Neuroimaging findings in Rasmussen's syndrome.
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.
Yacubian
E M
EM
Department of Neurology, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
Marie
S K
SK
Valério
R M
RM
Jorge
C L
CL
Yamaga
L
L
Buchpiguel
C A
CA
D000293:Adolescent; D000328:Adult; D001284:Atrophy; D001921:Brain / Q000473:pathology*; D001927:Brain Diseases / Q000175:diagnosis*; D002540:Cerebral Cortex / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004292:Dominance, Cerebral / Q000502:physiology; D004660:Encephalitis / Q000175:diagnosis; D004828:Epilepsies, Partial / Q000175:diagnosis; D005260:Female; D006429:Hemiplegia / Q000175:diagnosis; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D013577:Syndrome; D015899:Tomography, Emission-Computed, Single-Photon; D014057:Tomography, X-Ray Computed