A patient with rapidly progressive cognitive decline over an approximately four month period was suspected to have sporadic Creutzfeldt-Jakob disease. Features thought to support this diagnosis included psychiatric symptoms (anxiety and depression), visual hallucinations and a visual field defect. However, the finding of papilloedema broadened the differential diagnosis. Although standard brain...

A patient is described who showed subacute dementia and ataxia and, finally, acute asending polyradiculoneuritis. At necropsy, together with the cerebral and cerebellar lesions that are typical of Creutzfeldt-Jakob disease, the lesions of the spinal motor neurons, the spinal ganglia, and of the roots and peripheral nerves were analogous with those of the Guillain-Barré-Landry syndrome. The aeti...

Only a few years ago, pharmacotherapy of Creutzfeldt-Jakob disease was inconceivable. The enigmatic prion agent causing Creutzfeldt-Jakob disease, consisting solely of a misfolded conformational isoform, the scrapie prion protein, of the normal cellular prion protein was considered hard to treat by routine drug development. However, huge progress has been achieved in recent years, demonstrating...

BACKGROUND The determination of the form of prion disease and early diagnosis are important for prognostic, public health, and epidemiologic reasons. OBJECTIVE To describe a patient with sporadic Creutzfeldt-Jakob disease (sCJD) who had a clinical history and initial electroencephalogram and magnetic resonance imaging findings consistent with variant CJD (vCJD). RESULTS Results of a repeate...

Rapidly progressive dementias are a rare group of cognitive disorders that primarily require the exclusion large number potentially reversible causes. Prion diseases frequent in this disorders. Some hereditary forms Alzheimer's disease can also be aggressive, with onset at young age and autosomal dominant inheritance family. The article presents first case report patient verified Ile143Thr muta...

Creutzfeldt-Jakob disease (CJD) is a rare and rapidly fatal neurological disease. Diagnosis made through clinical features, imaging, electroencephalography, cerebrospinal fluid analysis. Sporadic CJD accounts for the majority of cases occurs due to somatic mutation in gene or random structural change prion protein. Coronavirus 2019 (COVID-19) known cause neurodegeneration, acceleration hypothes...

Creutzfeldt-Jakob disease is the most common form of human prion diseases. A 57-year-old woman was transferred to our Department from a local hospital, where she had been treated for two weeks due to consciousness disorders and convulsive epileptic attacks that progressed to refractory status epilepticus. Electroencephalography showed diffuse spike-wave complex discharges and development of non...

Necropsy findings consistent with spongiform encephalopathy of the Creutzfeldt-Jakob type are described in the brain of a 48-year-old woman whose prolonged course and clinical features had suggested Alzheimer's presenile dementia. Six other members of her family in two generations have also died of progressive presenile dementing illnesses of Alzheimer type, lasting 5-10 years. Autopsies showed...

The objective of this work is to describe the neuropathological findings of a patient clinically presenting with rapidly progressive nonspecific neurological symptoms suggestive of Creutzfeldt-Jakob disease. Methods used were clinical description with laboratory analyses, repeated electroencephalogram, cerebral computed tomography, magnetic resonance imaging studies and details on neuropatholog...

An attempt was made to ascertain all cases of Creutzfeldt-Jakob disease occurring in England and Wales during the 5 year period 1980-1984. The mean annual mortality rate was 0.49/million; women were more frequently affected than men. The age-specific mortality rate reached a peak in the seventh decade. A case-control study involving 92 of the 122 definite and probable cases ascertained failed t...