What Is Progressive Muscular Atrophy?

Progressive muscular atrophy (PMA), also known as progressive spinal muscular atrophy, is a subtype of amyotrophic lateral sclerosis (motor neuron disease). Degeneration is limited to alpha motor neurons in the anterior horn of the spinal cord. It is characterized by progressive muscle atrophy and muscle weakness, usually starting from the small muscles of the hand, spreading to the entire upper limb, the reflection disappears, and the sensory disturbance does not appear. Some patients may eventually develop amyotrophic lateral sclerosis.

Basic Information

nickname

Progressive spinal muscular atrophy

Visiting department

Neurology

Multiple groups

Middle-aged man

Common locations

Upper limbs, lower limbs

Common causes

Etiology unknown, often inherited

Common symptoms

Weak and weak hands, "claw-shaped hand" or "simian hand", involving arms, shoulders, and lower limbs, and irregular muscle tremor appeared

Causes of progressive muscular dystrophy

The exact cause is still unclear, often a chromosomal dominant or recessive genetic feature. Different cases can have different causes, such as cold, fatigue, infection, lead poisoning, trauma, and can be secondary to syphilis and polio.

Clinical manifestations of progressive muscular dystrophy

The onset is insidious. It occurs in middle-aged people and is mostly male. It is characterized by weak hands and weak hands, atrophy of the internal muscles of the hands, and deformities of "claw hands" and "simian hands". Fasten clothes, pick up small items, and have difficulty writing. Later muscle weakness spreads to adjacent muscle groups, involving the arms and shoulders, and then progresses to the lower limbs. There is also onset from the feet, extending to the lower limbs, and then to the upper limbs. Muscle atrophy develops weakly symmetrically, sometimes involving only one hand. Decreased muscle tone and weakened tendon reflexes. The sphincter has no dysfunction, and pathological reflexes are mostly absent, but it can be seen in frequent muscle bundle tremor. Muscle tremor can occur in uncertain locations, and can cause and aggravate muscle tremor when cold, mood swings, or mechanical stimuli occur. No spontaneous pain and paresthesia occurred during the course of the disease, and symptoms of tongue muscle atrophy and soft palate dyskinesia with dysphagia and dysphagia rarely occurred.

Progressive muscular dystrophy test

1. CSF (Cerebrospinal Fluid) may have a slight increase in protein.

2. SEP (Somatosensory Evoked Potential) measured normal nerve conduction velocity.

3. EMG (electromyography) shows the characteristics of lower motor neuron lesions.

Diagnosis of progressive muscular dystrophy

Diagnosis can be made based on clinical manifestations and related examinations.

Progressive muscular dystrophy treatment

Currently treated with amyotrophic lateral sclerosis. Excitatory antagonists are available, such as high concentrations of mecobalamin, free radical scavengers, and the like. A high-protein, low-fat diet should be given to ensure adequate rest.