"Neuro-Sweet Disease"

Abstract

We propose that there is an entity that is like Sweet disease, but with recurrent encephalitis characterized by an association with HLA-B54 and a high responsiveness to corticosteroid therapy, which we have tentatively named neuro-Sweet disease, that is distinct from the classic central nervous system involvement of Behçet disease.

Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation

“…Moreover, acute active lesions of NBD are often enhanced with gadolinium, but NSD lesions are not. 4 Both Patients #1 and Patient #2 were middle-aged women, and they responded well to steroid therapy with virtually no sequelae, suggesting that they had NSD. Also, the presence of high-intensity areas in the dorsal portion of the medulla oblongata of Patient #1 on FLAIR and diffusion-weighted images was suggestive of NSD.…”

“…This patient was also considered to have possible NSD, because she responded to steroids, and the symptoms disappeared except for slight hemiparesis and also met other diagnostic criteria for NSD. NSD often initially presents with skin symptoms: Hisanaga et al 4 reported that 27 of 29 NSD patients initially presented with cutaneous symptoms or both cutaneous and neurological symptoms, and that only 2 initially presented with neurological symptoms Our search of the literature found that 39 patients with NSD had developed meningitis, 5–12 but only 2 of them had initially presented with neurological symptoms, suggesting that such a case is rare (Table 1). In cases reported by Hisanaga et al 4 and Kokubo et al , 7 skin rashes appeared 29 and 64 months, respectively, after the onset of NSD; the diagnosis of NSD was established based on pathological findings.…”

“…These two diseases are considered to be closely related in that they are exudative immune diseases associated with a state of immune sensitivity, such as neutrophil hyperfunction. 4 NSD is characterized by: i) little gender preponderance unlike the marked male preponderance of NBD; ii) age at onset between 30 and 70 years; iii) frequent recurrence but showing a favorable response to steroids and being less likely to result in sequelae; and iv) being almost always positive for HLA-Cw1 and frequently positive for HLA-B54, unlike the high positive rate (about 80%) of HLA-B51 in NBD. 13 In addition, NBD has a predilection for the basal ganglia and brainstem, whereas NSD presents with abnormal signal intensity areas at various sites of the central nervous system at similar frequencies, and these signal abnormalities often resolve with the relief of symptoms.…”

Grammar disruption in a patient with Neuro-Sweet syndrome

“…Apart from a few studies such as Hisanaga et al (1999) in which cognition was only broadly assessed (WAIS-R total IQ) on one single occasion during a follow-up of 5 years and some anecdotal accounts of the patient's cognitive and mental state, there are no studies in which cognitive functions, behavioural symptoms or language were thoroughly investigated. To the best of our knowledge, this paper is the first to report neurolinguistic and (functional) neuroimaging 5 findings in a patient who primarily showed a range of neurobehavioural abnormalities secondary to Neuro-Sweet disease.…”

Sweet's syndrome associated with acute benign encephalitis

“…Hisanaga et al [14], described the first case of chronic recurrent encephalitis associated with Sweet's syndrome, emphasizing an association with HLA B54, which is not found in Behcet's disease, and proposed the term "neuro-Sweet disease".…”