Locked-in Syndrome: Woman diagnosed with rare disorder after what seemed like a harmless headache

But Maita had an episode of severe vomiting at around 3:30 a.m. Minutes later, she started looking weak and confused about what was happening, showing signs of a stroke. It was when Mark knew it was time to seek immediate medical assistance for his mother.

She arrived in the emergency room at 5:00 a.m., and after a few medical tests, it was revealed that a blood clot was formed in her brain.

"Inignore niya lang talaga 'yung sign dahil akala niya dadatnaan lang siya,” Mark told L!fe. "Dinatnan siya ng [menstruation] niya nung nasa ICU na kami. Nagkataon na pala na nag-sabay ang mens at ang stress, resulting in severe headache. Namamaga na pala ‘yung ugat niya sa brain."

The incident left Maita paralyzed, unable to control any part of her body except her eyes. "I’m still in shock because I can't believe that the woman I recently sang karaoke with is now in bed, unable to move nor speak,” her son opened up.

Diagnosed with a rare disorder called Locked-in Syndrome, Maita is now only able to communicate with her loved ones through her eyes—like she’s in a "coma" but is aware of her surroundings and feelings, as Mark put it.

"If her eyes go upward, it means yes. If she closes her eyes, it means no. If her eyes are looking at the bottom, it means something is hurting, or there is discomfort within her. That's the only communication we have in the meantime," he shared. "Imagine not being able to say what you need—that must be so frustrating on her end.”

What is Locked-in Syndrome? 

While it sounds like something out of a science fiction novel, Locked-in Syndrome is very real and is caused by severe damage to the brainstem, the part of the brain responsible for controlling motor functions and vital bodily processes. 

"Locked-in Syndrome is a condition where a part of the brainstem (puno ng brain) is affected. It is the part of the brain where all the nerves for movements ay dumadaan. Kung may sugat o lesion doon, then there are totally no movements at all from the person," Dr. Jose Antonio "Iggy" Luis Pantangco, an adult neurologist and neuro-rehabilitation specialist, said in an interview with L!fe.

The defining characteristic of LiS is complete paralysis, often sparing only eye movement. Patients retain full cognitive abilities, which means they can think, understand, and process information, but they are unable to express themselves in typical ways. 

Pantangco continued, "Kaya the patient is 'locked-in,' ibig sabihin hindi niya maigalaw ang kanyang katawan, hindi siya makapagsalita, hindi makakain, in some cases, hindi maka-hinga pero gising, nakikinig."

Those with LiS can only respond through eye movements, or, in rare cases, the ability to move a finger or toe, which is far different from being in a coma or a vegetative state. 

"Sa coma, hindi ka gising. If vegetative state ka, gising [ka] but not aware of the environment, [meaning], you cannot sense and react to the environment," Pantangco noted. 

LiS is typically caused by a catastrophic event that damages the brainstem.

"The most common cause of LiS is stroke—may nagbara o pumutok na ugat sa puno ng brain or brainstem, causing these symptoms. It's very small, but pwede din bukol or tumor or other neurological disease pwede mag-cause nito," Pantangco said. 

Other potential causes also include traumatic brain injury, brain hemorrhages, and certain diseases, such as amyotrophic lateral sclerosis (ALS).

Warning signs, diagnosis

While LiS has no specific or clear symptoms, remembering the acronym B.E.F.A.S.T. can help an individual spot the signs of stroke early.

According to Pantangco, diagnosing LiS can be tricky because its symptoms are often confused with a coma or other conditions that cause unresponsiveness. Neurologists rely on several diagnostic methods to distinguish LiS from other conditions, including physical examinations, neurological tests, brain imaging, and electroencephalography, which measures electrical activity in the brain and can help determine whether the person is conscious.

Treatments and therapies are currently available for patients with LiS, depending on its cause. "If the stroke is the cause, most likely the patient will need rehabilitation and hopefully, makapag-improve, and if tumor, [it can be treated through] surgery," the neurologist said.

Ultimately, great care is needed to manage patients with LiS as they will need help with the basics like eating, bathing, urinating, defecating, and sometimes, breathing.

At the moment, Maita is still intubated at the intensive care unit as her condition has significantly affected her breathing. "We visit her daily during the allowed visitation hours. We also make sure to massage her arms and legs as we were informed she is prone to cramps," Mark told L!fe.

No positive response has come from his mother so far, and Mark admitted feeling a mix of sadness and frustration "for not expressing my love for her enough."

He recalled having an argument with his mother that made him leave their family home prior to the incident. "I was so disappointed in myself dahil 'yun ang last na nagkausap kami ng mama. Totoo 'yung nasa huli ang pagsisisi and ever since this happened, araw-araw, kinakausap ko ang mama, humihingi ng sorry, at patuloy na kinekwentuhan ng mga plano namin 'pag umokay na siya."

Still, Mark noted that he and his family are not losing hope.

"We hope and pray to God to give my mother a chance that she deserves," he said, "and we will make sure that she will live the best of her life."