"The Red Flags" in Clinical Approach to Acute Ataxia-the Experience in Cohort of 76 Children
R Kravljanac1,2, A Golubovic3, B Vucetic Tadic4,5
1Pediatric Clinic, Institute for Mother and Child Healthcare of Serbia, Belgrade, Serbia. ruzica.kravljanac@gmail.com.
Insights
In children with acute ataxia (AA), immune-mediated/infective causes and intoxication are most common. "Cerebellar plus symptoms" and structural brain abnormalities are key indicators of a poor outcome.
Area of Science:
- Pediatric Neurology
- Neuroscience
- Clinical Pediatrics
Background:
- Acute ataxia (AA) in children presents a diagnostic challenge.
- Identifying frequent etiologies and predictive factors for outcome is crucial for timely intervention.
- Recognizing
- red flags
- is essential for effective diagnostic approaches.
Purpose of the Study:
- To define the most frequent causes, clinical presentations, and outcome predictors in pediatric acute ataxia.
- To identify critical
- red flags
- in the diagnostic process for children with AA.
Main Methods:
- Retrospective study of children (aged 1-18 years) with acute ataxia (evolution time ≤72 hours) treated between 2015 and 2021.
- Clinical presentation categorized into isolated cerebellar signs (CS) and CS-plus symptoms (CS with additional neurological deficits).
- Outcome assessed as complete or incomplete recovery; risk factors for poor outcome analyzed using univariate and multivariate logistic regression.
Main Results:
- The most common causes of AA were immune-mediated/infective cerebellar ataxia (35.5%) and intoxication (31.6%).
- Of 76 children, 46% presented with CS-plus symptoms.
- Presence of CS-plus symptoms (p=0.002) and structural abnormalities (p<0.001) were significantly associated with poor outcomes, confirmed by multivariate analysis (p=0.021 and p=0.002, respectively).
Conclusions:
- Most childhood AA cases have benign etiologies like intoxication or post-infectious ataxia with favorable outcomes.
- However, AA can be an initial sign of CNS neoplasms or paraneoplastic syndromes.
- Key
- red flags
- for poor prognosis include limb/facial palsy, hypotonia, altered consciousness (GCS < 15), vomiting, opsoclonus, headache, myoclonus, visual impairment, torticollis, and vertigo.
Abstract:
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.
