Prions and Prion Diseases of the Central Nervous System (Transmissible Neurodegenerative Diseases)

An ominous radiographic feature: cortical ribbon sign

“…The corresponding ADC (apparent diffusion coefficient) sequence demonstrates a classic cortical ribbon hypointensity as opposed to DWI [1][2][3][4]. The most characteristic clinical manifestation of sCJD is a rapidity of deterioration in neurological functions, typically over a period of a few weeks to months [1]. Clinical features of sCJD include myoclonic jerks, memory deficits, cerebellar signs (ataxia, nystagmus, tremors), pyramidal/corticospinal tract dysfunctions (spasticity, hyperreflexia, extensor plantar responses), extrapyramidal manifestations (bradykinesia, rigidity, tremors), visual disturbances (visual field cuts, cortical blindness, visual agnosia), and akinetic mutism [1,2].…”

“…Creutzfeldt-Jakob disease (CJD) was first described separately by two German physicians-Hans Gerhard Creutzfeldt (neuropathologist) and Alfons Maria Jakob (neurologist) in the 1920s [1]. CJD is the most common form of human prion disease.…”

“…The most characteristic clinical manifestation of sCJD is a rapidity of deterioration in neurological functions, typically over a period of a few weeks to months [1]. Clinical features of sCJD include myoclonic jerks, memory deficits, cerebellar signs (ataxia, nystagmus, tremors), pyramidal/corticospinal tract dysfunctions (spasticity, hyperreflexia, extensor plantar responses), extrapyramidal manifestations (bradykinesia, rigidity, tremors), visual disturbances (visual field cuts, cortical blindness, visual agnosia), and akinetic mutism [1,2]. A brain MRI is the most useful imaging tool in assisting the ante-mortem diagnosis of sCJD, and also helps to differentiate variant CJD (vCJD) [1,2].…”

“…DWI (diffusion-weighted imaging) sequence is typically a more sensitive modality for identifying the cortical ribbon sign as compared to FLAIR (fluid-attenuated inversion recovery) sequence. The corresponding ADC (apparent diffusion coefficient) sequence demonstrates a classic cortical ribbon hypointensity as opposed to DWI [1][2][3][4]. The most characteristic clinical manifestation of sCJD is a rapidity of deterioration in neurological functions, typically over a period of a few weeks to months [1].…”

“…CJD is the most common form of human prion disease. CJD is a rare and fatal neurogenerative disease, characterized by a rapidly progressive decline in memory, cognition, motor, and other brain functions [1]. It is caused by an infectious prion protein, defined as proteinaceous infectious particle without nucleic acid.…”