Diagnosis and treatment of Meijer syndrome

Meige syndrome is a group of extrapyramidal disorders first described by French neurologist Henry Meige in 1910, and is also an idiopathic dystonia involving the head and face, and occasionally the neck, upper limbs, and trunk. It is difficult to treat and often leads to low self-esteem and autism, prevents walking and reading, and prevents normal communication with others, thus seriously affecting the patient’s normal life and work. Major’s syndrome is more common in middle-aged and elderly women. The first symptoms are mostly bilateral blepharospasm, which can gradually involve the mouth, face and jaw. Patients exhibit frequent involuntary blinking, difficulty in opening the eyes, and involuntary muscle movements of the mouth and jaw in the form of “eyebrow squeezing”. In a few patients, the disease can start unilaterally and gradually involve the opposite side. The symptoms are often triggered and aggravated by stress, fatigue, bright light, and gaze, and improve during sleep, speech, singing, and mouth opening. Meijer syndrome needs to be distinguished mainly from facial myasthenia, which is often involved only unilaterally and rarely bilaterally. It mainly presents with involuntary jumping and twitching of the eyelid unilaterally and can involve the ipsilateral corner of the mouth and cheek, but does not present with involuntary movements of the oral and facial jaws bilaterally. Non-surgical treatment of Meijer syndrome Non-surgical treatment of Meijer syndrome consists mainly of Botox injections and medications. Botulinum toxin injections may result in symptomatic improvement in some patients, but relapse usually occurs within 3-6 months, and the effect further diminishes with repeated injections. The overall effect of drug treatment is not satisfactory. Commonly used medications include Antan, clonidine, baclofen, haloperidol, clonazepam and others. The drugs are ineffective in many patients, only partially improving symptoms in some patients, and the side effects caused by the drugs are often difficult to tolerate. Levodopa can aggravate the condition. Carbamazepine and sodium valproate are not effective. Surgical treatment of Meijer syndrome Surgical treatment is currently the best treatment for Meijer syndrome. In recent years, we have used deep brain electrical stimulation (DBS) to treat Meijer syndrome with good results. The majority of patients have achieved significant improvement in their symptoms after surgery. Some patients can easily open their eyes, which are usually almost closed, on the first day after surgery. Deep brain electrical stimulation (DBS) is the latest development in today’s technology, and the microelectrodes are accurately implanted into the relevant nuclei in the brain through a stereotactic technique. The patient’s symptoms are improved by correcting them. The DBS procedure has the advantages of being minimally invasive, safe, reversible and controllable, and long-lasting. The microelectrodes, wires and stimulators are embedded under the patient’s skin, and the patient looks like a normal person after the procedure, which does not affect the patient’s daily work and life. Compared with other therapies, DBS is currently the best treatment for Meijer syndrome.