Wernicke – Korsakoff syndrome

05 November 2004

Prevalence of Wernicke – Korsakoff syndrome (Necropsy Study)

2.1% of adults >15 years old in Sydney

15% psychiatric inpatients

24% homeless men

Wernicke syndrome

Carl Wernicke (1881) : polioencephalitis hemorrhagica superioris

Punctate hemorrhages in grey matter around third & fourth ventricles & aqueduct of Sylvius.

Abrupt onset of   1.gaze palsy

                           2.gait ataxia

                           3.mental confusion

Clinical Features

Only 10-20% of cases will meet traditional rigid triad criteria

1.Ocular signs 29%

          horizontal nystagmus 85%

          bilateral rectus palsy 54%

          conjugate gaze palsy 45%

          vertical nystagmus

          complete ophthalmoplegia

          retinal hemorrhages

          papilledema

2. Ataxia 23%

3. Mental state change 82%

4. Hypotension

5. Hypothermia

Korsakoff Syndrome

SS Korsakoff (1887): psychosis polyneuritica

Amnestic syndrome & polyneuropathy

Clinical Features

Defect in learning (anterograde amnesia)

Retrograde amnesia

Defect in short term memory but registration & immediate recall is normal.

Confabulation

Wernicke – Korsakoff syndrome

80% overlap between Korsakoff & Wernicke syndromes

Associated features

Wet beriberi (cardiomyopathy)

Dry beriberi (peripheral neuropathy) 80% association.

Reduced olfactory nerve discrimination

Aids to diagnosis

1. Caloric testing

2. CT/MRI: lesions in medial portion of thalami & midbrain, dilation of third ventricle, atrophy of mammillary bodies & cerebellum, widening of interhemispheric fissures between frontal lobes.

3. Increased blood pyruvate

4. Reduced RBC transketolase activity

5. CSF reduced  3-methoxy-4-hydroxyphenyglycol (noradrenaline metabolite)

6. Macropathology (75% sensitivity): greyish discoloration, shrinkage, congestion & Punctate hemorrhages in mammillary bodies, superior cerebellar vermis, dorsal medial thalamus, hypothalamic nuclei & other diencephalic structures.

7.Micropathology (gold standard): always bilateral, symmetrical necrosis & demyelination in mammillary bodies, superior cerebellar vermis, dorsal medial thalamus, hypothalamic nuclei & other diencephalic structures.

Prognosis

10-20% mortality (usually from sepsis or decompensated liver disease)

45-85% institutionalization

Ophthalmoplegia improves within days

60% residual nystagmus or ataxia

80% chronic memory disorder
 

Treatment

Parental thiamine urgently (always before glucose)

Oral maintenance thiamine (Minimum daily requirement 1.4 mg/d)

Multi B

Balanced diet (Thiamine enriched flour reduced incidence of WKS in Sydney)

Abstinence

Possible role for clonidine or fluvoxamine.

Minimal improvement with psychotherapy.

Nonalcoholic Causes

Prolonged intravenous feeding

TPN

Hyperemesis gravidarum

Anorexia nervosa

Prolonged fasting

Refeeding after starvation

Gastric plication

Dialysis