Introduction

Locked-in syndrome is a rare neurological disorder that can occur as a result of a stroke.¹ The management options for both of these conditions are relatively similar, focusing on adapting certain areas of a person’s life in order to increase their quality of life. Spotting the signs of an acute stroke at an early point can help reduce the long-lasting effects of stroke and the risk of further deterioration.

Stroke

What is a stroke?

A stroke is a neurological disorder that has two main subtypes: ischaemic stroke and haemorrhagic stroke. Ischaemic stroke affects blood vessels in the brain by blocking arteries and reducing the normal flow to important structures throughout the brain. Occasionally, when blood flow is blocked or disturbed, bleeding can occur within the brain, which is known as a haemorrhage.² Depending on which area the bleed or blockage occurs in, different symptoms can arise. It is essential to recognise these symptoms in yourself or others as soon as possible after they appear, as a stroke is a medical emergency that requires immediate medical attention. 

Types of stroke location and symptoms

• Middle cerebral artery (MCA) infarction: stroke affecting the lateral part of the brain, causing opposite-sided hemiparesis, facial paralysis, sensory loss to the face and arms, visual field loss, dysarthria and aphasia
• Anterior cerebral artery (ACA) infarction: stroke affecting the frontal area of the brain, causing opposite side sensory or motor loss in the lower part of the body only
• Posterior cerebral artery (PCA) infarction: stroke affecting the occipital lobe of the brain, causing changes to vision
• Lacunar stroke: causes pure motor or sensory loss of function and ataxia
• Vertebrobasilar stroke: stroke affecting either of two vertebral arteries or the basilar artery supplying the back of the brain, causing headaches, vomiting, visual changes and ataxia³

Risk factors of stroke

There are several factors that can increase the risk of stroke, some being more easily modifiable than others. Being over the age of 55 is one of the biggest non-modifiable risk factors, alongside being assigned male at birth (AMAB) and Asian ethnicity.⁴ Modifiable risk factors include having a poor quality diet that is high in fat and low in variety, a higher body mass index (BMI), smoking and drinking alcohol regularly. Other modifiable major risk factors for having a stroke include comorbidities such as diabetes and high blood pressure.⁴ Being mindful of lifestyle choices is important when thinking about stroke prevention. 

After having a stroke, there is an increased risk of cognitive impairment, such as the onset of dementia, and functional impairment. Recovery can potentially take a long time following a stroke, often requiring input from various medical specialities to regain functionality. There is a 10-25% risk of long-term stroke recurrence at 1 year and 40% at 5 years post-stroke.⁵

Locked-in syndrome

The term ‘locked-in syndrome” refers to the complete state of body paralysis and sensory loss due to damage in the brain. Most commonly, a lesion is found in a specific area of the brain known as the brainstem, which causes this condition to occur. Some of the most common causes of locked-in syndrome are a consequence of stroke, masses in the brain, infections, trauma, and demyelinating conditions like multiple sclerosis (MS).⁶ This can be a very difficult condition to identify, diagnose and manage for medical professionals, and is a life-altering diagnosis for the patient and their families. 

Symptoms of locked-in syndrome

• Whole body paralysis (quadriplegia)
• Whole body sensory impairment
• Preservation of eye movements and cognitive function
• Dizziness and vertigo
• Retention of hearing
• Continued understanding of language comprehension and orientation to time and place⁷

Stroke management

A stroke is a medical emergency, and urgent medical attention is required after first spotting any of the FAST signs. The ‘FAST’ acronym is a useful tool to follow when in a public setting and there is suspicion of stroke. This stands for Face, Arms, Speech and Time, using the three most common and noticeable changes in features that are often associated with stroke and ensuring that you act fast in order to treat the acute stroke.⁸ Doctors will need to conduct several tests to determine what type of stroke is occurring and where in the brain it is occurring, for the treatment to begin as soon as possible. This may involve a CT scan of the brain, amongst other tests, including blood tests and blood pressure monitoring.⁹ It is important that these are completed immediately so treatment can be given within the first 4.5 hours of symptom onset.

There are two main treatment options for an ischaemic stroke. This depends on the current state of the person and the time since symptoms began. Treatment can be either an intravenous drug option or a surgical removal of the clot, known as a mechanical thrombectomy.⁹

The main aims when managing stroke and locked-in syndrome include improving the quality of life of the patient and ensuring that the person is pain-free in their current state, which involves many healthcare professionals from different areas of medicine. Early rehabilitation started at around 1 month after the event occurred has been shown to improve recovery and reduce mortality rates. Involvement with speech and language teams, if tracheostomies are necessary, as well as teams focusing on lung function and physiotherapy, will be needed for those with locked-in syndrome.¹⁰

Summary

Locked-in syndrome is a deeply challenging condition that, while being rare, can impact everyone involved in the person's life. It can be associated with stroke in some cases, but can also have other implications relating to causation. Being able to spot the first signs of acute stroke is very critical in improving the health outcome and reducing any long-term side effects often associated with stroke. Acute care and management of stroke and locked-in syndrome have some similarities in terms of physiotherapy, pain management and establishing a quality of life for the person. Caring for someone with Locked-In syndrome or stroke can potentially be challenging; however, it is important to understand that every little bit can help significantly, and there are many places to seek help for everyone involved.

FAQs

What are the signs of acute stroke?

The ‘FAST’ acronym is a useful tool in spotting the first signs of a stroke. This means looking for any facial drooping, any asymmetry or movement in the arms, or slurring of speech. Additionally, if someone is suddenly incoherent when talking or has numb legs or arms, then they should also seek medical attention.

Who is involved in treating locked-in syndrome?

There are many different healthcare professionals who may be involved in the care of those with locked-in syndrome. These can include speech and language therapists, dieticians to help with calorie intake, intensive care doctors for long hospital stays, tracheostomy insertions, respiratory healthcare professionals to maintain good lung function, and occupational health practitioners, to name just a few.