Prion Diseases: actual clinical and diagnostic aspects

Recently, the problem of neurodegenerative diseases in the medical community has become increasingly relevant. This is due to many factors: from insufficiently studied mechanisms of development of some nosological units to low awareness of medical workers. Among neurodegenerative diseases in humans, prions constitute a very specific group, which are infectious protein particles with a unique morphological structure and capable of causing a number of incurable diseases. Despite years of research, no optimal remedy has yet been found to treat them. This review examines the already studied aspects of prion diseases as a class, including small historical background, features of ethiology, pathogenesis, course and outcome of the most common of them, as well as existing research on experimental methods of diagnostics, treatment and prevention of prion infections.

Periodic Sharp Wave Complexes observed by Repetitive EEGs in a Patient with Probable Sporadic Creutzfeldt-Jacob Disease

The periodic sharp wave complexes are the most characteristic EEG abnormalities in sCJD. We report the serial EEG findings in a patient with probable sCJD. The initial EEG showed diffuse theta and delta slowings only. However, it changed to the typical periodic sharp wave complexes according to the worsening clinical state. These changes occurred during a short-term interval. The serial EEGs within the short-term interval will be helpful for the earlier diagnosis of sCJD.

A Case of Creutzfeldt-Jacob Disease / 신경정신의학

We report a case of Creutzfeldt-Jacob disease and review the relevant literatures. This 56-year-old male patient was admitted to our hospital with a history of rapidly progressing cognitive dysfunction, visual disturbance, gait disturbance, weakness, involuntary movement of lower extremities, and the symptoms of delirium. On mental status examination, he showed psychomotor retardation, speech disturbance, perseveration, disorientation, impairement of short-term and long-term memory, and inability of abstract thinking. He had a rapid downhill course, along with shakiness of trunk, rigidity of the limbs, myoclonus, confusion, and finally, inability to speak or move on the bedside. On electroencephalogram(EEG), continuous multifocal slow delta activities were found in all leads, especially in the right frontal areas. Brain biopsy showed, spongiosis with neuronal loss and gliosis noted at the cerebral cortex. These findings were compatible with Creutzfeldt-jacob disease.