Periodic lateralized epileptiform discharges (PLEDs): a possible role for metabolic factors in pathogenesis

Periodic, lateralized, epileptiform discharges (PLEDs) in the electroencephalogram have been reported in association with a variety of structural lesions of the brain. Although a great deal of attention has been directed to the neuropathological basis of PLEDs, little emphasis has been placed on the functional basis of this EEG syndrome. In this paper, we reported a patient with multiple systemic problems whose EEG showed PLEDs. However, radiological and pathological studies revealed no neuropathological basis for the occurrence of this EEG syndrome. Thus, we presented evidence that metabolic factors may play a part in the pathogenesis of PLEDs.     

Invasion of visual cortex by the auditory system in the naturally blind mole rat.

Previously we have shown that the dorsal lateral geniculate body (LGB), which is strictly visual in sighted mammals, receives a strong auditory input in the naturally blind mole rat (Spalax ehrenbergi). Here we show with the 2-deoxyglucose technique and with single-unit recordings that in this species the initially non-degenerated visual cortex, as defined by its connection with LGB, is also activated by the auditory modality. These findings suggest that cross-modal compensation may occur as a natural consequence of the degeneration of a sense organ.     

Carrier detection by microsatellite analysis of Duchenne/Becker muscular dystrophy in Hungarian families

Duchenne and Becker muscular dystrophies are among the most severe and frequent inherited disorders. Being still incurable, medical treatment is concentrated on the carrier diagnosis of the members of the affected families. Here we report the results of the studies of 151 members of 41 Hungarian families, obtained with multiplex PCR amplification of 18 exons as well as the muscle specific promoter region, and haplotype analysis of two polymorphic (CA)_n repeat microsatellite loci in introns 45 and 49 of the dystrophin gene. The analysis of 15 deletion-type families revealed a frequency of new mutations not differing significantly from that in the other regions of Europe. We also compared the allele distributions of the two microsatellites in randomly selected normal individuals and affected family members. The allele distribution of STRP45 shows interesting differences between the two populations.