NEUROIMAGING FINDINGS IN RASMUSSENS-SYNDROME

Rasmussen's syndrome is a progressive childhood disease of unknown cau se characterized by severe epilepsy hemiparesis, mental deterioration, inflammation of one cerebral hemisphere, and brain atrophy. Computed tomography, single-photon emission computed tomography (SPECT), and ma gnetic resonance (MR) neuroimaging findings of 8 patients with patholo gically confirmed Rasmussen's syndrome were evaluated retrospectively. All patients showed a predominance of the atrophy in the temporo-insu lar region and cerebral hemispheric alterations on MR images in a simi lar extension as seen on SPECT studies. Focal increase in regional cer ebral blood flow was observed in the 4 patients presenting with epilep sia partialis continua at the time of hexamethylpropyleneamineoxime in jection. Extensive cortical hypoperfusion was noted in the other 4 pat ients who received the injection during the interictal state. Cerebell ar functional abnormalities were present in 6 patients, 2 of them with structural damage.