Abstract
Background
Acute necrotizing encephalopathy (ANE) is a rare disease characterized by multiple, symmetrical brain lesions, affecting thalami, brainstem tegmentum, and cerebellar medulla; more inconstantly, other structures are involved, i.e., internal capsules, posterolateral putamen, and deep periventricular white matter.
Features
The clinical picture consists of rapidly deteriorating acute monophasic encephalopathy preceded by prodromal febrile illness; the symptoms include hyperpyrexia, convulsions, recurrent vomiting, and coma within 24 h.
Prognosis
The outcome is usually poor and approximately 70% of the patients die within a few days from the onset of fever. There is no specific therapy for ANE but, in some patients, the clinical status improved with steroid treatment.
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References
Albayram S, Bilgi Z, Selcuc D, Cam H, Koçer N, Islak C (2004) Diffusion-weighted MRI findings of acute necrotizing encephalopathy. Am J Neuroradiol 25:792–797
Ashtekar CS, Jaspan T, Thomas D, Weston V, Gayatri NA, Whitehouse WP (2003) Acute bilateral thalamic necrosis in a child with Mycoplasma pneumoniae. Dev Med Child Neurol 45:634–637
Campistol J, Gassio R, Pineda M, Fernandez Alvarez E (1998) Acute necrotizing encephalopathy of childhood (infantile bilateral thalamic necrosis): two non-Japanese cases. Dev Med Child Neurol 40:771–774
De Vivo DC (1998) Leigh syndrome: historical perspective and clinical variations. Biofactors 7:269–271
Donnet A, Dufour H, Gambarelli D, Bruder N, Pellisser JF, Grisoli F (1996) Acute Weston-Hurst necrotizing hemorrhagic leukoencephalitis. Rev Neurol (Paris) 152:748–751
Goo HW, Choi CG, Yoon CH, Ko TS (2003) Acute necrotizing encephalopathy: diffusion MR imaging and localized proton MR spectroscopic findings in two infants. Korean J Radiol 4:61–65
Harada M, Hisaoka S, Mori K, Yoneda K, Noda S, Nishitani H (2000) Differences in water diffusion and lactate production in two different types of postinfectious encephalopathy. J Magn Reson Imaging 11:559–563
Huang SM, Chen CC, Chiu PC, Cheng MF, Lai PH, Hsieh KS (2004) Acute necrotizing encephalopathy of childhood associated with influenza type B virus infection in a 3-year-old girl. J Child Neurol 19:64–67
Hurst EW (1941) Acute haemorrhagic leuco-encephalitis: a previously undefined entity. Med J Aust 2:1–16
Kim JH, Kim IO, Lim MK, Park MS, Choi CG, Kim HW, Kim JE, Choi SJ, Koh YH, Yang DM, Choo SW, Chung MJ, Yoon HK, Goo HW, Lee M (2004) Acute necrotizing encephalopathy in Korean infants and children: imaging findings and diverse clinical outcome. Korean J Radiol 5:171–177
Kumada S, Kusaka H, Okaniwa M, Kobayashi O, Kusunoki S (1997) Encephalomyelitis subsequent to mycoplasma infection with elevated serum anti-Gal C antibodies. Pediatr Neurol 16:241–244
Mastroyianni SD, Voudris KA, Katsarou E, Gionnis D, Mavromatis P, Vagiakou EA, Skardoutson A (2003) Acute necrotizing encephalopathy associated with parainfluenza virus in a Caucasian child. J Child Neurol 18:570–572
Matsushita E, Takita K, Shimada A (1997) Suspected acute encephalopathy with symmetrical abnormal signal areas in the basal ganglia, thalamus, midbrain and pons diagnosed by magnetic resonance imaging. Acta Paediatr Jpn 39:454–458
Mizuguchi M (1997) Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev 19:81–92
Mizuguchi M, Abe J, Mikkaichi K, Noma S, Yoshida K, Yamanaka T, Kamoshita S (1995) Acute necrotizing encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry 58:555–561
Mizuguchi M, Hayashi M, Nakano I, Kuwashima M, Yoshida K, Nakai Y, Itoh M, Takashima S (2002) Concentric structure of thalamic lesions in acute necrotizing encephalopathy. Neuroradiology 44:489–493
Munakata M, Kato R, Yokoyama H, Haginoya K, Tanaka Y, Kayaba J, Kato T, Takayanagi R, Endo H, Hasegawa R, Ejima Y, Iinuma K (2002) Combined therapy with hypothermia and anticytokine agents in influenza A encephalopathy. Brain Dev 22:373–377
Neilson DE, Eiben RM, Waniewski S, Hoppel CL, Varnes ME, Bangert BA, Wiznitzer M, Warman ML, Kerr DS (2003) Autosomal dominant acute necrotizing encephalopathy. Neurology 61:226–230
Porto L, Lanferman H, Moller-Hartmann W, Jacobi G, Zanella F (1999) Acute necrotizing encephalopathy of childhood after exanthema subitum outside Japan or Taiwan. Neuroradiology 41:732–734
Ravid S, Topper L, Eviatar L (2001) Acute necrotizing encephalopathy presenting as a basal ganglia syndrome. J Child Neurol 16:461–462
Sazgar M, Robinson JL, Chan AKJ, Sinclair DB (2003) Influenza B acute necrotizing encephalopathy: a case report and literature review. Pediatr Neurol 28:396–399
Wang HS (1995) Acute necrotizing encephalopathy of childhood presenting with multifocal, symmetric lesions occurring outside Japan. J Neurol Neurosurg Psychiatry 59:661
Weitkamp JH, Spring MD, Brogan T, Moses H, Bloch KC, Wright PF (2004) Influenza A virus-associated acute necrotizing encephalopathy in the United States. Pediatr Infect Dis J 23:259–263
Yagishita A, Nakano I, Ushioda T, Otsuki N, Hasegawa A (1995) Acute encephalopathy with bilateral thalamo tegmental involvement in infants and children: imaging and pathology findings. AJNR Am J Neuroradiol 16:439–447
Yoshikawa H, Watanabe T, Abe T, Oda Y (1999) Clinical diversity in acute necrotizing encephalopathy. J Child Neurol 14:249–255
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Manara, R., Franzoi, M., Cogo, P. et al. Acute necrotizing encephalopathy: combined therapy and favorable outcome in a new case. Childs Nerv Syst 22, 1231–1236 (2006). https://doi.org/10.1007/s00381-006-0076-9
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DOI: https://doi.org/10.1007/s00381-006-0076-9