发淋巴瘤的影像学表现特点。结果：10例肺原发性淋巴瘤患者中,7例确诊为黏膜相关淋巴组织淋巴瘤（mucosa-associated lymphoid tissue, MALT）淋巴瘤,1例确诊为弥漫大B细胞淋巴瘤,2例确诊为外周T细胞淋巴瘤。8例患者的CT图像上病灶可见支气管充气征,9例增强CT均可见病灶轻度强化及血管漂浮征,8例可见晕征,而患者的临床表现均无特异性。结论：肺部原发性淋巴瘤患者的影像学表现复杂多样,且临床和影像学表现无特异性,空气支气管征、增强后病灶呈轻中度强化、血管漂浮征、晕征等为肺部淋巴瘤在CT图像上较为特征的征象。     

麻痹性痴呆5例临床分析

【目的】探讨麻痹性痴呆（GPI）患者的临床,实验室检测,脑电图及影像学改变特点,以提供早期诊断依据。【方法】回顾性分析5例经临床和实验室确诊的GPI患者的相关资料。患者智能的检测采用简易智力状态检查量表（MMSE）评分,入选条件是MMSE≤20分。【结果】：5例患者均有进行性记忆力减退,判断力和计算力下降和语言表达障碍等。糊涂4例,行为异常2例,幻觉2例,易冲动2例,抽搐发作2例。2例在3个月内出现明显痴呆,言语障碍。2例在5个月内出现糊涂,行为异常,言语表达障碍。1例半年后出现明显痴呆,人格改变及行为异常。头颅MRI检查5例均有不同程度脑萎缩,2例脑实质内多发异常信号,以双侧额叶,颞叶明显。脑电图3例广泛中度异常,1例广泛重度异常。【结论】慢性进行性痴呆进展快者,做血清及脑脊液快速血浆反应实验（RPR）,血清梅毒螺旋体特异性抗体（TP-Elisa）检测是早期诊断麻痹性痴呆的关键。     

Bickerstaff脑干脑炎--1例报告和文献复习

Bickerstaff脑干脑炎(BBE)在临床上较为罕见,本文报道了1例BBE患者的诊治过程,并进行相关文献复习,总结了BBE的临床特征及其诊治要点。磁共振成像(MRI)可显示脑干受累,是诊断BBE的可靠工具,对判定BBE预后具有重要的临床价值。BBE多样的临床特征与后遗症尚需更多的研究进一步分析。     

Aortic Root Dilation: Do Patients With Marfan Syndrome Fare Worse Than Those With Marfanoid Features?

Objective

To discover whether patients with aortic root dilation and leptosomic features but without a diagnosis of Marfan syndrome (MFS) fare similarly to patients with MFS.

黏液纤维肉瘤增强MRI“尾征”研究进展

黏液纤维肉瘤（MFS）是以黏液样成分（>50%）为主的恶性纤维组织细胞瘤，具有浸润性生长特征，MRI表现为边界清楚、逐渐变细的曲线样信号，呈特征性的尾巴样，即"尾征"。术前MRI显示"尾征"是MFS术后局部复发的独立危险因素。本文对MFS增强MRI"尾征"相关研究进展进行综述。     

A case of Bickerstaff encephalitis with overlapping Gullian Barre syndrome in a pediatric patient treated with therapeutic plasma exchange

Bickerstaff brain stem encephalitis (BBE) is a rare brainstem disorder characterized by acute onset of ophthalmoplegia, ataxia, and altered consciousness. Guillain Barre syndrome (GBS), Miller Fischer syndrome and BBE share certain similarities such as the presence of anti-ganglioside antibodies. The use of Therapeutic Plasma Exchange (TPE) has been reserved for severe to fulminant cases of BBE mostly as an 'off label' use. The role of TPE in the overlapping syndrome of BBE and GBS has not been explored much, especially in the paediatric population. Herein, we describe a case of 2-year-old male who presented with features of BBE and later evolved to an overlapping syndrome with BBE and GBS. A multi-disciplinary team managed the patient and TPE was initiated as a part of the treatment plan. Five cycles of TPE were done from day 24 after which the patient improved. In our case, TPE was used as rescue therapy in patients with BBE overlapping with GBS. The effectiveness of TPE can be further explored as a modality in such disorders.     

Miller Fisher syndrome: an uncommon acute neuropathy

The syndrome of ophthalmoplegia, ataxia, and areflexia was first described in 1956 by Miller Fisher. This syndrome has long been believed to be a variant of Guillain-Barré syndrome (GBS), mainly because of its areflexia, cerebrospinal fluid findings, and its postinfectious presentation. The case of an 11-year-old male with Miller Fisher syndrome (MFS) is described. MFS differs from GBS in several key clinical features and presents an extensive and challenging differential diagnosis. It is useful to recognize MFS as both a variant of GBS and a distinct entity with its own therapeutic considerations.     

Recovery of impaired motor function of the upper extremity after stroke.

Manual function score (MFS) of 174 hemiparetic stroke patients was examined every week for 8 weeks after starting the occupational therapy with programs based on MFS recovery profile, and the relation between the time since stroke onset (x) and MFS (y) was approximated by a hyperbolic function, y = A - B/x. The patients were classified into two groups, 125 cases (71.8%) with statistically significant fit of the function (fit group) and 49 without fit (non-fit). Using demographic and neurological variables of each patient, characteristics of the patients with regular recovery were analyzed. The results indicated that the gain of MFS during 8 weeks of the therapy was large in the fit group compared to the non-fit, and the regular recovery could be mostly expected in rather young patients, those with early start of the therapy, and those without ataxia, mental deterioration, and cortical lesions.     

Morse跌倒评估量表与Berg平衡量表应用于老年患者预测跌倒风险的效果分析

摘要 目的：测量并比较Morse跌倒评估量表(MFS)及Berg平衡量表（BBS）应用于老年患者的跌倒风险预测效果。 方法：两名测试护士使用MFS及BBS分别对161例老年患者进行跌倒风险评估,3个月后电话随访患者是否在此期间跌倒。分析量表不同诊断界值的敏感度、特异度及接受者操作特性（ROC）曲线。 结果：MFS的ROC曲线下面积（AUC）为0.893（P＜0.01）,其敏感度和特异度在诊断界值为25分时分别为100%和11%,在45分时分别为96%和36%,在50分时分别为96%和52%,在55分时相对平衡,分别为92%和64%。BBS的AUC为0.654（P＜0.05）,其敏感度和特异度在诊断界值为45分时分别为72%和40%,在40分时相对平衡,分别为68%和57%。 结论：两种量表应用于我国老年患者的预测效果均较好,推荐MFS的诊断界值为55分,BBS诊断界值为40分。MFS较BBS的预测效果好,临床需结合患者的特点选择合适的量表并由临床护士使用。     

Miller Fisher syndrome and plasmapheresis.

Treatment for Miller Fisher syndrome (MFS) is controversial, and even the natural history and prognosis are not fully understood. We retrospectively reviewed our cases of MFS for the last 3 years. The analysis of 4 MFS cases revealed that we had performed plasmapheresis or additional immunotherapy to each of 4 patients, and their symptoms resolved for up to 50 days after the onset (ataxia improved 20-35 days and ophthalmoplegia for 25-50 days) except for 1 patient, and that Guillain-Barré syndrome had been diagnosed in 1 patient who had developed profound muscle weakness. We also discovered that MFS patients had a deviated T-helper Type-1 (Th1)/T-helper Type-2 (Th2) polarization and that plasmapheresis can shift Th2-dominant status to Th1-dominant status in patients with MFS. Although plasmapheresis may remove humoral factors, including anti-GQ1b, and may induce a shift of the Th1/Th2 cytokine-producing cell balance in peripheral blood, the therapeutic rationale has not yet been established. Therefore, controlled clinical trials are required to show whether plasmapheresis leads to earlier recovery with fewer neurologic deficits in patients with MFS.