Epilepsy and prion diseases: A narrative review.

摘要: Abstract Epileptic seizures have been described as one feature of prion diseases, but are an unusual clinical presentation. The aim this narrative Review was to summarize current knowledge epileptic in the various forms from a perspective. Examination published literature identified no systematic studies; evidence base is largely anecdotal, consisting mainly case studies and small series. Hence, uncertainty prevails seizure frequency, semiology, treatment, pathogenesis diseases. Seizures probably occur around 10% sporadic cases less frequently iatrogenic familial forms, with possible exception E200K mutation. suggests predominance focal motor nonconvulsive status epilepticus. Electroencephalographic accompaniments include periodic lateralized or generalized epileptiform discharges (PLEDs, GPEDs), sometimes predating more typical sharp wave complexes. There convincing accounts successful antiepileptic drug therapy. underlying mechanisms epileptogenesis diseases may loss cellular protein function (PrPc) aggregation abnormally folded (PrPSc). need for trials expand surrounding epilepsy evident.