18th International Child Neurology Congress, Cape-Town, Güney Afrika, 6 - 10 Mayıs 2024, ss.1
Introduction: Myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune disorder characterized by inflammatory demyelination of the central nervous system. Despite its broad clinical spectrum, the occurrence of thalamic tremor in patients with MOGAD has not been previously reported in the literature. In this report, we aim to present a child diagnosed with MOGAD who developed thalamic tremor during the course of the disease. Case Report: A three-year-old boy was admitted to our pediatric emergency unit due to febrile status epilepticus. Following clinical stabilization, cerebrospinal fluid (CSF) analysis, initially performed with a suspected diagnosis of meningoencephalitis, showed normal findings. Magnetic resonance imaging (MRI) revealed hyperintense multifocal lesions on T2/FLAIR sequences consistent with demyelinating diseases involving the bilateral basal ganglia, cortical and subcortical regions, and left thalamus. Electroencephalography demonstrated diffuse slow background activity. The serum anti-MOG titer was positive, 1:1000. Subsequently, the patient received a diagnosis of MOGAD and underwent treatment with intravenous pulse steroid and then intravenous immunoglobulins. He exhibited worsening of his encephalopathy with rigidity and muscle weakness in his extremities. Additionally, a slow and large-amplitude tremor manifested in the right arm, potentially attributed to thalamic involvement. After the completion of his treatment regimen, he was discharged with maintained oral steroid therapy and physiotherapy. Conclusion: Thalamic tremor, also known as Holmes tremor, is characterized by a low-frequency and high-amplitude tremor, particularly with proximal predominance. Notably, MOGAD has not previously been reported as an underlying cause of thalamic tremor and can be considered an exceedingly rare etiology in such cases.