神经棘红细胞增多症(附1例报告)

目的通过舞蹈-棘红细胞增多症(ChAc)的临床资料分析,提高对ChAc的认识。方法对收治的1例ChAc病例的诊断过程进行分析,结合文献复习对神经棘红细胞增多症(NA)的遗传方式、影像学和实验室检查等特点进行总结。结果NA临床症状复杂多样,其发病特点与广泛的非特异性神经系统病变、棘红细胞增多密切相关。NA治疗以对症支持为主。结论NA是一种罕见的神经退行性疾病,预后差;早发现、早诊断、早治疗可改善预后。     

家族性神经棘红细胞增多症

目的：报道神经棘红细胞增多症一家系3例,方法：对先证者进行临床症、血生化、激素水平及红细胞形态的布氏相差显著微镜及扫描电镜观察,结果：此家系中患者均20岁左右起病的女性,主要症状为进行性舞蹈样动作、睡眠后舞蹈动作并不消失,,先证者有精神抑郁、棘形红细胞增多（扫描电镜观察百分比为44．3％）、血浆载脂蛋白B100水平明显降低（0．26g/L)及泌乳素水平显著升高（96．3μg/L)特征。结论先证者的临床表现、化验检查及电镜观察结果,符合神经－棘红细胞增多症的诊断。此病例除载脂蛋白B100缺乏处,还同时伴有高泌乳素血症。生物相差显著镜与扫描电镜观察能反映棘形红细胞增多的真实程度。     

神经棘红细胞增多症(附1例报道)

神经棘红细胞增多症是一种罕见的遗传性疾病,至今为止,国内报道的病例很少,现报道1例如下。1临床资料患者女性,32岁,已婚,因不自主运动4年,伴全身无力半年入院。患者4年前出现口舌不自主运动,不时咬舌,咬下唇,不能控制,并将舌及下唇咬伤,继而出现颈部的不自主扭动;数月后出现上肢舞动,步态不稳,易跌倒,并感乏力。情绪激动时,不自主运动加重,睡眠中明显减轻。不伴鬼脸样动作,无抽搐;2003年9月曾在本院住院治疗,诊断为“遗传性舞蹈病”,并给予氯硝安定、泰必利、安坦治疗。出院后,全身无力症状进行性加重,近半年来逐渐不能起床,不能行走,说话…     

舞蹈病-棘状红细胞增多症1例报道并文献复习

目的通过病例及相关文献复习本探讨舞蹈病-棘状红细胞增多症的临床表现、细胞学特点、治疗方法。方法回顾性分析湖北医药学院附属太和医院收治的1例舞蹈病-棘状红细胞增多症患者的临床资料。结果舞蹈病-棘状红细胞增多症的主要临床特点是不自主舞蹈样运动,表现为口唇咀嚼样动作、下颌不自主运动、舌唇自咬,伴有实验室检查外周血棘状红细胞增多。以对症支持治疗为主。结论舞蹈病-棘状红细胞增多症的典型临床表现、周围血棘红细胞计数增多是本病确诊的主要依据。本病预后差,因此对疑似病例需及时行外周血涂片及透视扫描电子显微镜等检查以便早诊断、早治疗。     

舞蹈病-棘红细胞增多症1例报告

<正>神经棘红细胞增多症(neuroacanthocytosis,NA)是一类临床罕见的伴有外周血棘红细胞增多的神经系统遗传性疾病,于1967年由CRITCHLEY等~[1]与LEVINE等~[2]首先发现。NA通常包括舞蹈病-棘红细胞增多症(Chorea-acanthocytosis,Ch Ac)、Mcleod综合征(Mcleod neuroacanthocytosis syndrome,MLS)、类亨廷顿病2型(Huntington’s disease-like 2,HDL2)以及泛酸激酶相关性的神经退化性疾病(Pantothenate kinase-associated neurodegeneration,PKAN)     

舞蹈-棘红细胞增多症1例报告

1病例资料患者李某,男性,46岁,国家公务员。主因"四肢及头部不自主抖动3a,加重1周"入院。患者于3a前出现不自主点头,吃饭时易咬内侧颊部,并出现坐位时不自主伸腿,为治疗曾拔掉3颗磨牙,症状未改善。后出现驾车时身不由己晃动,逐步出现咬舌,右侧无名指不自主抖动,并发展到右手、右上肢抖动,最终四肢及头部抖动。患者抖动呈阵发性,情     

外周血涂片棘红细胞阴性的舞蹈病-棘红细胞增多症一例

1病例报告 患者男 ,34 岁. 主因"渐进性四肢舞动 3 年余"于2019年3月入院.患者于2016年底逐渐出现右下肢不自主舞蹈样动作,情绪激动时加重,睡眠时消失,后进展至右上肢,伴频繁咬舌.当地医院行肌电图检查示周围神经病变,左小指展肌、左拇短展肌及右胫前肌呈神经源性损害.血涂片示:细胞形态未见明显异常.     

舞蹈病-棘状红细胞增多症(附二例报告)

目的了解舞蹈病-棘状红细胞增多症（chorea-aeanthocytosis，ChAc）的临床表现及辅助检查特点，以便早期做出诊断。方法回顾分析2例ChAc患者的诊断过程，总结其临床表现、实验室检查、肌电图、头颅MRI及红细胞形态变化的特点。结果ChAc以舞蹈样不自主运动为主要临床表现，伴有肌酶增高，肌电图检查结果示失神经电位，MRI检查示脑萎缩，外周血可见棘状红细胞。结论ChAc是一种罕见的进展型、致命性神经系统运动障碍性疾病，目前治疗以对症支持为主，早期诊断有助于延长患者生命。     

神经棘红细胞增多症的临床及神经影像学特征

目的 分析神经棘红细胞增多症的临床及头颅影像学特征.方法 回顾分析8例患者的临床资料并复习文献,周围血棘红细胞检测采用外周血涂片、红细胞盐水诱发实验和扫描电镜方法.结果 男性2例,女性6例;发病年龄10～35岁,平均发病年龄22岁,4例以口面舌部肌张力障碍为首发突出症状,3例以四肢远端不自主运动为首发突出表现,且于发病过程中累及口面部肌张力;1例以帕金森症状为主要临床表现.4例有癫痫发作,为全身强直阵挛发作.4例智力减退.6例肌张力低、腱反射低.8例患者外周血涂片光镜下7例棘红细胞增多,另1例外周血涂片正常,但红细胞盐水诱发实验和电镜下棘红细胞增多.所有患者肌酸激酶不同程度轻度升高.8例患者头颅MRI中,6例示不同程度侧脑室扩大,前角明显增宽;尾状核和豆状核萎缩.4例肌电图和神经传导速度检查,2例正常,2例示上、下肢神经源性损害,感觉纤维轴突受累为著.外周血棘红细胞检测:7例患者外周血涂片光镜下棘红细胞增多;8例红细胞盐水诱发实验阳性,2例电镜检查符合棘红细胞增多症.结论 神经棘红细胞增多症是一种主要累及基底节的进行性神经系统变性病,主要表现为口面肌张力障碍、舞蹈症、智力减退、癫痫发作,头颅影像学示不同程度的尾状核和豆状核萎缩,周围血的棘红细胞检测方式对该病有确诊意义.     

神经棘红细胞增多症误诊为强迫症一例

患者　女,31岁。平素性格开朗,1年前患口腔溃疡,因担心治不好而时有焦虑。溃疡治愈后,自认为口腔有瘢痕影响说话,常出现吸吮动作,并不时咬下唇,她不想这样做,但不能控制,很痛苦,偶有双侧面部肌肉痉挛。未发现其他神经体征,诊断为强迫症。2004年1月住外院治疗,予氯丙咪嗪50mg/d渐加至200mg/d,至第6周,症状大部缓解出院。20d后症状再现,并出现步态不稳,时有跌倒,将下唇咬烂;曾为避免咬下唇而咬伤手指或咬毛巾。2004年6月1日住本院神经内科。入院检查:意识清,焦虑,迫切求治,语言欠流利。口唇黏膜破损,下唇部分缺失。进餐时,舌不由自主将食…     

Mycoplasma pneumoniae infection and obsessive-compulsive disease: a case report

It has been demonstrated that obsessive-compulsive disease and/or tic syndromes in children may be triggered by an antecedent infection especially with group A beta-hemolytic streptococci, and this subgroup of children has been designated by the acronym PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections). Other infectious agents such as viruses and bacteria have also been reported to be associated with the acute onset or dramatic exacerbation of obsessive-compulsive disease or Tourette syndrome, and another acronym, PITAND (pediatric infection-triggered autoimmune neuropsychiatric disorder) has appeared in the literature. The involvement of other infectious agents such as Mycoplasma pneumoniae has been described in single case reports. We describe a case of a 5.5-year-old boy who suddenly developed obsessive-compulsive disease symptoms during a M. pneumoniae pneumonia. After treatment with oral clarithromycin, all his obsessive-compulsive disease symptoms disappeared. To our knowledge, this is the first report that shows the association between Mycoplasma pneumoniae infection and obsessive-compulsive disease.     

Successful use of maintenance electroconvulsive therapy in the treatment of clozapine-associated obsessive-compulsive symptoms in schizophrenia: a case report

Denovo obsessive-compulsive (OC) symptoms are associated with the use of atypical antipsychotics, clozapine in particular. Treatment of clozapine-associated OC symptoms is challenging and often difficult because continuation of clozapine can result in worsening of OC symptoms, whereas discontinuation may result in worsening of psychosis. We report, for the first time, the use of maintenance electroconvulsive therapy, in clozapine-associated OC symptoms, in a patient with schizophrenia.     

Quetiapine and obsessive-compulsive symptoms (OCS): case report and review of atypical antipsychotic-induced OCS

The atypical antipsychotics have advanced the treatment of schizophrenia and have proved to be effective agents in treating other disorders with or without psychotic features. We review the literature concerning an increasingly reported and interesting adverse effect, atypical antipsychotic-induced obsessive-compulsive symptoms (OCS). The first known report of quetiapine exacerbating OCS in a 43-year-old man with obsessive-compulsive disorder (OCD), trichotillomania, delusional disorder and bipolar II disorder is presented. Mechanisms, including 5-HT2A and 5-HT2C antagonism, serotonergic regulation of dopamine systems and putative dopaminergic subtypes of OCS and OCD, are discussed. Given the paradoxical efficacy of the atypical antipsychotics in pure OCD, the neurobiology and comorbidity of OCD and schizophrenia, as well as the increasing use of atypical antipsychotics, a cautious and rational pharmacotherapeutic treatment approach is recommended.     

Aripiprazole augmentation in poor insight obsessive-compulsive disorder: a case report

Background  

Obsessive-compulsive disorder is associated with a relevant impairment in social and interpersonal functioning and severe disability. This seems to be particularly true for the poor insight subtype, characterised by a lack of consciousness of illness and, consequently, compliance with treatment. Poor responsiveness to serotonergic drugs in poor insight obsessive-compulsive patients may also require an augmentation therapy with atypical antipsychotics.