Understanding Meige Syndrome: Clinical Treatments and Differences from Facial Spasms

Image Source | Internet

『Meige Syndrome (MS)』 is a focal/segmental cranial dystonia that involves eyelid spasms and/or involuntary contractions of the submandibular muscles, leading to segmental dystonia. As the disease progresses, it gradually affects the entire facial and even neck muscles.

Currently, MS is classified into 4 types based on the affected areas of dystonia:

(1)Eyelid Spasm Type (BSP): Primarily involves involuntary movements or tonic spasms of the orbicularis oculi muscle, often presenting as frequent blinking or difficulty keeping the eyes open, with pre-symptomatic sensations of dryness, burning, or foreign body sensation.

(2)Oral Submandibular Dystonia Type (OMD): Often involves involuntary spasms of the orbicularis oris and/or masticatory muscles, manifesting as involuntary spasms around the mouth, submandibular muscles, and even the tongue, such as teeth grinding, jaw clenching, and lip and jaw twisting.

(3)Eyelid Combined Oral Dystonia Type: This is the classic type of MS, characterized by involuntary contractions of the eyelids combined with the submandibular muscles, which can develop from the above types, often resulting in bizarre facial expressions in patients.

(4)Other Types: These types combine neck, trunk, and other body parts with dystonia, presenting with speech difficulties, breathing problems, and limited movement.

These types can occur individually or develop from BSP or OMD. Such involuntary contractions can generally spread to other body parts within 5 years.

The exact pathogenesis of Meige Syndrome is currently unclear; however, long-term use of psychiatric medications, facial nerve infections, and psychological factors may trigger MS.

Differential Diagnosis

『Facial Spasms (HFS) primarily involves unilateral involuntary spasms of facial muscles, rarely affecting both sides, while MS often presents bilaterally. Additionally, in HFS patients, magnetic resonance angiography usually shows vascular compression of the facial nerve on the affected side, whereas no vascular compression has been found in MS patients.

『Myasthenia Gravis』 This disease can also present with difficulty keeping the eyes open, but in the former, there is abnormal strength in the levator palpebrae with symptoms that worsen throughout the day, and the edrophonium test is positive (injection of edrophonium improves symptoms after 20 minutes, lasting 2-3 hours), with chest CT often showing thymic hyperplasia or thymoma; whereas in the latter, the difficulty in keeping the eyes open is due to involuntary contractions of the orbicularis oculi, with normal strength in the levator palpebrae, the edrophonium test is negative, and chest CT often shows no abnormality of the thymus.

Treatment Methods

Medication Treatment: Currently, the effectiveness of medication treatment is limited, and the duration of efficacy is often short. Commonly used medications include anticholinergic drugs, such as benztropine, which can reduce the frequency of eyelid spasms; sedative or antipsychotic medications, such as diazepam and haloperidol, can effectively alleviate symptoms but have significant side effects; muscle relaxants, such as baclofen.

Botulinum Toxin Injection: After receiving type A botulinum toxin treatment, symptoms may alleviate but are prone to recurrence; repeated injections remain effective, with adverse reactions mainly including eyelid ptosis, facial paralysis, dry mouth, and local swelling, which will completely disappear after a certain period.

Surgical Treatment: The main methods include selective facial nerve sectioning, eyelid surgery, and DBS.

• Selective sectioning of the facial nerve to alleviate BSP involves primarily cutting the zygomatic or buccal branches of the facial nerve to reduce frequent involuntary blinking and closing while preserving the ability to close the eyelids voluntarily.
• Alleviating BSP by excising part of the eyelid muscles (part of the orbicularis oculi, pretarsal muscle, preseptal muscle, and corrugator muscle) is another method.
• DBS is the most commonly used surgical method with the most clinical research. DBS surgery, also known as deep brain stimulation, is a new surgical method for treating Meige syndrome that has emerged with the development of stereotactic technology. It involves implanting fine electrodes in the brain to emit electrical pulses to stimulate specific nuclei in the brain, controlling the abnormal excitation of neurons, thereby improving motor symptoms, with characteristics of reversibility and controllability.

Speculation

As one of the difficult-to-cure conditions, MS poses challenges for both patients and doctors, especially for patients who suffer not only physically but also psychologically.

Is there a better direction for treatment in the future? A complete reflex arc requires receptors – afferent nerves – central – efferent nerves – effectors. Some researchers have found that performing radiofrequency treatment on the trigeminal nerve for trigeminal neuralgia and on the facial nerve for facial spasms allows patients to open their eyes and reduces masticatory muscle strength post-surgery. Cutting the relevant responsible nerves controlling muscle movement in MS patients (such as selective facial nerve sectioning) can stop signal transmission.

So, would targeting different types of MS with radiofrequency treatment on the facial nerve and/or trigeminal nerve effectively alleviate MS symptoms? Perhaps this is a direction worth exploring.

References

Liu Xiaolan, Xu Shuangshuang, Zhao Wei, et al. Clinical Diagnosis and Treatment Progress of Meige Syndrome [J]. Chinese Journal of Pain, 2022, 18(3): 419-423.

『Voice of Pain』 is a learning team formed by young doctors in the field of pain medicine (anesthesiology) at the undergraduate, master’s, doctoral, and postdoctoral levels, if you are also eager to learn, join us! Professional content writing guides and excellent seniors are waiting for you to chat (scan the code below).

Do you have any experience in treating Meige Syndrome? Add the WeChat of the administrator below to join the group chat! Pain Doctors Group Chat // Anesthesia (Pain) Master’s and Doctoral Club // Anesthesia (Pain) Training Group // National Anesthesia (Pain) Graduate Circle, there’s bound to be a group chat that suits you

Author | Yao Weijie

Editor | Ma Xiaoxuan