Neuroimaging findings in Rasmussen's syndrome

J Neuroimaging. 1997 Jan;7(1):16-22. doi: 10.1111/jon19977116.


Rasmussen's syndrome is a progressive childhood disease of unknown cause characterized by severe epilepsy, hemiparesis, mental deterioration, inflammation of one cerebral hemisphere, and brain atrophy. Computed tomography, single-photon emission computed tomography (SPECT), and magnetic resonance (MR) neuroimaging findings of 8 patients with pathologically confirmed Rasmussen's syndrome were evaluated retrospectively. All patients showed a predominance of the atrophy in the temporoinsular region and cerebral hemispheric alterations on MR images in a similar extension as seen on SPECT studies. Focal increase in regional cerebral blood flow was observed in the 4 patients presenting with epilepsia partialis continua at the time of hexamethylpropyleneamineoxime injection. Extensive cortical hypoperfusion was noted in the other 4 patients who received the injection during the interictal state. Cerebellar functional abnormalities were present in 6 patients, 2 of them with structural damage.

MeSH terms

  • Adolescent
  • Adult
  • Atrophy
  • Brain / pathology*
  • Brain Diseases / diagnosis*
  • Cerebral Cortex / pathology
  • Child
  • Child, Preschool
  • Dominance, Cerebral / physiology
  • Encephalitis / diagnosis
  • Epilepsies, Partial / diagnosis
  • Female
  • Hemiplegia / diagnosis
  • Humans
  • Intellectual Disability / diagnosis
  • Magnetic Resonance Imaging*
  • Male
  • Syndrome
  • Tomography, Emission-Computed, Single-Photon*
  • Tomography, X-Ray Computed*