急性共济失调临床治疗的“危险信号”——76例儿童队列的经验。

IF 2.7 3区 医学 Q3 NEUROSCIENCES Cerebellum Pub Date : 2024-08-01 Epub Date: 2023-11-27 DOI:10.1007/s12311-023-01639-w
R Kravljanac, A Golubovic, B Vucetic Tadic, S Ostojic, I Cerovic, J Savkic
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引用次数: 0

摘要

本研究的目的是确定急性共济失调(AA)儿童最常见的病因、临床表现和预后预测因素,并确定AA儿童诊断方法中的“危险信号”。回顾性研究纳入2015 - 2021年在该所治疗的AA患者。纳入标准为1-18岁儿童,共济失调72 h内发生时间,经医师诊断。排除标准为未经医生证实的共济失调的记忆资料、慢性/持续性共济失调、心因性或后发性共济失调。临床表现分为两类:(1)孤立性小脑征象(CS):步态共济失调、构音障碍、运动障碍、意向性震颤、构音障碍、眼球震颤;(2) CS +症状,包括伴有任何其他症状的CS,如脑病(GCS < 15)、意识障碍、呕吐、头痛、新发肢体或面部轻瘫、斜颈、张力低下和斜视。结果在住院结束时进行评估,并定义为完全恢复或不完全恢复。该研究包括76名儿童,平均年龄为5.7岁(IQR 2.1-8.3)。最常见的原因是免疫介导/感染性小脑共济失调27例(35.5%),中毒24例(31.6%),其次是前庭共济失调、阵挛-肌阵挛-共济失调综合征和颅内扩张过程。42例(56%)为孤立性CS, 35例(46%)为CS +。62例(81.6%)患者完全康复。分析了一些危险因素(性别、年龄、既往感染、“小脑合并症状”和结构异常/神经影像学异常)及其与预后的关系。分析显示,共济失调的附加症状,即所谓的“小脑加症状”(p = 0.002)和结构异常(p < 0.001)的存在,在统计学上具有更高的不良预后频率。单变量分析仍有统计学意义。多变量logistic回归分析中包含的重要数据也显示,“小脑+症状”(p = 0.021)和结构异常(p = 0.002)的存在与预后不良有关。大多数AA患儿有“良性”病因,如中毒和感染后/副感染性小脑性共济失调,预后良好。另一方面,AA可能是中枢神经系统肿瘤或副肿瘤现象的首发表现。与小脑症状相关的“危险信号”包括肢体或面瘫、张力低下、GCS < 15、呕吐、阵挛、头痛、肌阵挛、视力障碍、斜颈和眩晕。这些症状和/或脑结构异常的存在与嗜酒成瘾儿童的不良预后有关。
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"The Red Flags" in Clinical Approach to Acute Ataxia-the Experience in Cohort of 76 Children.

The aim of our study is to define the most frequent etiology, clinical presentation, and predictive factors of outcome in children with acute ataxia (AA) and to determine "the red flags" in the diagnostic approach to children with AA. The retrospective study included the patients with AA treated in the institute from 2015 to 2021. The inclusion criteria were children aged 1-18 years, evolution time of ataxia within 72 h, and diagnosis made by a physician. The exclusion criteria were anamnestic data about ataxia without confirmation by any physician, chronic/persistent ataxia, and psychogenic or postictal ataxia. Clinical presentation was divided into two categories: (1) isolated cerebellar signs (CS): ataxic gait, dysmetria, dysdiadochokinesia, intention tremor, dysarthria, and nystagmus; (2) CS-plus symptoms which included CS associated with any of other symptoms such as encephalopathy (GCS < 15), awareness disturbances, vomiting, headache, a new onset limb or facial paresis, torticollis, hypotonia, and opsoclonus. The outcome was assessed at the end of hospitalization and was defined as complete or incomplete recovery. The study included 76 children, with a mean age of 5.7 years (IQR 2.1-8.3). The most frequent causes of AA were immune-mediated/infective cerebellar ataxia in 27 (35.5%), and intoxication in 24 (31.6%) cases, followed by vestibular ataxia, opsoclonus-myoclonus-ataxia syndrome, and intracranial expansive process. Forty-two (56%) cases experienced isolated CS, and 35 (46%) cases had CS-plus. Complete recovery was experienced by 62 (81.6%) patients. Analysis of some risk factors (sex, age, presence of previous infection, "cerebellar plus symptoms," and structural abnormalities/neuroimaging abnormalities) and their relation to outcome was performed. Analysis showed that presence of additional symptoms to ataxia, so called "cerebellar plus symptoms" (p = 0.002) and structural abnormalities (p < 0.001), had statistically higher frequency of poor outcome. Statistical significance remained in the univariate analysis. Significant data was included in multivariate logistic regression analysis which also showed that presence of "cerebellar plus symptoms" (p = 0.021) and structural abnormalities (p = 0.002) is related to a poor outcome. Most of the children with AA have "benign" etiology such as intoxication and post/parainfectious cerebellar ataxia with favorable outcomes. On the other hand, AA might be the first manifestation of CNS neoplasm or paraneoplastic phenomena. "The red flags" associated with cerebellar signs are limbs or facial palsy, hypotonia, GCS < 15, vomiting, opsoclonus, headache, myoclonus, visual impairment, torticollis, and vertigo. The presence of those signs and/or structural brain abnormalities was related to poor outcomes in children with AA.

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来源期刊
Cerebellum
Cerebellum 医学-神经科学
CiteScore
6.40
自引率
14.30%
发文量
150
审稿时长
4-8 weeks
期刊介绍: Official publication of the Society for Research on the Cerebellum devoted to genetics of cerebellar ataxias, role of cerebellum in motor control and cognitive function, and amid an ageing population, diseases associated with cerebellar dysfunction. The Cerebellum is a central source for the latest developments in fundamental neurosciences including molecular and cellular biology; behavioural neurosciences and neurochemistry; genetics; fundamental and clinical neurophysiology; neurology and neuropathology; cognition and neuroimaging. The Cerebellum benefits neuroscientists in molecular and cellular biology; neurophysiologists; researchers in neurotransmission; neurologists; radiologists; paediatricians; neuropsychologists; students of neurology and psychiatry and others.
期刊最新文献
Correction: Systematic Review and Meta-Analysis of the Diagnostic Accuracy of a Graded Gait and Truncal Instability Rating in Acutely Dizzy and Ataxic Patients. Correction: Long-Term Follow-Up Before and During Riluzole Treatment in Six Patients from Two Families with Spinocerebellar Ataxia Type 7. Correction: Silica Nanoparticles from Melon Seed Husk Abrogated Binary Metal(loid) Mediated Cerebellar Dysfunction by Attenuation of Oxido-inflammatory Response and Upregulation of Neurotrophic Factors in Male Albino Rats. Clinical Heterogeneity of Essential Tremor: Understanding Neural Substrates of Action Tremor Subtypes. The Neuroimmune System and the Cerebellum.
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