Congenital toxoplasmosis and audiological outcome: from a case series to a suggestion of patient-based schedule.

Introduction: Sensorineural hearing loss (SNHL) has been suggested to be possibly related to congenital toxoplasmosis (CT), although its prevalence varies from 0% to 26%. This variance appears to be dependent especially on early timing of treatment. However, the available data are based on outdated studies conducted on small groups of patients that lack homogeneity. Therefore, to establish evidence-based guidelines for audiologic monitoring in CT, we conducted a comprehensive evaluation of a large case series over a long period of time. Patients and methods: This is a single-center, retrospective cohort that enrolled all infants and children who were exposed in utero to Toxoplasma gondii and/or congenitally infected between September 1980 and December 2022. They underwent standard serial audiological evaluations to detect possible SNHL at an early stage. The first evaluation was performed during the initial assessment to define the onset of congenital toxoplasmosis, with another evaluation conducted at least at 12 months of life. Results: We collected data from 1,712 patients, and 183 (10.7%) were diagnosed with CT. Among these cases, 78 children (42.6%) presented with symptomatic CT at the onset, exhibiting ocular findings (21.1%), clinical cerebral manifestations (6.1%), and/or abnormal findings on neuroimaging (35.5%). Therapy was administrated at the onset in 164 patients (89.6%) with 115 of them starting treatment prior to 2.5 months of age (0-388, median 32.00 ± 92.352 days of life). Only one patient presented with SNHL at the onset, but this was apparently unrelated to CT. The median number of audiological assessments was 2.2 ± 1.543 (2-10). No patients developed any grade of delayed hearing loss, both in treated and untreated groups. The median age at last audiological evaluation was 2.3 ± 2.18 years (1-8), although the median follow-up period was 12.4 years (±6.3), ranging from 1 to 27 years. Conclusions: Based on these data, it appears that SNHL may be less frequent in CT than previously assumed. We recommend conducting an audiological assessment at the onset (within the first 2.5 months of life) to comprehensively define the type of CT onset, and then conducting another evaluation within 9 months of life.

Development and validation of a new mealtime assessment tool (MAT) for patients with dysphagia: A cross sectional study.

PURPOSE: This study aimed to create a new quantitative and qualitative Mealtime Assessment Tool (MAT) to be administered by the not institutional caregiver to explore the dysphagic patient's mealtime behavior. METHODS: The development and validation process consisted of three steps: firstly, identification of items for the questionnaire, then pilot testing phase of the first version of the instrument and finally a psychometric evaluation in which the test was administered to the target population (adult inpatients with diagnosed and undiagnosed oropharyngeal dysphagia). RESULTS: A factorial analysis explained 51% of the variance and grouped the 12 items of the scale into three categories that defined: a) structural setting and compliance at mealtime, b) behavioral attitude at mealtime, c) structural setting as a function of possible distractibility. The concurrent validity using the Dysphagia Outcome Severity Scale and Three Oz Water Swallow Test, as the gold standard, was r =0.01. Cronbach's alpha was 0.709. Stability test-retest reliability had ICC values of 0.980 p=0.01. The ROC curve using as state variable the De Pippo test as a dichotomius variable showed the under-curve area as 0.830 (95% CI, 0.763 to 0.897). CONCLUSIONS: The validation process led to the conclusion that the MAT is a valid, reliable and stable scale for caregivers.

Effect of reactive oxygen species promoted by delta-aminolevulinic acid on porphyrin biosynthesis and glucose uptake in rat cerebellum.

1. delta-Aminolevulinic acid (ALA) has been reported to promote reactive oxygen species (ROS). Overproduction and accumulation of ALA, as it occurs in acute intermittent porphyria (AIP), can be the origin of an endogenous source of ROS, which can then exert their oxidative damage to cell structures. 2. To investigate the induction of lipid peroxidation by ALA, thiobarbituric acid reactive substances and conjugated diene formation were measured by using minimal tissue units (MTUs) obtained from rat cerebellum. Malondialdehyde levels increased with ALA concentration and incubation time (72% at 1.0 mM ALA and 127% at 4.0 mM ALA for 4 hr), and conjugated diene formation was enhanced 50% in incubations with 1.0 mM ALA for 4 hr. 3. ALA-promoted ROS by exposure of cerebellum MTUs to 1.0 mM ALA during different intervals (1-4 hr) was partly reduced by the addition of antioxidants such as superoxide dismutase (SOD; 50 U/ml), catalase (4.5 microM) and dimethylsulfoxide (150 mM), demonstrating the involvement of O2-., H2O2 and OH. in ALA autooxidation. 4. Porphobilinogen biosynthesis was 170% increased when cerebellum MTUs were incubated with 1.0 mM ALA for 4 hr in the presence of SOD, suggesting that protein damage was promoted by ALA autooxidation. 5. These findings provide the first experimental evidence of the involvement of ALA-promoted ROS in the damage of proteins related to porphyrin biosynthesis, specially ALA-D. Oxidation of this enzyme would lead to further accumulation of ALA in AIP patients, which may be the origin of the well-known neuropsychiatric manifestations.

GAP1 activity is dependent on cAMP in Saccharomyces cerevisiae.

General amino acid permease (GAP1) activity was evaluated in adenylate cyclase-deficient Saccharomyces cerevisiae to determine the effect of cAMP levels on GAP1 activity. Lowering cAMP concentrations in the culture media led to a decrease in the initial rates of L-citrulline uptake. Kinetics of the amino acid transport system showed a partial loss of transport capacity, with no apparent modifications in permease affinity.

The treatment of anomia resulting from output lexical damage: analysis of two cases.

This study describes a treatment project, carried out with two anomic subjects, RBO and GMA failed to name pictures correctly as a consequence of damage to phonological lexical forms; their ability to process word meaning was unimpaired. Words that were consistently comprehended correctly, but produced incorrectly by each subject, were identified. Some words were treated, whereas some served as the control set. A significant improvement was observed in both subjects. As predicted by the model of lexical-semantic processing used as the theoretical background for the study, improvement was restricted to treated items and did not generalize to untreated words, not even to words that were semantically related to those administered during treatment. Improvement was long-lasting, as shown by the fact that 17 months post-therapy GMA's performance on treated words was still significantly better than before treatment. These results are discussed in relation to the claim that cognitive models can be profitably used in the treatment of language disorders.

[Rehabilitation of oro-pharyngeal dysphagia of neurogenic etiology using radiological examination: preliminary results]. / Riabilitazione della disfagia oro-faringeaad eziologia neurogena guidata dall'indagine radiologica: risultati preliminari.

Dysphagia is more frequently observed in patients with neurologic diseases (stroke, bulbar or pseudo-bulbar syndrome, amyotrophic lateral sclerosis, cranial trauma). Furthermore, the presence of this pathology is obviously more frequently noted in the light of the increase in the length of the human life span. It has become evident that alternative feeding procedures such as the nasogastric tube or gastrostomy may bring about complications and deprive patients of the oral phase of deglutition which plays a leading role in stimulating digestive functions. The Authors report a systematic research on the rehabilitation aspects of neurogenous dysphagia. All the patients studied underwent a neurological examination and oropharyngeal functional evaluation using echo-videorecording of the oral phase of deglutition and fluoro-videorecording of the pharyngeal phase. The data obtained allowed for the selection of five patients considered suitable for the rehabilitation program. One of them had a multi-infarct encephalopathy, two a spastic hemiplegia f.b.c., a fourth a cerebellar syndrome and the last a sequela of meningioma removal of the ponto-cerebellar angle with peripheral paralysis of the right VII, IX, X, XI cranial nerves. This last patient also underwent a crico-pharyngeal myotomy. Therapy consisted in making the patient sensitive to swallowing movements and in training them to assume a compensatory posture as well as functional rehabilitation of the organs involved in deglutition. The first datum emerging from the study is the lack of etiological homogeneity found in the cases treated with evident variability in different deglutition organ impairment, even though there was the common denominator of the dysphagia symptom. With regard to the results obtained, there was a complete resolution in one patient, while in the other four there was such an improvement as to allow the patients a safe autonomous oral assumption of food. The positive results obtained are not only linked to the recovery of damaged organs, but also to the development of compensatory strategies such as the choice of appropriate food consistency and the assumption of postures which protect the respiratory tract from aspiration and favor crico-pharyngeal relaxation.