Case Files Neurology, Second Edition (LANGE Case Files) by Tintner Ron & Toy Eugene & Simpson Ericka

Author:Tintner, Ron & Toy, Eugene & Simpson, Ericka [Tintner, Ron]
Language: eng
Format: epub
Publisher: McGraw-Hill
Published: 2012-09-04T00:00:00+00:00

CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the most common of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann–Straussler–Scheinker disease (GSS). Kuru was identified in people of an isolated cannabalistic tribe in Papua, New Guinea, and has now almost disappeared along with cannibalistic practices. FFI and GSS are extremely rare hereditary diseases, found in just a few families around the world. Other TSEs are found in specific kinds of animals. These include bovine spongiform encephalopathy (BSE), which is found in cows and is often referred to as “mad cow” disease; scrapie, which affects sheep and goats; mink encephalopathy; and feline encephalopathy. Similar diseases have occurred in elk, deer, and exotic zoo animals.

CJD is characterized by rapidly progressive dementia. Initially, patients experience problems with muscular coordination, personality changes, including impaired memory, judgment, and thinking, and impaired vision. Affected patients also can experience insomnia, depression, or unusual sensations. CJD does not cause a fever or other flu-like symptoms. As the illness progresses, the patients’ mental impairment becomes severe. They often develop involuntary muscle jerks called myoclonus, and they may go blind. They eventually lose the ability to move and speak and enter a coma. Pneumonia and other infections often occur in these patients and can lead to death.

There are several known variants of CJD. These variants differ somewhat in the symptoms and course of the disease. For example, a variant form of the disease—called new variant or variant (nv-CJD, v-CJD), described in Great Britain and France—begins primarily with psychiatric symptoms, affects younger patients than other types of CJD, and has a longer than usual duration from onset of symptoms to death. Another variant, called the panencephalopathic form, occurs primarily in Japan and has a relatively long course, with symptoms often progressing for several years. Scientists are trying to learn what causes these variations in the symptoms and course of the disease. Some symptoms of CJD can be similar to symptoms of other progressive neurologic disorders, such as Alzheimer or Huntington disease. However, CJD causes unique changes in brain tissue that can be seen at autopsy. It also tends to cause more rapid deterioration of a person’s abilities than Alzheimer disease or most other types of dementia.

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