title: Functional cortical deafferentation from the subcortical structures in a patient with early myoclonic encephalopathy: a functional neuroimaging study.
abstract: We, for the first time, used functional neuroimaging analyses for a girl with early myoclonic encephalopathy (EME). The interictal single photon emission computed tomography (SPECT) and [18F]-fluoro-D-deoxyglucose positron emission tomography (FDG-PET) at 1 month of age showed hypoperfusion and hypometabolism of bilateral basal ganglia, thalami, and the right parietooccipital cerebral cortices, showing that there is profound dysfunction of the basal ganglia and thalamus as well as cerebral cortex. On the other hand, subtraction ictal SPECT of tonic spasms clearly showed hyperperfusion of the bilateral basal ganglia, thalami, brainstem, and deep cortical layer of bilateral frontoparietal cortices. The present study suggests that functional deafferentation of the cortex from subcortical structures exists in EME, and that these imaging abnormalities may provide insight into the pathophysiology of suppression-burst pattern in EME.
authors.affiliation: Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: Mieko
authors.initials: M
authors.lastname: Hirose
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Haginoya
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Yokoyama
authors.forename: Soichiro
authors.initials: S
authors.lastname: Tanaka
authors.forename: Mitsugu
authors.initials: M
authors.lastname: Uematsu
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Kaneta
authors.forename: Masaru
authors.initials: M
authors.lastname: Takayanagi
authors.forename: Kimiya
authors.initials: K
authors.lastname: Kon
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Yoshihara
authors.forename: Kazuie
authors.initials: K
authors.lastname: Iinuma
authors.forename: Shigeru
authors.initials: S
authors.lastname: Tsuchiya
mesh_terms: D000344:Afferent Pathways / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001921:Brain / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001931:Brain Mapping; D002540:Cerebral Cortex / Q000000981:diagnostic imaging / Q000503:physiopathology*; D002648:Child; D004292:Dominance, Cerebral / Q000502:physiology; D004569:Electroencephalography; D004734:Energy Metabolism / Q000502:physiology; D004831:Epilepsies, Myoclonic / Q000000981:diagnostic imaging / Q000503:physiopathology*; D005260:Female; D019788:Fluorodeoxyglucose F18; D006801:Humans; D007091:Image Processing, Computer-Assisted; D049268:Positron-Emission Tomography; D012815:Signal Processing, Computer-Assisted; D013382:Subtraction Technique; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D019788:Fluorodeoxyglucose F18