EEG in Creutzfeldt-Jakob disease.

Electroecenphalography (EEG) is an integral part of the diagnostic process in patients with Creutzfeldt-Jakob disease (CJD). The EEG has therefore been included in the World Health Organisation diagnostic classification criteria of CJD. In sporadic CJD (sCJD), the EEG exhibits characteristic changes depending on the stage of the disease, ranging from nonspecific findings such as diffuse slowing and frontal rhythmic delta activity (FIRDA) in early stages to disease-typical periodic sharp wave complexes (PSWC) in middle and late stages to areactive coma traces or even alpha coma in preterminal EEG recordings. PSWC, either lateralized (in earlier stages) or generalized, occur in about two-thirds of patients with sCJD, with a positive predictive value of 95%. PSWC occur in patients with methionine homozygosity and methionine/valine heterozygosity but only rarely in patients with valine homozygosity at codon 129 of the prion protein gene. PSWC tend to disappear during sleep and may be attenuated by sedative medication and external stimulation. Seizures are an uncommon finding, occurring in less than 15% of patients with sCJD. In patients with iatrogenic CJD, PSWC usually present with more regional EEG findings corresponding to the site of inoculation of the transmissible agent. In genetic CJD, PSWC in its typical form are uncommon, occurring in about 10%. No PSWC occur in EEG recordings of patients with variant CJD.     

Fronto-ethmoidal mucocoeles: a histopathological analysis.

From a series of 118 fronto-ethmoidal mucocoeles, 40 specimens have been submitted to detailed histological analysis. The frequency of each epithelial and sub-epithelial component is considered and reveals features which support a dynamic theory of bone resorption and formation rather than that of pressure erosion in the pathogenesis of mucocoeles.     

Clinical application of epiderm growth factor in patients with most severe eye burns

20 eyes of 11 patients suffering from most severe eye burns of grade 4 or worse were treated in addition to various other drugs with epiderm growth factor (EGF) or placebo in a double-blind test. Among 10 eyes treated with EGF, 6 achieved complete regeneration of the corneal epithelium, and 1 additional case, of the conjunctival epithelium. Among the eyes receiving placebo, only 3 out of 10 healed; all of them were also treated with fibronectin. Although in these very difficult cases EGF could not be investigated as a single therapy, and various other undefined factors may have been effective, in the extended course of the disease the period of EGF treatment was marked by a significant better regeneration of the epithelium.     

Usher syndrome: clinical findings and gene localization studies

The issue of genetic heterogeneity is a critical problem in the localization of the gene(s) for Usher syndrome. Based on the data obtained on families studied to date, the differences between type I and type II Usher syndrome appear quite distinct with regard to auditory and vestibular function. Although the majority of families can be confidently diagnosed as typical type I or type II, clinical investigations revealed four families with findings that did not fit into either of the two more common subtypes. These findings emphasize the critical importance of an in-depth clinical analysis concomitant with the linkage investigation to assure accurate subtyping of Usher syndrome. Based on an analysis of only those families with definite type I or type II Usher syndrome, approximately 17% of the genome can be excluded as a potential site of the gene for type I, and 14% can be excluded as the site for the type II gene. This study will continue until the Usher gene(s) is successfully localized.     

Ascorbinsäure,ein Vorstadium des C-Vitamins. II

Zusammenfassung Es wird eine Mikromethylenblaumethode zur quantitativen Bestimmung der Serumascorbinsäure mitgeteilt.Durch vergleichende Untersuchungen mitLund undLiecks Methylenblaumethode wird die Auffassung bekräftigt, daß die Ascorbinsäure ein Vorstadium des C-Vitamins ist.     

MR imaging of the spine in children

MR imaging was reviewed in 66 pediatric spinal cord patients with diagnoses of posterior fossa tumor (n = 8), primary spinal cord tumor (n = 3), metastatic disease (n = 11), neuroectodermal disorder (n = 8), congenital malformation (n = 14), trauma (n = 2), and demyelinating, neurodegenerative, or metabolic disorders (n = 9). MR proved to be useful in delineating the extent of posterior fossa and cord tumor including metastasis to the cord. MR was of limited value in demyelinating and metabolic disorders. Arnold Chiari malformations, syringomyelia, tethered cord and meningoceles were all easily evaluated using MR.