title: [Evaluation of two patients with SCA2 with frontal lobe dysfunction using brain SPECT with three-dimensional stereotactic surface projections (3D-SSP)].
abstract: We evaluated the cognitive impairment of two patients with genetically confirmed spinocerebellar ataxia type 2 (SCA2). Neurological examination revealed ignorance of his illness and Gegenhalten phenomenon in patient 1, and emotional incontinence in patient 2. Although their mental status evaluated by HDS-R or Mini-Mental State Examination (MMSE) was almost normal, the results of WAIS-R and Wisconsin Card Sorting Test (WCST) revealed the existence of intellectual decline and executive dysfunction. 3D-SSP SPECT demonstrated distinct hypoperfusion in bilateral frontal lobes, whereas brain MRI revealed no apparent cerebral atrophy in both patients. These results raise the possibility that frontal lobe dysfunction was observed in the early stages of SCA2, and that 3D-SSP SPECT is useful for evaluating the involvement of frontal lobe dysfunction in SCA2.
authors.affiliation: Department of Neurology, Brain Research Institute, Niigata University.
authors.forename: Takayoshi
authors.initials: T
authors.lastname: Shimohata
authors.forename: Yoko
authors.initials: Y
authors.lastname: Matsuzawa
authors.forename: Koumei
authors.initials: K
authors.lastname: Tanaka
authors.forename: Osamu
authors.initials: O
authors.lastname: Onodera
authors.forename: Keiko
authors.initials: K
authors.lastname: Tanaka
authors.forename: Masatoyo
authors.initials: M
authors.lastname: Nishizawa
mesh_terms: D000328:Adult; D005260:Female; D005625:Frontal Lobe / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D021621:Imaging, Three-Dimensional; D008279:Magnetic Resonance Imaging; D008297:Male; D008609:Mental Status Schedule; D008875:Middle Aged; D009460:Neurologic Examination; D020754:Spinocerebellar Ataxias / Q000000981:diagnostic imaging* / Q000523:psychology; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods