Acute encephalopathy with bilateral striatal necrosis: favourable response to corticosteroid therapy

Eur J Paediatr Neurol. 1997;1(1):41-5. doi: 10.1016/s1090-3798(97)80010-4.


A case of acute encephalopathy with selective bilateral symmetrical striatal lesions is reported. The patient was a previously healthy 4-year-old boy who became obtunded after a febrile illness and fell into a state of delirium with severe pain in the feet. He showed abnormal postures: hyperextension of the neck and upper limbs and extreme flexion of both lower limbs, and abnormal involuntary movements of the limbs: tremor, athetotic movement and right hemiballismus. Analysis of serum antibody titres suggested recent primary infection of herpes simplex type 1 (HSV-1). Cranial T2-weighted magnetic resonance imaging (MRI) demonstrated areas of high-signal intensity involving the whole basal ganglia bilaterally. He showed rapid clinical improvement after the initiation of corticosteroid therapy; complete clinical recovery was noted 3 months after the onset. Serial MRI studies demonstrated a rapid reduction of the lesions, resulting in only slight T2-hyperintense areas in both caudate nuclei. The pathogenesis of the disorder remains unknown, though an autoimmune mechanism has been speculated. The clinical and laboratory findings in this case suggested a possible role of HSV-1 in the pathomechanism of the disorder and a beneficial effect of early corticosteroid therapy.

Publication types

  • Case Reports

MeSH terms

  • Acute Disease
  • Basal Ganglia Diseases / diagnosis
  • Basal Ganglia Diseases / drug therapy*
  • Child, Preschool
  • Corpus Striatum / drug effects*
  • Corpus Striatum / pathology
  • Dominance, Cerebral* / drug effects
  • Encephalitis, Viral / diagnosis
  • Encephalitis, Viral / drug therapy*
  • Glucocorticoids / administration & dosage*
  • Herpes Simplex / diagnosis
  • Herpes Simplex / drug therapy*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Necrosis
  • Prednisolone / administration & dosage*


  • Glucocorticoids
  • Prednisolone