What is hockey stick sign?

The hockey stick sign refers to the hyperintense signal involving the pulvinar and dorsomedial thalamic nuclei bilaterally on FLAIR, in cases of variant Creutzfeldt-Jakob disease, which has the shape of a hockey stick.

What is cortical Ribboning?

Cortical ribboning is the term given to an imaging appearance characterized by hyperintense signal changes seen in cerebral cortex and basal ganglia in diffusion-weighted magnetic resonance imaging (MRI).

What is the pulvinar sign?

The pulvinar sign refers to bilateral FLAIR hyperintensities involving the pulvinar thalamic nuclei. It is classically described in variant Creutzfeldt-Jakob disease. It is also described in other neurological conditions: Fabry disease: the hyperintense signal is seen on T1 rather than T2.

What do prions do?

Prions are misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize several fatal and transmissible neurodegenerative diseases in humans and many other animals.

Do all prions cause disease?

All known prion diseases in mammals affect the structure of the brain or other neural tissue; all are progressive, have no known effective treatment, and are always fatal….Transmissible spongiform encephalopathies.

Affected animal(s) Disease
Human Fatal familial insomnia (FFI)
Familial spongiform encephalopathy

What causes cortical Ribboning?

It is caused by an infectious prion protein, defined as proteinaceous infectious particle without nucleic acid. CJD is divided into four subtypes and includes sporadic, variant, genetic/familial, and iatrogenic.

What is pulvinar hip?

Pulvinar (P) = fibrofatty tissue between acetabulum and femoral head, more evident in DDH due to femoral head not pressing against it in the acetabulum. α pulvinar.

What are florid plaques?

Plaques in the cerebral cortex were often surrounded by a zone of spongiform changes, giving them a florid appearance. Prion protein plaques of this type were quite similar, morphologically and in their immunoreactivity to anti-PrP antibodies, to those found in the brain of a patient with nvCJD (Figure 2, C and D).

Is Alzheimer’s a prion disease?

Prion diseases are caused by the toxic misfolding and clumping of the prion protein, PrP. Although Alzheimer’s is not a prion disease, and the PrP, Aβ, and tau proteins each normally fold into distinct 3D shapes, upon misfolding, all three proteins can all form aggregates that have a very specific structural pattern.

Which diseases are caused by prions?

Identified Prion Diseases

  • Creutzfeldt-Jakob Disease (CJD)
  • Variant Creutzfeldt-Jakob Disease (vCJD)
  • Gerstmann-Straussler-Scheinker Syndrome.
  • Fatal Familial Insomnia.
  • Kuru.

How do humans get prions?

A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. Prion diseases can affect both humans and animals and are sometimes spread to humans by infected meat products. The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD).

What does anoxic brain injury look like on MRI?

The imaging findings of diffuse cerebral anoxia include obscured gray-white matter junctions, abnormal appearance of deep gray matter nuclei, infarctions in regions between major arterial territories, and laminar necrosis (1–7).

Is the pulvinar part of the thalamus?

As a group they make up the collection called the pulvinar of the thalamus (pulvinar thalami), usually just called the pulvinar. The pulvinar is usually grouped as one of the lateral thalamic nuclei in rodents and carnivores, and stands as an independent complex in primates.

Where are the pulvinar nuclei located in the brain?

Anatomical terms of neuroanatomy. [edit on Wikidata] The pulvinar nuclei or nuclei of the pulvinar (nuclei pulvinares) are the nuclei (cell bodies of neurons) located in the thalamus (a part of the vertebrate brain). As a group they make up the collection called the pulvinar of the thalamus (pulvinar thalami), usually just called the pulvinar.

Which is the posterior most aspect of the thalamus?

Posterior. The posterior most aspect of the thalamus is known as the pulvinar. Each pulvinar is lateral to the pineal gland, the Habenular and posterior commissures, posterolateral to the corpora quadrigemini (superior and inferior colliculi), and superior to the medial and lateral geniculate bodies.

What are the effects of damage to the pulvinar?

Unilateral lesions of the pulvinar result in a contralateral neglect syndrome resembling that resulting from lesions of the posterior parietal cortex. The pulvinar is susceptible to damage in epilepsy and prion disorders, and may contribute to cognitive and visual manifestations of Lewy body disease.