Symmetrical basal ganglia involvement in pediatric neuropsychiatric lupus: MRI findings in a rare presentation
TL;DR
An adolescent girl with a recent diagnosis of systemic lupus erythematosus who presented with acute neuropsychiatric deterioration, including altered mental status and seizures is reported, highlighting the importance of including NPSLE in the differential diagnosis of symmetrical basal ganglia lesions in children, particularly when systemic autoimmune features are present.
Abstract
Bilateral basal ganglia involvement is an uncommon but treatable manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE) that is rarely described in pediatric patients and is frequently omitted from the standard differential diagnosis for bilateral basal ganglia lesions in childhood. We report an adolescent girl with a recent diagnosis of systemic lupus erythematosus who presented with acute neuropsychiatric deterioration, including altered mental status and seizures. Brain magnetic resonance imaging (MRI) demonstrated symmetrical T2-weighted/fluid-attenuated inversion recovery (T2/FLAIR) hyperintensity of the bilateral anterior basal ganglia without diffusion restriction, consistent with vasogenic edema. Concomitant findings included a left cerebellar subacute cortical infarct and diffuse leptomeningeal enhancement, a constellation postulated to reflect diffuse systemic lupus erythematosus-related endothelial dysfunction and blood–brain barrier disruption. Infectious and metabolic etiologies were excluded. The patient was managed with pulse methylprednisolone and azathioprine without significant improvement. Subsequent initiation of plasmapheresis resulted in clinical improvement, supporting a humorally mediated mechanism of injury. This case highlights the importance of including NPSLE in the differential diagnosis of symmetrical basal ganglia lesions in children, particularly when systemic autoimmune features are present, to ensure timely escalation of therapy.