title: Progressive apraxic agraphia with micrographia presenting as corticobasal syndrome showing extensive Pittsburgh compound B uptake.
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, (11)C-Pittsburgh compound B positron emission tomography (PiB-PET) showed the predominantly right-sided accumulation of amyloid β in the cortices and striatum. (18)F-fluoro-deoxy-glucose PET and single photon emission computed tomography with a (99m)Tc-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.
authors.affiliation: Department of Neurology, Mitsui Memorial Hospital, 1, Kanda-Izumi-cho, Chiyoda-ku, Tokyo 101-8643, Japan. ysakurai-tky@umin.ac.jp
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Sakurai
authors.forename: Kenji
authors.initials: K
authors.lastname: Ishii
authors.forename: Masahiro
authors.initials: M
authors.lastname: Sonoo
authors.forename: Yuko
authors.initials: Y
authors.lastname: Saito
authors.forename: Shigeo
authors.initials: S
authors.lastname: Murayama
authors.forename: Atsushi
authors.initials: A
authors.lastname: Iwata
authors.forename: Kensuke
authors.initials: K
authors.lastname: Hamada
authors.forename: Izumi
authors.initials: I
authors.lastname: Sugimoto
authors.forename: Shoji
authors.initials: S
authors.lastname: Tsuji
authors.forename: Toru
authors.initials: T
authors.lastname: Mannen
mesh_terms: D000368:Aged; D000381:Agraphia / Q000000981:diagnostic imaging* / Q000503:physiopathology; D000814:Aniline Compounds; D001057:Apolipoproteins E / Q000134:cerebrospinal fluid; D001072:Apraxias / Q000000981:diagnostic imaging* / Q000503:physiopathology; D001480:Basal Ganglia Diseases / Q000000981:diagnostic imaging* / Q000503:physiopathology; D001921:Brain / Q000473:pathology; D002540:Cerebral Cortex / Q000000981:diagnostic imaging* / Q000503:physiopathology; D003545:Cysteine / Q000031:analogs & derivatives; D018450:Disease Progression; D056344:Executive Function; D005260:Female; D006236:Handwriting; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007361:Intelligence Tests; D019636:Neurodegenerative Diseases / Q000000981:diagnostic imaging* / Q000503:physiopathology; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D015609:Organotechnetium Compounds; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D011932:Reading; D013844:Thiazoles; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: C475519:2-(4'-(methylamino)phenyl)-6-hydroxybenzothiazole; D000814:Aniline Compounds; D001057:Apolipoproteins E; D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; D013844:Thiazoles; C059659:technetium Tc 99m bicisate; D003545:Cysteine