Spongiform encephalopathy

Spongiform Encephalopathy is a type of neurodegenerative disease that can affect both humans and animals. The term "spongiform" refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges when viewed under a microscope.

Types of Spongiform Encephalopathy

There are several types of spongiform encephalopathies, including:

• Creutzfeldt-Jakob disease (CJD)
• Variant Creutzfeldt-Jakob disease (vCJD)
• Kuru
• Fatal familial insomnia (FFI)
• Gerstmann–Sträussler–Scheinker syndrome (GSS)
• Transmissible mink encephalopathy (TME)
• Chronic wasting disease (CWD)
• Bovine spongiform encephalopathy (BSE), also known as "mad cow disease"

Causes

Spongiform encephalopathies are caused by prions, which are misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize a number of fatal and transmissible neurodegenerative diseases in humans and many other animals.

Symptoms

The symptoms of spongiform encephalopathy can vary depending on the specific disease, but they often include dementia, ataxia (problems with movement and coordination), and myoclonus (sudden, involuntary jerking of a muscle or group of muscles).

Diagnosis

Diagnosis of spongiform encephalopathy can be challenging, as it often involves a range of neurological tests, and may also include a brain biopsy. The definitive diagnosis, however, can usually only be confirmed post-mortem.

Treatment

There is currently no cure for spongiform encephalopathy. Treatment is focused on relieving symptoms and making the patient as comfortable as possible.

See also

• Prion
• Neurodegenerative disease
• Creutzfeldt-Jakob disease
• Bovine spongiform encephalopathy