Partial body atrophy

Introduction Progressive hemifacial atrophy (progressivehemifacialatrophy) is also called Parry-Romberg syndrome, a progressive unilateral tissue dystrophic disease, a small number of lesions involving the limbs or body, called progressive half atrophy Its clinical features are chronic progressive atrophic muscle fibers of focal subcutaneous fat and connective tissue on one side of the face and are not affected by severe cartilage and bone. Most scholars believe that this disease and sympathetic dysfunction are related to various causes of sympathetic nerve damage, causing facial tissue neurotrophic disorders, and finally lead to facial tissue atrophy. Other doctrines involve local or systemic infection damage, genetic degeneration of trigeminal connective tissue disease.

Cause

1. Topal lobe lesions Some patients with parietal lobe tumors may have contralateral muscle atrophy and are considered to have diagnostic value. This muscle atrophy is mostly limited to the upper limb. In the proximal part of the upper limb, there is often a dislocation of the shoulder joint. It is also associated with cortical sensory disturbances, autonomic disorders in the upper limbs and hands, and even subcutaneous tissue and skeletal abnormalities. It has been suggested that flaccid monoterpene or hemiplegia, cortical sensory disturbance, and muscular atrophy are triads of parietal lesions.

2. Cerebrovascular disease hemiplegia can also appear muscle atrophy, seen in two cases, early in the early days usually appear within a few weeks after the onset, the other case is late, occurs several months after the onset. Often manifested in the muscle atrophy of the distal extremity, and sometimes limited to the scapula, especially in the deltoid muscle, so there is often shoulder dislocation. Lower extremity muscle atrophy is rare and often belongs to disuse atrophy.

3. Congenital eccentric muscle atrophy is characterized by uniform muscle atrophy, no obvious muscle loss and sacral reflex changes, often associated with congenital parietal dysplasia.

4. Progressive partial muscle atrophy is characterized by hyperreflexia, partial dysfunction, and deep lesions in the contralateral cerebral hemisphere or cerebral hemisphere, especially in the space-occupying lesions of the thalamus.

an examination

Related inspection

Brain CT examination of nervous system examination

(A) splenomegaly: In most cases, the spleen is swollen. For those who do not touch the spleen under the ribs, other tests should be performed to confirm whether the spleen is swollen. The spleen area scan after injection of 99m , 198 gold or 113m indium colloid helps to estimate the size and shape of the spleen. Computerized tomography can also measure spleen size and spleen lesions. However, the degree of splenomegaly is not necessarily proportional to the degree of hypersplenism.

(b) Hematocytopenia: Red blood cells, white blood cells or platelets can be reduced individually or simultaneously. In general early cases, only white blood cells or, in advanced cases, complete cytopenia.

(3) Bone marrow is a hematopoietic cell hyperplasia: some cases may also have maturity disorders at the same time, or a large number of peripheral blood cells may be destroyed, and mature cells are released too much, causing similar maturity disorders.

(D) changes in splenectomy: After splenectomy can make the number of blood cells close to or return to normal, unless the bone marrow hematopoietic function has been damaged.

(5) Radionuclide scanning: 51Cr-labeled platelets or red blood cells were injected into the body and scanned on the body surface. It was found that the amount of 51Cr in the spleen area was 2 to 3 times larger than that in the liver, suggesting that platelets or red blood cells were destroyed excessively in the spleen. When considering the diagnosis of spleen, the previous three are particularly important.

Differential diagnosis

Only in the early stages need to be identified with the following diseases:

1. Congenital lipodystrophy (also known as Lawrence-Seip syndrome) This disease mainly manifests the autosomal recessive inheritance of fat atrophy in the body, limbs or face. Onset in infancy, often complicated by genital hypertrophy, hyperhidrosis, head hirsutism, black acanthosis later developed into diabetes can occur liver, kidney dysfunction or cardiac hypertrophy, and acromegaly.

2. Localized scleroderma disease may be confused at the beginning, but the head and face are not the site of scleroderma, and the skin scleroderma is not easy to pinch with the underlying tissue, and there is no knife-mark distribution to help identify.

3. Facial-capped dystrophy (facioscapulohumeral mucular dystrophy) occurs in adolescents with a slow facial muscle atrophy, a special "myopathy face", a slight drooping of the upper jaw, the frontal and nasolabial folds disappear, The expression movement is weak or disappears, because the hard hypertrophy of the sacral muscles appears thicker and slightly tilted (cat face). With closed eyes is not tight, blowing air can not be able to squat, shoulder, face muscle atrophy, upper arm lift weakness, upper limbs flat stroke when the shoulder bones show a wing-like breakthrough. The activities of serum citrate kinase (CPK) and pyruvate kinase (PK) are increased.

4. Progressive lipodystrophy (lipodystrophy) is more common in women, more than 5 to 10 years old, and often symmetry distribution, slow progress. It is characterized by progressive subcutaneous fat loss or weight loss, onset on the face, cheeks and ankles concave, skin loose, loss of normal elasticity, eyelids deep, followed by the neck, shoulders, arms and trunk. In some cases, the lesion is confined to the face or half of the side, half of the body, may be confused with Parry-Rombery's syndrome, but the former biopsy only subcutaneous adipose tissue disappeared.

It still needs to be differentiated from diseases such as muscular dystrophy of syringomyelia muscle amyotrophic lateral sclerosis.

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