Wernicke Korsakoff, Cerebellar Flashcards
What is Wernicke Korsakoff syndrome?
Wernicke Korsakoff syndroms is a spectrum of diseases resulting from thiamine deficiency - usually related to alcohol abuse. Wernicke’s encephalopathy is a classic triad: mental confusion, ataxia and nystagmus/opthalmoplegia (paralysis of muscles within/surrounding eye). Korkasoff’s syndrome is the late manifestation of the condition when Wernicke’s encephalopathy has not been adequately treated.
Prevalent in low socio-economic areas - 50-60yrs.
Risk factors for Wernicke Korsakoff syndrome?
1) Thiamine deficiency - Decreased intake, malabsorption from GI tract, deficiency due to increased demand.
2) Alcoholism - High risk of thiamine deficiency - inflammation of stomach lining due to alcohol, and poor nutrition and diet lacking essential vitamins.
Pathophys of Wernicke Korsakoff?
Wernicke’s encephalopathy will progress to Kosakoff’s if not treated, due to damage of the hypothalamus and cerebral atrophy resulting from a deficiency of thiamine.
(Thiamine plays a role with carbohydrate metabolism thus deficiency will interfere with numerous cellular functions).
Clinical presentation of Wernicke Korsakoff’s syndrome?
Wernicke’s: Ataxia, confusion, nystagmus, ophthalmoplegia, altered GCS, peripheral sensory neuropathy.
Korsakoff’s: Memory loss, inability to make new memories, confabulation (invented memory - retrograde amnesia), lack of insight and apathy.
Ddx of Wernicke Korsakoff?
1) Drug abuse
2) Dementia
3) Delirium Tremens
4) Viral encephalitis
Diagnosis of Wernicke Korsakoff?
1) U&Es to check for hypernatraemia, hypercalcaemia, uraemia.
2) LFT’s
3) Serum thiamine (B1) levels
4) Red cell transketolase activity is decreased in thiamine deficiency (not diagnostic)
Treatment of Wernicke Korsakoff?
Failure to treat appropriately will result in either death, or the rest will be left with Korsakoff’s syndrome (1/4 reversible and 1/4 long term institutional care).
1) Check glucose levels for hypoglycaemia, if so GIVE THIAMINE FIRST before glucose so that wernicke’s is not precipitated by glucose administrations.
2) IV Thiamine over 1 week (then oral supplementation until no longer at risk)
3) IV Magnesium Sulphate - Thiamine deficiency is associated with magnesium deficiency as well.
Pathophys of cerebellar disorder/syndrome?
Contains motor and sensory representation of the whole body, and controls timing and pattern of motor activation during movement - balance and coordination. Most important layer is the Purkinje layer and this is the only output of the cerebellum - Purkinje cells fire at a very high frequency and when holding posture they are constantly firing, therefore Purkinje cell layer degeneration or damage will result in dysfunction, therefore ataxia. Ataia affects movement, coordination and balance.
Aetiology of cerebellar syndrome?
1) Freidrich’s ataxia - Autosomal recessive, early-onset hereditary, 10-15yrs onset, present with gait ataxia and kyphoscoliosis.
2) Ataxic telangectasia - Autosomal recessive, present with ataxia and telangiectasia.
3) Neoplasm - cerebellar haemangioma
3) Stroke, MS, hypothyroidism, alcohol
4) Drugs - phenytoin, lead poisoning
Pathophys of cerebellar disorder?
Unilateral cerebellar lesions cause IPSILATERAL SIGNS
Presentation of cerebellar disorder?
DANISH D - Dysdiochokinesia, dysmetria, drunk appearance A - Ataxia N - Nystagmus I - Intention Tremor S - Slurred, stoccato speech H - Hypotonia
Treatment of cerebellar disorder?
Treat cause
