Related Subjects:
|Acute Stroke Assessment (ROSIER&NIHSS)
|Causes of Stroke
|Ischaemic Stroke
|Cancer and Stroke
|Cardioembolic stroke
|CT Basics for Stroke
Introduction
- ๐ง Locked-In Syndrome (LIS) results from damage to the brainstem, particularly the ventral pons.
- It presents with a spectrum of severity, from partial to complete forms.
- ๐ First described by Plum and Posner in 1966.
๐ Key concept: LIS is characterised by quadriplegia + anarthria but preserved consciousness and vertical eye movements โ "conscious but trapped."
๐ฌAnatomy
- ๐ฆต Bilateral ventral pons damage โ quadriparesis.
- ๐ Facial and bulbar paralysis are common (loss of speech/swallowing).
- ๐ Vertical eye movements and blinking are usually preserved (midbrain intact); lateral gaze is lost.
- ๐ง Awareness and consciousness remain intact despite profound motor deficits.
- Other deficits depend on associated injuries โ blindness, ataxia, or sensory changes.
๐งฌ Aetiology
- Most common: โ๏ธ Pontine stroke (ischaemic/haemorrhagic) from basilar artery perforator occlusion.
- Other causes:
- โก Central pontine myelinolysis (rapid Naโบ correction).
- ๐งฌ Demyelination (e.g. Multiple Sclerosis).
- ๐ง Advanced Motor Neurone Disease (ALS).
- ๐๏ธ Tumours compressing the ventral pons.
- ๐ก๏ธ Guillain-Barrรฉ (severe, mimicking LIS).
- ๐ช Myasthenia gravis (fulminant form).
- ๐ค Trauma to brainstem.
๐ฉบ Clinical Features
- ๐ Often emerges after coma โ patient regains consciousness but is quadriplegic.
- ๐๏ธ Horizontal gaze palsy; vertical gaze and blinking usually intact โ primary mode of communication.
- ๐ฃ๏ธ Speech absent (mute), but comprehension preserved.
- ๐ฆต Quadriparesis varies depending on corticospinal tract damage.
- ๐ Vigilance: eye movements inconsistent, small, and fatiguable.
- ๐ง Cognition: usually preserved, with only mild deficits if any.
๐ Exam pearl: LIS โ coma. In LIS, the patient is awake and aware but cannot move or speak. Vertical eye movement is the diagnostic clue.
๐ Investigations
- ๐ฉป CT: Useful for excluding haemorrhage but may miss brainstem infarcts.
- ๐งฒ MRI: Gold standard for identifying pontine lesions.
- ๐ EEG: Demonstrates wakefulness and rules out diffuse cortical dysfunction.
๐ Management
- โก Acute: Depends on cause.
- ๐งฉ Basilar artery occlusion โ consider urgent thrombectomy.
- Electrolyte derangements โ correct carefully.
- ๐ฅ Supportive / Long-term:
- PEG feeding due to bulbar dysfunction.
- Respiratory support in acute phase (prevent aspiration pneumonia).
- Early physiotherapy and communication training (e.g. eye-tracking devices).
- โค๏ธ Many chronic LIS patients report meaningful quality of life. Requests for euthanasia are rare.
- โ๏ธ Patients must be supported to live with dignity, with access to rehab, pain control, and autonomy in end-of-life decisions.
๐ Clinical takeaway: Always distinguish LIS from coma or persistent vegetative state. Early recognition, supportive care, and communication aids are key to preserving dignity and quality of life.