Abstract
A previously healthy 24-day-old boy presented with a 2-day history of fever and had a convulsion on the day of admission. MRI showed abnormal signal in the thalami, caudate nuclei and central white matter. Acute necrotising encephalopathy was diagnosed, other causes having been excluded after biochemical and haematological analysis of blood, urine and CSF. He recovered, but with spastic quadriparesis. At the age of 28 months, he suffered sudden deterioration of consciousness and motor weakness of his right limbs. MRI was consistent with an acute cerebrovascular accident. Angiography showed bilateral middle cerebral artery stenosis or frank occlusion with numerous lenticulostriate collateral vessels consistent with moyamoya disease.
Similar content being viewed by others
References
Mizuguchi M, Abe J, Mikkaichi K, et al (1995) Acute necrotizing encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry 58:555–561
Yoshikawa H, Watanabe T, Abe T, et al (1999) Clinical diversity in acute necrotizing encephalopathy. J Child Neurol 14:249–255
Nagal T, Yagishita A, Tsuchiya Y, et al (1993) Symmetrical thalamic lesions on CT in influenza A virus infection presenting with or without Reye syndrome. Brain Dev 15:67–73
Oki J, Tokumitsu A, Takahashi S, et al (1995) Serial neuroimages of acute necrotizing encephalopathy associated with human herpesvirus 6 infection. Brain Dev 17:356–359
Porto L, Lanfermann H, Moller-Hartmann W, et al (1999) Acute necrotizing encephalopathy of childhood after exanthema subitum outside Japan or Taiwan. Neuroradiology 41:732–734
Mizuguchi M, Hayashi M, Nakano I, et al (2002) Concentric structure of thalamic lesions in acute necrotizing encephalopathy. Neuroradiology 44:489–493
Nakano I, Otsuki A, Hasegawa A (1993) Acute stage neuropathology of a case if infantile acute encephalopathy with thalamic involvement: widespread symmetrical fresh necrosis of the brain. Neuropathology 13:315–325
Yagishita A, Nakano I, Ushioda T, et al (1995) Acute encephalopathy with bilateral thalamotegmental involvement in infants and children: imaging and pathologic findings. AJNR 16:439–447
Kim YJ, Chun KA, Kim KT, et al (2001) MR imaging of acute necrotizing encephalopathy: a report of two cases (in Korean). J Korean Radio Soc 44:633
Brismar J, Aqeel A, Brismar G, et al (1990) Maple syrup urine disease: findings on CT and MR scans of the brain in 10 infants. AJNR 11:1219–1228
Duchen LW, Jacobs JM (1992) Nutritional deficiencies and metabolic disorders. In: Adams JH, Duchen LW (eds) Greenfield's neuropathology, 5th edn. Oxford University Press, New York, pp 813–817, 846–847
Debrun G, Sauvegrain J, Aicardi J, et al (1975) Moyamoya, a nonspecific radiological syndrome, Neuroradiology 8:241–244
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kim, TK., Eun, BL., Cha, S.H. et al. Moyamoya disease in a child with previous acute necrotizing encephalopathy. Pediatr Radiol 33, 644–647 (2003). https://doi.org/10.1007/s00247-003-0955-0
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-003-0955-0