POEMS syndrome is a potentially fatal disease, and prediction of prognostic factors for POEMS syndrome is important for clinicians. Papilloedema is an early sign of the syndrome. The present study was carried out to evaluate whether papilloedema is a prognostic factor for POEMS syndrome. Between January 2003 and December 2012, 94 patients with POEMS syndrome were enrolled in this study. The patients were divided into groups with and without papilloedema. Logistic regression was performed to identify risk factors related to papilloedema. Prognostic factors were analysed with Cox proportional hazard regressions for POEMS syndrome-related prognoses, and survival curves were plotted using the Kaplan-Meier method and compared by the log-rank test. Papilloedema was found in 52.1 % of the patients with POEMS syndrome. Raised intracranial pressure [hazard ratio (HR) 3.06, 95 % confidence interval (CI) 1.24-7.41; P = 0.011] and elevated cerebrospinal fluid protein levels (HR 2.03, 95 % CI 1.07-4.51; P = 0.043) were independently associated with papilloedema. Papilloedema, decreased diffusing capacity of the lung for carbon monoxide (DLCO) and treatment with corticosteroids alone were related to poor prognosis in POEMS syndrome. In multivariate analysis, papilloedema (HR 1.58, 95 % CI 1.05-2.46; p = 0.027) and decreased DLCO (HR 2.17, 95 % CI 1.12-3.39; p = 0.023) were independent factors for POEMS syndrome-related death. Papilloedema and decreased DLCO are important prognostic factors for patients with POEMS syndrome, which can help clinicians predict the risk of mortality and provide better medical care for these patients.

Background and purposeWe investigated the baseline demographics of patients with severe unilateral atherosclerotic stenosis of the middle cerebral artery (MCA) using multimodal MRI and evaluated the haemodynamic impairments and plaque characteristics of patients who had a recurrent stroke.Materials and methodsWe retrospectively recruited consecutive patients with severe unilateral atherosclerotic MCA stenosis who underwent arterial spin labelling (ASL) with postlabelling delay (PLD) of 1.5 and 2.5 s, and vessel wall MRI. For each PLD, cerebral blood flow (CBF) maps were generated. Hypoperfusion volume ratio (HVR) from 2 PLD CBF was calculated. An HVR value ≥50% was considered as severe HVR. Plaque areas, plaque burden, plaque length and remodelling index were measured. Plaque enhancement at maximal lumen narrowing site were graded. Baseline clinical and imaging characteristics were compared between patients with (event+) and without (event−) 1 year ischaemic events.ResultsForty-three patients (47.23±12.15 years; 28 men) were enrolled in this study. Seven patients had an HVR ≥50%. During the 1-year follow-up, 7 patients had experienced a recurrent stroke. HVR were significantly higher in the event+ than event− (53.17%±29.82% vs 16.9%±15.57%, p=0.0002), whereas no significant difference was detected in plaque areas, plaque burden, remodelling index, plaque length and plaque enhancement grade. The multivariable analysis revealed that a severe HVR was significantly associated with a recurrent stroke (Odds ratio=12.93, 95% confidence interval 1.57 to 106.24, p=0.017) after adjusted by hypertension and smoking.ConclusionHVR obtained from two PLD ASL may be a useful imaging predictor of recurrent stroke.

The purpose was to assess the difference of collaterals in symptomatic versus asymptomatic patients with unilateral middle cerebral artery (MCA) stenosis by comparing cerebral blood flow (CBF) at two post labeling delays (PLD) using three-dimensional pseudo-continuous arterial spin labeling (3D pCASL). Eighty-one patients (49 symptomatic and 32 asymptomatic) with unilateral MCA stenosis ≥50% who underwent pCASL with two PLDs were included. Mean CBF and CBF subtraction images between two PLDs of MCA territories were compared in symptomatic and asymptomatic groups, respectively. Compared with the asymptomatic group, patients with symptomatic MCA stenosis had significantly lower CBF in the MCA territory of stenotic side at each PLD. The CBF of stenotic territory showed greater increase than that of normal side from PLD 1.5 to 2.5 s. The CBF of asymptomatic MCA territory increased similarly with that of symptomatic MCA territory from PLD of 1.5 to 2.5 s in stenotic side, while symptomatic patients experienced significantly slower antegrade flow. On CBF subtraction images, asymptomatic patients showed larger volume of differences between PLD of 1.5 and 2.5 s compared with those of symptomatic patients ( p = 0.037). The results suggest that more robust collateral perfusion on two-delay 3D pCASL is present in asymptomatic patients compared with symptomatic patients.

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NB should be kept in mind in patients with autonomic dysfunction, especially disorders of urination and defecation. Hearing loss due to vestibulocochlear nerve injury seems to be typical for NB. The high incidence of sequelae may be related to a long disease course and the involvement of the central nervous system. Early detection, diagnosis and treatment could decrease mortality and sequelae.