The first case of bovine spongiform encephalopathy (BSE) in Switzerland was diagnosed in November 1990, although the risk factors identified in the United Kingdom were not all present in Switzerland. At 29 December 1998, a total of 282 BSE cases (all animals born in Switzerland), had been recorded. The number of cases is declining, with fourteen cases diagnosed in 1998 (at 29 December 1998). Th...

The possibility of the agent causing bovine spongiform encephalopathy (BSE) infecting small ruminants is of serious concern for human health. Among scrapie cases, the CH1641 source in particular appears to have certain biochemical properties similar to the BSE strain. In France, several natural scrapie cases were identified as "CH1641-like" natural scrapie isolates in sheep and goats. The Tg(Ov...

Recognition potential (RP), a recently discovered electrophysiological response of the brain, is sensitive to semantic aspects of stimuli. Given its peak values (about 250 ms), RP may be a good candidate for the study of semantic processing during its occurrence. However, its topography and neural generators are largely unknown. To improve this state of affairs, high-resolution electroencephalo...

We report transmission of atypical L-type bovine spongiform encephalopathy to mouse lemurs after oral or intracerebral inoculation with infected bovine brain tissue. After neurologic symptoms appeared, transmissibility of the disease by both inoculation routes was confirmed by detection of disease-associated prion protein in samples of brain tissue.

The first case of spongiform encephalopathy in a zebu (Bos indicus) was identified in a zoo in Switzerland. Although histopathologic and immunohistochemical analyses of the central nervous system indicated a diagnosis of bovine spongiform encephalopathy (BSE), molecular typing showed some features different from those of BSE in cattle (B. taurus).

The determination of blood transketolase before and serially after thiamine administration, and the response of clinical symptomatology after thiamine are reported in two normomagnesaemic patients and one hypomagnesaemic patient with acute Wernicke-Korsakoff encephalopathy. The response of the depressed blood transketolase and the clinical symptoms was retarded in the hypomagnesaemic patient. C...

Introduction Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) can involve multiple systems and cause stroke-like episodes and status epilepticus. Case Presentation A 48-year-old female with history of early fatigability, migraine-type headaches, and bilateral sensory-neural hearing loss presented 3 episodes of serial seizures. On admission she was affected by We...

Acute axonal polyneuropathy and Wernicke-Korsakoff encephalopathy developed simultaneously in three patients. Nerve conduction studies (NCS) detected markedly decreased compound muscle action potentials (CMAPs) and sensory nerve action potentials (SNAPs) with minimal conduction slowing; sympathetic skin responses (SSRs) were also notably decreased. Sural nerve biopsies showed only mild axonal d...

novel data, which together served to define and explicate an understanding of the alcoholic Wernicke-Korsakoff Syndrome (WKS) from the clinical and basic science perspectives. The symposium's five presentations covered neuropathology, epidemiology, neural and genetic mechanisms, neuropsychological and neuroradiological signs of Wernicke's encephalopathy (WE) and Korsakoff's syndrome (KS), and t...

In anticipation of the emergence of more variants of bovine spongiform encephalopathy (BSE), a semiquantitative display of the following four independent molecular diagnostic prion parameters was designed: N terminus, proteinase K (PK) resistance, glycoprofile, and mixed population. One H BSE case, three L BSE cases, six C BSE cases, and one unusual classical BSE (C BSE) case are reported.