Skip to main content

Advertisement

SpringerLink
Log in

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

  • Original Communication
  • Published:
Journal of Neurology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Rapcsak SZ, Beeson PM (2002) Neuroanatomical correlates of spelling and writing. In: Hillis AE (ed) The handbook of adult language disorders. Integrating cognitive neuropsychology, neurology, and rehabilitation. Psychology Press, New York, pp 71–99

  2. Ullrich L, Roeltgen DP (2011) Agraphia. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology, 5th edn. Oxford University Press, New York, pp 130–151

    Google Scholar 

  3. Otsuki M, Soma Y, Arai T, Otsuka A, Tsuji S (1999) Pure apraxic agraphia with abnormal writing stroke sequences: report of a Japanese patient with a left superior parietal haemorrhage. J Neurol Neurosurg Psychiatry 66:233–237

    Article  PubMed  CAS  Google Scholar 

  4. Sakurai Y, Onuma Y, Nakazawa G, Ugawa Y, Momose T, Tsuji S et al (2007) Parietal dysgraphia: characterization of abnormal writing stroke sequences, character formation, and character recall. Behav Neurol 18:99–114

    PubMed  Google Scholar 

  5. Alexander MP, Fischer RS, Friedman R (1992) Lesion localization in apractic agraphia. Arch Neurol 49:246–251

    Article  PubMed  CAS  Google Scholar 

  6. Heilman KM, Coenen A, Kluger B (2008) Progressive asymmetric apraxic agraphia. Cogn Behav Neurol 21:14–17

    Article  PubMed  Google Scholar 

  7. Passov V, Gavrilova RH, Strand E, Cerhan JH, Josephs KA (2011) Sporadic corticobasal syndrome with progranulin mutation presenting as progressive apraxic agraphia. Arch Neurol 68:376–380

    Article  PubMed  Google Scholar 

  8. Kezuka M, Kawamura M, Yano Y, Shiroyama H (1995) A peculiar kind of agraphia due to degenerative processes predominant in the right hemisphere: comparison with apraxic agraphia. Shinkeishinrigaku 11:196–205

    Google Scholar 

  9. Croisile B, Brabant MJ, Carmoi T, Lepage Y, Aimard G, Trillet M (1996) Comparison between oral and written spelling in Alzheimer’s disease. Brain Lang 54:361–387

    Article  PubMed  CAS  Google Scholar 

  10. Hughes JC, Graham N, Patterson K, Hodges JR (1997) Dysgraphia in mild dementia of Alzheimer’s type. Neuropsychologia 35:533–545

    Article  PubMed  CAS  Google Scholar 

  11. Rapcsak SZ, Arthur SA, Bliklen DA, Rubens AB (1989) Lexical agraphia in Alzheimer’s disease. Arch Neurol 46:65–68

    Article  PubMed  CAS  Google Scholar 

  12. Sakurai Y, Tsuchiya K, Oda T, Hori K, Tominaga I, Akiyama H et al (2006) Ubiquitin-positive frontotemporal lobar degeneration presenting with progressive Gogi (word-meaning) aphasia. A neuropsychological, radiological and pathological evaluation of a Japanese semantic dementia patient. J Neurol Sci 250:3–9

    Article  PubMed  Google Scholar 

  13. Ichikawa H, Takahashi N, Hieda S, Ohno H, Kawamura M (2008) Agraphia in bulbar-onset amyotrophic lateral sclerosis: not merely a consequence of dementia or aphasia. Behav Neurol 20:91–99

    PubMed  Google Scholar 

  14. Sakurai Y, Hashida H, Uesugi H, Arima K, Murayama S, Bando M et al (1996) A clinical profile of corticobasal degeneration presenting as primary progressive aphasia. Eur Neurol 36:134–137

    Article  PubMed  CAS  Google Scholar 

  15. Platel H, Lambert J, Eustache F, Cadet B, Dary M, Viader F et al (1993) Characteristics and evolution of writing impairment in Alzheimer’s disease. Neuropsychologia 31:1147–1158

    Article  PubMed  CAS  Google Scholar 

  16. Neils J, Roeltgen DP, Constantinidou F (1995) Decline in homophone spelling associated with loss of semantic influence on spelling in Alzheimer’s disease. Brain Lang 49:27–49

    Article  PubMed  CAS  Google Scholar 

  17. Doody RS, Jankovic J (1992) The alien hand and related signs. J Neurol Neurosurg Psychiatry 55:806–810

    Article  PubMed  CAS  Google Scholar 

  18. Sakurai Y, Sakai K, Sakuta M, Iwata M (1994) Naming difficulties in alexia with agraphia for kanji after a left posterior inferior temporal lesion. J Neurol Neurosurg Psychiatry 57:609–613

    Article  PubMed  CAS  Google Scholar 

  19. Sakurai Y, Yagishita A, Goto Y, Ohtsu H, Mannen T (2006) Fusiform type alexia: pure alexia for words in contrast to posterior occipital type pure alexia for letters. J Neurol Sci 247:81–92

    Article  PubMed  Google Scholar 

  20. Sakurai Y, Matsumura K, Iwatsubo T, Momose T (1997) Frontal pure agraphia for kanji or kana: dissociation between morphology and phonology. Neurology 49:946–952

    Article  PubMed  CAS  Google Scholar 

  21. Dubois B, Slachevsky A, Litvan I, Pillon B (2000) The FAB: a frontal assessment battery at bedside. Neurology 55:1621–1626

    Article  PubMed  CAS  Google Scholar 

  22. Shaw LM, Vanderstichele H, Knapik-Czajka M, Clark CM, Aisen PS, Petersen RC et al (2009) Cerebrospinal fluid biomarker signature in Alzheimer’s disease neuroimaging initiative subjects. Ann Neurol 65:403–413

    Article  PubMed  CAS  Google Scholar 

  23. Schoonenboom NS, Reesink FE, Verwey NA, Kester MI, Teunissen CE, van de Ven PM et al (2012) Cerebrospinal fluid markers for differential dementia diagnosis in a large memory clinic cohort. Neurology 78:47–54

    Article  PubMed  CAS  Google Scholar 

  24. Friston KJ, Holmes AP, Worsley KJ, Poline J-P, Frith CD, Frackowiak RSJ (1995) Statistical parametric maps in functional imaging: a general linear approach. Hum Brain Mapp 2:189–210

    Article  Google Scholar 

  25. Matsuda H, Mizumura S, Soma T, Takemura N (2004) Conversion of brain SPECT images between different collimators and reconstruction processes for analysis using statistical parametric mapping. Nucl Med Commun 25:67–74

    Article  PubMed  CAS  Google Scholar 

  26. Crary MA, Heilman KM (1988) Letter imagery deficits in a case of pure apraxic agraphia. Brain Lang 34:147–156

    Article  PubMed  CAS  Google Scholar 

  27. Levine DN, Mani RB, Calvanio R (1988) Pure agraphia and Gerstmann’s syndrome as a visuospatial–language dissociation: an experimental case study. Brain Lang 35:172–196

    Article  PubMed  CAS  Google Scholar 

  28. Boeve BF, Lang AE, Litvan I (2003) Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 54(Suppl 5):S15–S19

    Article  PubMed  Google Scholar 

  29. Ishii K (2002) Clinical application of positron emission tomography for diagnosis of dementia. Ann Nucl Med 16:515–525

    Article  PubMed  Google Scholar 

  30. Hassan A, Whitwell JL, Josephs KA (2011) The corticobasal syndrome-Alzheimer’s disease conundrum. Expert Rev Neurother 11:1569–1578

    Article  PubMed  Google Scholar 

  31. McKhann GM, Knopman DS, Chertkow H, Hyman BT, Jack CR Jr, Kawas CH et al (2011) The diagnosis of dementia due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimers Dement 7:263–269

    Article  PubMed  Google Scholar 

  32. Blennow K, Hampel H (2003) CSF markers for incipient Alzheimer’s disease. Lancet Neurol 2:605–613

    Article  PubMed  CAS  Google Scholar 

  33. Moghekar A, Goh J, Li M, Albert M, O’Brien RJ (2012) Cerebrospinal fluid Abeta and tau level fluctuation in an older clinical cohort. Arch Neurol 69:246–250

    Article  PubMed  Google Scholar 

  34. Grimmer T, Riemenschneider M, Forstl H, Henriksen G, Klunk WE, Mathis CA et al (2009) Beta amyloid in Alzheimer’s disease: increased deposition in brain is reflected in reduced concentration in cerebrospinal fluid. Biol Psychiatry 65:927–934

    Article  PubMed  CAS  Google Scholar 

  35. Shelley BP, Hodges JR, Kipps CM, Xuereb JH, Bak TH (2009) Is the pathology of corticobasal syndrome predictable in life? Mov Disord 24:1593–1599

    Article  PubMed  Google Scholar 

  36. Josephs KA, Whitwell JL, Boeve BF, Knopman DS, Petersen RC, Hu WT et al (2010) Anatomical differences between CBS-corticobasal degeneration and CBS-Alzheimer’s disease. Mov Disord 25:1246–1252

    Article  PubMed  Google Scholar 

  37. Kim J, Basak JM, Holtzman DM (2009) The role of apolipoprotein E in Alzheimer’s disease. Neuron 63:287–303

    Article  PubMed  CAS  Google Scholar 

  38. Christensen DZ, Schneider-Axmann T, Lucassen PJ, Bayer TA, Wirths O (2010) Accumulation of intraneuronal Abeta correlates with ApoE4 genotype. Acta Neuropathol 119:555–566

    Article  PubMed  CAS  Google Scholar 

  39. Cordato NJ, Halliday GM, McCann H, Davies L, Williamson P, Fulham M et al (2001) Corticobasal syndrome with tau pathology. Mov Disord 16:656–667

    Article  PubMed  CAS  Google Scholar 

  40. Boeve BF, Maraganore DM, Parisi JE, Ahlskog JE, Graff-Radford N, Caselli RJ et al (1999) Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology 53:795–800

    Article  PubMed  CAS  Google Scholar 

  41. Williams DR, Holton JL, Strand K, Revesz T, Lees AJ (2007) Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy. Mov Disord 22:2235–2241

    Article  PubMed  Google Scholar 

  42. Yoshida T, Yamadori A, Mori E (1989) A case of micrographia with the right hand due to left putaminal infarction. Rinsho Shinkeigaku 29:1149–1151

    PubMed  CAS  Google Scholar 

  43. Sakurai Y, Yoshida Y, Sato K, Sugimoto I, Mannen T (2011) Isolated thalamic agraphia with impaired grapheme formation and micrographia. J Neurol 258:1528–1537

    Article  PubMed  Google Scholar 

  44. Scarmeas N, Hadjigeorgiou GM, Papadimitriou A, Dubois B, Sarazin M, Brandt J et al (2004) Motor signs during the course of Alzheimer disease. Neurology 63:975–982

    Article  PubMed  CAS  Google Scholar 

  45. Heilman KM, Rothi LJ (2011) Apraxia. In: Heilman KM, Valenstein E (eds) Clinical neuropsychology, 5th edn. Oxford University Press, New York, pp 214–237

    Google Scholar 

  46. Sugishita M (1986) The Western Aphasia Battery, Japanese edn. Igaku-Shoin, Tokyo

    Google Scholar 

Download references

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

Authors and 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
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kenji Ishii
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Masahiro Sonoo
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Yuko Saito
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Shigeo Murayama
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Atsushi Iwata
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Kensuke Hamada
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Izumi Sugimoto
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Shoji Tsuji
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Toru Mannen
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Yasuhisa Sakurai.

Rights and permissions

Reprints and permissions

About this article

Cite this article

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

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

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

Keywords

Search

Navigation