Related Subjects:
|Acute Stroke Assessment (ROSIER&NIHSS)
|Causes of Stroke
|Ischaemic Stroke
|Cancer and Stroke
|Cardioembolic stroke
|CT Basics for Stroke
Introduction
- 🧠 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.
- 📖 First described by Plum and Posner in 1966.
🔑 Key concept: LIS is characterised by quadriplegia + anarthria but preserved consciousness and vertical eye movements → "conscious but trapped."
🔬Anatomy
- 🦵 Bilateral ventral pons damage → quadriparesis.
- 🙂 Facial and bulbar paralysis are common (loss of speech/swallowing).
- 👀 Vertical eye movements and blinking are usually preserved (midbrain intact); lateral gaze is lost.
- 🧍 Awareness and consciousness remain intact despite profound motor deficits.
- Other deficits depend on associated injuries → blindness, ataxia, or sensory changes.
🧬 Aetiology
- Most common: ⛏️ Pontine stroke (ischaemic/haemorrhagic) from basilar artery perforator occlusion.
- Other causes:
- ⚡ Central pontine myelinolysis (rapid Na⁺ correction).
- 🧬 Demyelination (e.g. Multiple Sclerosis).
- 🧓 Advanced Motor Neurone Disease (ALS).
- 🎗️ Tumours compressing the ventral pons.
- 🛡️ Guillain-Barré (severe, mimicking LIS).
- 💪 Myasthenia gravis (fulminant form).
- 🤕 Trauma to brainstem.
🩺 Clinical Features
- 🛌 Often emerges after coma → patient regains consciousness but is quadriplegic.
- 👁️ Horizontal gaze palsy; vertical gaze and blinking usually intact → primary mode of communication.
- 🗣️ Speech absent (mute), but comprehension preserved.
- 🦵 Quadriparesis varies depending on corticospinal tract damage.
- 👓 Vigilance: eye movements inconsistent, small, and fatiguable.
- 🧠 Cognition: usually preserved, with only mild deficits if any.
📝 Exam pearl: LIS ≠ coma. In LIS, the patient is awake and aware but cannot move or speak. Vertical eye movement is the diagnostic clue.
🔎 Investigations
- 🩻 CT: Useful for excluding haemorrhage but may miss brainstem infarcts.
- 🧲 MRI: Gold standard for identifying pontine lesions.
- 📉 EEG: Demonstrates wakefulness and rules out diffuse cortical dysfunction.
💊 Management
- ⚡ Acute: Depends on cause.
- 🧩 Basilar artery occlusion → consider urgent thrombectomy.
- Electrolyte derangements → correct carefully.
- 🏥 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).
- ❤️ Many chronic LIS patients report meaningful quality of life. Requests for euthanasia are rare.
- ⚖️ Patients must be supported to live with dignity, with access to rehab, pain control, and autonomy in end-of-life decisions.
🌟 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.