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• Vol.63 No.4
• CLINICA NEUROL, 63: 214−220, 2023
• Vol.63 No.4 contents

Case Report

A case of Neuropsychiatric systemic lupus erythematosus with varying stenotic lesions in several cerebral major arteries and a vertebral artery aneurysm

Seiji Sugiyama, M.D.¹⁾, Akihiro Kitamura, M.D., Ph.D.¹⁾²⁾, Shunya Kaneko, M.D.¹⁾, Isamu Yamakawa, M.D., Ph.D.¹⁾, Mitsuru Sanada, M.D., Ph.D.¹⁾ and Makoto Urushitani, M.D., Ph.D.¹⁾²⁾

¹⁾ Department of Neurology, Shiga University of Medical Science
²⁾ Molecular Neuroscience Research Center, Shiga University of Medical Science

A 42 years old female suffered from systemic lupus erythematosus (SLE) about 20 years ago. While steroid was tapered for a steroid-induced psychiatric disorder, she presented with an acute confusional state and was diagnosed with neuropsychiatric SLE (NPSLE). MRI showed acute infarction mainly in the cortex of the right temporal lobe and MRA demonstrated dynamic subacute morphological changes such as stenosis and dilation in several major intracrainal arteries. The right vertebral artery diffusely dilated and subsequently formed an aneurysm in a week. Contrast enhanced MRI vessel-wall imaging showed a remarkable enhancement of the aneurysm wall, which might indicate an unstable unruptured aneurysm. The prompt introduction of intravenous cyclophosphamide improved both clinical and radiological signs. Our case indicates that intensive immunosuppressive treatments should be considered in NPSLE patients with varying vasospasm and aneurysm, indicating exacerbated disease activity.
Full Text of this Article in Japanese PDF (3093K)

(CLINICA NEUROL, 63: 214−220, 2023)
key words: NPSLE, cerebral aneurysm, multiple vasospasm, RCVS

(Received: 22-Jul-22)