Hepatic myelopathy

Introduction to hepatic myopathy Hepatic myelopathy (hepatic myelopathy), also known as portal-cavity shunt myelopathy, is a special type of neurological complication of liver disease, characterized by slow progressive paraplegia, lateral and post-myelin sheathing. Pathological changes are predominant, more common in surgery or natural formation of portal-cavity circulation shunt, most cases coexist with hepatic encephalopathy, often spinal cord symptoms are covered by serious brain disease consciousness and movement disorders can not make a diagnosis until pathological examination The demyelination of the posterior cord and lateral cord of the spinal cord was discovered. basic knowledge The proportion of the disease: the proportion of the disease in a specific group is 0.1% - 0.2% Susceptible people: more common in young adults Mode of infection: non-infectious Complications: hepatic encephalopathy shock ascites primary liver cancer portal vein thrombosis

Causes of hepatic myelopathy

Viral hepatitis (40%):

Other factors (30%):

Hepatic myelopathy prevention

1, Chinese medicine believes that physical decline, drinking, food, depression, over-eating fat and so on, may cause liver changes.

2, vegetables are commonly used in people's lives, rich in nutrients, and beneficial, can be eaten regularly. Vegetables are not only rich in vitamins, but also contain a lot of cellulose, lignin, fruit acid, inorganic salts, etc., which are essential nutrients in the recovery process of liver patients.

Hepatic myelopathy complications Complications, hepatic encephalopathy, shock, ascites, primary liver cancer, portal vein thrombosis

During hepatic encephalopathy, patients may have transient visual impairment, dizziness, loss of computational power, and spinal cord disease. The patient's lower extremities gradually develop into bilateral symmetrical paraplegia, walking in gait, scissors gait, individual cases have Atrophy of the lower extremities or atrophy of the hands muscles.

In addition, there are the following complications:

1. Hepatic encephalopathy is the most common cause of death. It is a comprehensive disorder of central nervous system dysfunction based on metabolic disorders. The main clinical manifestations are disturbance of consciousness, behavioral disorder and coma.

2, a large number of upper gastrointestinal bleeding often manifested as hematemesis and black feces, if the amount of bleeding is not much, only black feces, a large number of bleeding can cause shock, and induce ascites and hepatic encephalopathy, and even death.

3, infection.

4, primary liver cancer.

5, liver and kidney syndrome is characterized by oliguria or anuria, azotemia, hyponatremia and low urinary sodium.

6 portal vein thrombosis.

Symptoms of hepatic myelopathy common symptoms jaundice spider sputum liver splenomegaly fatigue visual impairment dizziness upper gastrointestinal bleeding anorexia blurred bloating

It is more common in young adults, but the age of onset varies with the cause of the disease. Hepatic myelopathy caused by lenticular nucleus degeneration often occurs in adolescence, and cirrhosis often occurs in middle-aged and young, and the latter is common.

The disease mostly occurs in the decompensated period of cirrhosis, liver function decline and portal hypertension. The majority of patients have repeated upper gastrointestinal bleeding, portal-to-body venous shunt and spleno-renal venous anastomosis, no history of surgery. There are often obvious abdominal varices, suggesting that the portal-body vein shunt has naturally formed.

The time of onset of hepatic myelopathy is usually 4 months to 10 years after portal-body vena cava anastomosis or spleno-renal venous anastomosis; patients with natural shunt occur with symptoms of liver damage such as jaundice, ascites, and hematemesis. The time of spinal cord symptoms is 6 months to 8 years, and some cases have direct spinal cord symptoms without hepatic encephalopathy. Even those with liver disease after simultaneous and early neurological symptoms are clinically based on the symptoms of domestic scholars. Divided into 3 phases:

1. The liver symptom stage (pre-neuropathy) is mainly the manifestation of chronic liver damage, such as anorexia, abdominal distension, fatigue, hepatosplenomegaly, ascites, spider mites, elevated ALT, decreased serum total protein, and A/G ratio inversion. , elevated blood ammonia, esophageal varices, abdominal varicose veins and upper gastrointestinal bleeding.

2. Hepatic encephalopathy (sexual paraplegia) can repeatedly appear symptoms of transient encephalopathy, mainly manifested as euphoria, poor sleep, excitement or retardation and other emotional abnormalities; unconscious hyperactivity, runaway and other behavioral abnormalities; memory and orientation Mental distress and other mental abnormalities; mental disorder, madness, confusion, and other mental abnormalities; tachycardia, facial and chest skin flushing, calf and foot abnormal cold sense and other autonomic symptoms and flapping tremor, dysarthria, Other neurological symptoms such as transient visual impairment, dizziness, loss of computing power, life can still take care of themselves, but some patients lack the period of encephalopathy, and the period of liver symptoms directly into the paralytic paraplegia.

3. Spinal cord disease and encephalopathy symptoms are not parallel and long-term, brain symptoms are characterized by repeated transient hair, while myelopathy is slowly progressively aggravated. Spinal cord disease often occurs after encephalopathy, but can also occur in Before the encephalopathy period, even in the absence of encephalopathy, the lower limbs have a heavy feeling, walking and feeling hard, the muscles of both lower limbs are trembling, the activity is not flexible, and gradually develop into bilateral bilateral paraplegia, and the early stage is straight and paralytic paraplegia. Increased muscle tone, strong and straight, straight extension of the knee and ankle, "folding knife phenomenon", walking squat gait, scissors gait, late flexion paralysis, a small number of quadriplegia, but still The lower extremity is heavier. During the examination, the lower extremity muscle strength is reduced, the muscle tension is increased, the tendon reflex is hyperthyroidism, and the sputum sputum and sputum sputum sputum are positive, the abdominal wall reflex and the cremaster reflex disappear, the pyramidal tract sign is positive and other pathological signs, the limb Symptoms are generally symmetrical, with proximal and distal symptoms. Individual cases have lower limb muscle atrophy or atrophy of the hands, normal EMG or neurogenic damage, and a small number of patients can be combined. Tip neuropathy, occurs on both sides of the symmetry of socks like light sensory impairment, occasional deep sense of loss, sphincter barrier-free, when accompanied by hepatic encephalopathy, an individual who has incontinence or urinary retention.

Examination of hepatic myelopathy

1. Hepatic myelopathy with faster progress has more elevated transaminase, decreased albumin, elevated globulin and other liver dysfunction. Chronic onset is characterized by elevated blood ammonia as an important laboratory feature, but blood ammonia level and brain-spinal The severity of the damage is not in a parallel relationship.

2. Most of the cerebrospinal fluid is normal, and some proteins are mild or moderately elevated.

3. Serum ceruloplasmin, vitamin B12, folic acid and syphilis serum were normal.

4. In patients with hepatolenticular degeneration complicated with spastic paraplegia, corneal KF pigment ring, serum ceruloplasmin serum oxidase and total serum copper are reduced under the slit lamp or in the naked eye, serum direct reaction copper and 24h urinary tract An abnormality in copper metabolism such as an increase in the amount of copper.

5. Electromyography showed impaired performance of upper motor neurons, and EEG showed mild to moderate diffuse abnormalities.

6. Spinal MRI helps to rule out other spinal cord lesions.

Diagnosis and diagnosis of hepatic myelopathy

diagnosis

1. A history of acute, chronic liver disease and cirrhosis.

2. Do a portal-to-body anastomosis, TIPS or a wide range of natural collateral formation and other signs of liver disease.

3. Chronic encephalopathy and symptoms and signs of upper motor neuron damage, slow onset of young adults, progressive exacerbation of paraplegia of both lower extremities, and repeated episodes of mental and mental disorders should be highly suspected as hepatic myelopathy .

4. The obvious increase of blood ammonia is an important basis for the diagnosis of this disease. Generally, there is no muscle atrophy, sensory disturbance and sphincter dysfunction, cerebrospinal fluid is normal, serum copper oxidase is normal, and there is no corneal pigment ring.

Differential diagnosis

1. Hepatolenticular degeneration: more common in adolescents with a positive family history, mainly manifested as myotonia, limb tremor, mental disorders, language disorders, corneal pigment ring, serum copper and ceruloplasmin decreased, increased urinary copper, liver Biopsy liver tissue increased copper content, brain CT scan can show ventricular enlargement or brain parenchymal softening.

2. Amyotrophic lateral sclerosis: more often after the middle age, the disease progresses slowly, and the multiple manifestations are neuromuscular spasm of the upper and lower limbs. The hand muscle atrophy is obvious, often accompanied by muscle fibrillation, and may also involve the tail group sports brain. Nerve, dysphagia, dysarthria, atrophy of the lingual and sternocleidomastoid muscles, no sensory disturbance, and no liver disease.

3. Hereditary spastic paraplegia: mostly in childhood, with slow progression of both lower extremity paralytic paraplegia and scissor gait, accompanied by mild ataxia, with stable or improved condition with age, more Significant family history, no liver disease.

4. Subacute spinal cord combined degeneration: middle-aged onset, subacute or chronic progression, long course of disease, clinical manifestations of anemia symptoms such as burnout, fatigue, glossitis, diarrhea and pale skin mucosa, etc. The cone-shaped lesion of the cord may be accompanied by peripheral nerve damage, showing a collaterality of ataxia, and it is difficult to close the eye.

5. Multiple sclerosis (MS): There is a history of relapsing remission, oligoclonal bands appear in the brain fluid, and hormone therapy is effective.

6. Acute myelitis: caused by viral infection, lesions involving the cervical and upper thoracic spinal cord or all, MRI enhanced scan showed mild focal patch enhancement of spinal cord lesions.

7. Vascular malformation and spinal cord occupying lesions: spinal iodine angiography, angiography and spinal CT, MRI, examination can be clearly diagnosed, in addition to neurosyphilis, HTLV (human T cell leukemia lymphoma virus) - type I spinal cord Identification of the disease.

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