Acute encephalopathy and brain abnormalities on magnetic resonance imaging during combination therapy with adrenocorticotropic hormone and vigabatrin for infantile spasms

Case presentation: A 11-month-old female patient with Trisomy 21 (Down Syndrome) who developed infantile spasms at 6-months-old. She was diagnosed West Syndrome and first treated Vigabatrin (VGB). positive response observed, control of the spams regression hypsarrhythmia electroencephalogram register. well controlled until 10-months-old, when returned developmental regression. treatment synthetic adrenocorticotropic hormone (ACTH) started in a low dose, but partial spasms. It decided to increase dose ACTH we observed recovery milestones. An oral corticosteroid withdrawal maintained. evolved drowsiness, diarrhea, tachycardia, hypotension abnormal movements, characterized by sudden limb movements (ballismus) chorea on face. for sepsis initiated, improvement laboratory parameters hypotension, she persisted encephalopathy, paroxysmal tachycardia diarrhea. cranial tomography (CT) performed, showing symmetrical bilateral image hypoattenuation basal nuclei. All clinical abnormalities stopped after withdrawing VGB. Magnetic Resonance Imaging (MRI) findings showed T2/FLAIR hypersignal nuclei diffusion restriction.