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01-08-2013 | Original Communication

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

Authors: Yasuhisa Sakurai, Kenji Ishii, Masahiro Sonoo, Yuko Saito, Shigeo Murayama, Atsushi Iwata, Kensuke Hamada, Izumi Sugimoto, Shoji Tsuji, Toru Mannen

Published in: Journal of Neurology | Issue 8/2013

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.

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

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

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 66:233–237PubMedCrossRef

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 18:99–114PubMed

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

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

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

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

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 54:361–387PubMedCrossRef

10.

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

11.

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

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 250:3–9PubMedCrossRef

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 20:91–99PubMed

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 36:134–137PubMedCrossRef

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 31:1147–1158PubMedCrossRef

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 49:27–49PubMedCrossRef

17.

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

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 57:609–613PubMedCrossRef

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 247:81–92PubMedCrossRef

20.

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

21.

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

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 65:403–413PubMedCrossRef

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 78:47–54PubMedCrossRef

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 2:189–210CrossRef

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 25:67–74PubMedCrossRef

26.

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

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 35:172–196PubMedCrossRef

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–S19PubMedCrossRef

29.

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

30.

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

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 7:263–269PubMedCrossRef

32.

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

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 69:246–250PubMedCrossRef

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 65:927–934PubMedCrossRef

35.

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

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 25:1246–1252PubMedCrossRef

37.

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

38.

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

39.

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

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 53:795–800PubMedCrossRef

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 22:2235–2241PubMedCrossRef

42.

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

43.

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

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 63:975–982PubMedCrossRef

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

46.

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

Title: Progressive apraxic agraphia with micrographia presenting as corticobasal syndrome showing extensive Pittsburgh compound B uptake
Authors: Yasuhisa Sakurai
Kenji Ishii
Masahiro Sonoo
Yuko Saito
Shigeo Murayama
Atsushi Iwata
Kensuke Hamada
Izumi Sugimoto
Shoji Tsuji
Toru Mannen
Publication date: 01-08-2013
Publisher: Springer Berlin Heidelberg
Published in: Journal of Neurology / Issue 8/2013
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-013-6908-0

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

Abstract

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