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The first time Rosa Alarcón realised she couldn’t open her eyes was while trying to cross the street in her native Barcelona.
“My eyes closed and I couldn’t open them. The cars started honking and I got scared because I didn’t know where to move,” the Spanish politician told the El Diario website.
“Luckily a man yelled at me: ‘Madam, what are you doing?’ He grabbed me and took me to the pavement.”
Alarcón, a Spanish politician whose eyelids close involuntarily and sometimes stay shut for days, has focused attention on a rare condition which seldom is talked about.
Meige syndrome, is a neurological condition that leads to abnormal movement of the eyelids (blepharospasm) and spasms in the jaw and tongue, sometimes also affecting the neck.
It was first described by Henri Meige, a French neurologist, at the turn of the last century. It used to be called Brueghel syndrome after Pieter Brueghel the Elder, the 16th century Dutch painter.
Tom Warner, a professor of clinical neuroscience at the UCL Institute of Neurology, explained: “One of his portraits had someone grimacing and wincing and they thought it might be a historic example of Meige syndrome.”
What is Meige syndrome?
Meige syndrome is a form of a much wider spread problem called dystonia, which describes involuntary muscle spasms that can affect any part of the body.
Prof Warner said: “It is a bizarre neurological condition, but overall it’s not that uncommon. There are even more severe forms which are often genetic that often affect children from an early age. But most of the forms like Meige come on in older life, at middle age or beyond.”
Alarcón, a Barcelona city councillor involved in local politics for more than 25 years, was diagnosed with Meige syndrome in April 2022, three years after she began experiencing symptoms.
What are the symptoms of Meige Syndrome?
The first symptoms to appear are an increased rate of blinking, uncontrollable squinting of eyes, light sensitivity, eyes closing during speech and uncontrollable eyes closing shut.
It is more common in women than men. “For many years, we didn’t know why that was. What now seems to be likely is that we know many parts of the brain are very susceptible to hormones, not least oestrogen and it may be something to do with oestrogen receptors.”
He adds: “For most of these cases of dystonia, we can do brain scans and you see no abnormalities at all, so it’s not like Parkinson’s where brain cells pack up and die. It seems to be a dysfunction of the pathways controlling movements, rather than of cell death. So all the tests you do are normal.”
It is therefore frequently difficult for sufferers to receive diagnosis, and when Alarcón sought help at a health centre following the incident, she was told she had dry eyes.
After a more extended episode a few months later in which her eyes stayed shut for 10 days, Alarcón got serious about getting expert medical advice and obtaining a diagnosis.
“Seventy years ago, many cases were thought to be psychiatric in origin. It’s become quite clear it’s a neurological disorder,” said Prof Warner.
Treatment for dystonia and Meige Syndrome
This month is Dystonia Awareness month and the Dystonia Society which offers support for sufferers. Prof Warner is one of the society’s medical advisers. There are thought to be about 100,000 cases of dystonia of all types in the UK.
Prof Warner said: “Most general practices will have a couple of patients with dystonia. Meige is one of the rarer ones and one of the more debilitating ones. If you have spasms of the jaw, it might force your food out of your mouth, which isn’t under your control.”
There is currently no cure, only treatment of symptoms. He added: “For some of the more severe forms of dystonia, particularly those affecting children, there is a type of brain surgery called deep brain stimulation and that can be quite effective.
“It’s also used in Parkinsons. But for focal dystonia, that only affects one part of the body, like Meige, Botulinum toxin (Botox) injections are most effective.”
This helps to reduce the amount of spams by relaxing the muscles and allows people to keep their eyes open.
“It’s not curative,” said Prof Warner. “It reduces symptoms rather than cures them and it’s less effective for jaw and tongue spasms. So it’s very stigmatising. When people see someone grimacing as they walk down the street, they think they look odd.”
In 33 per cent of patients with Meige syndrome, emotional stress may be a factor as their primary symptoms are anxiety, depression, and sleep problems.
Alarcón tried to return to work after her diagnosis and intended to fight this year’s local election for the Catalan Socialist Party. But she resigned, saying she needed to accept that she needs more time for relaxation than council work allows for.
She now has more than 6,000 followers on TikTok and some of her videos have been viewed around half a million times, after she began to share videos to explain the difficulties of coping with her condition, as well as boost awareness and understanding of the day-to-day challenges faced by the visually impaired.
“I thought that, beyond life in politics, TikTok is a way to reach a larger number of people to make the illness more visible,” Alarcón said.