What Is Stiff Person Syndrome?

Stiff man syndrome refers to a rare and serious central nervous system disease characterized by excessive contraction of the trunk and lower limb muscles, accompanied by myalgia. A variety of high-titer autoantibodies can be detected in patients with other autoimmune diseases. Clinical manifestations of the trunk, limbs, and neck muscles are persistent or fluctuating stiffness, abdominal muscles are plate-like solid, active and antagonist muscles can be affected at the same time. Benzodiazepines are currently the drug of choice for the treatment of this disease. The prognosis is good if the disease is free of complications.

Basic Information

nickname: Systemic muscle stiffness syndrome, Moersch-Wollman syndrome
English name: stiffman syndrome
Visiting department: Neurology

Common causes: The etiology of this disease may be related to heredity, neurophysiology, immune response, etc.
Common symptoms: Abdomen, trunk, joints are fixed, free movement is limited, severe pain, sweating, arrhythmia, emotional disorders, etc.

Causes of Zombie Syndrome

The etiology of this disease may be related to heredity, neurophysiology, and immune response.

1. Although the cause of the disease is unclear, spinal motor neurons continue to excite, and the level of norepinephrine metabolites in patients' urine increases, suggesting that the imbalance of the central nervous system's GABA inhibitory system and the norepinephrine system may be caused by an imbalance Causes of symptoms of this disease.

2. In recent years, many studies have shown that the cloned region of the brain and spinal cord is positive, and lgG synthesis is increased. May be related to immune abnormalities, but remains to be confirmed.

Clinical manifestations of stiff man syndrome

The onset of stiff man syndrome is chronic, often fluctuating, and there is often a history of infection before the illness, which ranges from 5 days to April. Paroxysmal aches in the abdomen, torso, and muscles at the beginning, with tight sensation, non-specific and transient, and then affected the limbs. With the progress of the disease, the trunk, limbs, and neck muscles are often stiff or volatile, the abdominal muscles are plate-like or stone-like solid, and the active and antagonist muscles are affected at the same time. The joints are fixed and free movement is limited. In severe cases, the joints are fixed in a stiff posture. Masticatory muscles are common in China, followed by cervical muscles. In addition to muscle stiffness and dyskinesia, a few may be associated with pyramidal signs. Symptoms progressed, followed by tightening of the lumbar, neck, trunk, and proximal muscles of the extremities, stiffness, restricted autonomy, and stiffness when walking, but not bending over, but stiffness disappeared after sleep.

Paroxysmal pain produces spasms and triggering factors. A slight stimulus can induce painful spasms of painful limbs and lumbar muscles, which can be induced by passive exercise or sudden emotional stimulation. During the attack, the patient has severe pain, crying, panic, loud howling, sweating, arrhythmia, etc. In severe cases, it can cause fractures or muscle tears. Rarely affects facial and throat muscles. Examination of the nervous system showed no abnormalities except for myotonia and normal intelligence. A few patients have difficulty swallowing, respiratory muscle involvement, sternocleidomastoid muscle tension, and some still have emotional disorders such as depression, insomnia, hallucinations, delusions, etc.

Stiff man syndrome test

EMG

The resting potential is a continuous normal action potential, which suddenly increases during the spontaneous spasm period after somatosensory stimulation or passive exercise. Myoelectric activity weakens or disappears after falling asleep or intravenous diazepam, general or spinal anesthesia, and blockade of norflucaine or arrow poison. Due to increased central excitability and increased sympathetic nerve activity, chewing up.

2. Muscle biopsy

Often normal, mild hyaline degeneration of muscle fibers is seen in individual cases.

3. EEG

Usually normal.

4. Cerebrospinal fluid examination

The number of cells can be normal, and a small amount of protein or immunoglobulin lgG, lgA, lgM slightly increased.

5. Blood test

Can detect autoimmune antibodies.

6. CT and MRI of the head

Most were abnormal, and CT of the head showed small calcifications. MRI of individual patients may show transient inflammation-like changes or atrophy of the brainstem or high cervical spinal cord.

7. Other

There may be an increase in urinary creatine excretion, but nonspecific changes.

Diagnosis of Zombie Syndrome

According to the clinical characteristics of the disease, its diagnostic criteria are as follows:

1. The axillary muscles (including facial muscles and masticatory muscles) continue to be stiff and rigid, showing a "plate shape" (proximal limb muscles can be affected);

2. Abnormal body position (usually excessive lordosis of the lumbar spine);

3. Sudden painful cramps, caused by random movements, emotional disturbances or unexpected auditory and somatosensory stimuli, alleviating or disappearing after bedtime;

4. Continuous movement unit activity (CMUA) on at least one body axis machine;

5. The electromyographic resting potentials can be issued with normal action potentials, which are enhanced during seizures and weakened or disappeared after injection stabilization.

6. Treatment with diazepam has special effects;

7. Metastases caused by tumors should be excluded.

Differential diagnosis of stiff man syndrome

This disease should be distinguished from the following:

Tetanus

Antipsychotic drugs have specific effects on this disease, but tetanus is ineffective. The EEG of this disease is normal, and about 50% of tetanus has abnormal EEG.

2. Differentiation from Congenital Myotonia

The disease is characterized by continued contraction of the striated muscle after stopping exercise, a myoglobal response to tapping, and typical myotonic myocardial electromyogram, which is effective with procainamide.

3.Isacs-Mertens syndrome (that is, myotonia-dwarfism-diffuse bone disease)

The disease is manifested by extensive muscular rigidity, with the affected areas mostly in the eyes and face. Patients are usually short, have bone deformities and special faces, and respond well to phenytoin sodium. Electromyogram shows continuous motor neuron potentials during muscle breaks.

4. Organophosphorus pesticide poisoning

Patients have a history of pesticide exposure, with or without pesticide poisoning in the early stage. Most patients have muscle tremor, individual muscles appear plate-like rigidity, they are not effective with antipsychotics, and atropine and phosphorotropic drugs have special effects.

5. The disease should also be distinguished from rickets, polymyositis, extrapyramidal diseases, pseudomyotonia, arthritis, and cervical spondylosis.

Zombie Syndrome Complications

This disease is often accompanied by severe episodic autonomic dysfunction, such as fear, pain, loud howling, sweating, shortness of breath, tachycardia, dilated pupils, increased blood pressure, and elevated body temperature. Diffuse muscle stiffness is common throughout the body.

Zombie Syndrome Treatment

Benzodiazepines

Is currently the drug of choice for the treatment of this disease. Diazepam is widely used, and its muscle relaxation can be achieved by increasing GABA-induced presynaptic inhibition in the spinal cord. The dose varies from person to person. After improvement, it decreases by 20 mg daily until discontinuation.

2. clonazepam

Intravenous or oral, the effect is very good. Individual severe patients may die from suffocation or breathing, cardiac arrest.

3.10% Chloraldehyde Enema

Satisfactory results can be achieved, but no report has been reported yet.

4.SMS is an autoimmune disease

Immunosuppressants (intravenous cyclophosphamide), corticosteroids, intravenous immunoglobulins or plasma exchange therapy are effective in treating this disease.

5. Intrathecal injection of baclofen in SMS patients

Reduces stiffness and cramps. Baclofen is a GA derivative, which can inhibit the transmission between spinal monosynaptic and polysynaptic neurons. It also inhibits the high central nervous system and has a significant muscle relaxation effect.

6. Traditional Chinese Medicine

Traditional Chinese medicine believes that the disease is caused by physical exhaustion, fluid loss, and tendon vein loss due to maintenance. Yiheying should be used to relieve muscles, relieve soothing items, pass through live genus, and activate blood circulation to remove stasis.

Zombie Syndrome Prevention

Prevent and treat respiratory and urinary tract infections, pay attention to avoid sound and light stimulation, maintain emotional stability, and prevent various triggering factors.