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Wernicke's Encephalopathy

 

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Wernickes Encephalopathy

Wernicke-Korsakoff syndrome is a brain disorder involving loss of specific brain functions caused by a thiamine deficiency . Dr Carl Wernicke, a Polish neurologist, described it in 1881 as a triad of acute mental confusion, ataxia, and ophthalmoplegia. Wernicke's encephalopathy onsets acutely, and usually presents with nystagmus , gaze palsies, ophthalmoplegia (especially of the abducens nerve , CN VI), gait ataxia , confusion , and short-term memory loss. The heart, vascular, and nervous system are involved. Symptoms include amnesia, confabulation, attention deficit, disorientation, and vision impairment. This deficiency develops because most alcoholics, for example, tend to neglect their diet and often drink instead of eating. An initial magnetic resonance image (MRI) of the brain, with fluid-attenuated inversion recovery, demonstrated abnormal hyperintensity of the mamillary bodies and periaqueductal gray matter (Panel A, arrows), with associated abnormal enhancement on T 1 -weighted images that were obtained with the use of contrast material (Panel B, arrows). Commonly associated with chronic alcoholism but also starvation, persistent vomiting and systemic diseases such as cancer, liver failure, disseminated tuberculosis and uraemia. Treatment includes an intravenous (IV) or intramuscular (IM) injection of thiamine , as well as the active exclusion of central nervous system (CNS) diseases or other metabolic disturbances. Patients are usually dehydrated, and so rehydration to restore blood volume should be started. If the condition is treated early, recovery may be rapid and complete.

Wernicke's encephalopathy is a severe syndrome characterised by loss of short-term memory . Wernicke's encephalopathy is a degenerative brain disorder caused by the lack of thiamine (vitamin B1). It may result from alcohol abuse, dietary deficiencies, prolonged vomiting, eating disorders, or the effects of chemotherapy. The most common cause of an onset is severe alcoholism , though there are several other causes. An inherited problem in metabolising thiamine may leave certain people particularly vulnerable to thiamine deficiency. The patient was given thiamine, with marked improvement in her confusion, memory loss, slurred speech, and extraocular movements during the next seven days. Thiamine pyrophosphate (TPP) is a co-enzyme required for linking glycolysis to the Krebs cycle in the aerobic metabolism of glucose as well as several other steps in carbohydrate metabolism. Although Wernicke's and Korsakoff's may appear to be two different disorders, they are generally considered to be different stages of the same disorder, which is called Wernicke-Korsakoff syndrome . Wernicke's encephalopathy represents the "acute" phase of the disorder, and Korsakoff's amnesic syndrome represents the "chronic" phase.

Causes of Wernicke's Encephalopathy

The common Causes of Wernicke's Encephalopathy :

  • This disease is caused by a lack of thiamin (vitamin B1), which leads to problems with the normal functioning of the brain.
  • Hyperemesis gravidarum (continuous nausea and vomiting during pregnancy)
  • Thiamine deficiency associated with Alcoholism.
  • Wernicke-Korsakoff syndrome is a brain disorder involving loss of specific brain functions caused by a thiamine deficiency .
  • Other less common etiologies are forced or self-imposed starvation, protein-energy malnutrition resulting from inadequate diet or malabsorption (from sprue), conditions associated with protracted vomiting (eg, hyperemesis gravidarum)
  • Chronic renal failure, carbohydrate loading in the presence of marginal thiamine stores (feeding after starvation), bariatric surgery and other gastric bypass surgeries, absence of thiamine from the diet (in the case of infants fed formula without the addition of thiamine), and congenital transketolase function abnormalities.

Symptoms of Wernicke's Encephalopathy

Some common Symptoms of Wernicke's Encephalopathy :

  • Muscle atrophy,
  • Cold skin.
  • Loss of memory, can be profound.
  • Swallowing difficulties,
  • Double vision.
  • Abnormal eye movements.
  • Inability to form new memories.
  • Dry skin,
  • Staggering gait.
  • Eye movement abnormalities.
  • Difficulty with speech,
  • Confabulation (making up stories).
  • Reduced eye movement.

Treatment of Wernicke's Encephalopathy

  • Intravenous thiamin is given to the patient for a few days until it can be given by mouth. The most effective treatment is to eat properly.
  • Because patients with Wernicke encephalopathy present with altered mental status in the prehospital setting, focus prehospital care on stabilizing the airway, ensuring oxygenation, and maintaining blood pressure and euvolemia.
  • Antidepressants that increase levels of serotonin may also be helpful, although the reasons why are not clear since these drugs are not effective with other memory disorders.
  • Start thiamine prior to or concurrently with treatment of intravenous glucose solutions, and continue until the patient resumes a normal diet.
  • Treatment involves replacement of thiamine and providing proper nutrition and hydration. In some cases, drug therapy is also recommended.
  • A Diagnosis can be made by treating the patient with thiamin and watching the symptoms improve.  There are blood and urine tests available to check the level of thiamin, but giving the patient thiamin and seeing the response is the best way to diagnose this condition.