Case Report

A Rare Case of Capgras Syndrome in Moyamoya Disease
Kazuma Koda, MD, Yoshihisa Otsuka, MD, Yukihiro Yoneda, MD,
Ryo Tsukamoto, MD, and Yasufumi Kageyama, MD

Moyamoya disease is a rare cerebrovascular disorder with unknown etiology and
psychiatric symptoms occasionally manifest initially. Capgras syndrome is a unique
neuropsychiatric symptom that is a delusional misidentification of a familiar person
replaced by an identical imposter. We report the case of a 51-year-old woman with
frontal lobe ischemic stroke caused by moyamoya disease, presenting with Capgras
syndrome. Dysfunction of frontal areas may be attributable to development of
Capgras syndrome.
Key Words: Capgras syndrome—Moyamoya diseasel—Stroke—Ischemic
© 2020 Elsevier Inc. All rights reserved.

Case description
A 51-year-old woman without past psychiatric history
was admitted to our hospital with headache and difficulty
in speaking. Neurological examination revealed a transcortical motor aphasia, left-right disorientation, and finger
agnosia. There were no motor and sensory deficits. Blood
test showed no remarkable findings. Brain diffusionweighted magnetic resonance imaging and fluid-attenuated inversion recovery imaging demonstrated an acute
ischemic lesion in the left frontal area (Fig. 1A,B). Magnetic resonance angiography showed occlusive lesions of
bilaterally distal internal carotid arteries (Fig. 1C) and
abnormal vascular network in the basal ganglia (Fig. 1D).
Brain single-photon emission computed tomography
revealed extensive hypoperfusion in the bilateral frontal
areas (Fig. 1E). After definite diagnosis of an ischemic
stroke in moyamoya disease, anti-thrombotic medication
was commenced with oral aspirin and intravenous
From the Department of Neurology, Hyogo Prefectural Amagasaki
General Medical Center, Amagasaki City, Japan.
Received June 15, 2020; revision received October 20, 2020; accepted
October 25, 2020.
Financial Disclosures: None.
Grant Support: None.
Address correspondence to Yoshihisa Otsuka, MD, Department of
Neurology, Hyogo Prefectural Amagasaki General Medical Center 217-77 Higashi Naniwa, Amagasaki City, 660-8550, Japan. Tel: +81-66480-7000; Fax: +81-6-6480-7001. E-mail: yotsuka@hp.pref.hyogo.jp.
1052-3057/$ - see front matter
© 2020 Elsevier Inc. All rights reserved.
https://doi.org/10.1016/j.jstrokecerebrovasdis.2020.105432

argatroban. On the third day, her aphasia began improving. The patient suddenly claimed that her mother had
previously died. When the patient listened to her mother’s
voices on the telephone and met her in the hospital, she
repeatedly and consistently described her mother’s voice
and face as “recording” and “dummy”, respectively.
Capgras syndrome was diagnosed by a psychiatrist. Antipsychotic medication with oral quetiapine was commenced, and her symptoms resolved over the next week.
Six months after her stroke, left superficial temporal
artery-middle cerebral artery bypass surgery was performed.

Discussion
To our knowledge, this is the first case of Capgras syndrome due to moyamoya disease. In adult moyamoya disease1 3 psychiatric symptoms manifest initially,
representing 14% of patients.1 Furthermore, 66% of moyamoya disease patients had widespread cognitive impairments such as memory disturbance, executive
dysfunction, and aphasia.1 Capgras syndrome occurs in
functional disorders such as schizophrenia, schizoaffective and bipolar affective disorders, and psychotic depression. In 43% of Capgras syndrome patients, the syndrome
develops in organic diseases including dementia, traumatic brain injury, drug related and cerebrovascular
diseases.4 8
Neuroanatomical mechanisms of Capgras syndrome
have yet to be elucidated. Among 28 patients with organic
Capgras syndrome, including 17 with cerebrovascular

Journal of Stroke and Cerebrovascular Diseases, Vol. 30, No. 1 (January), 2021: 105432

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K. KODA ET AL.

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Fig. 1. (A, B) Diffusion-weighted magnetic resonance imaging and fluid-attenuated inversion recovery imaging revealing high-intensity lesions in the left frontal lobe. (C, D). Magnetic resonance angiography showing occlusions of bilateral distal internal carotid arteries and abnormal vascular network in the basal ganglia (arrow). (E). Brain single-photon emission computed tomography revealing left-sided dominant hypoperfusion of bilateral frontal lobes.

accidents,7 18 (64%) patients had right-sided frontal lobe
damage. However, some cases with damage on the left
have also been reported.9 Nagahama et al. found that
hypoperfusion of the left hippocampus, insula, ventral
striatum, and bilateral inferior frontal gyri is related to
misidentification syndromes in Lewy-body dementia,
including Capgras syndrome.10 Thus, they suggested that
impaired memory and emotional function—associated
with limbic-paralimbic system dysfunction—might
explain the misidentification in said dementia.10 In our
patient, direct ischemic damage of the left frontal area and
hemodynamic hypoperfusion of bilateral frontal areas
due to moyamoya disease may explain the symptoms.
Capgras syndrome is commonly treated with antipsychotic or antidepressant medications, with 65% showing a
good response,4 including our patient who was administered oral quetiapine.

Declarations of Competing Interest
None

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