Locked in Syndrome

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 described by Plum and Posner in 1966

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.

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 syndrome
Myasthenia
Trauma

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

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