Worster-Drought syndrome (WDS), also known as congenital suprabulbar paresis, is a rare neurodevelopmental disorder characterized by feeding, swallowing, drooling, and speech disturbances. Currently, it is classified as a subtype of cerebral palsy. However, the limited number of studies and the clinical and radiological overlap with related entities such as congenital bilateral perisylvian syndrome (CBPS) and Foix-Chavany-Marie syndrome (FCMS) have contributed to persistent uncertainty regarding its proper classification. In this review, we summarize the current knowledge on the WDS based on data from published case series. Special emphasis is placed on proposed etiological mechanisms, including recent genetic findings potentially contributing to WDS, as well as on the diagnostic process, ongoing classification dilemmas, and spectrum-based perspective. We point out the need to establish standardized diagnostic criteria and conduct large-scale genetic and neurodevelopmental research. Addressing these gaps may help clarify the underlying pathophysiology, reappraise the classification framework, and ultimately minimize misdiagnosis and time to proper diagnosis to improve outcomes for individuals affected by WDS.
Background: Several strategies such as premedication or allergologic work-up have been used to facilitate safe iodinated contrast media (ICM) application. The aim of this study was to assess the negative predictive value of skin tests and complete allergologic work-up with iodinated contrast media in real-life settings. Methods: We retrospectively enrolled 210 patients with a history of immediate hypersensitivity reactions to ICMs. The stepwise protocol of selecting safe ICMs included skin prick tests (SPTs), intracutaneous tests (ICTs), and intravenous drug provocation tests (DPTs). The tests were performed with one or more of the following medications: iohexol, iodixanol, iomeprol, iopromide, and ioversol. At least 6 months after selecting safe ICMs, a telephone follow-up was scheduled. Based on this data, negative predictive values of skin tests and the whole work-up were calculated. Results: Overall, we obtained the following rates of positive tests: SPT—12% (55/441); ICT—10% (40/383); and DPT—5% (10/195). The highest rates of positive work-ups were found in patients tested within 1 year after the reaction and patients with a history of urticaria/angioedema after ICMs. The skin tests and DPTs determined a safe ICM in 184/210 (87%) patients. The negative predictive value of skin tests in reference to DPTs in hospital settings was 95%. Finally, a telephone follow-up demonstrated that 75/184 participants (41%) had been given the recommended ICM, including 68 cases (37%) with no subsequent hypersensitivity reaction and 7 (4%) with a reaction. The negative predictive value of allergologic work-up consisting of SPTs, ICTs and DPTs in real-life settings was 90%. Conclusions: Skin tests and intravenous drug provocation tests with ICMs are characterised by high negative predictive value, and enable the determination of safe ICMs for most patients.