橄榄桥脑小脑萎缩11例临床与MRI表现

目的:分析橄榄桥脑小脑萎缩(OPCA)的临床表现及MRI特征,以利早期诊断。方法:对11例OPCA病人的临床表现及头颅MRI特征进行回顾性分析。结果:OPCA男性多于女性,平均年龄55岁,其临床表现多种多样,以小脑症状,植物神经症状及锥体外系症状多见,头颅MRI比头颅CT效果好,以小脑和脑干萎缩为主,大脑皮质萎缩轻。结论:成年人出现小脑性共济失调、植物神经功能紊乱和锥体外系症状,应高度怀疑OPCA,且MRI有助于早期诊断。     

16例多系统萎缩的临床与MRI分析

目的探讨多系统萎缩（MSA）的临床及MRI特征。方法对临床诊断的16例MSA患者的临床及MRI资料进行回顾性分析。结果根据临床资料及MRI结果分为3种类型：（1）11例橄榄体桥脑小脑萎缩（OPCA）,其中小脑共济失调为主10例,脑MRI明显可见延髓萎缩、桥脑萎缩及小脑萎缩,其中桥脑十字征9例,小脑中脚萎缩5例;（2）3例Shy-Drager综合征（SDS）,10例中以植物神经功能紊乱为主要表现2例,壳核边缘高信号和壳核萎缩;（3）2例纹状体-黑质变性（SND）,首发症状均因锥体外系表现而早期误诊为帕金森病（PD）,脑MRI示壳核萎缩,其中1例T2WI壳核及纹状体低信号。结论 MSA是一组神经系统多部位变性综合征,MRI对MSA的诊断有较肯定的意义。     

帕金森叠加综合征10例临床分析

目的 分析帕金森叠加综合征的临床特点、影像学特征,为临床诊断提供依据.方法 回顾性分析10例帕金森叠加综合征患者的临床及影像学资料.结果 诊断多系统萎缩(MSA)-C型5例,MSA-P型2例,进行性核上性麻痹(PSP)3例.MSA-C型以小脑症状突出,MSA-P型以锥体外系症状表现为主,PSP以姿势异常、躯干僵直、核上性眼球运动障碍为主要表现.头颅MRI显示MSA-C型的主要病变在脑干、小脑,MSA-P型病变在壳核,PSP的主要病变在中脑.结论 临床表现结合头颅MRI检查可提高帕金森叠加综合征的诊断率.脑干、小脑萎缩有助于MSA-C型诊断,壳核萎缩及壳核背外缘T2WI低信号支持MSA-P型诊断,中脑萎缩支持PSP诊断.     

多系统萎缩的临床与MRI特征

目的探讨多系统萎缩(MSA)的临床与MRI特征及其对临床亚型诊断的意义。方法回顾性分析28例MSA患者的临床及MRI资料。结果橄榄脑桥小脑萎缩(OPCA)以小脑体征(75.0%)突出,MRI表现为脑桥萎缩(91.7%)、小脑蚓部萎缩(91.7%),第四脑室扩大(83.8%),T2WI出现脑桥、小脑对称性高信号(63.6%)及脑桥十字征;纹状体黑质变性(SND)以锥体外系症状(80.0%)明显,MRI改变多位于基底节核团,表现为壳核萎缩(60.0%),T2WI示壳核外侧缘缝隙样高信号(80.0%);ShyDrager综合征(SDS)以自主神经症状(81.8%)为主,出现早且重,MRI未见特异性变化。结论MRI有助于提高MSA及其亚型诊断。脑桥萎缩、T2WI高信号改变,尤其是脑桥十字征的出现有助于OPCA诊断;壳核萎缩与T2WI壳核外侧缘缝隙样高信号改变支持SND诊断。     

多系统萎缩17例临床分析和一例尸检报告

目的 分析多系统萎缩的临床、病理特点和诊断标准。方法 按Gilman诊断标准,回顾性分析了17例多系统萎缩的临床资料和其中1例病理资料。结果 按Gilman诊断标准,本组17例中,确诊多系统萎缩1例,拟诊12例,可疑者4例。首发症状表现为植物神经功能障碍者8例,锥体外系体征8例。病程中出现姿位性低血压的有12例;排尿障碍16例,阳痿9例;帕金森综合征症状12例;共济失调13例;皮质兴髓束损害12例。头颅MRI发现10例脑萎缩。1例经尸检证实存在少突胶质细胞包涵体。结论 多系统萎缩是累及植物神经、锥体外系、小脑和皮质脊髓束,并具少突胶质细胞包涵体等特定神经病理表现的变性疾病。Gilman诊断标准具有较强的临床可操作性。     

多系统萎缩17例临床分析和一例尸检报告

目的分析多系统萎缩的临床、病理特点和诊断标准.方法按Gilman诊断标准,回顾性分析了17例多系统萎缩的临床资料和其中1例病理资料.结果按Gilman诊断标准,本组17例中,确诊多系统萎缩1例,拟诊12例,可疑者4例.首发症状表现为植物神经功能障碍者8例,锥体外系体征8例.病程中出现姿位性低血压的有12例;排尿障碍16例,阳痿9例;帕金森综合征症状12例;共济失调13例;皮质脊髓束损害12例.头颅MRI发现10例脑萎缩.1例经尸检证实存在少突胶质细胞包涵体.结论多系统萎缩是累及植物神经、锥体外系、小脑和皮质脊髓束,并具少突胶质细胞包涵体等特定神经病理表现的变性疾病.Gilman诊断标准具有较强的临床可操作性.     

多系统萎缩的临床与影像学分析

目的 探讨多系统萎缩(MSA)的临床特点、影像学特征与临床表现的相关性，为临床诊断提供依据。方法 按Gilman诊断标准，回顾性分析26例MSA病人临床资料、一般辅助检查结果及脑CT、MRI资料。结果 拟诊MSA2l例，可能MSA5例，其中橄榄桥脑小脑萎缩(OPCA)14例，Slay-Drager综合征(SDS)8例，纹状体黑质变性(SND）4例。MRI显示OPCA的主要病变部位在小脑、桥脑、延髓；SDS仅部分有小脑病变，大部分正常；SND主要病变在壳核。而小脑、桥脑、延髓病变不明显。脑CT改变均不明显。结论 临床表现与MRI结合可提高MSA中OPCA、SND的诊断率。在SDS病人MRI改变不明显。一般辅助检查、脑CT对MSA诊断意义不大。     

Shy-Drager综合征16例临床分析

Shy-Drager综合征少见。本文报告16例临床资料。男12例，女4例，以47～60岁居多（14例）。都有典型的小脑症状（眼震、构音障碍和共济失调）、体位性低血压及排尿淋漓及男性阳萎植物神经功能障碍。部分尚有锥体外系和锥体束征。CT／MRI扫描显示小脑和脑干萎缩。本症缺乏有效的治疗方法，合理提高血压可预防体位性低血压晕厥。     

小脑梗死38例临床分析

目的 通过对38例小脑梗死临床诊断与治疗分析,提高对该病的认识.方法 回顾38例小脑梗死的病因、临床表现及影像学资料,对该病有进一步认识.结果 小脑梗死病因主要为高血压动脉硬化,其次为心源性脑栓塞.临床表现缺乏特异性.头颅CT、MRI检查有助早期诊断.结论 小脑梗死是神经科少见疾病,易漏诊,对疑诊小脑梗死的患者,应尽早行头颅MRI及CT检查.     

小脑梗死38例临床分析

目的 通过对38例小脑梗死临床诊断与治疗分析,提高对该病的认识.方法 回顾38例小脑梗死的病因、临床表现及影像学资料,对该病有进一步认识.结果 小脑梗死病因主要为高血压动脉硬化,其次为心源性脑栓塞.临床表现缺乏特异性.头颅CT、MRI检查有助早期诊断.结论 小脑梗死是神经科少见疾病,易漏诊,对疑诊小脑梗死的患者,应尽早行头颅MRI及CT检查.     

橄榄脑桥小脑萎缩与“十字征”

目的:探讨橄榄脑桥小脑萎缩(OPCA)的临床和影像学特点。方法:对1例橄榄脑桥小脑萎缩患者的临床资料进行回顾性分析并文献复习。结果:本病以小脑共济失调为首发表现,部分出现植物神经系统受损、锥体束或锥体外系症状。影像学除脑干变细、小脑体积变小外,可出现特征性的十字征表现。结论:因该病和某些小脑变性疾病表现相重叠,且无有意义的生化标记物,故其影像学十字征的发现对此病的诊断和鉴别诊断意义重大。     

橄榄-脑桥-小脑萎缩与“十字征”八例

目的探讨MRI脑桥十字征的橄榄-脑桥-小脑萎缩(OPCA)患者临床与影像学特点。方法总结8例经临床诊断为OPCA,分析其临床特点及影像学特征表现。结果 8例患者的临床表现均以小脑性共济失调和脑干功能受损伴有自主神经功能障碍为主,MRI在脑桥轴位T2WI上均出现典型的"十字征"。结论以小脑性共济失调和脑干功能受损为主要表现的OPCA患者,多伴有自主神经功能障碍,脑桥十字征是MRI特征性表现之一,有助于该病的诊断。     

橄榄桥脑小脑萎缩的临床观察及随访研究

目的 :探讨橄榄桥脑小脑萎缩 (OPCA)患者病情的发生、发展和转归的规律及可能影响因素。方法 :对确诊的 38例 (家族性 2例 ,散发性 36例 )进行脑脊液、头颈 CT、 MRI检查及临床特点分析 ,并对其中 2 8例进行了 1～ 12年随访。结果 :1SOPCA和 FOPCA平均发病年龄分别为 4 6 .9岁和 2 0岁 ;平均病程分别为 6 .7年和 14 .5年。 2受累系统症状出现频率为小脑共济失调 92 .1% ,锥体束征 86 .8% ,构音障碍 84 .2 % ,自主神经损害 76 .3%。 3病程第一年内头颅 CT和 MRI分别有 16 .7%和 70 %的异常。 4平均确诊时间为 4 .1年 ,目前治疗无法改变其转归 ,随访 2 8例死亡 3例 ,死因分别为吸入性肺炎、窒息及全身衰竭。结论 :OPCA受累系统以小脑最多 ,无遗传家族史者病情发展较快 ,多死于并发症。核磁共振为首选辅助检查 ;随访有助于早期确诊。     

橄榄脑桥小脑萎缩20例临床分析

目的探讨橄榄脑桥小脑萎缩的临床表现和影像学检查特点,以便早期诊断并改善患者预后。方法回顾性分析经临床诊断的20例橄榄脑桥小脑萎缩患者的相关资料。结果橄榄脑桥小脑萎缩患者临床以小脑性共济失调、言语不清、排尿功能障碍、帕金森症状等最常见。16例患者头颅磁共振检查结果示脑萎缩,主要为小脑和脑干萎缩。结论橄榄脑桥小脑萎缩临床以小脑性共济失调为主要表现,言语障碍较继往报道发生率高,头颅磁共振可表现为小脑、脑干萎缩,并可一定程度上反映病程。     

Idiopathic cerebellar ataxia of late onset: natural history and MRI morphology.

Twenty eight patients with the clinical diagnosis of idiopathic late onset cerebellar ataxia were examined clinically and by magnetic resonance imaging (MRI) or computed tomography (CT). In addition, the clinical records of all patients were analysed retrospectively. On the basis of their clinical presentation they were subdivided into patients with a pure cerebellar syndrome (n = 9) and patients with a cerebellar syndrome and additional non-cerebellar symptoms (n = 13). No attempts were made to classify patients with a disease duration of less than four years (n = 6) because the retrospective analysis showed that the disease started almost invariably with a pure cerebellar syndrome and additional symptoms came later. Patients with a lasting pure cerebellar syndrome had a significantly better prognosis than patients with additional non-cerebellar involvement (annual progression rate rate: 0.40 versus 0.80). Calculated median lifetime from onset of symptoms was 20.7 years in patients with a pure cerebellar syndrome and 7.7 years in patients with additional non-cerebellar symptoms. Among the latter, disease progression was faster the earlier non-cerebellar symptoms occurred. All of them presented with Parkinsonian symptoms, whereas bulbar symptoms, vertical gaze paresis, pyramidal deficits, dementia and urinary incontinence were encountered less frequently. MRI or CT showed cerebellar atrophy without apparent involvement of brainstem structures in all patients with a pure cerebellar syndrome suggesting the diagnosis of cerebellar cortical atrophy (CA). The majority of the patients with additional non-cerebellar symptoms had evidence of an atrophy of the cerebellum and the brainstem suggesting the presence of olivo-ponto-cerebellar atrophy (OPCA). In two of them, however, MRI morphology was not compatible with the diagnosis of OPCA.     

Analysis of follow-up survey in spinocerebellar degeneration

Spinocerebellar degeneration (SCD) is associated with other various degeneration of the nervous systems such as the optic tract, pyramidal pathway, extrapyramidal system, nuclei of the brain stem and autonomic nervous system as well as changes of heart. The clinical pattern, also have the great variability. We investigated the mode of progression of clinical symptoms and signs in 214 cases of SCD which were examined 2 times at intervals of about 10 years. 79 of 214 cases were reported to be died at the last examination. 135 alive cases included 3 with the Holmes type, 14 with late cortical cerebellar atrophy (LCCA) 10 with Menzel type, 18 with olive-ponto-cerebellar atrophy (OPCA), 33 with spinocerebellar form (SCF), 6 with Friedreich's ataxia, 18 with hereditary spastic paraparesis (HSP) and 33 with the other type. 79 dead cases included 0 with the Holmes type, 6 with LCCA, 5 with Menzel type, 32 with OPCA, 16 with SCF, 1 with Friedreich's ataxia, 4 with HSP and 15 with the other type. The disability of daily living in SCD revealed slower progression in the advanced stage than in the early stage. Every type of SCD had some different progression of disability each other. In the early stage, Friedreich's ataxia showed the highest progression of disability, but in the advanced stage, Holmes type and the OPCA did. Holmes type showed progression of ataxia without any remarkable change of other systems. LCCA showed increase of abnormality in the eye movements, pyramidal tract and autonomic nervous system in addition to the cerebellar system. OPCA involved multiple systems as ataxia worsening, but Menzel type had no remarkable changes of incidence in eye movement disorder.(ABSTRACT TRUNCATED AT 250 WORDS)     

The development of infratentorial atrophy in patients with idiopathic cerebellar ataxia of late onset: a CT study

Summary The development of infratentorial atrophy in six patients suffering from idiopathic cerebellar ataxia of late onset was studied by a retrospective evaluation of consecutive computed tomography (CT) scans. Four patients had evidence of olivopontocerebellar atrophy (OPCA) both on clinical testing and magnetic resonance imaging (MRI). In these four patients, atrophy of the cerebellum and brain stem became visible at the same time and progressed in a roughly parallel manner, whereas in the remaining two the brain stem was left intact. In all patients with OPCA, definite brain-stem atrophy was visible earlier than the appearance of non-cerebellar clinical symptoms. The present data suggest that CT investigations at regular intervals may be of prognostic value in cerebellar ataxias.     

Multiple system atrophy. Clinical and MR observations on 42 cases

Probable or possible multiple system atrophy (MSA) was diagnosed on strict clinical criteria in 42 patients: 20 with combined parkinsonism and cerebellar ataxia, 9 with striatonigral degeneration (SND) and 13 with olivopontocerebellar atrophy (OPCA). All patients were then studied with 0.5 and/or 1.5 Tesla magnetic resonance (MR) units. MR imaged putaminal abnormalities in all 9 patients with SND and posterior fossa obnormalities consistent with OPCA in all 13 patients with this diagnosis. Of the 20 patients with parkinsonism and cerebellar involvement, classified as probable MSA, 7 presented putaminal abnormalities only, 3 abnormalities consistent with OPCA only and 10 a combination of both. These findings show strong MRI support for the clinical diagnosis of MSA.

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Sommario In base a precisi criteri clinici 42 pazienti furono riconosciuti affetti da atrofia multisistemica (MSA) probabile o possibile. Venti pazienti presentavano parkinsonismo e atassia cerebellare; in 9 fu fatta diagnosi di degenerazione strio-nigrica (SND) e in altri 13 di atrofia olivopontocerebellare (OPCA). Tutti i pazienti furono sottoposti a risonanza magnetica 0.5 e/o 1.5 T. La RM mostrava alterazioni nei putamen nei 9 pazienti con SND e alterazioni in fossa posteriore come attese nell'OPCA nei 13 casi diagnosticati affetti da OPCA. In 10 dei 20 pazienti con parkinsonismo e atassia cerebellare le alterazioni nei putamen e in fossa posteriore erano associate. I nostri dati confermano che la RM è un supporto diagnostico fondamentale nella diagnosi di MSA.