Tuesday, December 08, 2009

대사성 뇌질환 - 1. Wernicke-Korsakoff syndrome’s Overview

Wernicke-Korsakoff syndrome's Overview

Wernicke-Korsakoff syndrome(also called wet brain, Korsakoff psychosis, alcoholic encephalopathy, Wernicke's disease, and alcoholic encephalopathy, alcoholic psychosis) is a severe memory disorder usually associated with chronic excessive alcohol consumption, although the direct cause is a deficiency in the B1 vitamin thiamin. It mainly causes vision changes, ataxia and impaired memory.
The syndrome is a combined manifestation of two eponymous disorders, Korsakoff's Psychosis and Wernicke's encephalopathy, named after Drs. Sergei Korsakoff and Carl Wernicke.

The disorder was first identified in the late nineteenth century. The first phase of the condition, called Wernicke's encephalopathy, was described by German neurologist and psychiatrist Karl Wernicke in 1881. He noted three key symptoms in three patients - two with alcoholism and one who had swallowed sulfuric acid. These patients suffered from mental confusion, eye movement disorders, and ataxia (poor motor coordination).

A few years later, S. S. Korsakoff, a Russian psychiatrist, began publishing reports describing a syndrome of anterograde amnesia - an inability to form new memories - and confabulation in individuals with severe alcoholism or certain medical illnesses(Confabulation refers to the practice of filling in gaps in memory by fabrication).

By 1900, researchers and clinicians studying alcoholism recognized a connection between the two conditions.

The typical syndrome begins with acute Wernicke's encephalopathy, with Korsakoff syndrome emerging when the acute phase resolves. The symptoms of Wernicke's encephalopathy appear suddenly. The most prominent symptom initially is mental confusion including memory problems.

On examination, patients have difficulty moving their eyes to follow a visual stimulus due to paralysis of the muscles controlling eye movements. For instance, a patient may have trouble looking upward or to the side with one or both eyes.

Problems maintaining balance while standing or walking, a condition known as ataxia, are frequently observed as well. If left untreated, most of these symptoms may resolve spontaneously, but the severe memory disorder characteristic of Korsakoff syndrome remains.

The typical person with Korsakoff syndrome appears fairly normal on first impression. Intelligence is intact, and individuals with the syndrome can carry on a conversation quite naturally. They are usually able to recall and talk about incidents that took place before the onset of the disorder and recognize family members and old friends without much difficulty.

The ability to form new memories is nearly absent, however. In the course of conversation, people with Korsakoff syndrome may repeat comments or questions several times. They will fail to recognize people they met minutes before or greet a friend with excitement and surprise after a brief trip to another room.

These are the characteristics of anterograde amnesia. Research shows that anterograde amnesia results from a failure of memory formation and storage. New information is processed normally, but almost immediately forgotten, never making it into the regions of the brain where memories of the past are stored.

People with Korsakoff syndrome thus have no memories of events that happened after the onset of the illness. Many previously stored memories are still available, however, explaining why individuals with Korsakoff syndrome can usually remember the distant past quite well.

Wernicke's encephalopathy is characterized by;

Confusion
Nystagmus
Ophthalmoplegia
Anisocoria
Ataxia
Sluggish pupillary reflexes
Coma and death if untreated

Korsakoff's psychosis is characterized by;

Anterograde and retrograde amnesia
Confabulation
Hallucinations

Synonyms

Wernicke-Korsakoff syndrome
Korsakoff psychosis
Alcoholic encephalopathy
Wernicke's disease
Wernicke's encephalopathy