Learning objectives
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Introduction
- Damage to the brainstem especially the ventral pons can result in a locked in syndrome
- Cases vary in their degree and there is a spectrum
- First descrbed by Plum and posner in 1966
Anatomy
- Bilateral damage to the pons can result in quadriparesis
- Facial and Bulbar paralysis
- Preservation of Midbrain IIIrd/IVth eye movements but loss of lateral gaze
- Awareness and consciousness
- Whatever deficits causes by other strokes beyond pons e.g. Blindness, ataxia etc.
Aetiology
- Pontine stroke Infarct or haemorrhage affecting ventral pons usually due to damage to ventral perforators from Basilary artery
- Central pontine myelinosis
- Demyelination
- Late stage Motor neurone disease
- Tumour affecting ventral pons
- Guillain Barre synbdrome
- Myasthenia
- Trauma
Clinical
- May wake up after a period of coma. Horizontal gaze palsy
- Appears awake - can open eyes to command and move eyes depending on nuclei damaged
- Usually mute and unable to speak due to bulbar weakness but understands and can produce non verbal language
- Quadriparesis which depends on extend of damage to corticospinal tracts in ventral pons
- Vigilance is fluctuating and eye movements may be inconsistent, very small, and easily exhausted
- Maybe some cognitive deficits but not usually severe
Investigations
- CT scan may show bleed. MRI is best to show anatomy of any lesion
- EEG: will show wakefulness
Management
- Acute stroke management depending on cases e.g. Thrombectomy for basilar artery occlusion
- Once medically stable, and given appropriate medical care, life expectancy increases to several decades.
- Long term will need PEG tube for feeding
- May need respiratory support and will be at high risk for aspiration
- Chronic LIS patients typically self-report meaningful quality of life and their demand for euthanasia is surprisingly infrequent.
- Patients suffering from LIS should not be denied the right to die - and to die with dignity
- They should not be denied the right to live - with dignity and the best possible revalidation, and pain and symptom management
References and further reading
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