Case Report Vascular Corticobasal Syndrome Caused by Unilateral Internal Carotid Artery Occlusion Yosuke Miyaji, MD,* Kazuo Koyama, MD, PhD,* Takashi Kurokawa, MD,* Mutsumi Mitomi, MD,* Yume Suzuki, MD, PhD,† and Yoshiyuki Kuroiwa, MD, PhD† A 65-year-old man developed progressive worsening of right-sided limb-kinetic apraxia and extrapyramidal dysfunction. His left internal carotid artery was found to be occluded, and there was general atrophy and severely decreased cerebral blood flow in the left hemisphere. He had experienced an acute infarction in the left watershed area before superficial temporal artery to middle cerebral artery bypass surgery. After surgery, the cerebral blood flow in the left hemisphere was remarkably improved. Unilateral internal carotid artery occlusion may result in clinical manifestations similar to corticobasal degeneration. Key Words: Corticobasal syndrome— internal carotid artery occlusion. Ó 2013 by National Stroke Association Case Report A 65-year-old right-handed man with diabetes mellitus who was employed as a sushi chef was seen in our hospital complaining of a tendency to fall toward the right. Seven months before visiting, his family noticed his forgetfulness. Five months before visiting, he retired from his job because of his difficulty making sushi. His MiniMental State Examination score was 11 of 30. He lacked spontaneity and showed right-sided limb-kinetic apraxia and constructional apraxia. He had right upper limb rigidity and a wide-based gait with slightly short steps. A magnetic resonance imaging (MRI) scan of the brain revealed mild atrophy of the left hemisphere and no infarction. Technetium-99 bicisate single-photon emission computed From the *Department of Neurology, Fujisawa City Hospital, Fujisawa; and †Department of Neurology and Stroke Medicine, Yokohama City University, Yokohama City, Japan. Received March 19, 2012; revision received June 12, 2012; accepted July 9, 2012. Address correspondence to Yosuke Miyaji, MD, Department of Neurology, Fujisawa City Hospital, 2-6-1, Fujisawa, Fujisawa-shi, Kanagawa, Japan. E-mail: mmmyosuke@yahoo.co.jp. 1052-3057/$ - see front matter Ó 2013 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2012.07.005 tomography (ECD-SPECT) revealed severely decreased cerebral blood flow in the left hemisphere and crossed cerebellar diaschisis. Magnetic resonance angiography revealed occlusion of the origin of the left internal carotid artery (Fig 1 A-D). Angiography revealed collateral blood flow through the right anterior communicating artery and the left ophthalmic artery to the left middle cerebral artery. He was admitted for superficial temporal artery to middle cerebral artery (STA-MCA) bypass surgery. On the fourth day after admission, he suffered transient aphasia lasting for 2 hours. An MRI scan of the brain revealed acute infarction in the left corona radiata (Fig 1 E), and he was started on aspirin and argatroban. On the seventh day, he developed aphasia, dysarthria, and right hemiparesis. The ischemic lesions had spread to the left watershed area (Fig 1 F). On day 31, he underwent STA-MCA bypass. The cerebral blood flow in the left hemisphere was remarkably improved (Fig 1 G), but his symptoms remained. Discussion Rebeiz et al1 described 3 patients with progressive asymmetrical bradykinesia, involuntary movement, and apraxia as corticodentatonigral degeneration with neuronal achromasia. Gibb et al2 described this type of Journal of Stroke and Cerebrovascular Diseases, Vol. 22, No. 7 (October), 2013: pp 1193-1195 1193 1194 Y. MIYAJI ET AL. Figure 1. The right side of this figure shows the patient’s left side. (A) Axial fluid-attenuated inversion recovery magnetic resonance imaging revealing mild atrophy and no infarctions in the left hemisphere. (B) Technetium-99 bicisate single-photon emission computed tomography revealing severely decreased cerebral blood flow in the left hemisphere and right cerebellum. (C and D) Magnetic resonance angiography revealing occlusion of the origin of the left internal carotid artery. (E) Diffusion-weighted magnetic resonance imaging revealing acute infarction in the left corona radiata on day 4 after admission. (F) Ischemic lesions that had spread to the left watershed area on day 7 after admission. (G) Improvement of cerebral blood flow in the left hemisphere. pathology and these clinical features in detail and called the disorder corticobasal degeneration (CBD). Boeve et al3 advocated the definition of corticobasal syndrome (CBS) as the constellation of clinical features initially con- sidered characteristic of CBD, and CBD as the histopathologic disorder. Recently, multiple cerebral infarctions presenting as CBD have been reported.4-6 In the present case, decreased cerebral blood flow and atrophy without VASCULAR CORTICOBASAL SYNDROME infarctions in the left hemisphere because of the occlusion of the left internal carotid artery resulted in clinical manifestations mimicking CBD. Although we cannot yet confirm the pathology and cannot estimate improvement in his symptoms after the STA-MCA bypass because of deterioration of his condition related to cerebral infarctions that occurred before surgery, the marked improvement in cerebral blood flow after the STA-MCA bypass supports the diagnosis of CBS, not CBD. References 1. Rebeiz JJ, Kolodny EH, Richardson EP Jr. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 1968;18:20-33. 1195 2. Gibb WRG, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain 1989;112:1171-1192. 3. Boeve BF, Lang AE, Litvan I. Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 2003; 54(Suppl 5):S15-S19. 4. Kreisler A, Mastain B, Tison F, et al. Multiinfarct disorder presenting as corticobasal degeneration (DCB): Vascular pseudo-corticobasal degeneration? Rev Neurol (Paris) 2007;163:1191-1199. 5. Kim YD, Kim JS, Lee ES, et al. Progressive ‘‘vascular’’ corticobasal syndrome due to bilateral ischemic hemispheric lesions. Intern Med 2009;48:1699-1702. 6. Engelen M, Westhoff D, de Gans J, et al. A 64-year old man presenting with carotid artery occlusion and corticobasal syndrome: A case report. J Med Case Rep 2011; 5:357.