Reversible parainfectious bilateral "striatal necrosis"

Pediatr Neurol. 2012 Jan;46(1):51-3. doi: 10.1016/j.pediatrneurol.2011.10.003.

Abstract

Bilateral striatal necrosis is usually associated with either endogenous or exogenous toxins, and with poor neurodevelopmental outcomes. We describe two patients with acute bilateral striatal clinical syndrome and magnetic resonance signal changes who made a complete clinical and radiologic recovery within 3 months. After an uneventful pregnancy, normal birth, and normal development, both boys presented at ages 3 and 5 years, respectively, after a viral illness with slurring of speech, bradykinesia, and an extrapyramidal movement disorder. On examination, both manifested bilateral cog wheel rigidity, with a broad-based gait and flexor plantar response. Cranial magnetic resonance imaging in both children indicated bilateral, symmetric, high signal changes in the lentiform nucleus, predominately in the putamen, with sparing of the globus pallidi bilaterally. The brain parenchyma was otherwise normal. Neurometabolic investigations produced normal results in both patients. The pathogenesis is uncertain, but could be immune-mediated. Both children, at 3-year and 1-year follow-ups, respectively, are doing well neurologically and academically. Our patients demonstrate that abnormal imaging findings during acute stages do not preclude good clinical and radiologic recovery.

Publication types

  • Case Reports

MeSH terms

  • Aspartic Acid / analogs & derivatives
  • Aspartic Acid / metabolism
  • Child, Preschool
  • Corpus Striatum / pathology*
  • Corpus Striatum / physiopathology*
  • Encephalomyelitis / physiopathology*
  • Female
  • Functional Laterality*
  • Humans
  • Longitudinal Studies
  • Magnetic Resonance Imaging
  • Magnetic Resonance Spectroscopy
  • Male
  • Necrosis / pathology
  • Pregnancy
  • Pregnancy Complications / pathology

Substances

  • Aspartic Acid
  • N-acetylaspartate