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VOLUME 22 , ISSUE 7 ( 2018 ) > List of Articles


Acute leukoencephalopathy with restricted diffusion

Mahesh Kamate

Keywords : Acute encephalopathy, acute encephalopathy with biphasic seizures and restricted diffusion, magnetic resonance imaging brain Acute Leukoencephalopathy with Restricted Diffusion

Citation Information : Kamate M. Acute leukoencephalopathy with restricted diffusion. Indian J Crit Care Med 2018; 22 (7):519-523.

DOI: 10.4103/ijccm.IJCCM_139_18

License: CC BY-ND 3.0

Published Online: 01-03-2015

Copyright Statement:  Copyright © 2018; The Author(s).


Treatment and outcome of children with acute encephalopathy depend on the cause, prompt treatment of the underlying cause, and use of adequate supportive measures. Many novel causes of acute encephalopathy are emerging where lumbar puncture, computed tomography of the head, and routine biochemical testing can be normal such as acute disseminated encephalomyelitis and febrile infection-related refractory epilepsy syndrome. Magnetic resonance imaging (MRI) plays an important role in the workup of children with acute leukoencephalopathy. Despite this in few cases, a correct diagnosis is not possible and novel conditions have been described in the last decade. One such condition is acute encephalopathy with biphasic seizures and restricted diffusion also called as acute leukoencephalopathy with restricted diffusion. Here, the routine MRI sequences such as T1, T2, and fluid-attenuated inversion recovery sequences can be normal. Here, we have reviewed the etiology, types, clinicoradiological features, and treatment of this condition.

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  1. Okumura A, Kidokoro H, Tsuji T, Suzuki M, Kubota T, Kato 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;30:825-30.
  2. Mizuguchi M, Abe J, Mikkaichi K, Noma S, Yoshida K, Yamanaka T, et al. Acute necrotising encephalopathy of childhood: A new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry 1995;58:555-61.
  3. Tada H, Takanashi J, Barkovich AJ, Oba H, Maeda M, Tsukahara H, et al. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion. Neurology 2004;63:1854-8.
  4. Takanashi J, Oba H, Barkovich AJ, Tada H, Tanabe Y, Yamanouchi H, et al. Diffusion MRI abnormalities after prolonged febrile seizures with encephalopathy. Neurology 2006;66:1304-9.
  5. Singh V, Tomar V, Kumar A, Phadke RV. Acute leucoencephalopathy with restriction of diffusion – A case report. Eastern J Med 2012;17:149-52.
  6. Ito Y, Natsume J, Kidokoro H, Ishihara N, Azuma Y, Tsuji T, et al. Seizure characteristics of epilepsy in childhood after acute encephalopathy with biphasic seizures and late reduced diffusion. Epilepsia 2015;56:1286-93.
  7. Yamaguchi H, Tanaka T, Maruyama A, Nagase H. Septic encephalopathy characterized by acute encephalopathy with biphasic seizures and late reduced diffusion and early nonconvulsive status epilepticus. Case Rep Neurol Med 2016;2016:7528238.
  8. Tadokoro R, Okumura A, Nakazawa T, Hara S, Yamakawa Y, Kamata A, et al. Acute encephalopathy with biphasic seizures and late reduced diffusion associated with hemophagocytic syndrome. Brain Dev 2010;32:477-81.
  9. Takanashi J, Tada H, Terada H, Barkovich AJ. Excitotoxicity in acute encephalopathy with biphasic seizures and late reduced diffusion. AJNR Am J Neuroradiol 2009;30:132-5.
  10. Saitoh M, Shinohara M, Hoshino H, Kubota M, Amemiya K, Takanashi JL, et al. Mutations of the SCN1A gene in acute encephalopathy. Epilepsia 2012;53:558-64.
  11. Saitoh M, Shinohara M, Ishii A, Ihara Y, Hirose S, Shiomi M, et al. Clinical and genetic features of acute encephalopathy in children taking theophylline. Brain Dev 2015;37:463-70.
  12. Rivas-Coppola MS, Shah N, Choudhri AF, Morgan R, Wheless JW. Chronological evolution of magnetic resonance imaging findings in children with febrile infection-related epilepsy syndrome. Pediatr Neurol 2016;55:22-9.
  13. Kelley BP, Patel SC, Marin HL, Corrigan JJ, Mitsias PD, Griffith B, et al. Autoimmune encephalitis: Pathophysiology and imaging review of an overlooked diagnosis. AJNR Am J Neuroradiol 2017;38:1070-8.
  14. Matsuo M, Maeda T, Ono N, Sugihara S, Kobayashi I, Koga D, et al. Efficacy of dextromethorphan and cyclosporine a for acute encephalopathy. Pediatr Neurol 2013;48:200-5.
  15. Nakazawa M, Akasaka M, Hasegawa T, Suzuki T, Shima T, Takanashi J, et al. Efficacy and safety of fosphenytoin for acute encephalopathy in children. Brain Dev 2015;37:418-22.
  16. Yamanouchi H, Kawaguchi N, Mori M, Imataka G, Yamagata T, Hashimoto T, et al. Acute infantile encephalopathy predominantly affecting the frontal lobes. Pediatr Neurol 2006;34:93-100.
  17. Maegaki Y, Kondo A, Okamoto R, Inoue T, Konishi K, Hayashi A, et al. Clinical characteristics of acute encephalopathy of obscure origin: A biphasic clinical course is a common feature. Neuropediatrics 2006;37:269-77.
  18. Freeman JL, Coleman LT, Smith LJ, Shield LK. Hemiconvulsion-hemiplegia-epilepsy syndrome: Characteristic early magnetic resonance imaging findings. J Child Neurol 2002;17:10-6.
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