“The red flags” in clinical approach to acute ataxia – the experience in cohort of 76 children

Abstract

Objectives: 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. Methods: The retrospective study included the patients with AA treated in Institute from 2015 - 2021. The inclusion criteria were: children aged 1 - 18 years; evolution time of ataxia within 72 hours, diagnosis made by a physician. The exclusion criteria were: anamnestic data about ataxia without confirmation by any physician; chronic/persistent ataxia; 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. Evaluated parameters in patients are demographic features, etiology, the age of ataxia onset, clinical presentation and symptoms associated with ataxia, neuroimaging, comorbidities, treatment, and the outcome. The predictive value of different outcomes was analyzed using univariate and multivariate logistic regression analyses. Results: 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. Univariate analysis showed that the presence of CS-plus symptoms (p=0.007) and structural abnormalities (p=0.001) were related to poor outcomes. In multivariate logistic regression analysis of these factors, statistical significance remained (p=0.021 and p=0.002) respectively. Conclusions: Most of the children with AA have “benign” etiology with favorable outcomes such as intoxication and post/parainfectious cerebellar ataxia. 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.