Clinical and Brain Imaging Findings of Children with Acute Ataxia: Ten Years of Experience at a Major Pediatric Referral Center

Abstract

Background: Ataxia is a symptom of a wide range of disorders, which manifests as a lack of coordinated movements. It is commonly associated with cerebellar disorders. Objectives: To evaluate the neuroimaging findings of children with acute ataxia (AA) and to identify the association between these findings and clinical results. Patients and Methods: This cross-sectional study was conducted using the electronic medical records of patients, presenting to a major pediatric referral center over 10 years from March 2009 to February 2020. Patients were included in the study if they were younger than 18 years, were diagnosed with AA (< 30 days), and underwent magnetic resonance imaging (MRI), computed tomography (CT) scan, or both. The exclusion criteria were as follows: A history of neurological or medical disorders, explaining ataxia; pseudo-ataxia; a traumatic brain injury; severe loss of consciousness; and missing key information in the medical records (e.g., no brain imaging findings). Patient data, including the demographic data, clinical history, laboratory findings, imaging results, and in-hospital outcomes, were collected. A P-value less than 0.05 was considered statistically significant, and the confidence interval was set at 95%. Results: A total of 119 patients were included in this study (51% female; mean age, 4.9 years). Clinically urgent neurological pathology (CUNP) was detected in 37 (31.09%) patients. The most common pathologies on images were tumors, acute disseminated encephalomyelitis (ADEM), and stroke. However, the most common diagnosis was acute post-infectious cerebellar ataxia (APCA; 24.4%), followed by brain tumors (16.8%) and Guillain-Barre syndrome (GBS; 15.1%), respectively. Overall, 11 variables were significantly different between patients with and without CUNP. Most notably, the duration of symptoms (P < 0.01), ophthalmoplegia (OR = 13.93; 95% CI: 3.5 - 54.7), focal neurologic deficit (OR = 7.26; 95% CI: 2.6 - 20.5), and fever (OR = 3.33; 95% CI: 1.1 - 9.8) were associated with a higher risk of CUNP. On the other hand, some features, such as a recent history of febrile illness (presence of fever or body temperature above 38°C in the last month) (OR = 0.36; 95% CI: 0.16 - 0.8) and hyporeflexia (P < 0.01), were associated with a lower risk of CUNP. Conclusion: Acute post-infectious cerebellar ataxia (APCA) was the most common diagnosis of AA in children, undergoing neuroimaging studies in our center. Nevertheless, tumor was the most common pathology detected on the images of patients with CUNP. Some pathologies might not be detected by some imaging modalities. Considering the associations identified in this study, patients with risk factors, such as a longer duration of symptoms, ophthalmoplegia, focal neurologic deficit, and fever, need to undergo another imaging modality with higher sensitivity if there are no positive findings in the initial imaging study. On the other hand, patients with protective factors, such as hyporeflexia and a recent history of febrile illness, could benefit more from other diagnostic modalities.