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.     

Critical ovarian hyperstimulation syndrome in a coasted in-vitro fertilization patient

We report an instance of critical ovarian hyperstimulation syndrome in a highly responsive in-vitro fertilization patient despite the preventive measure of a 4 day 'coast' interval during which no gonadotrophins were administered while gonadotrophin-releasing hormone agonist therapy continued until serum oestradiol concentrations fell below 3000 pg/ml.      

Pemphigus vulgaris: the role of IL-1 and IL-1 receptor antagonist in pathogenesis and effects of intravenous immunoglobulin on their production

Intravenous immunoglobulin (IVIG) is increasingly being used for the treatment of autoimmune diseases. In the present report, the role of IVIG on in vivo and in vitro production of IL-1 and IL-1 receptor antagonist (Ra) was studied in patients with pemphigus vulgaris (PV). Serum samples from 20 untreated patients with active PV prior to initiation of systemic therapy, 20 patients receiving IVIG treatment, 20 patients in clinical remission after conventional therapy, and 20 normal human controls were studied to determine the serum levels of IL-1alpha, IL-1beta, and IL-1Ra. The in vitro production of these cytokines was measured in the culture supernatant of peripheral blood mononuclear cells (PBMC) from 10 PV patients immediately before and after IVIG therapy and from age and sex-matched 10 healthy donors simultaneously. Elevated levels of IL-1alpha and IL-1beta were detected (i) in the serum of untreated PV patients with active disease prior to systemic therapy and (ii) before IVIG infusions in patients receiving IVIG therapy. These increased levels are statistically significant when compared to the levels in healthy controls (P < 0.01). A marked reduction of IL-1alpha and IL-1beta was detected (i) in the serum of patients in prolonged clinical remission and (ii) immediately after IVIG infusion in those patients on IVIG therapy. Increased level of IL-1Ra was detected in PV patients in prolonged clinical remission and after IVIG infusion in those receiving IVIG therapy. These differences were statistically significant when compared to the levels in normal controls and to the levels in the sera of patients with active disease (P < 0.01) or just before the beginning of IVIG infusion (P < 0.01). Similar differences in the levels of IL-1alpha, IL-1beta, and IL-1Ra were found in the culture supernatant of PBMC isolated from the PV patients pre and post IVIG therapy. These observations suggests that, compared to normal controls, patients with active PV have reversed levels of IL-1alpha, IL-1beta, and IL-1Ra. IVIG therapy may down-regulate production of IL-1alpha and IL-1beta and enhance production of IL-1Ra, in vivo and in vitro. This might be one of the important mechanisms by which IVIG produces its early therapeutic effects in pemphigus vulgaris.     

Extensive somatosensory innervation in infants with obstetric brachial palsy

In the pre-operative screening of infants with obstetric brachial palsy (OBP), the results of routine electromyography are often overly optimistic when compared to the peri-operative findings. This prompted us to include investigation of the sensory innervation of these infants using the N20 (the first cortical response to a peripheral stimulation) of the somatosensory evoked potentials (SSEP). Three to seven months after birth, SSEP were recorded at the skull after stimulation of the thumb and middle finger in infants with obstetric rupture of the upper trunk or avulsion of roots C5, C6, or C7, and in whom no clinical improvement of motor function was observed in the biceps brachii and deltoid muscles. In most infants, a normal N20 could be evoked, indicating the existence of peripheral sensory pathways. From the thumb, these sensory pathways would necessarily bypass the upper trunk and dorsal roots of spinal nerves C5 and C6, and from the middle finger bypass the middle trunk and dorsal root C7, before extending into the dorsal column and projecting toward the thalamus and cerebral cortex. These data suggest that in infancy the segmental sensory innervation of the hand is more diverse than is described in most textbooks.     

Mycoplasma pneumoniae-associated nephritis in children

 Mycoplasma pneumoniae infection is a rare cause of acute nephritis. Six children (2 girls) aged 5–10 years, admitted for nephritis, had serological tests showing recent Mycoplasma pneumoniae infection. The diagnosis of Mycoplasma pneumoniae infection was based on the presence of serum IgM, detected either by immunofluorescence (IF) (n=1) or enzyme-linked immunosorbent assay (n=5). Four children had a renal biospy, with analysis of parenchymal Mycoplasma pneumoniae components by indirect IF and polymerase chain reaction. Extrarenal symptoms were: respiratory (n=3), ear, nose, and throat (n=2), gastrointestinal (n=3), hepatic (n=1), neurological (n=1), articular (n=1), and hematological (n=3). The patients presented with acute nephritis (1 had a nephrotic syndrome) or with acute renal failure and proteinuria. Pathological findings included type 1 membranoproliferative glomerulonephritis (MPGN, n=1), proliferative endocapillary glomerulonephritis (n=2), and minimal change disease (n=1). The patient with type 1 MPGN progressed rapidly towards end-stage renal failure because of a congenital solitary kidney. Among the patients with endocapillary glomerulonephritis, 1 relapsed 6 months later and remained proteinuric, while the other recovered, as did the child with minimal change disease. The search for Mycoplasma pneumoniae antigens and nucleic acids in renal tissue was negative. However, the absence of the microorganism in the kidney is a common feature of post-streptococcal glomerulonephritis. We conclude that Mycoplasma pneumoniae is a rare yet potential cause of acute glomerulonephritis. Received: 13 September 1996 / Revised: 16 June 1998 / Accepted: 18 June 1998     

Practice parameters for the identification and testing of patients at risk for dominantly inherited colorectal cancer--supporting documentation

It should be recognized that these guidelines should not be deemed inclusive of all proper methods of care or exclusive of methods of care reasonably directed to obtaining the same results. The ultimate judgment regarding the propriety of any specific procedure must be made by the physician in light of all of the circumstances presented by the individual patient. Prepared By The Standards Task Force The American Society of Colon and Rectal Surgeons