WERNICKE’S DISEASE

 

WERNICKE’S DISEASE

This is a brain disease characterised by memory, visual impairment and ataxia due to thiamine deficiency found in vitamin B₁. It is usually associated with chronic excessive alcohol consumption it is sudden onset.  

This disease was first identified by a German Neurologist and Psychiatrist called Karl Wernicke in the 19^th Century in 1881. Wernicke noted and compared the key symptoms in three patients in which one had swallowed sulfuric acid and the other two with alcoholism. He observed that these patients suffered from mental confusion, eye movement disorders and ataxia poor motor coordination.

AETIOLOGY

-Vitamin B₁ (Thiamine) deficiency. Thiamine helps in the production of energy needed by neurons for proper functioning, production of neural transmitters and lipid (fat) metabolism. Therefore, insufficient thiamine can lead to damage or death of the brain cells (neurons).

PREDISPOSING FACTORS

i)                 ALCOHOLISM

-        It has been observed that many heavy drinkers have poor eating habits and their diets lack the essential nutrients required by the body especially the brain.

-        Alcohol can also interfere with the conversion of thiamine into its active form called THIAMINE PYROPHOSPHATE.

-        Alcohol leads to the inflammation of the stomach lining (gastritis) causing frequent abdominal upsets, such as vomiting. Hence the poor absorption of essential vitamins (avitaminosis).

-        Alcohol can as well lead to inflammation of the liver cells making it difficult for the liver to store vitamins and also leads to the increase in hepatic enzymes. The rise in the hepatic enzymes decreases the availability of the thiamine in the general circulation, hence the deficient supply to the neurons.

 

ii)               POST GASTERECTOMY

This is a surgical procedure performed to correct a gastric problem, such as peptic ulcers, which may have failed to medical intervention, bleeding excessively or perforated. Gasterectomy may result in reduction of the stomach size and also impairment to the pyloric sphincter resulting in reduced time of storage of food in the stomach.

 

iii)              LIVER CIRRHOSIS

This also interferes with storage of the vitamin by the liver and also increases the metabolisation of thiamine by the increased hepato-enzymes resulting in insufficient thiamine reaching the general circulation.

 

iv)              MALNUTRION

 

v)               GASTRO-INTESTINAL DISORDERS

The conditions like acute gastro – enteritis, Crohn’s disease etc.

 

vi)              Patient undergoing kidney dialysis which reduces vitamin B₁ re absorption.

 

INCIDENCE

1/8 (one eighth) of alcoholics are diagnosed with this disorder. It is more common in the under privileged communities and affects more males between the ages of 45 and 65 years with long standing history of alcohol consumption than women.

 

PATHOPHYSIOLOGY

Thiamine deficiency damages regions of the brain particularly the Thalamus and the Mamillary bodies. The Thalamus is a structure deep in the brain that serves many important functions. It is often called the major relay station of the brain, and many neurons make connections in the thalamus.

The mammillary bodies are part of the Hypothalamus located just below the Thalamus.  The Mammillary receive many neural connections from another part of the brain called Hippocampus which appears to be the first part of the brain involved in the formation of memory. Neurons in the Mammillary bodies make connections with Thalamus which in turn connects with the cortex of the brain, where long term memories are stored. This may explain why damage to the Mammillary bodies and Thalamus can lead to amnesia. Memories formed in the Hypothalamus are never stored since connections between

 

Hippocampus and cortex are disrupted.

Eye movement disorders are probably due to damage to other nearby brain regions that make connections to the nerves controlling eye muscles, i.e. the Superior Rectus, Inferior Rectus muscle, Medial Rectus etc.

 

SIGNS AND SYMPTOMS

-        Double vision

-        Drooping/dropping upper eye lid due to the weakness in the Lavetor Palpbrae Superior’s muscle.

-        Weakness in the up and down or side to side eye movement

-        Loss of muscle coordination

-        Loss of memory.

-        Difficult to understand the meaning of the information.

-        Hallucinations

-        Hypothermia

 

DIAGNOSIS

-        Presenting signs and symptoms of Thiamine deficiency, i.e. loss of memory, ataxia and visual disturbances.

-        History of chronic excessive alcohol consumption.

-        Malnutrition –

-        Gastro-intestinal disorders leading to Gasterectomy, Crohn’s disease etc.

-        Physical examination may reveal – old incision scar, suggesting surgical operation on the abdomen

-        Patient may look malnourished – showing wasted thin, puffy and pale body, thin hair etc.

-        On palpation, organomegally – hepatomegally may show.

-        Blood analysis may show – increased alcohol levels, reduced thiamine levels, liver functions disorders.

-        Abdominal Ultra sound may confirm hepatomegally.

-        Neuro-imaging findings show degeneration of the Thalamus and mammillary bodies, loss of brain volume in the area surrounding the fourth ventricle.

-        EEG and CT scan will show abnormalities.    

 

 

PROGNOSIS

If not well treated, Wernicke’s disease may progress into Korsakoff’s syndrome. The prognosis for fully recovery is poor once the chronic Wernicke’s – Korsakoff’s syndrome sets in.

Approximately 80% of patients will never fully recover the ability to learn and remember new events and information and may lead to death. Therefore, urgent medical intervention is required as this may reverse many of the neurological symptoms of Wernicke’s disease.            

 

MANAGEMENT

AIMS ARE:

-To save life

- To educate patient the importance of having balanced diet.

-To introduce or replace vitamin B₁ back to the body.

- To eradicate the possible cause/contributing factors

 

CHEMOTHERAPY

-        IV Thiamine Hydrochloride 500mg over 30 minutes OD for three days.

-        Treat other causes or contributing factors- such as liver cirrhosis, gastrointestinal disturbances etc.

-        Treat for alcoholism – Especially alcoholic withdrawal (Delirium Tremens) and give support during the withdrawal therapy.

 

 

NURSING CARE

ENVIRONMENT

-        Nurse in an environment free from noise, hazards, and well lit because the patient is confused, lacking muscle coordination and impaired vision.

POSITION:

 Nurse in a position patient is most comfortable with.

 

OBSERVATIONS:

 Observe for the vital signs especially Temperature, which is an indicator of the Hypothalamus disturbance.

REST:

 Allow the patient time to rest so that he/she can conserve energy to the brain cells.

NUTRITION:

Give food rich in B₁ that is in small frequent and attractive. The food should be warm enough to avoid further chilling of the patient. Most of the patients in this state are likely to be malnourished, so provide food rich in protein to repair worn out tissues, food rich in carbohydrates to provide energy. Food rich in fats will provide the adipose tissue, hence conserving heat generated by the body.

In case of liver cirrhosis, diet rich in proteins should be given with caution.

As regards Hygiene and Elimination, nurse according to the needs of the patient.

PSYCHOLOGICAL CARE

-        Explain all procedures in simple and straight forward language that the patient best understands.

 

-        Explain what the disease entails and what the health care provider’s team is doing to help the patient recover.

 

-        Allow the patient and the relatives to ask questions and verbalise their fears. The questions be answered truthfully and refer to expertise you fail to provide the right responses.

 

-        Reassure the patient by saying, as health care provider; you are doing everything possible to help the patient recover.

 

 

INFORMATION, EDUCATION AND COUNSELLING

-        Educate patient and the relatives on the predisposing factors of the illness, its prevention and the importance of avoiding alcohol consumption.

-        Teach the patient and the relatives on the need to take food rich in vitamins

-        Tell the patient the need to seek medical advice whenever they are unwell and this must be emphasized.

-        For those that are unable to stop drinking completely, or among individuals like homeless drinkers, thiamine supplements should be given whenever they take beer or brewers should be taught the importance of supplementing alcohol beverages with thiamine.