Interviewed expert: Wang Lin, Director of the Second Department of Neurology, Chief Physician, Aviation General Hospital.
Editor: In life, the eyelids occasionally jump may not be taken seriously, at most muttering a left eyelid jump is a good thing, but have you ever thought that this may also be a disease. So what is the disease, and how to treat it? Xiaobian specially consulted Wang Lin, director of the Second Department of Neurology (Functional Neurosurgery Ward) of Aviation General Hospital, to explain the relevant knowledge about Major syndrome.
1. Editor: Director Wang, can you tell us what is Major syndrome?
Director Wang Lin: Major syndrome was first described by French neurologist Henry Meige in 1910, which is manifested as segmental dystonia, which mainly causes eyelid spasm and various forms of dystonia in the face, jaw and neck of the low position, and is an adult-onset facial dystonia disease. Also known as idiopathic eyelid spasm-oral-mandibular muscle tone syndrome, Brueghel syndrome, some scholars believe that it is a type of adult ADHD.
2, Editor: Is that limb shaking also counted as Major syndrome? How do you distinguish between epilepsy and Major syndrome?
Director Wang Lin: If the simple limb shaking is generally not Major syndrome, if the transient shaking it may be physiological (such as twitching after muscle fatigue, etc.); if it is repeated and prolonged shaking, it may be a manifestation of some dystonia disease. Like Parkinson's disease, idiopathic tremor, tics, etc., most of them also have other symptoms to distinguish them. The fourth type of Major syndrome can also be combined with limb twitching, but he must also have eyelid spasm and manifestations of various forms of dystonia in the face, jaw, and neck at a low level.
Epilepsy is commonly known as "sheep's horn wind" or "sheep epilepsy", which is a chronic disease of sudden abnormal firing of brain neurons, resulting in transient brain dysfunction, and the clinical manifestations are complex and diverse, which can be manifested as episodic motor, sensory, autonomic, conscious and mental disorders. Most patients have transient impaired consciousness, limb twitches and other symptoms, while patients with Major syndrome mainly present with symmetrical eyelid spasm and various forms of dystonia in the face, jaw and neck at a low level, and very few have limb twitches, but there is never a conscious disorder, and there is generally no abnormality in EEG. Unless it is a relatively rare focal epilepsy that causes facial convulsions, the clinical manifestations are easily confused, but an EEG can generally be identified.
3. Editor: In which groups is Meijaer syndrome generally more likely to occur?
Director Wang Lin: Usually onset in old age, mostly 40 to 70 years old, usually 50 or 60 years old, mostly in women, male: female ratio of 1:2 ~ 3. Incidence is around 1:20,000.
4. Editor: What are the causes of Major syndrome?
Director Wang Lin: The etiology of Major syndrome is very complicated, and the cause of primary Major syndrome is not fully understood at present, and it is currently believed that it is mainly related to the following factors:
(1) Cortical-striatal-globus pal-thalamus circuit dysfunction;
(2) Dopaminergic, cholinergic, γ-aminobutyric acid neuron dysfunction in the thalamus and basal ganglia regions, resulting in neurotransmitter imbalance and receptor hypersensitivity;
(3) Dysfunction of r-aminobutyric acidergic neurons in the substantia nigra weakens the inhibition of dopaminergic neurons;
(4) Overactivation of some interneurons in the basal ganglia, midbrain, and pontine that are related to the orbicularis muscle reflex; the absence of control excitatory and inhibitory pathways eventually leads to the failure of integration of interactive muscle activity and spontaneous motor control.
(5) Catecholamine metabolism disorders, autoimmune dysfunction;
(6) Family genetic factors are related to the situation (there is a homozygous mutant gene 6-PTS activity in the CNS).
(7) Psychosocial factors (55-80%).
Causes of secondary Major syndrome include long-term use of antipsychotics, chickenpox infection, head trauma, nasopharyngeal radiation therapy, basal ganglia calcification or ischemic injury, dental procedures, and environmental factors.
5. Editor: What are the main symptoms of Major syndrome?
Director Wang Lin: The clinical manifestations of Meijie syndrome are mainly characterized by double eyelid spasm and involuntary movements like facial muscle tone disorders.
Usually onset is slow, preceded by irritation in one or both eyes, increased shyness and blinking frequency, dry eyes, and later develops eyelid spasm, difficulty opening the eyes, and functional blindness. Some patients gradually develop downward from eyelid spasm, and there is symmetrical irregular hyperactive contraction of the oral and maxillary muscles, which is manifested by involuntary opening, closing, pouting, lip shrinking, cheek biting, tongue biting, tooth contusion, tongue spitting, etc. Mandibular muscle tension prevents chewing, swallowing, and speaking, and can involve spastic dysphonia and dyspnea when violating the laryngeal and respiratory muscles. Neck muscle involvement: presents with neck discomfort, torticollis, shaking, head tilting, and shoulder shruging.
Severely ill patients can cause ptosis, difficulty opening the eyes, functional blindness, dyspnea, dysphagia, and spastic dysarthria.
One of the characteristics of Meige syndrome is a dramatic reduction in symptoms that can be seen when yawning, eating, coughing, singing, playing the piano, guessing, playing the harmonica, whistling, etc. (Tricks phenomenon - sensory trickery).
6. Editor: In what environment is it easy to stimulate the onset of Major syndrome?
Director Wang Lin: Symptoms worsen during fatigue, solar stimulation, gaze, and nervousness, reduce when the mind is focused on other things other than eyelid spasms, and disappear during sleep.