桥本脑病22例临床分析

目的探讨桥本脑病临床特点,提高诊断与治疗水平。方法与结果对22例桥本脑病患者的临床表现、实验室和影像学特点、治疗及预后进行回顾分析,临床主要表现为急性或亚急性发病的认知功能障碍(9例)、精神症状(6例)、癫发作(5例)、共济失调(5例)、卒中样发作(6例)、锥体外系症状(3例)和意识障碍(3例),且血清抗甲状腺过氧化物酶抗体(19例)和抗甲状腺球蛋白抗体(20例)水平明显升高。其中21例经糖皮质激素冲击治疗,17例病情不同程度改善。结论桥本脑病是一种伴抗甲状腺抗体水平升高的脑病,大多数患者对糖皮质激素冲击治疗反应良好,早期诊断、及时治疗对改善患者预后尤为重要。对于不明原因的脑病患者,应考虑桥本脑病的可能,推荐血清抗甲状腺抗体作为常规实验室检查项目。     

桥本脑病22例临床分析

目的 探讨桥本脑病临床特点,提高诊断与治疗水平.方法与结果 对22例桥本脑病患者的临床表现、实验室和影像学特点、治疗及预后进行回顾分析,临床主要表现为急性或亚急性发病的认知功能障碍（9例）、精神症状（6例）、癫（痫）发作（5例）、共济失调（5例）、卒中样发作（6例）、锥体外系症状（3例）和意识障碍（3例）,且血清抗甲状腺过氧化物酶抗体（19例）和抗甲状腺球蛋白抗体（20例）水平明显升高.其中21例经糖皮质激素冲击治疗,17例病情不同程度改善.结论 桥本脑病是一种伴抗甲状腺抗体水平升高的脑病,大多数患者对糖皮质激素冲击治疗反应良好,早期诊断、及时治疗对改善患者预后尤为重要.对于不明原因的脑病患者,应考虑桥本脑病的可能,推荐血清抗甲状腺抗体作为常规实验室检查项目.     

桥本脑病2例及文献复习

目的探讨桥本脑病的临床特点,以提高对本病的认识。方法回顾性分析2例确诊桥本脑病患者的临床资料并复习相关文献。结果 2例均为急性起病,1例表现为精神症状及意识障碍,另1例表现为运动及感觉障碍。2例抗甲状腺抗体均增高,抗甲状腺过氧化物酶抗体增高明显,2例脑电图均广泛异常,1例脑脊液蛋白明显升高。2例糖皮质激素治疗均有效。结论桥本脑病临床表现错综复杂,容易漏诊和误诊,当临床上遇到无法解释的中枢神经系统疾病,应常规检查抗甲状腺抗体,确诊后应尽早用糖皮质激素治疗。     

桥本脑病2例及文献复习

目的探讨桥本脑病的临床特点,以提高对本病的认识。方法回顾性分析2例确诊桥本脑病患者的临床资料并复习相关文献。结果 2例均为急性起病,1例表现为精神症状及意识障碍,另1例表现为运动及感觉障碍。2例抗甲状腺抗体均增高,抗甲状腺过氧化物酶抗体增高明显,2例脑电图均广泛异常,1例脑脊液蛋白明显升高。2例糖皮质激素治疗均有效。结论桥本脑病临床表现错综复杂,容易漏诊和误诊,当临床上遇到无法解释的中枢神经系统疾病,应常规检查抗甲状腺抗体,确诊后应尽早用糖皮质激素治疗。     

桥本脑病的临床分析

目的 研究桥本脑病的临床特点,以提高对本病的诊治水平.方法 对5例临床诊断为桥本脑病的患者进行回顾研究,并对其发病机制、临床表现、治疗及预后进行分析.结果 5例患者中有3例既往有甲状腺疾病病史,其中1例有自身免疫性甲状腺疾病家族史.临床表现有认知功能障碍者4例,精神行为异常者3例,锥体外系改变者4例,抽搐者2例,意识障碍者1例,运动障碍者1例.甲状腺功能正常者4例,亚临床甲状腺功能亢进者1例.血清ATPO及ATG阳性率达100%.1例脑脊液蛋白轻度增加.2例脑电图呈弥漫性慢波.5例头部MRI均无特异性改变.4例接受糖皮质激素治疗,其中1例合用人血丙种球蛋白,基本治愈1例,有效3例,无死亡.结论 目前可能存在对桥本脑病诊断率较低的情况.内分泌功能异常不能作为桥本脑病的病因,可认为其为一种自身免疫性疾病.当遇有难以解释的脑病时,应将检测血清抗甲状腺抗体作为常规检查,当其滴度显著增高时应高度怀疑本病,及时予以糖皮质激素治疗.     

桥本脑病17例临床分析

目的探讨桥本脑病(HE)的临床特点及治疗方法,以提高HE的诊治效果。方法回顾性分析本院收治的17例HE患者的临床资料,包括临床表现、实验室、影像学及EEG检查结果以及治疗方案。结果17例HE患者中,表现为认知功能减退的有10例,精神症状的有10例,癫痫发作的有8例,表现为卒中样发作的有7例,1例为肌阵挛发作,1例患者表现为单肢胀痛无力,1例患者表现为锥体外系症状。17例患者血清甲状腺抗过氧化物酶抗体明显增高,13例患者血清甲状腺球蛋白抗体增高。有12例患者接受了EEG检查,均有异常表现,其中8例为慢波增多,4例出现痫样放电。接受糖皮质激素治疗后有9例患者临床症状明显改善,7例患者临床症状部分改善,1例患者无明显好转。结论 HE的临床表现多样,缺乏特异性,易被误诊。其EEG多有异常表现,抗甲状腺抗体水平显著增高,经糖皮质激素治疗后大都反应良好。故对于原因不明的脑病,应考虑HE可能,及时治疗。     

桥本脑病的临床、影像学及病理学特点(附1例报告)

目的 研究桥本脑病的临床、影像学和病理学特点.方法 回顾性分析1例桥本脑病患者的临床资料.结果 本例患者的主要临床表现为快速进展的认知功能障碍及癫(癎)发作.血清抗甲状腺抗体显著增高,甲状腺功能基本正常.颅脑MRI显示双侧额、顶、颞、枕叶皮质及皮质下多发性长T1、长T2病灶,以皮质更加明显.增强扫描全部病灶均无强化.病理学检查显示反应性胶质细胞、小血管增生及少量淋巴细胞浸润.电镜检查显示神经细胞部分空泡样变性和线粒体肿胀,白质内可见小囊腔形成.经用糖皮质激素和免疫抑制剂治疗,患者的症状和影像学改变均得到了明显的缓解.结论 桥本脑病的临床特点为亚急性大脑皮质功能障碍,血抗甲状腺抗体增高而甲状腺功能正常,糖皮质激素治疗有效.影像学表现为广泛脑皮质及皮质下异常病灶;病理学改变无特异性.     

儿童桥本脑病:病例报道及文献综述

桥本脑病是一种少见的自身免疫系统疾病,在儿童中尤为少见。其临床表现多种多样,诊断主要依靠早期识别临床特征以及甲状腺抗体的检查。本文报道1例以精神行为异常首发症状的儿童桥本脑病病例,结合实验室检查,影像学检查以及治疗进行分析;同时本文总结以往相关文献,对桥本脑病特别是儿童桥本脑病的流行病学、临床表现、实验室检查及影像学特征、诊断及治疗等方面进行讨论。     

桥本脑病的病因、临床表现和治疗

桥本脑病是桥本甲状腺炎的脑部并发症[1],于1966年首先由Brain等[2]报道,是一种伴有抗甲状腺过氧化物酶抗体和(或)抗甲状腺球蛋白抗体增高,以持续性或波动性神经和精神功能缺陷及对糖皮质激素治疗反应良好为特征的综合征,患者通常甲状腺功能正常或仅有轻度甲状腺功能减退[3].近40年来,国外文献报道约100余例桥本脑病患者[4],而国内只有3例个案报道[5-7].     

桥本脑病临床及实验室分析

目的探讨桥本脑病的临床、实验室、影像学特点。方法回顾性分析2例桥本脑病的临床资料和治疗效果,结合相关文献进行分析。结果 2例患者均有以记忆力下降为主的认知障碍,颅内均有多发病灶。1例有幻视、癫痫发作和明确的血管狭窄,1例有反复头晕、肢体力弱,无血管狭窄。2例均存在抗甲状腺抗体阳性和甲状腺功能亢进,且糖皮质激素治疗有效。结论认知障碍合并抗甲状腺抗体的增高是本病的特点之一,可以合并颅内血管狭窄,确诊后可早期糖皮质激素治疗。     

Hashimoto’s encephalopathy mimicking presenile dementia

Hashimoto’s encephalopathy (HE), which carries kaleidoscopic clinical presentations, is easily misdiagnosed in clinical practice. Early diagnosis and prompt initiation of steroid therapy are associated with good prognosis. We describe a 50-year-old female patient who had subclinical hypothyroidism and who presented herself with gradual cognitive impairment, accompanied with auditory hallucination and delusion. Increased anti-thyroid antibodies titers were found in her serum and cerebrospinal fluid. The HE diagnosis was confirmed using the laboratory test for anti-thyroid antibodies along with the patient’s clinical presentation. We treated her with steroid pulse therapy, and the results were favorable. We highlight this case to call for early diagnosis and prompt intervention of HE in clinical practice.     

Acquired hepatocerebral degeneration and hepatic encephalopathy: correlations and variety of clinical presentations in overt and subclinical liver disease

Acquired hepatocerebral degeneration (AHD) and hepatolenticular degeneration can have similar clinical presentations, but when a chronic liver disease and atypical motor findings coexist, the distinction between AHD and hepatic encephalopathy (HE) can be even more complicated. We describe three cases of AHD (two having HE) with different neuroimaging findings, distinct hepatic diseases and similar motor presentations, all presenting chronic arterial hypertension and weight loss before the disease manifestations. The diagnosis and physiopathology are commented upon and compared with previous reports. In conclusion, there are many correlations among HE, hepatolenticular degeneration and AHD, but the overlapping of AHD and HE could be more common depending on the clinical knowledge and diagnostic criteria adopted for each condition. Since AHD is not considered a priority that affects the liver transplant list, the prognosis in AHD patients remains poor, and flow interruption in portosystemic shunts must always be taken into account.     

18例HIV阴性的神经梅毒诊断

目的 分析HIV阴性的神经梅毒的临床特点及诊断.方法 回顾18例HIM阴性的神经梅毒患者的临床分型、临床特点、血清及脑脊液检查和影像学改变.结果 患者年龄33～62岁,平均(46.0±9.2)岁;分为脑膜血管型3例,麻痹性痴呆8例,脊髓痨3例,视神经萎缩1例,混合型3例.最常见的症状是认知减退、阿一罗氏瞳孔、步态不稳和共济失调、双下肢腱反射消失、精神行为异常等.误诊率高达72.2％,其中误诊为病毒性脑炎4例.血清梅毒螺旋体特异性抗体检测均为阳性,血清和CSF梅毒螺旋体明胶凝集试验(TPPA)阳性率高,CSF白细胞数增加、蛋白升高;影像学表现缺乏特异性.结论 无法根据单一的实验室检查或临床表现确诊神经梅毒.血清梅毒螺旋体特异性抗体是重要的初筛实验,血清、脑脊液RPR和TPPA可作为确证实验,将临床表现与脑脊液白细胞计数、蛋白含量、神经影像学特点结合起来,综合分析,才能避免误诊和漏诊.     

An unusually presenting case of sCJD--the VV1 subtype

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive neurodegenerative disease caused by prions. Typically CJD presents with a triad of rapidly progressive dementia, abnormal movements (e.g., myoclonus) and electroencephalographic (EEG) changes. Recently, CJD has been subdivided into subtypes based on host genetic polymorphisms and the characteristics of the pathological prion protein. Different subtypes likely have different clinical and laboratory presentations. We describe a case of sporadic CJD of the VV1 subtype. We describe our patient's clinical symptoms, time course, laboratory workup, structural and functional neuroimaging data, EEG data and CJD biomarkers. Our patient presented with clinical symptoms atypical for CJD. Because of that, her clinical symptoms were initially attributed to psychiatric reasons. After extensive clinical and laboratory investigation, we concluded that the patient probably had CJD. Postmortem neuropathological results confirmed this clinical hypothesis. We compare our patient's clinical, laboratory and neuroimaging data to the data on typical CJD as well as the data on the few CJD VV1 cases described in the literature. We discuss our case's relevance to the diagnosis of CJD.     

Pediatric macrophagic myofasciitis associated with motor delay

BACKGROUND: Macrophagic myofasciitis (MMF) is a rare inflammatory myopathy characterized by accumulation of perifascicular macrophages without muscle fiber necrosis. Few sporadic pediatric cases have been described, and MMF is recognized as a possible reaction to intramuscular injections of aluminum-containing vaccines. The association of MMF and motor delay is unclear in the pediatric population. We report the clinical evaluation and follow-up of 4 young children with MMF and review of 4 cases previously reported of sporadic, pediatric MMF to better determine the possible association of sporadic MMF in children presenting with motor delay. PATIENTS AND METHODS: Described our 4 case reports in which we observed children presenting for evaluation of motor delay with unrevealing clinical and laboratory evaluations for common causes of motor delay and histopathological evaluations consistent with macrophagic myofasciitis. Muscle data was obtained by quadriceps muscle biopsy. RESULTS: Clinical presentations were similar in all children and were characterized by motor delay, hypotonia, and failure to thrive with an unrevealing evaluation for central nervous system disease, congenital, and mitochondrial myopathies. CONCLUSIONS: Our cases and those previously reported in the literature demonstrate MMF should be considered in the evaluation of children with failure to thrive, hypotonia, and muscle weakness, as clinical outcome appears to be favorable.     

Hyperammonemic encephalopathy associated with valproate acid

We report 4 clinical cases of hyperammonemic encephalopathy (HE) associated with valproate acid (VPA) and review the literature on its pathophysiology. In all four cases, hepatic function was normal and valproic levels were within the therapeutic range. Elevated ammonium levels were found as the only biochemical abnormality. Patients showed decreased level of consciousness, confusion, ataxia and seizures. In 1 case the EEG showed diffused triphasic waves with frontal predominance. After suppression of treatment with VPA there was remission of clinical manifestations, and ammonium levels returned to normal. In order to obtain the correct diagnosis of HE, in all patients treated with either VPA mono or polytherapy, ammonium levels should be considered.     

A reassessment of hemoglobin and hematoma expansion in intracerebral hemorrhage

BackgroundIn patients with spontaneous intracerebral hemorrhage (ICH), prior studies identified an increased risk of hematoma expansion (HE) in those with lower admission hemoglobin (Hgb) levels. We aimed to reproduce these findings in an independent cohort.MethodsWe conducted a cohort study of patients admitted to a Comprehensive Stroke Center for acute ICH within 24 hours of onset. Admission laboratory and CT imaging data on ICH characteristics including HE (defined as >33% or >6 mL), and 3-month outcomes were collected. We compared laboratory data between patients with and without HE and used multivariable logistic regression to determine associations between Hgb, HE, and unfavorable 3-month outcomes (modified Rankin Scale 4-6) while adjusting for confounders including anticoagulant use, and laboratory markers of coagulopathy.ResultsAmong 345 patients in our cohort (mean [SD] age 72.9 [13.7], 49% male), 71 (21%) had HE. Patients with HE had similar Hgb versus those without HE (mean [SD] 13.1 [1.8] g/dl vs. 13.1 [1.9] g/dl, p=0.92). In fully adjusted multivariable models, Hgb was not associated with HE (OR per 1g/dl 1.01, 95% CI 0.86 -1.17, p = 0.94), however higher admission Hgb levels were associated with lower odds of unfavorable 3-month outcome (OR 0.83 per 1 g/dl Hgb, 95% CI 0.72–0.96, p=0.01).ConclusionWe did not confirm a previously reported association between admission Hgb and HE in patients with ICH, although Hgb and HE were both associated with poor outcome. These findings suggest that the association between Hgb and poor outcome is mediated by other factors.