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

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.

Pearls & Oy-sters: Neuro-Sweet disease presenting as ischemic stroke and aseptic meningitis

“…Cerebral infarction in NSD has been reported in 3 other cases previously. 3,4 The latter 2 cases presented similarly to our patient, such that there was neutrophilic meningoencephalitis with stroke in the absence of cutaneous findings. In another case of NSD, 5 the patient presented with headaches and fatigue and was found to have several areas of T2-weighted hyperintensities that enhanced with gadolinium.…”

Increase of a wide range of bioactive substances in an active phase of neuro-Sweet disease

“…25 Neurological involvement usually follows skin lesions, but it can also be an initial manifestation of NSD. 26 The development of neurological symptoms in our patient's case was rapid compared with past reports. 27 28 NSD can mimic NBD, and it appears that NSD cannot be differentiated from NBD by neurological symptoms alone.…”

Neuro‐Sweet's disease

“…Diagnosing NSD is difficult without rash, as the diagnosis hinges on skin lesions. However, the rash does not necessarily coincide with neurologic symptoms . In such possible NSD cases, careful follow‐up for the development of skin lesions is important.…”