L-Dopa-responsive Parkinson's syndrome in association with phenylketonuria: In vivo dopamine transporter and D2 receptor findings.

Reports of parkinsonism in phenylketonuria are exceedingly rare. We report on a patient who had received a delayed diagnosis of phenylketonuria as an infant and subsequently developed levodopa-responsive parkinsonism at the age of 33. Single-photon emission computed tomography (SPECT) using (123)I-FP-CIT ([(123))I]-2 beta-carbomethoxy-3beta-(-4-iodophenyl)-N-(3-fluoropropyl)-nortropane) used to measure dopamine transporter levels on two occasions, 7 and 9 years after the onset of neurological symptoms, were normal. Iodine-123-iodo-lisuride SPECT (IBZM) imaging, however, showed reduced caudate over putamen binding. This combination of imaging findings indicates a possible upregulation of postsynaptic D2 receptors in the context of intact presynaptic dopamine nerve terminal density.     

Fetal magnetic resonance imaging in the prenatal diagnosis of cerebellar hemorrhage.

We report the case of a fetus with a sonographic mid-gestation diagnosis of hyperechogenic cerebellum suspected to be of hemorrhagic origin on fetal brain magnetic resonance imaging (MRI). No etiological factors for fetal hemorrhage were found other than a maternal heterozygocity for factor V Leiden. Following termination of the pregnancy, autopsy confirmed the prenatal diagnosis of massive cerebellar hemorrhage without underlying vascular anomaly. As an additional tool to ultrasonography, fetal brain MRI can affirm the hemorrhagic origin of hyperechogenic cerebellar lesions, especially by showing a high signal on T1-weighted images.     

Morphological analysis of the acute effects of overdistension on the extracellular matrix of the rat urinary bladder wall.

PURPOSE: To investigate the morphological effects of acute overdistension in the structure of the extracellular matrix of the bladder wall in rats. MATERIALS AND METHODS: The bladders of a group of 6 male Wistar rats were transurethrally overdistended for 3 hours. Another identical group (the control group) was only submitted to a sham operation. Specimens from the bladder dome were analyzed with light microscopy (LM), transmission electron microscopy (TEM) and scanning electron microscopy (SEM). RESULTS: LM--The control group bladders had a 4 to 5 layer urothelium, a lamina propria, and a smooth muscle layer with longitudinal and transversal fibers. The overdistended bladders presented an intense interstitial infiltrate in the lamina propria, and a less intense infiltrate among the smooth muscle fibers. TEM--The cells of the overdistended bladders had a significant amount of vacuoles, unlike the control bladders, where such vacuoles were scarce or absent. SEM--A delicate three-dimensional mesh of collagen fibrils was observed in the lamina propria of the bladder walls from the control group. Whilst for the control group this mesh consisted of distinct geometric structures, with mostly circular cellular spaces surrounded by the fibrils, the overdistended group showed evidence of distortion of the mesh, with flattened and elongated cellular spaces. CONCLUSIONS: Acute bladder overdistension induces structural modifications, altering the arrangement and interaction of collagen fibrils, as well as incipient tissue damage as edema in the lamina propria and smooth muscle layers.     

Colonization with methicillin-resistant Staphylococcus aureus after liver transplantation.

Methicillin-resistant Staphylococcus aureus (MRSA) is a frequent cause of infection after orthotopic liver transplantation (OLT). Colonization with MRSA is associated with a higher risk of infection. Previous studies have shown a high prevalence of MRSA colonization among OLT candidates. However, the risk of colonization with MRSA after OLT is still unclear. The objective of this study was to estimate the incidence and the factors associated with colonization with MRSA after OLT. This was a prospective cohort study including patients submitted to OLT between the years 2000 and 2002. Surveillance cultures of nasal swab specimens were performed within the 1st 72 hours of hospital admission and, subsequently, on weeks 2, 6, 13, and 26. Patients whose baseline cultures revealed nasal carriage of MRSA were excluded. A total of 60 patients were included in the study. The median follow-up was 72 days. A total of 9 patients (15%) became colonized. In multiple logistic regression analyses, the use of a urinary catheter for > or =5 days (P = .006), postoperative bleeding at the surgical site (P = .009), and preoperative use of fluoroquinolones (P = .08) were associated with a higher risk of colonization. Patients without any of these risk factors did not become colonized. In conclusion, nasal carriage of MRSA is frequently acquired after OLT. Periodic postoperative screening for MRSA carriage should be an integral component in programs designed to reduce nosocomial MRSA transmission in these patients. Further studies are needed to set up and validate a predictive model that could allow targeting postoperative screening to high-risk OLT recipients.     

Stability of skeletal Class III malocclusion after combined maxillary and mandibular procedures: titanium versus resorbable plates and screws for maxillary fixation.

PURPOSE: The aim of this study was to evaluate skeletal stability after double jaw surgery for correction of skeletal Class III malocclusion to assess if there were any differences between resorbable plate and screws and titanium rigid fixation of the maxilla. PATIENTS AND METHODS: Twenty-two Class III patients had bilateral sagittal split osteotomy for mandibular setback stabilized with rigid internal fixation. Low level Le Fort I osteotomy for maxillary advancement was stabilized with conventional titanium plate and screws in 12 patients (group 1) and with resorbable plate and screws in 10 patients (group 2). Lateral cephalograms were taken before surgery, immediately postoperatively, 8 weeks after surgery, and 1 year postoperatively. RESULTS: Before surgery both groups were balanced with respect to linear and angular measurements of craniofacial morphology. One year after surgery, maxillary stability was excellent in both groups. In group 1 no significant correlations were found between maxillary advancement and relapse. In group 2, significant correlations were found between maxillary advancement and relapse at A point and posterior nasal spine. No significant differences in postoperative skeletal and dental stability between groups were observed. CONCLUSION: Surgical correction of Class III malocclusion after combined maxillary and mandibular procedures appears to be a fairly stable procedure for maxillary advancements up to 5 mm independently from the type of fixation used to stabilize the maxilla. Resorbable devices should be used with caution for bony movements of greater magnitude until their usefulness is evaluated in studies with large maxillary advancements.     

Gastro-oesophageal and bile reflux--simultaneous quantitative assessment with gastric and gallbladder emptying evaluation: clinical applicability of a new computerized gammagraphic method.

The aim of this study was to develop a new computerized gammagraphic method to evaluate gastrooesophageal (GER), bileo-oesophageal (BER) and bileogastric reflux (BGR) simultaneously with gastric and gallbladder emptying: a long duration (60 min) and a dual energy detection (113Inm-sulphur colloid and 99Tcm-HIDA) test of the different refluxes during slow gastric emptying of an enteric feeding liquid meal. Forty patients with oesophagitis and 18 normal volunteers were evaluated. Good reproducibility of all the quantified parameters was found with r Spearman between 0.75 (P < 0.05) and 1.0 (P < 0.001). Patients with oesophagitis have a tendency for slower gastric emptying patterns (gastric emptying T1/2, GT1/2 P < 0.05). The calculated specificity for gallbladder emptying parameters was 77.8% for the gallbladder T1/2 BT1/2) and 94.4% for gallbladder residue at 60 min (BR60). The calculated accuracy for the GER index was 90%. The predictability for positive values was 95%, owing to a 90% sensitivity and 89% specificity. The predictability for negative values was 80%. The mean GER index in oesophagitis was greater than in controls (P < 0.001). The calculated specificity for BGR was 94.4% and reflux was detected in 12 out of 40 patients. The calculated specificity for BER was 83.3% for a '+' index and 94.4% for a '++' BER index. In 14 patients a positive BER index has been determined ('+' in three and '++' in 11 cases).(ABSTRACT TRUNCATED AT 250 WORDS)     

The brain as a target for adrenocortical steroids: cognitive implications.

It is well established that a reciprocal control exists between the brain and glucocorticoid hormones. The brain regulates adrenocortical function via hypothalamic corticotrophin releasing hormone-41 (CRH-41), glucocorticoids act at specific receptors in the hippocampus, thus promoting negative feedback mechanisms. Because the hippocampus is a major site for memory processes, a role for excessive/long-lasting plasma glucocorticoid levels has been suggested in conditions of mental impairment. Major depression, Cushing's disease, and dementia of the Alzheimer type are disorders which share hyperactivity of the hypothalamo-pituitary-adrenal axis, as well as symptoms of cognitive decline. Although the mechanisms leading to hypercortisolemia appear to be different in each case, the neuropsychological features of these three disorders accord with the hypothesis of glucocorticoid-associated brain damage. It therefore is important to find pharmacological strategies that will avert or reduce these potential consequences on brain function.     

Skin cancers and precancerous lesions in Parkinson's disease patients.

Our objective was to evaluate the frequency of neoplastic and preneoplastic skin lesions in Parkinson's disease (PD) patients when compared with an aged-matched population. We performed a cross-sectional survey in PD patients and in an age-matched control group. Patients and controls were examined by a movement disorder specialist and a dermatologist. 150 PD patients and 146 controls were included. Thirty-five PD patients (23.3%) presented skin lesions that could be classified as neoplastic or preneoplastic vs. 20 subjects in the control group (13.7%) (OR 95%, CI 1.92 [1.05, 3.51]). However, this difference lost statistical significance when adjusted for gender (recruitment of controls was matched just for age with an over representation of males in the PD group). Twenty-nine PD patients (19%) presented actinic keratosis and basal cell carcinoma was diagnosed in 4 patients (3%). Although nonconclusive, our results are in agreement with previous studies suggesting an increased risk of skin cancer in PD patients. The frequency of actinic keratosis in PD patients and the associated risk to develop melanoma recommends its screening in future epidemiological studies.     

Peripheral Foot Blockade Versus Popliteal Fossa Nerve Block: A Prospective Randomized Trial in 51 Patients

The majority of foot and ankle operations are performed on an outpatient basis and often under some form of regional anesthesia. In this prospective, randomized study of 51 patients undergoing elective unilateral forefoot procedures, we compared 2 different anesthetic techniques: the peripheral foot blockade and the popliteal sciatic nerve block. Variables assessed included the quality of surgical anesthesia, postoperative analgesia, and the incidence of postoperative complications. The anesthesia was classified as effective if it was the sole anesthetic technique for the forefoot surgery. We found successful results in both groups: 92% in the foot block group and 96% in the popliteal block group. Analysis of time required to perform the anesthetic procedure showed a significant difference between the 2 groups, with foot block being considerably faster (14.3 minutes vs 19.2 minutes for popliteal block) (P = .0078). Foot block patients demonstrated 10.96 hours of analgesia, whereas popliteal block patients exhibited 14.32 hours (P = .132). With a mean follow-up of 5.7 months, we did not find anesthesia-related complications in any of the patients. Both techniques showed a high level of safety and efficacy, with no significant difference detected between them. Our patients showed a high rate of satisfaction with both procedures (96% for foot block patients and 96.1% for popliteal block patients) and reported a good discharge disposition. These data show that both procedures are safe and effective anesthetic techniques and well suited to forefoot ambulatory surgery.