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Neuroimaging Findings in Rasmussen's Syndrome
Author(s) -
Yacubian Elza M. T.,
Marie Sueli K. N.,
Valério Rosa M. F.,
Jorge Carmen L.,
Yamaga Liliam,
Buchpiguel Carlos A.
Publication year - 1997
Publication title -
journal of neuroimaging
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.822
H-Index - 64
eISSN - 1552-6569
pISSN - 1051-2284
DOI - 10.1111/jon19977116
Subject(s) - epilepsia partialis continua , medicine , hemiparesis , neuroimaging , atrophy , ictal , magnetic resonance imaging , cerebral blood flow , insular cortex , epilepsy , cerebral atrophy , single photon emission computed tomography , radiology , pathology , cardiology , angiography , status epilepticus , psychiatry
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.