Acute leukoencephalopathy with restricted diffusion – Case Series

Abstract

Acute leukoencephalopathy with restricted diffusion (ALERD) is a clinicopathological diagnosis that is characterized by severe encephalopathy and seizures with extensive areas of restricted diffusion in cerebral parenchyma. Case 1: Apparently healthy 13-month-old male child presented with acute febrile encephalopathy with a flurry of seizures with raised intracranial pressure. Investigations were consistent with multiorgan dysfunction along with severe metabolic acidosis, ketosis, and hyperammonemia. Initial magnetic resonance imaging (MRI) Brain was normal. However subsequent MRI brain suggested features of diffuse ALERD. With immunotherapy and supportive management, he improved and was discharged without sequelae. Case 2: A 9-month-old male child who was premorbidly normal presented with fever, status epilepticus, and encephalopathy. MRI Brain was suggestive of central sparing ALERD. He was discharged with sequelae with the advice of rehabilitation. Unfortunately, he got succumbed due to aspiration. Case 3: A 10-day-old neonate presented with the refusal of feeds, multiple seizures, and encephalopathy. MRI Brain revealed central sparing ALERD. Improved with immunotherapy and was developmentally normal at five months of age. ALERD is a clinic-radiological syndrome characterized by acute encephalopathy with restricted areas of diffusion in subcortical white matter on MRI. Reported two categories are Diffuse and Central sparing ALERD. Diffuse ALERD has severe manifestations. However, Case 1 had a good outcome, suggesting a variable prognosis. Central sparing ALERD is a milder form, but when basal ganglia are involved, it may have a worsened outcome, as seen in Case 2. Case 3 had central sparing ALERD, and had a good prognosis as described in literature. This study highlights the varied signs and symptoms of ALERD, including neonatal age of onset. Diagnosis is based on the restricted diffusion in white matter on diffusion-weighted imaging and the apparent diffusion coefficient in MRI of the brain. MRI may be normal in the initial course; hence, it’s important to repeat if strongly suspected. Prompt immunotherapy and supportive care are emphasised.