RESULTS OF THE COLLABORATIVE STUDY ON NEUROFIBROMATOSIS TYPE-1 (NF1) IN ITALY

The results of a collaborative Italian study on Neurofibromatosis type 1 (NF1) among the Pediatric Institute of Siena and 63 Italian Pediatric Institutions are reported. Data regarding 375 NF1 cases have been obtained from the Italian Registry of Neurocutaneous Syndromes established in 1987. The study allowed us to obtain data about the frequency of the main findings of the disease. Some of these findings, such as macrocephaly and multiple areas of increased signal intensity on T2-weighted images at brain MRI (Unidentified Bright Objects or UBOs) are not included in the diagnostic criteria; however they appear to be important from the diagnostic point of view because of their high incidence. UBOs have been observed in 56% of cases in which MRI was performed and, since they did not show an invasive nature, it is important that these images are well known in order to avoid a misdiagnosis of cerebral tumors. DNA linkage analysis, using probes linked to NF1 locus in 9 families with 2 or 3 generations of NF1-patients, indicated that 6 of these families resulted informative and a prenatal diagnosis would be possible whenever requested. The same molecular study allowed us to exclude the disease in 3 at risk individuals coming from 3 different families. Two recent international Seminars and the istitution of the Italian Association of Neuro Ectodermosis (A.I.N.E.) have greatly contribute to improve the knowledge about the genetic and clinic implications of the disease among physicians and general population. We think useful to obtain a widespread collaboration among all physicians who take care of NFI-patients and to operate in order to obtain from the politic authorities an adequate assistence to these patients and their families

Understanding Factors Associated With Psychomotor Subtypes of Delirium in Older Inpatients With Dementia

Objectives: Few studies have analyzed factors associated with delirium subtypes. In this study, we investigate factors associated with subtypes of delirium only in patients with dementia to provide insights on the possible prevention and treatments. Design: This is a cross-sectional study nested in the “Delirium Day” study, a nationwide Italian point-prevalence study. Setting and Participants: Older patients admitted to 205 acute and 92 rehabilitation hospital wards. Measures: Delirium was evaluated with the 4-AT and the motor subtypes with the Delirium Motor Subtype Scale. Dementia was defined by the presence of a documented diagnosis in the medical records and/or prescription of acetylcholinesterase inhibitors or memantine prior to admission. Results: Of the 1057 patients with dementia, 35% had delirium, with 25.6% hyperactive, 33.1% hypoactive, 34.5% mixed, and 6.7% nonmotor subtype. There were higher odds of having venous catheters in the hypoactive (OR 1.82, 95% CI 1.18-2.81) and mixed type of delirium (OR 2.23, CI 1.43-3.46), whereas higher odds of urinary catheters in the hypoactive (OR 2.91, CI 1.92-4.39), hyperactive (OR 1.99, CI 1.23-3.21), and mixed types of delirium (OR 2.05, CI 1.36-3.07). We found higher odds of antipsychotics both in the hyperactive (OR 2.87, CI 1.81-4.54) and mixed subtype (OR 1.84, CI 1.24-2.75), whereas higher odds of antibiotics was present only in the mixed subtype (OR 1.91, CI 1.26-2.87). Conclusions and Implications: In patients with dementia, the mixed delirium subtype is the most prevalent followed by the hypoactive, hyperactive, and nonmotor subtype. Motor subtypes of delirium may be triggered by clinical factors, including the use of venous and urinary catheters, and the use of antipsychotics. Future studies are necessary to provide further insights on the possible pathophysiology of delirium in patients with dementia and to address the optimization of the management of potential risk factors