Concurrent thalamic and cerebellar involvement in pediatric neuropsychiatric lupus: Expanding the radiologic spectrum

Abstract

Background

Neuropsychiatric systemic lupus erythematosus (NP-SLE) in children is uncommon but often severe, with a wide spectrum of neurologic presentations and sometimes atypical or initially normal neuroimaging. Among reported manifestations, acute cerebellitis is rare, and simultaneous thalamic involvement has scarcely been described. Differentiating inflammatory NP-SLE from mimics such as posterior reversible encephalopathy syndrome (PRES), infection, or ischemia is essential, since management strategies and prognosis differ substantially.

Case

We describe a 13-year-old female with newly diagnosed SLE who developed acute cerebellitis accompanied by bilateral medial thalamic signal abnormalities and non-convulsive status epilepticus after extensive infectious and cerebrospinal fluid evaluation. MRI demonstrated cerebellar cortical diffusion restriction and faint bilateral thalamic T2-FLAIR/DWI hyperintensity without corresponding ADC reduction, consistent with vasogenic edema, and without evidence of vascular occlusion. Management required high-dose corticosteroids, antiseizure therapy, plasmapheresis, cyclophosphamide, and rituximab. Her course was complicated by cytopenias, renal involvement, and encephalopathy, but she achieved gradual neurologic recovery with rehabilitation therapies and hydroxychloroquine maintenance.

Conclusions

Simultaneous cerebellar and thalamic involvement expands the radiologic spectrum of pediatric NP-SLE and highlights thalamocerebellar network vulnerability underlying impaired consciousness. Recognition of this pattern supports early advanced MRI, continuous EEG monitoring, and prompt, multimodal immunotherapy to optimize outcomes.