Wernicke's encephalopathy

Wernicke's encephalopathy ( syn.: Wernicke-Korsakoff syndrome, engl. Wernicke 's encephalopathy ) is a degenerative enzephaloneuropathische of the brain in adulthood disease. It occurs in vitamin B1 deficiency.

The first description dates back to Carl Wernicke (1848-1905), who in 1881 a study on hemorrhagic changes in the gray matter of the mammillary bodies in three alcoholics wrote and consider as the Polioencephalopathia hemorrhagic superior.

Affected

A Wernicke 's encephalopathy is found mainly in malnourished alcoholics, but also in patients with chronic gastritis, chronic inflammatory bowel disease ( such as Crohn's disease), during prolonged vomiting eg chemotherapy or after obesity surgery.

In 2003, several diseased babies who were exclusively thiaminfreie food, to the syndrome.

Formation

This error is due a vitamin B1 deficiency (thiamine ), which leads to disturbances in carbohydrate metabolism caused by failure of a high-energy phosphorylation. After an edematous swelling of the brain occurs later for hemorrhage and Kapillarsprossung at certain locations of the brain ( mammillary bodies, hypothalamic nuclei to the III. Ventricle, and periaqueductal gray lamina tecti with Okulomotoriuskerngebieten ). The following is a atrophy of these structures.

The transition to Korsakoff's syndrome is fluid and can be done quickly, so the disease is often called Wernicke-Korsakoff syndrome. The damage can be irreversible.

Symptoms

Wernicke's encephalopathy is classically described by a triad of symptoms:

• Brain organic brain syndrome ( HOPS ) with mental changes such as memory loss, psychosis, confusion and confabulations, disorientation
• Gait and unsteadiness ( cerebellar ataxia)
• Eye movement disorders and ocular palsies ( horizontal nystagmus, ophthalmoplegia, anisocoria, double vision (diplopia ) ).

At the beginning are often double vision, slurred speech, unsteady gait, and tingling paresthesia of the legs. Can be added in addition to the clinical triad reflex disturbances, disturbances of consciousness, apathy, somnolence, impaired fine motor skills with dysdiadochokinesia, bulbar speech disorder ( dysarthria ), difficulty swallowing (dysphagia ), sleep disturbances ( insomnia), autonomic disturbances such as hypotension, hypothermia and hyperhidrosis.

Diagnosis

The vitamin B1 levels can be detected in the blood, the plasma levels which may cause false - negative. The whole blood test is considered sensitive.

Therapy

Treatment is by administration of thiamine. Since the absorption oral thiamine is changing and difficult to monitor, an intravenous dose must be done in an emergency. While there are no studies on the optimal dosage is given 200 mg of thiamine after a survey in 2012 mostly over two days. Partly also three times daily 500 mg for two days, followed by one 500 mg thiamine is recommended for five more days. After that, it is generally recommended a more long-term oral administration.

A glucose infusion in alcoholics with unexplained disturbance of consciousness may fire when a latent thiamine deficiency a Wernicke's encephalopathy or Korsakoff's syndrome, because the metabolism of glucose requires thiamine as a coenzyme.