Functional cortical deafferentation from the subcortical structures in a patient with early myoclonic encephalopathy: A functional neuroimaging study

Mieko Hirose, Kazuhiro Haginoya, Hiroyuki Yokoyama, Soichiro Tanaka, Mitsugu Uematsu, Tomohiro Kaneta, Masaru Takayanagi, Kimiya Kon, Yasushi Yoshihara, Kazuie Iinuma, Shigeru Tsuchiya

Research output: Contribution to journalArticlepeer-review

10 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)699-702
Number of pages4
JournalEpilepsia
Volume51
Issue number4
DOIs
Publication statusPublished - 2010 Apr

Keywords

  • EIEE
  • EME
  • Early myoclonic encephalopathy
  • PET
  • Subtraction ictal SPECT
  • Suppression-burst pattern

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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