Journal of Neurology

, Volume 256, Issue 9, pp 1455–1460

Agraphia in intellectually normal Japanese patients with ALS: omission of kana letters

  • Masayuki Satoh
  • Katsuhiko Takeda
  • Shigeki Kuzuhara
Original Communication


To investigate the occurrence of a writing defect, omission of kana letters (OKL), in intellectually normal Japanese patients with amyotrophic lateral sclerosis (ALS), and define the neuroimaging profile of OKL. Sixteen Japanese adults (10 men and 6 women), similar in age (mean 62.9 ± 9.9 years) and level of education (mean 12.6 ± 2.13 years), with early-stage, classical ALS (mean duration 15.9 ± 5.45 months) were investigated, including tests of motor function and ALS progression; intellectual function including writing ability; and neuroimaging, with follow-up of 1 year. Main outcome measures were as follows: Raven’s Colored Progressive Matrices (RCPM: intellect and psychomotor speed); one-minute verbal fluency measurement; paired associate word-learning test (PAWLT); Western Aphasia Battery (WAB); moraic segmentation test; magnetic resonance imaging (MRI); and 123I-isopropyl amphetamine (123I-IMP) or 99mTc-ethylcysteinate dimmer (ECD) single photon emission tomography (SPECT). Three patients (18.8%) showed OKL (WAB), with disturbance in moraic segmentation. One patient showed decreased blood flow to the bilateral frontal lobes by 123I–IMP-SPECT. Patients with OKL did not differ significantly from those without in the RCPM (intellect), RCPM (time), verbal fluency, or PAWLT (p = 0.10, 0.84, 0.63, 0.55). Although motor dysfunction and weakness progressed during follow-up, none developed symptoms of dementia. The OKL may develop as a relatively early cognitive symptom in intellectually normal Japanese patients with classical ALS. The neuroimaging profile of OKL remains uncertain.


Amyotrophic lateral sclerosis (ALS) Kana Omission of kana letters (OKL) Moraic segmentation disturbance 


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Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Masayuki Satoh
    • 1
    • 3
  • Katsuhiko Takeda
    • 2
  • Shigeki Kuzuhara
    • 3
  1. 1.Mie St. Cross HospitalKomonoJapan
  2. 2.Department of NeurologyMita Hospital, International Medicine and Welfare UniversityTokyoJapan
  3. 3.Department of Neurology, School of MedicineMie UniversityTsuJapan

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