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Early clinical, radiological and EEG improvement following L-arginine infusion in SMART syndrome

Mehta, AR; Mallon, DH; Rees, JH; (2023) Early clinical, radiological and EEG improvement following L-arginine infusion in SMART syndrome. Brain Disorders , 12 , Article 100103. 10.1016/j.dscb.2023.100103. Green open access

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Abstract

Objectives: To report the clinical, radiological (MRI) and neurophysiological (EEG) changes in a case of SMART (stroke-like migraine attacks after radiation therapy) syndrome following treatment with intravenous L-arginine. Methods: A 60-year-old woman had, ten years prior, been diagnosed with primary CNS diffuse large B cell lymphoma, and was successfully treated with curative chemotherapy and whole brain radiotherapy. She presented acutely with left-sided headache, teichopsia and dysphasia following a chest infection. MRI of the brain showed striking left parieto-occipital gyral swelling, diffusion restriction, leptomeningeal enhancement, and increased cerebral blood volume. Her EEG showed an excess of slow activity diffusely, particularly over the left temporal lobe. A diagnosis of SMART syndrome was made. Intravenous L-arginine (0.5 g/kg) was administered. Results: A few hours post infusion, her migrainous headache subsided and her mentation improved. Her MRI brain performed six days post infusion showed reduced cortical swelling and hyperperfusion, and her EEG showed less temporal slowing. She continued to improve cognitively. Discussion: This is the first report of SMART syndrome with a response to L-arginine, reflected clinically by a measurable improvement in cognition, brain perfusion and EEG parameters, encouraging further clinical studies.

Type: Article
Title: Early clinical, radiological and EEG improvement following L-arginine infusion in SMART syndrome
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.dscb.2023.100103
Publisher version: https://doi.org/10.1016/j.dscb.2023.100103
Language: English
Additional information: This work is licensed under a Creative Commons License. The images or other third-party material in this article are included in the Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
URI: https://discovery.ucl.ac.uk/id/eprint/10212857
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