Retrospective study on PET-SPECT imaging in a large cohort of myotonic dystrophy type 1 patients

Vincenzo Romeo, E. Pegoraro, F. Squarzanti, G. Sorarù, C. Ferrati, M. Ermani, P. Zucchetta, F. Chierichetti, C. Angelini

Research output: Contribution to journalArticlepeer-review


The aim was to study brain involvement in myotonic dystrophy type 1 by single photon emission tomography (SPECT) and positron emission tomography (PET). 58 DM1 patients were subjected to SPECT; 17 to both SPECT and PET. SPECT patients were grouped as 'normally perfused' and 'abnormally perfused'; PET patients as 'normal performers' and 'abnormal performers'. To quantify hypoperfusion and/or hypometabolism, we used a semi-quantitative scale. To localize focal hypoperfusion/hypometabolism, nine cerebral areas of involvement were identified. The Chi-square, Wilcoxon, McNemar tests were used for statistics. SPECT showed abnormalities in 52/58 patients. PET showed an abnormal glucidic uptake in 15/17. Hypoperfusion was mild/moderate in 50/58 patients, mostly involving the left supratentorial areas. Abnormal glucidic uptake was mainly observed in the left frontal lobe. Abnormalities in blood perfusion and/or glucose metabolism are frequent in DM1. These abnormalities involve the left more often than the right hemisphere, the frontal lobe more than other lobes. Such abnormalities are more often cortical than subcortical.

Original languageEnglish
Pages (from-to)757-763
Number of pages7
JournalNeurological Sciences
Issue number6
Publication statusPublished - Dec 2010


  • Brain involvement
  • DM1
  • Myotonic dystrophy type 1
  • PET

ASJC Scopus subject areas

  • Clinical Neurology
  • Psychiatry and Mental health
  • Dermatology


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