The maturation of the central auditory conduction in preterm infants until three months post term. III. The middle latency auditory evoked response (MLR)

Middle latency auditory evoked responses (MLRs) were recorded in 64 premature infants; serially in 54 of them. The last recording sessions occurred at 50-52 weeks conceptional age (CA), defined as the gestational age (GA) added to the chronological age. The MLRs were analyzed for the components PO, Na and Pa, and the interpeak latency difference (IPLD) Na-PO. The detectability rate of PO and Na reached 80-90% at about 30 weeks CA. Pa reached the highest rate of about 60% at 52 weeks CA. The degree of prematurity did not result in clear differences with respect to the parameter values. Also, the side of stimulation did not influence the MLR parameter values. The latency values of the MLR components are strongly age dependent. Topographic differences were found between the sides ipsi- and contralateral to stimulation. They are, however, different for PO, Na and Pa. The influence of the state of vigilance on the parameter values could generally only be established at CA levels of about 32 and 52 weeks CA. The amplitude values at 52 weeks CA are especially sensitive for sleep or awake state. The particular pattern of age dependency of the different components and their topographic differences are consistent with a differential generation of bilateral nature. The early appearance of the response supports the generation of an early functioning subcortical structure in the auditory pathway.     

Abnormal EMG and SSEP in a young child with an ependymoma

We report our findings on a case of ependymoma in a one-year-old boy. A partial paresis of the left arm was found but the EEG and BAEP were normal. The EMG showed fibrillations and positive sharp waves in the paretic muscles and the SSEP showed a far field negativity. After removal of the cerebral process all neurophysiologic findings normalized. We discuss a hypothesis for the established clinical neurophysiological findings.     

Immunogenic properties of octasaccharide-protein conjugates derived from Klebsiella serotype 11 capsular polysaccharide.

The tetrasaccharide repeating unit of the capsular polysaccharide of Klebsiella serotype 11, K11PS, comprises the following sequence: [----3)-beta-D-GlcpA-(1----3)-alpha-D-Galp-(1----3)-beta-D-Glcp-(1 ----] with a 4,6-O-(1-carboxyethylidene)-alpha-D-galactopyranosyl residue linked to O-4 of the glucuronic acid residue. Octasaccharide (OS) derived from K11PS by bacteriophage phi 11-associated glycanase, was coupled to bovine serum albumin and to keyhole limpet hemocyanin. The immunogenicity of various antigens after intraperitoneal immunization was studied by measuring the levels of circulating antibodies. Injection of BALB/c mice with K11PS resulted in induction of 2-mercaptoethanol-sensitive immunoglobulin M antibodies. The responses observed in BALB/c nu/nu mice and in male (CBA/N X C3H/HeN)F1 mice indicate that K11PS is a thymus-independent type 2 antigen. Immunization of BALB/c mice with either OS-bovine serum albumin or OS-keyhole limpet hemocyanin resulted in the induction of circulating 2-mercaptoethanol-resistant immunoglobulin G antibodies. Results in BALB/c nu/nu mice indicate that the OS-protein conjugates are thymus-dependent antigens. Since the OS-keyhole limpet hemocyanin conjugate induced antibodies in both (CBA/N X C3H/HeN)F1 females and males, we propose to refer to this kind of antigen as a thymus-dependent type 1 antigen, whereas OS-bovine serum albumin, which evoked immunoglobulins in (CBA/N X C3H/HeN)F1 females only, can be referred to as a thymus-dependent type 2 antigen.     

Sjögren-Larsson syndrome: clinical and MRI/MRS findings in FALDH-deficient patients

OBJECTIVE: To determine the spectrum of clinical and MRI/1H MRS features of patients with fatty aldehyde dehydrogenase (FALDH) deficiency. BACKGROUND: The Sjogren-Larsson syndrome (SLS) was originally defined as a clinical triad consisting of ichthyosis, spastic di- or tetralegia, and mental retardation, with autosomal recessive inheritance. By now, both the deficiency of the enzyme FALDH, and the genetic mutations on chromosome 17 responsible for this deficiency, have been identified. SLS, defined by fibroblast FALDH deficiency, seems to be a much broader syndrome. METHODS: The clinical findings of 11 FALDH-deficient patients of different ages and one patient with the characteristic SLS-like ichthyosis, but without FALDH deficiency, were evaluated in relation to their cerebral MRI, and to 1H MRS in six patients. RESULTS: The severity of neurologic symptoms showed considerable variation. Fundoscopic perifoveal glistening dots and the characteristic SLS-like ichthyosis were present in all patients. Serial MRI findings showed evidence of retarded myelination and a variable degree of dysmyelination. 1H MRS showed an accumulation of free lipids in the periventricular white matter, even before the stage of visible dysmyelination. CONCLUSIONS: The neurologic consequences of FALDH deficiency show considerable variation. The characteristic pattern of ichthyosis and retinal degeneration are seen consistently, yet they are not pathognomonic. MRI and 1H MRS findings suggest an accumulation of long-chain fatty alcohol intermediates, resulting in retarded myelination and dysmyelination.     

Responsiveness of the generic EQ-5D summary measure compared to the disease-specific EORTC QLQ C-30

Introduction: We investigated whether the sensitivity of the generic health-related quality of life (HRQoL) EQ-5D summary measure (or index) to detect changes over time in a clinical setting is comparable with that of a disease-specific HRQoL questionnaire. Methods: Patients with liver metastases (n = 75) filled out the five domains of the EQ-5D self-classifier, the EQ VAS, and the EORTC QLQ C-30 (a disease-specific (cancer) HRQoL questionnaire). The HRQoL instruments were completed before intervention, and 1/2 month and 3 and 6 months after intervention. Three analyses were performed. First, the EQ-5D index (based on self-classification) was compared to the EQ VAS. Second, the EQ-5D domains were compared to corresponding EORTC QLQ C-30 scales. Third, EQ-5D index and EQ VAS were compared with the EORTC QLQ C-30 global health-status scale. Effect size was chosen as the metric of responsiveness. Results: The EQ-5D index was slightly less responsive than the EQ VAS. Overall, the responsiveness of the EQ-5D index and EQ VAS was equal to the EORTC QLQ C-30 global health-status scale. Conclusion: Despite its generic principle and the apparent crudeness of its framework, the responsiveness of the EQ-5D proved to be comparable to that of a disease-specific HRQoL questionnaire in this specific clinical setting.     

Idiopathic neuralgic amyotrophy in children. A distinct phenotype compared to the adult form

Two cases of neuralgic amyotrophy (idiopathic brachial plexus neuropathy) in children are presented and combined with a review of the literature. Difficulties in establishing the diagnosis are illustrated, and we give an overview of the phenotype of childhood neuralgic amyotrophy and its distinctions from the adult type. Pain, in adult cases present in over 95% of the cases, is less frequent in children, and its absence by no means excludes the diagnosis. In children under 8 weeks of age, the literature shows that a subsequent osteomyelitis of the shoulder or arm always seems to be involved, which warrants a close follow-up. Overall, recovery is less favourable in children, but when they fully recover they seem to do so in a shorter period of time than adults. We conclude that neuralgic amyotrophy in children is distinct from the adult variety, and that it has a definite place in the differential diagnosis of a sudden limp arm, even if it is painless.     

Societal Effects Are a Major Factor for the Uptake of the Coronavirus Disease 2019 (COVID-19) Digital Contact Tracing App in The Netherlands

ObjectivesOur study investigates the extent to which uptake of a COVID-19 digital contact-tracing (DCT) app among the Dutch population is affected by its configurations, its societal effects, and government policies toward such an app.MethodsWe performed a discrete choice experiment among Dutch adults including 7 attributes, that is, who gets a notification, waiting time for testing, possibility for shops to refuse customers who have not installed the app, stopping condition for contact tracing, number of people unjustifiably quarantined, number of deaths prevented, and number of households with financial problems prevented. The data were analyzed by means of panel mixed logit models.ResultsThe prevention of deaths and financial problems of households had a very strong influence on the uptake of the app. Predicted app uptake rates ranged from 24% to 78% for the worst and best possible app for these societal effects. We found a strong positive relationship between people’s trust in government and people’s propensity to install the DCT app.ConclusionsThe uptake levels we find are much more volatile than the uptake levels predicted in comparable studies that did not include societal effects in their discrete choice experiments. Our finding that the societal effects are a major factor in the uptake of the DCT app results in a chicken-or-the-egg causality dilemma. That is, the societal effects of the app are severely influenced by the uptake of the app, but the uptake of the app is severely influenced by its societal effects.     

Inflammatory biomarkers at hospital discharge are associated with readmission and death in patients hospitalized for COVID-19

European Journal of Clinical Microbiology & Infectious Diseases -