title: Reversible extensive leukoencephalopathy in Sweet disease: a case report.
abstract: Sweet disease, also known as acute febrile neutrophilic dermatosis, is an idiopathic multisystem inflammatory disorder characterised by erythematous skin lesions and fever. "Neuro-Sweet disease" is a rare central nervous system involvement that coexists with the characteristic cutaneous lesions of Sweet disease. Here, we report a case of Sweet disease complicated with acute encephalopathy. This case showed extensive lesions in the cerebral white matter on magnetic resonance imaging (MRI) and high protein concentration in the cerebrospinal fluid (CSF) without pleocytosis. After steroid therapy, the patient's clinical syndrome recovered completely with no significant neurological deficits or abnormal findings on brain MRI, and normalization of the high CSF protein concentration. Both clinical and MRI findings suggested that the marked leukoencephalopathy in this case was mainly due to reversible oedema rather than destructive structural alterations in the cerebral parenchyma.
authors.affiliation: Department of Internal Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan. fuku@hsp.md.shinshu-u.ac.jp
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Fukushima
authors.forename: Akiyo
authors.initials: A
authors.lastname: Hineno
authors.forename: Minori
authors.initials: M
authors.lastname: Kodaira
authors.forename: Kazuko
authors.initials: K
authors.lastname: Machida
authors.forename: Wataru
authors.initials: W
authors.lastname: Ishii
authors.forename: Tomoki
authors.initials: T
authors.lastname: Kaneko
authors.forename: Hisashi
authors.initials: H
authors.lastname: Shimojo
authors.forename: Hisashi
authors.initials: H
authors.lastname: Uhara
authors.forename: Kanji
authors.initials: K
authors.lastname: Yamamoto
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Morita
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yoshida
authors.forename: Shu-ich
authors.initials: SI
authors.lastname: Ikeda
mesh_terms: D002556:Cerebrospinal Fluid Proteins / Q000378:metabolism; D006801:Humans; D007564:Japan; D007964:Leukocytosis / Q000150:complications; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D054038:Posterior Leukoencephalopathy Syndrome / Q000134:cerebrospinal fluid / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D013256:Steroids / Q000627:therapeutic use; D016463:Sweet Syndrome / Q000134:cerebrospinal fluid / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D055815:Young Adult
chemical_list: D002556:Cerebrospinal Fluid Proteins; D013256:Steroids