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Neurology International
Volume 4
Issue 1
10.4081/ni.2012.e5
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Neurology International is published by MDPI from Volume 12 Issue 3 (2020). Previous articles were published by another publisher in Open Access under a CC-BY licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Case Report

Two Cases of Possible Neuro-Sweet Disease with Meningoencephalitis as the Initial Manifestation

by
Go Makimoto
1,
Yasuhiro Manabe
1,*,
Chizuru Yamakawa
1,
Daiki Fujii
1,
Yasuko Ikeda-Sakai
1,
Hisashi Narai
1,
Nobuhiko Omori
1 and
Koji Abe
2
1
Department of Neurology, National Hospital Organization Okayama Medical Center, Okayama, Japan
2
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, Japan
*
Author to whom correspondence should be addressed.
Neurol. Int. 2012, 4(1), e5; https://doi.org/10.4081/ni.2012.e5
Submission received: 4 August 2011 / Revised: 20 October 2011 / Accepted: 17 January 2012 / Published: 9 February 2012

Abstract

We report 2 cases that were considered to be neuro-Sweet disease. They initially manifested with meningoencephalitis and no skin lesions, and rapidly improved with corticosteroid therapy. In both cases, patients complained of meningitic symptoms such as fever and headache, and HLA-B54 and -Cw1 turned out to be positive over the clinical course. Cerebrospinal fluid analysis showed increased levels of lymphocytes and protein. In case #1, fluid-attenuated inversion recovery (FLAIR), magnetic resonance imaging (MRI) and diffusion-weighted images (DWI) showed high-intensity signals in the right dorsal medulla oblongata, bilateral dorsal midbrain, and left thalamus. In case #2, FLAIR and DWI showed high-intensity signals in the bilateral cerebellar cortex and left caudate nucleus. Symptoms and MRI images were markedly improved in both cases after corticosteroid pulse therapy. According to published diagnostic criteria, these 2 cases were considered possible neuro-Sweet disease. These cases suggest that the combination of meningoencephalitis and HLA specificity is important to consider the possibility of neuro-Sweet disease, even without skin lesions.
Keywords: neuro-Sweet disease; meningoencephalitis; neuro-neutrophilic disease neuro-Sweet disease; meningoencephalitis; neuro-neutrophilic disease

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MDPI and ACS Style

Makimoto, G.; Manabe, Y.; Yamakawa, C.; Fujii, D.; Ikeda-Sakai, Y.; Narai, H.; Omori, N.; Abe, K. Two Cases of Possible Neuro-Sweet Disease with Meningoencephalitis as the Initial Manifestation. Neurol. Int. 2012, 4, e5. https://doi.org/10.4081/ni.2012.e5

AMA Style

Makimoto G, Manabe Y, Yamakawa C, Fujii D, Ikeda-Sakai Y, Narai H, Omori N, Abe K. Two Cases of Possible Neuro-Sweet Disease with Meningoencephalitis as the Initial Manifestation. Neurology International. 2012; 4(1):e5. https://doi.org/10.4081/ni.2012.e5

Chicago/Turabian Style

Makimoto, Go, Yasuhiro Manabe, Chizuru Yamakawa, Daiki Fujii, Yasuko Ikeda-Sakai, Hisashi Narai, Nobuhiko Omori, and Koji Abe. 2012. "Two Cases of Possible Neuro-Sweet Disease with Meningoencephalitis as the Initial Manifestation" Neurology International 4, no. 1: e5. https://doi.org/10.4081/ni.2012.e5

APA Style

Makimoto, G., Manabe, Y., Yamakawa, C., Fujii, D., Ikeda-Sakai, Y., Narai, H., Omori, N., & Abe, K. (2012). Two Cases of Possible Neuro-Sweet Disease with Meningoencephalitis as the Initial Manifestation. Neurology International, 4(1), e5. https://doi.org/10.4081/ni.2012.e5

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