TY - JOUR
T1 - Functional cortical deafferentation from the subcortical structures in a patient with early myoclonic encephalopathy
T2 - A functional neuroimaging study
AU - Hirose, Mieko
AU - Haginoya, Kazuhiro
AU - Yokoyama, Hiroyuki
AU - Tanaka, Soichiro
AU - Uematsu, Mitsugu
AU - Kaneta, Tomohiro
AU - Takayanagi, Masaru
AU - Kon, Kimiya
AU - Yoshihara, Yasushi
AU - Iinuma, Kazuie
AU - Tsuchiya, Shigeru
PY - 2010/4
Y1 - 2010/4
N2 - 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.
AB - 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.
KW - EIEE
KW - EME
KW - Early myoclonic encephalopathy
KW - PET
KW - Subtraction ictal SPECT
KW - Suppression-burst pattern
UR - http://www.scopus.com/inward/record.url?scp=77951656674&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77951656674&partnerID=8YFLogxK
U2 - 10.1111/j.1528-1167.2009.02399.x
DO - 10.1111/j.1528-1167.2009.02399.x
M3 - Article
C2 - 19922588
AN - SCOPUS:77951656674
VL - 51
SP - 699
EP - 702
JO - Epilepsia
JF - Epilepsia
SN - 0013-9580
IS - 4
ER -