WERNICKE'S SYNDROME (contd) Clinical features: Acute in onset
Horizontal and vertical nystagmus is evident.
Unilateral or bilateral VI nerve paresis commonly occur. Gaze palsies are less common. Retinal haemorrhages occasionally occur.
Pupillary involvement and complete ophthalmoplegia are rare.
Polyneuropathy is present in 80% of cases. Vestibular disturbances will occur occasionally and accentuate the ataxia. Autonomic disturbance is common.
Disinterested and inattentive. Coma may dominate presentation.
Withdrawal symptoms of: alcohol
- agitation, delusions and hallucinations - develop following admission to hospital. Ataxia - is often the presenting symptom. May be mild or severe with inability to stand. Lower limb (heel to shin) ataxia is modest.
Upper limbs are spared.
Haematological and biochemical evidence of alcohol/nutritional deficiency, e.g. elevated MCV, abnormal LFTs, elevated y GT.
The erythrocyte transketolase (enzyme in hexose monophosphate shunt), an index of thiamine levels, is reduced.
The blood pyruvate is elevated. MRI may show mamillary body atrophy. Treatment
100 mg of thiamine i.v. is given on suspicion of diagnosis. Thereafter daily infusions of 500 mg until a normal diet supplemented by 100 mg thiamine three times per day is tolerated. Never give glucose infusions prior to thiamine as this may precipitate or aggravate the disorder.
With treatment - Eyes improve - in days, though nystagmus may persist for months.
- Ataxia improves - in weeks. Overall mortality: 15% -* coma -* death. KORSAKOFF'S PSYCHOSIS
When patients survive Wernicke's syndrome up to 80% develop this condition. Pathology
Lesions are identical in distribution to those of Wernicke's syndrome without haemorrhagic change. Clinical features
There is a disturbance of memory in which new information cannot be stored. In addition the normal temporal sequence of established memories is disrupted, resulting in a semifictionalised account of the circumstances which the patient may find him/herself in (.confabulation). This memory disturbance can only be tested for when the confusion of Wernicke's disease has cleared. Treatment
Oral thiamine 100 t.d.s. should be continued for some months, although only 20% of patients show improved memory function.
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