Blepharospasm - oro-submental dystonia

Introduction

Eyelid - Introduction to the temporomandibular dysfunction The blepharospasm-oromanbulardystonia is also known as Meige's syndrome, or Brueghel syndrome. It is a group of unexplained syndromes, mainly showing binocular palsy, combined with facial dystonia-like involuntary movement. French scholar Heury Meige (1910) first described a group of cases, the disease is dystonia, the muscles of the affected muscles contracted tonic, from seconds to minutes, and then extended to other parts. Despite electrophysiological and MRI studies, it is speculated that the disease may be related to the midbrain and basal ganglia, but the exact cause is still unknown. Some people think that certain sedative drugs can also cause the disease. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: Affective Disorders Depression Anxiety Disorder

Cause

Eyelid - the cause of humeral dysfunction

(1) Causes of the disease

The cause of Meige syndrome is unclear and the cause may be:

1 Upper brain stem, abnormal basal ganglia.

2 brain choline energy system is overactive.

3 dopamine receptor hypersensitivity.

(two) pathogenesis

1. Excessive activation of the midbrain and basal ganglia, resulting in excessive activity of intramedullary interneurons involved in orbicularis muscle reflex.

2. Dopamine receptor hypersensitivity, overreaction.

3. The brain's cholinergic nervous system is overactive in the basal ganglia.

Pathology: Garcia-Albea et al. (1981) reported no abnormalities in the pathology of orbital and Meige syndrome. Ahrocchi et al (1983) reported a case of Meige syndrome with plaque-like neuron loss and glue on the dorsal striatum. Hyperplasia, Zweig et al. (1988) reported that one case of Meige syndrome had more severe neuronal loss in the brainstem nucleus (substantia nigra pars compacta, blue spot, nucleus, pons nucleus); There is a small amount of neurofibrillary tangles in the substantia nigra with neuromelanin staining in the blue spot.

Prevention

Eyelid - prevention of dystonia in the lower jaw

Strengthening clinical care is of great significance to improving the quality of life of patients.

Complication

Eyelid - humeral dystonia complications Complications, affective disorder, depression, anxiety

A considerable number of patients with emotional disorders, such as depression, anxiety; patients in need of daily life, often need to use a thumb and finger to push the upper eyelids, so that the eyes can be opened, see the outside world, continue this action for a long time, Will cause the two eyebrows to fall off, affecting the beauty of the face.

Symptom

Eyelids - sacral dystonia symptoms common symptoms eyelid relaxation and upper lip thickening eyelid ecstasy light eye movement crisis anxiety dry eye depression

The clinical manifestations of Meige syndrome can be divided into 3 types:

1 eyelid type.

2 eyelid combined mouth, mandibular dystonia type.

3 mouth, mandibular dystonia type.

Jankovic said that the orbital dysplasia is a complete type, the rest of the mandibular dystonia is incomplete, and the proportion of each type is very different, but the eyelid type and the eyelid are combined, and the mandibular dystonia is The type accounts for the majority.

Double eyelids are the most common first symptom (76% to 77%), and some of them start from a single eye, gradually with both eyes. They often have eye irritation, dry eyes, photophobia and blinking. The frequency of seizures often ranges from sparse to frequent, and sputum can last from a few seconds to 20 minutes. The functional blindness is caused by the unconstrained sustainable contraction. Patients often need to pull up the double upper jaws by hand and dare not go out alone or cross the road.

The mouth, jaw and tongue are often expressed as mouth opening, biting the jaws, shrinking the lips, licking the mouth, sticking out the tongue, etc., causing the facial expression to be strange and special (Brueghel's syndrome), which can cause dislocation of the lower jaw, tooth wear, can still affect the vocalization and Swallowing, mouth, and jaw are often triggered by speech and chewing.

In addition to eyelids and mouth, abnormal mandibular muscle tension, Meige syndrome can still be accompanied by torticollis, head tilting forward flexion, etc., generally no intelligence disorder, no pyramidal lesions, cerebellar lesions and paresthesia, about 1/3 of patients There are emotional disorders such as depression, anxiety, forced personality, and schizophrenic personality changes.

Examine

Eyelid - examination of humeral dystonia

Blood electrolytes, trace elements and biochemical tests help to differentiate the diagnosis.

1. There is no characteristic change in MRI and CT scans.

2. During the blink reflex examination, the frequency of blinking increased, the latency of R1 component (reflecting single synaptic reflex), the latency of R2 component (reflecting multi-synaptic reflex) was prolonged, and the time period of electro-induced corneal reflex was prolonged.

Diagnosis

Diagnosis and differentiation of orbital sacral muscle dystonia

diagnosis

The diagnosis of this disease is mainly based on the patient's clinical manifestations. Involuntary closure of bilateral eyelids is accompanied by irregular contraction of symmetrical facial and facial muscles. Symptoms are aggravated in patients with emotional or glare, symptoms are relieved when calm, and symptoms disappear after sleep. Characteristic performance, no characteristic changes in MRI and CT scans, sedative treatment effect is not obvious.

Differential diagnosis

The disease should be differentiated from idiopathic eyelids and facial muscle twitches. The diseases that need to be identified are:

1 Delayed dyskinesia: There is a long-term use of phenothiazines, a history of butyrylbenzene antipsychotic drugs, the affected muscles are often peristaltic rather than muscle spasm.

2 lateral hemiplegia: often limited to one side and facial nerve innervating muscle, without mouth, mandibular dystonia-like involuntary movement, even involving both sides, but bilateral sputum is not synchronized with Meige syndrome.

3 neurosis: can occur at any age, often accompanied by emotional instability, sleep disorders, more symptoms, fluctuations, and effective psychological treatment.

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