A29. Wernicke-Korsakoff syndrome

Question 1

Wernicke-Korsakoff Syndrome (WKS)- what is it

Answer 1

is brain damage caused by thiamine (vitamin B1) deficiency,
usually secondary to chronic alcoholism or malnutrition.

Question 2

what is Thiamine and what is it involved in

Answer 2

• is an essential vitamin,
• its phosphorylated derivatives are involved in many cellular processes, including the catabolism of sugars and amino acids.

Question 3

what are the most vulnerable organs to thiamine deficiency

Answer 3

The brain and cardiovascular system are
the most vulnerable
* hence CNS and cardiac symptoms (hypotension, tachycardia)
are seen in WKS.

Question 4

what are the CNS and cardiac symptoms
seen in Wernicke-Korsakoff Syndrome WKS and why

Answer 4

• hypotension
• tachycardia

because brain and cardiovascular system are the most vulnerable to thiamine deficiency

Question 5

Foods rich in thiamine

Answer 5

• lean pork,
• rice,
• spinach,
• peas,
• whole wheat

Question 6

Wernicke-Korsakoff syndrome is by definition

Answer 6

when symptoms of two different conditions are seen together;
* Wernicke´s Encephalopathy (WE) and
* Korsakoff Syndrome (KS).

Question 7

when symptoms of:
* Wernicke´s Encephalopathy (WE) and
* Korsakoff Syndrome (KS)
are seen together

Answer 7

Wernicke-Korsakoff syndrome

Question 8

Wernicke encephalopathy
-is it acute or chronic?
-reversible or irreversible?

Answer 8

acute and reversible

Question 9

Wernicke encephalopathy Symptoms commonly show the classic triad of:

Answer 9

1. Oculomotor dysfunction
   ○ Horizontal and vertical nystagmus, diplopia, ophtalmoplegia, ocular nerve palsy, gaze palsy, anisocoria, dysconjugated eye movements, ptosis
2. Trunk and gait ataxia
3. Encephalopathy
   ○ Confusion, apathy, disorientation, memory problems, psychosis, delirium, if severe can progress to stupor and coma

Question 10

Oculomotor dysfunction symptoms in Wernicke encephalopathy

Answer 10

• Horizontal and vertical nystagmus,
• diplopia,
• ophtalmoplegia,
• ocular nerve palsy,
• gaze palsy
• anisocoria,
• dysconjugated eye movements,
• ptosis

Question 11

Encephalopathy symptoms in Wernicke encephalopathy

Answer 11

• Confusion,
• apathy,
• disorientation,
• memory problems,
• psychosis,
• delirium,
• if severe can progress to stupor and coma

Question 12

Wernicke encephalopathy where are the lesions?

Answer 12

Lesions are
* bilateral
* around 3 rd and 4 th ventricle
* and the cerebral aqueduct.

Question 13

are lesion unilateral or bilateral in Wernicke encephalopathy

Answer 13

bilateral

Question 14

if Wernicke encephalopathy is left untreated what happens

Answer 14

Can lead to fatal lactic acidosis if left untreated

Question 15

Korsakoff Syndrome is it acute or chronic
reversible or irreversible

Answer 15

chronic
* only 20 % reversible with treatment

Question 16

is Korsakoff Syndrome reversible?

Answer 16

only 20 % reversible with treatment

Question 17

Korsakoff Syndrome symptoms

Answer 17

memory symptoms
* Anterograde amnesia (problems learning new information) and short-term memory loss
* Confabulations (make up information to fill memory gaps)
* Disorientation in time and space

Question 18

which of the 2 precedes the other
wernicke encephalopathy and korsakoff syndrome

Answer 18

• Wernicke Encephalopathy usually precede Korsakoff syndrome, and
• symptoms of WE tends to
  decrease as KS develops.

Question 19

how do symptoms of WE change as KS develops

Answer 19

symptoms of WE tends to
decrease as KS develops.

Question 20

Wernicke-Korsakoff Syndrome is suspected if

Answer 20

If symptoms of Wernicke´s encephalopathy is associated with memory disturbances

Question 21

Wernicke-Korsakoff syndrome Etiology

Answer 21

Deficiency of vitamin B1 (thiamine), due to:
- Chronic alcoholism (most common)
- Malnutrition (starvation, eating disorders, gastric surgery, cancer, prolonged vomiting etc) →
decreased absorption of thiamine
- Kidney dialysis can also decrease absorption of thiamine
- AIDS can also be seen in background of WKS

Question 22

Deficiency of vitamin B1 (thiamine), can be due to:

Answer 22

• Chronic alcoholism (most common)
• Malnutrition (starvation, eating disorders, gastric surgery, cancer, prolonged vomiting etc) →
  decreased absorption of thiamine
• Kidney dialysis can also decrease absorption of thiamine
• AIDS can also be seen in background of WKS

Question 23

Diagnosis of Wernicke-Korsakoff Syndrome

Answer 23

Clinical diagnosis, based on
- Clinical evaluation and history
- Looking for possible causes by lab screenings (e.g albumin for malnutrition or kidney/liver problems) and LFTs
- Strengthen suspicion with thiamine and erythrocyte transketolase activity (both reduced)
- ECG to look for cardiac symptoms
- CT / MRI to rule out other CNS causes (tumor, stroke etc.) – can also show signs of
Wernicke-Korsakoff syndrome (shrunken mammillary bodies and lesions for example)
- Treatment improve symptoms

Question 24

Treatment of Wernicke-Korsakoff Syndrome

Answer 24

• Thiamine replacement therapy (iv, because abdominal absorption is often hampered)
• Treat underlying condition (alcohol abstinence, psychotherapy etc)

Question 25

Prognosis of Wernicke-Korsakoff Syndrome

Answer 25

• Depends of disease progression before treatment
• Without treatment the mortality is high

Question 26

what lab screening are done for possible causes of Wernicke-Korsakoff Syndrome

Answer 26

• (e.g albumin for malnutrition or kidney/liver problems) and
• LFTs

Question 27

which tests Strengthen suspicion of Wernicke-Korsakoff Syndrome ?

Answer 27

• thiamine
• erythrocyte transketolase activity (both reduced)

Question 28

why is ECG is done in Wernicke-Korsakoff Syndrome

Answer 28

to look for cardiac symptoms

Question 29

CT / MRI is done in suspicioun of Wernicke-Korsakoff Syndrome to rule out what?

Answer 29

• other CNS causes (tumor, stroke etc.)
• can also show signs of
  Wernicke-Korsakoff syndrome (shrunken mammillary bodies and lesions for example)

Question 30

sign on MRI/CT of Wernicke-Korsakoff syndrome

Answer 30

• can also show signs of
  Wernicke-Korsakoff syndrome (shrunken mammillary bodies and lesions for example)

Question 31

hows the mortality Without treatment in Wernicke-Korsakoff Syndrome

Answer 31

mortality is high

Question 32

Thiamine replacement therapy - how is it administered and why?

Answer 32

(iv, because abdominal absorption is often hampered)