Neuroimaging Findings in Rasmussen's Syndrome

• Published in: Journal of neuroimaging 1997-01, Vol.7 (1), p.16-22
• Main Authors: Yacubian, Elza M. T., Marie, Sueli K. N., Valério, Rosa M. F., Jorge, Carmen L., Yamaga, Liliam, Buchpiguel, Carlos A.
• Format: Article
• Language: English
• Subjects: 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
• ISSN: 1051-2284; 1552-6569
• 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 temporo‐insular 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 hexamethyl‐propyleneamineoxime 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.