Cao, a patient in Jiangxi, suddenly developed dry eyes, itchy eyes, and frequent blinking two years ago, and at first thought it was due to his busy schedule and excessive eye use. He was unable to take care of himself and became literally “blind”. After the disease, Cao went to major hospitals across the country and was misdiagnosed with “dry eye”, “blepharospasm”, “facial spasm” and “myasthenia gravis”. He tried western medicine, Chinese medicine, acupuncture, and local botulinum toxin injections, all of which did not work. After many inquiries, he found the director of the neurology center of the General Hospital of Aviation of China Medical University, Wang Lin, who made a detailed analysis of the case and confirmed the diagnosis of refractory Meige syndrome, and led the team to perform brain pacemaker surgery for the patient. A few days ago, the patient drove himself to Hangzhou and saw the beautiful scenery in front of him, so he couldn’t help but call Director Wang Lin to express his gratitude. What is Meige syndrome? Director Wang Lin said, “Meige syndrome is a limited dystonia disorder that belongs to adult hyperactivity and was first reported by French neurologist Meige in 1910. The etiology and pathophysiological mechanisms of Meige syndrome are still unclear. Most scholars believe that the pathogenesis of the disease may be related to damage to the basal ganglia of the brain and hypofunction of nigrostriatal r-aminobutyric acidergic neurons, resulting in dopaminergic receptor hypersensitivity or dopamine transmitter imbalance and cholinergic imbalance. The disease has been reported to be associated with the use of certain medications, such as long-term use of psychostimulants, anticonvulsant paralytics, and anxiolytics. It has also been reported that the disease is associated with reduced cortical inhibition due to environmental factors and genetic susceptibility, autoimmune and psychosomatic factors. It has also been shown that facial trauma, including dental surgery, can cause oromandibular dystonia, which is particularly pronounced in susceptible populations. Meige’s syndrome usually begins in middle-aged and old age, mostly in women, with a male to female ratio of 1:2 to 3. It usually starts slowly with a feeling of irritation or discomfort in one or both eyes, shyness and increased blinking frequency, and dry eyes, and later develops into eyelid spasm. The symptoms worsen with fatigue, sunlight irritation, gaze, and stress, diminish when the mind is focused on something other than blepharospasm, and disappear during sleep. Most patients have paroxysmal blepharospasm as the first symptom. Blepharospasm is dominated by contraction of the orbicularis oculi muscle and contraction of the frontal and nasal muscles centered between the eyebrows, manifested by increased transients and tearing, and a few develop functional blindness after 1 to 2 years, eventually developing into severe bilateral blepharospasm, difficulty opening the eyes, and even loss of self-care. Some patients start with blepharospasm and gradually progress to the lower face, showing symmetrical irregular and hyperactive contraction of the mandibular muscles of the mouth. The specific symptoms of Meige syndrome can be divided into the following categories: eyelid involvement: in mild cases, eye discomfort, dry eyes, photophobia, and increased transients can be manifested, and some are misdiagnosed as “conjunctivitis”; in more severe cases, there are episodes of eye closure and difficulty in opening the eyes, requiring the use of fingers to open the eyelids; in severe cases, functional blindness can result. Mouth-mandibular involvement: Involuntary mouth opening, mouth closing, pouting, lip retraction, cheek biting, tongue biting, and tooth bruising. Neck muscle involvement: manifests as neck discomfort, sloping neck, head shaking, head tilting back, shrugging shoulders, etc. In severe cases, it is difficult to maintain normal head position. Others: Involvement of tongue muscles manifests as involuntary movements such as tongue retraction or tongue extension, tongue twisting or tongue root tightening and stiffness; involvement of pharynx may show pharyngeal discomfort, cough, slurred pronunciation and difficulty in swallowing; involvement of frontal muscles shows forehead tightening and frowning; involvement of hands and feet and limbs shows postural tremor, writing spasm, foot inversion and involuntary twitching; involvement of chest and abdomen may show local involuntary twitching, accompanied by chest tightness and breath-holding. Treatment of Meige syndrome Wang Lin said that the current treatment for Meige syndrome is mainly symptomatic treatment, treatment methods include oral medication, surgical treatment, local injection of botulinum toxin type A treatment. For patients whose medication is ineffective or less effective, or whose medication has obvious side effects and whose symptoms significantly affect their lives, neurosurgical intervention is also a more effective treatment option. The most advanced international treatment method is Deep Brain Stimulation,
DBS, commonly known as “brain pacemaker” surgery, is minimally invasive, reversible, adjustable and personalized, and is a safe and effective treatment with fewer side effects. According to Wang Lin, director of the Department of Neurosurgery of the Aviation General Hospital, there is still no special cure for Meige’s syndrome, and pacemaker surgery has been introduced into China for nearly 16 years, mainly for the treatment of Parkinson’s disease and other dystonic diseases. The only disadvantage is the high cost of the pacemaker, which was the exclusive monopoly of Medtronic, but now the domestic pacemaker from Tsinghua University has been marketed, and the cost is only about half of the imported pacemakers, bringing a boon to the majority of patients with Meige syndrome and Parkinson’s disease. The indications for “brain pacemaker” surgery Wang Lin said that Meige syndrome has no special cure due to its complex etiology and unclear pathogenesis, and the commonly used treatment methods mainly include neurological medication, local botulinum toxin injection and neurosurgery, but medication is only effective in some patients. However, drug treatment is only effective in some patients, and for patients with effective drug treatment, drug tolerance will become less effective as the time of taking the drug increases, resulting in an increase in the type and dose of drugs and thus the side effects of drugs will increase significantly. Local botulinum toxin injections are suitable for patients with limited dystonia and are also effective in some patients, while long-term high-dose injections can easily lead to local muscle paralysis. Therefore, for patients with poor efficacy of medication or obvious side effects of medication and without serious heart and lung diseases, all patients are suitable for deep brain electrical stimulation (“brain pacemaker surgery”) treatment.