Journal of Neurology

, Volume 260, Issue 8, pp 1982–1991

Progressive apraxic agraphia with micrographia presenting as corticobasal syndrome showing extensive Pittsburgh compound B uptake

  • Yasuhisa Sakurai
  • Kenji Ishii
  • Masahiro Sonoo
  • Yuko Saito
  • Shigeo Murayama
  • Atsushi Iwata
  • Kensuke Hamada
  • Izumi Sugimoto
  • Shoji Tsuji
  • Toru Mannen
Original Communication

DOI: 10.1007/s00415-013-6908-0

Cite this article as:
Sakurai, Y., Ishii, K., Sonoo, M. et al. J Neurol (2013) 260: 1982. doi:10.1007/s00415-013-6908-0

Abstract

A 65-year-old woman developed progressive apraxic agraphia, characterized by poorly formed graphemes, a kanji (Japanese morphograms) recall impairment, relatively preserved oral spelling of kanji characters, and incorrect stroke sequences on writing accompanied by micrographia over a 3-year period. She also showed minor degrees of rigidity, limb-kinetic apraxia, and ideomotor apraxia of the left hand. Although asymmetric rigidity and limb-kinetic apraxia strongly suggested corticobasal degeneration, 11C-Pittsburgh compound B positron emission tomography (PiB-PET) showed the predominantly right-sided accumulation of amyloid β in the cortices and striatum. 18F-fluoro-deoxy-glucose PET and single photon emission computed tomography with a 99mTc-ethylcysteinate dimer (ECD-SPECT) also revealed predominantly right-sided hypometabolism and hypoperfusion in the primary sensorimotor cortex, posterior cingulate gyrus, temporoparietal cortices, frontal cortices, thalamus, and basal ganglia, a pattern characteristic of both corticobasal degeneration and Alzheimer’s disease. The findings suggest that progressive apraxic agraphia with micrographia presenting as corticobasal syndrome can show an Alzheimer’s disease pathology. It is also suggested that ideomotor apraxia of the left hand can occur without a callosal lesion, and is caused by hypometabolism or hypoperfusion in the right frontal and parietal cortices, as revealed by PET and SPECT.

Keywords

Apraxic agraphiaNon-callosal ideomotor apraxia of the left handMicrographiaCorticobasal degenerationAlzheimer’s disease

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Yasuhisa Sakurai
    • 1
  • Kenji Ishii
    • 2
  • Masahiro Sonoo
    • 3
  • Yuko Saito
    • 4
  • Shigeo Murayama
    • 5
  • Atsushi Iwata
    • 6
  • Kensuke Hamada
    • 1
  • Izumi Sugimoto
    • 1
  • Shoji Tsuji
    • 6
  • Toru Mannen
    • 1
  1. 1.Department of NeurologyMitsui Memorial HospitalTokyoJapan
  2. 2.Positron Medical Center, Tokyo Metropolitan Institute of GerontologyTokyoJapan
  3. 3.Department of NeurologyTeikyo University School of MedicineTokyoJapan
  4. 4.National Center of Neurology and PsychiatryTokyoJapan
  5. 5.Tokyo Metropolitan Institute of GerontologyTokyoJapan
  6. 6.Department of NeurologySchool of Medicine, University of TokyoTokyoJapan