Acute cerebellar ataxia in a patient with maple syrup urine disease: a case report

Introduction: Maple syrup urine disease (MSUD) is an inborn error of amino acid metabolism, potentially resulting in metabolic intoxication crises accompanied by neurological manifestations. Case report: This article reports a case involving a six-year-old boy with MSUD who developed a post-infectious neurological disorder following COVID-19. Initially presenting with fever, cough, and ataxia, he was diagnosed with a metabolic crisis due to COVID-19. Despite an initial recovery, the patient experienced a relapse characterized by ataxia, tremor, and dysarthria, necessitating readmission. A subsequent investigation ruled out an MSUD crisis. A lumbar puncture and MRI failed to rule out a demyelinating acute disorder. Methylprednisolone treatment yielded no improvement, prompting the initiation of intravenous immunoglobulin, which led to improvement after 14 days. Discussion: The case posed challenges due to the overlapping symptoms between MSUD crises and post-infectious neurological conditions. This highlights the complexity of managing these patients, particularly due to the potential for more vigorous manifestations and slower recovery.