Radiological Insights into Acute Leukoencephalopathy with Restricted Diffusion: A Case Series Analysis

Abstract

Objectives The study aimed to investigate the clinical and radiological characteristics of acute leukoencephalopathy with restricted diffusion (ALERD) among pediatric patients presenting with acute encephalopathy, seizures, and fever.

Materials and Methods A retrospective analysis was conducted on 48 pediatric patients who underwent magnetic resonance imaging (MRI) between April 2022 and August 2023 at Bapuji Hospital, J.J.M. Medical College, Davanagere, Karnataka, India. Inclusion criteria comprised acute-onset encephalopathy, fever, seizures, and MRI evidence of acute subcortical white matter involvement. Clinical and radiological data were collected and analyzed to delineate ALERD characteristics.

Results Among the study cohort, 18.7% of cases met the diagnostic criteria for ALERD, predominantly affecting male children with a mean age of 32 months. Clinical presentations varied, including biphasic and monophasic courses. Two distinct patterns of brain involvement, diffuse and central-sparing, were identified. Laboratory abnormalities and differential diagnoses were elucidated, providing insights into distinguishing ALERD from other conditions. MRI, particularly diffusion-weighted imaging, emerged as a crucial tool for ALERD diagnosis, revealing characteristic restricted diffusion patterns.

Conclusion The study underscores the importance of recognizing ALERD as a distinct clinical-radiological syndrome associated with infections, aiding in timely diagnosis and management. Understanding the varied clinical presentations and radiological patterns enhances diagnostic accuracy, emphasizing the role of MRI in ALERD diagnosis. These findings contribute to a comprehensive understanding of ALERD, facilitating improved patient outcomes and prognosis.

Keywords

acute leukoencephalopathy with restricted diffusion (ALERD) - diffusion-weighted imaging (DWI) - apparent diffusion coefficient (ADC) - acute encephalopathy - acute encephalopathy with biphasic seizures and late reduced diffusion (AESD)

Publication History

Article published online:
25 September 2024

• References

• 1 Kamate M. Acute leukoencephalopathy with restricted diffusion. Indian J Crit Care Med 2018; 22 (07) 519-523
• 2 Ranjan RS, Arya G, Yadav VK. Acute encephalopathy with biphasic seizures and late reduced diffusion (central sparing type)-MRI and MR spectroscopy findings. Indian J Radiol Imaging 2019; 29 (04) 426-430
• 3 Lawrence RM, Aripirala P, Reddy N. et al. The spectrum of acute leukoencephalopathy with restricted diffusion (ALERD): a case series and review of literature. Eur J Paediatr Neurol 2021; 33: 86-93
• 4 Singh V, Tomar V, Kumar A, Phadke RV. Acute leucoencephalopathy with restriction of diffusion - a case report. East J Med 2012; 17: 149-152
• 5 Tadokoro R, Okumura A, Nakazawa T. et al. Acute encephalopathy with biphasic seizures and late reduced diffusion associated with hemophagocytic syndrome. Brain Dev 2010; 32 (06) 477-481
• 6 Schaefer PW, Grant PE, Gilberto Gonzalez R. Diffusion-weighted MR imaging of the brain. Radiology 2000; 217 (02) 331-345
• 7 Takanashi J, Oba H, Barkovich AJ. et al. Diffusion MRI abnormalities after prolonged febrile seizures with encephalopathy. Neurology 2006; 66 (09) 1304-1309 , discussion 1291
• 8 Okumura A, Kidokoro H, Tsuji T. et al. Differences of clinical manifestations according to the patterns of brain lesions in acute encephalopathy with reduced diffusion in the bilateral hemispheres. AJNR Am J Neuroradiol 2009; 30 (04) 825-830
• 9 Tada H, Takanashi JI, Terada H, Tajima K. Severe form of acute influenza encephalopathy with biphasic seizures and late reduced diffusion. Neuropediatrics 2008; 39 (02) 134-136
• 10 Maeda M, Okanishi T, Miyamoto Y. et al. Predicting the onset of acute encephalopathy with biphasic seizures and late reduced diffusion by using early laboratory data. Front Neurol 2021; 12: 730535