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

Journal of Neurology volume 260, pages 1982–1991 (2013)Cite this article

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.

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Acknowledgments

The study was partly supported by a Grant-in-Aid for Comprehensive Research on Dementia (No 1103404) from the Ministry of Health, Labour, and Welfare of Japan (K.I.).

Conflicts of interest

The authors declare that they have no conflict of interest.

Author information

Affiliations

  1. Department of Neurology, Mitsui Memorial Hospital, 1, Kanda-Izumi-cho, Chiyoda-ku, Tokyo, 101-8643, Japan

    Yasuhisa Sakurai, Kensuke Hamada, Izumi Sugimoto & Toru Mannen

  2. Positron Medical Center, Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan

    Kenji Ishii

  3. Department of Neurology, Teikyo University School of Medicine, Tokyo, Japan

    Masahiro Sonoo

  4. National Center of Neurology and Psychiatry, Tokyo, Japan

    Yuko Saito

  5. Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan

    Shigeo Murayama

  6. Department of Neurology, School of Medicine, University of Tokyo, Tokyo, Japan

    Atsushi Iwata & Shoji Tsuji

Authors
  1. Yasuhisa Sakurai
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  2. Kenji Ishii
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  3. Masahiro Sonoo
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Correspondence to Yasuhisa Sakurai.

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Sakurai, Y., Ishii, K., Sonoo, M. et al. Progressive apraxic agraphia with micrographia presenting as corticobasal syndrome showing extensive Pittsburgh compound B uptake. J Neurol 260, 1982–1991 (2013). https://doi.org/10.1007/s00415-013-6908-0

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  • DOI: https://doi.org/10.1007/s00415-013-6908-0

Keywords

  • Apraxic agraphia
  • Non-callosal ideomotor apraxia of the left hand
  • Micrographia
  • Corticobasal degeneration
  • Alzheimer’s disease