This neurodegenerative disease killed Dr Lee Wei Ling, Lee Kuan Yew's daughter

Consultant neurologist Dr Lee Wei Ling, daughter of the late former prime minister of Singapore Lee Kuan Yew, was not shy about sharing her journey with progressive supranuclear palsy, which she was diagnosed with in 2020 and passed away from on Oct 9. — The Straits Times/ANN filepic

A prominent doctor and famous former first daughter of our neighbouring country recently died of a disease called progressive supranuclear palsy. What is this disease? How come I have never heard of it?

You’ve probably never heard of it because it is extremely rare.

Progressive supranuclear palsy is a rare brain disease.

It affects your walking, balance, eye movements, and also swallowing.

It is also called Steele-Richardson-Olszewski syndrome.

It is a disease that has no cure, unfortunately.

It becomes worse over time, and can lead to difficult complications.

It affects people in their 60s and 70s, and is rare in people under 40.

What does the word “palsy” mean?

Palsy is simply a medical word that means “paralysis” or “paresis”, which is partial paralysis.

Palsy is usually accompanied by body weakness, loss of sensation and uncontrolled body movements.

It is a very old word – you can even find it in the Bible.

Are there many types of “palsy”?

There are indeed several types, and are usually named according to what they affect. They are:

• Bell’s palsy – a partial facial paralysis that may be caused by inflammation, named after Scottish surgeon Sir Charles Bell who first described it
• Bulbar palsy – caused by lesions to the bulbar nerves
• Cerebral palsy – where there are lesions in the brain (cerebral) matter, causing neural problems
• Conjugate gaze palsy – where the ability to move your eyes is affected
• Brachial palsy – paralysis of the arm due to injury to the brachial plexus nerves
• Spinal muscular atrophy – a muscle-wasting disease
• Squatter’s palsy – a nerve palsy that may be triggered by squatting
• Third nerve palsy – involves the third cranial nerve
• Progressive supranuclear palsy.

How would I know if someone has progressive supranuclear palsy?

Progressive supranuclear palsy involves progressive damage to cells in only certain areas of the brain, particularly the brainstem.

Brain cells in an area called substantia nigra start dying.

Tau proteins then start accumulating and spreading to other parts of the brain.

This causes cells to die and neural connections to be severed.

All of this progressively interferes with your normal functioning, especially in the parts of the brain that control your body movements and thinking.

Therefore, you may experience a loss of balance while walking, especially with a tendency to fall backwards.

You cannot aim your eyes properly, such as looking downward.

You may experience blurring of vision or double vision.

You may be sensitive to bright light.

Other people may perceive you as being uninterested in conversation because patients with progressive supranuclear palsy may not look at other people due to these eye issues.

You may have stiffness, especially of the neck.

Your face may appear rigid.

Your speech becomes slow and slurred.

You have trouble swallowing.

You cannot sleep properly.

You lose interest in things, and have anxiety or depression.

You may exhibit impulsive behaviour.

The worst part is that the disease inevitably progresses.

What causes progressive supranuclear palsy?

No one knows.

It does not appear to be inherited.

Some theories suggest that it may be caused by random changes in particular cells or genes, exposure to some environmental factor (e.g. chemicals) that slowly damages some areas of your brain, or free radicals damaging these same cells.

But one thing for sure is that Alzheimer’s disease also has clumps of the same protein, tau.

Let’s say someone has progressive supranuclear palsy. What happens next? How long can the disease take to progress?

If you have progressive supranuclear palsy, there can be complications.

And one day, one of these complications may lead to death.

If your muscle movements become slow and difficult, then you may have:

• Falling, leading you to a higher risk of fractures and injuries.

  Some of these injuries may be very serious.

• Inability to focus your eyes, which may also lead to falling and injuries.
• Trouble swallowing, which can lead to choking and aspiration pneumonia.

In fact, pneumonia the commonest cause of death in patients with progressive supranuclear palsy.

Can progressive supranuclear palsy be treated?

There is no cure, so progressive supranuclear palsy is treated symptomatically.

It is possible to live with aid and careful attention.

To ease symptoms, medications like those for Parkinson’s disease can help with easing movement, and antidepressants can be used.

Botox can be injected around the eyes to improve spasms.

Special eyeglasses with prism lenses can be used to enable patients to look downward without moving their eyes.

And physical therapy to improve balance can be done.

Dr YLM graduated as a medical doctor, and has been writing for many years on various subjects such as medicine, health, computers and entertainment. For further information, email [e-mail]. The information provided is for educational and communication purposes only, and it should not be construed as personal medical advice. Neither The Star nor the author gives any warranty on accuracy, completeness, functionality, usefulness or other assurances as to such information. The Star and the author disclaim all responsibility for any losses, damage to property or personal injury suffered directly or indirectly from reliance on such information.