Clinical Characteristics and Effects of Steroid Therapy in Children with Acute Cerebellar Ataxia

Abstract

Purpose Acute cerebellar ataxia (ACA) is characterized by unsteady gait and instability of the trunk, and is caused by secondary autoimmune responses to infection or vaccination in healthy children. Although its prognosis is usually very good, full symptom recovery generally takes 2 to 3 months. This study aimed to investigate clinical symptoms, neuroimaging findings, and laboratory findings in children with ACA, and to evaluate the effects of steroid therapy on ACA according to the method of administration (intravenous methylprednisolone vs. oral prednisolone). Methods We retrospectively analyzed nine patients diagnosed with ACA or acute cerebellitis (AC) who received steroid therapy. Results Nine children were included in this study (mean age, 3.71±2.89 years). The mean duration between prodromal febrile illness and cerebellar symptoms was 9.63±4.66 days. Ataxia (limb and/or truncal) was the most common cerebellar sign. Steroids were administered in two ways: methylprednisolone (20 to 30 mg/kg/day) was changed to an oral steroid (prednisolone, 1 mg/kg/day) after 2 to 3 days of administration; an oral steroid was used from the beginning of treatment. The cerebellar symptoms began to improve within 2 to 4 days of steroid therapy. All patients fully recovered without sequelae. The mean interval until full recovery of the cerebellar symptoms was 28.0±19.3 days, and was not significantly different between patients who received an oral steroid after methylprednisolone pulse therapy and patients who only received an oral steroid (P>0.05). Conclusion Regardless of the method of drug administration, steroid therapy helps to improve cerebellar symptoms in children with ACA/AC.

Keywords

• cerebellar ataxia
• cerebellar diseases
• steroids