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• Vol.45 No.1
• CLINICA NEUROL, 45: 22−26, 2005
• Vol.45 No.1 contents

Case Report

Evaluation of two patients with SCA2 with frontal lobe dysfunction using brain SPECT with three-dimensional stereotactic surface projections (3D-SSP)

Takayoshi Shimohata, M.D.¹⁾, Yoko Matsuzawa, M.D.¹⁾, Koumei Tanaka, M.D.¹⁾, Osamu Onodera, M.D.²⁾, Keiko Tanaka, M.D.¹⁾ and Masatoyo Nishizawa, M.D.¹⁾

¹⁾Department of Neurology, Brain Research Institute, Niigata University
²⁾Resource Branch for Brain Disease Research, Center for Bioresource-Based Researches, Brain Research Institute, Niigata University

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.

(CLINICA NEUROL, 45: 22−26, 2005)
key words: spinocerebellar ataxia type 2, SCA2, frontal lobe dysfunction, SPECT, 3D-SSP

(Received: 29-Jan-04)