title: Perfusion SPECT findings in a suspected case of Rasmussen encephalitis.
abstract: We report the case of a 35-year-old who was referred for brain perfusion single photon emission computed tomography (SPECT) for pharmacoresistant epilepsy. Her medical history included brain surgery for an inflammatory lesion of unknown origin at age 23 before partial epilepsy occurred. The seizures became refractory to standard antiepileptic drugs and she developed epileptic negative myoclonus of the right upper limb, nocturnal motor seizures, and progressive intellectual impairment. Neurological symptoms slowly worsened with mild aphasia and right visual neglect. Inter-ictal brain SPECT showed decreased cerebral blood flow on the left hemisphere corresponding on magnetic resonance imaging (MRI) with moderate left hemiatrophy, a left frontal defect in accordance with the history of surgery, and a crossed cerebellar diaschisis. Clinical and imaging data were in favor of a late-onset form of Rasmussen encephalitis. Rasmussen syndrome is a rare unilateral devastating disease with childhood onset that can also occur in adulthood, characterized by intractable epileptic seizures associated with progressive neurological deterioration and unilateral progressive atrophy. Brain perfusion SPECT can speed up the diagnosis when exhibiting a strictly unilateral hemispheric hypoperfusion in such a clinical context. It can also guide brain biopsy in cases of inconclusive MRI.
authors.affiliation: Fédération de Médecine Nucléaire CHU-CAL, Nice, France. laetg4@free.fr
authors.forename: Laetitia
authors.initials: L
authors.lastname: Tessonnier
authors.forename: Pierre
authors.initials: P
authors.lastname: Thomas
authors.forename: Danielle
authors.initials: D
authors.lastname: Benisvy
authors.forename: Stephane
authors.initials: S
authors.lastname: Chanalet
authors.forename: Jean Philippe
authors.initials: JP
authors.lastname: Chaborel
authors.forename: Françoise
authors.initials: F
authors.lastname: Bussière
authors.forename: Jacques
authors.initials: J
authors.lastname: Darcourt
mesh_terms: D000328:Adult; D017668:Age of Onset; D001921:Brain / Q000098:blood supply / Q000000981:diagnostic imaging* / Q000503:physiopathology; D002560:Cerebrovascular Circulation; D003937:Diagnosis, Differential; D004569:Electroencephalography; D004660:Encephalitis / Q000175:diagnosis* / Q000000981:diagnostic imaging* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D055420:Perfusion Imaging; D015899:Tomography, Emission-Computed, Single-Photon