Unilateral facial atrophy

Introduction Hemifacial atrophy, also known as Romberg's disease, with progressive facial atrophy.

Cause

The cause is unknown. May be a genetic disease. It is considered to be the central or peripheral damage of the autonomic nervous system, causing dystrophic dysfunction of the unilateral tissue, and the unilateral tissue is atrophied, and the subcutaneous adipose tissue and connective tissue atrophy are more obvious. 15% with cerebral hemisphere atrophy, may be ipsilateral, contralateral or bilateral.

an examination

Related inspection

Facial nerve examination of the head and face palpation

It is characterized by one or all progressive atrophy of the lateral side tissue.

Women accounted for about 3/5, and those who developed the disease before the age of 20 accounted for 3/4. The left side is more common, the facial atrophy starts from a part, and the progress speed is uncertain. Generally, it develops into a partial atrophy in about 2 to 10 years. 5% of cases involve both sides of the face, in addition to facial atrophy, often involving soft palate, tongue and oral mucosa, occasionally the ipsilateral neck, chest and even the trunk and extremities atrophy (about 10%). The side of the affected area is old and has a sharp contrast with the healthy side. The hair, eyebrows and eyelashes often fall off, and there are white spots and skin blemishes. There may be facial pain or migraine, and sensory disturbances are rare. In addition to the affected side sweat glands and lacrimal gland dysregulation, Horner's syndrome can be seen. A few patients have seizures, and about half of them have paroxysmal activity. Patients with brain atrophy may have hemiplegia, partial sensory disturbance, hemianopia, and aphasia.

Differential diagnosis

(a) progressive systemic sclerosis (progressive systemic sclerosis) is a rheumatic disease, one type is confined to the skin, and the other type is also visceral. Often women of childbearing age between the ages of 20 and 50, the ratio of men to women is about 1:2 to 3. Often there is Raynaud's phenomenon (90%) or symmetrical finger swelling can be stiff, skin lesions are generally seen on the fingers and hands or face, swelling and edema, no indentation (swelling period), followed by skin thickening and hardening like leather , dull (hardening period), and finally skin atrophy (atrophic period), skin lesions gradually spread to the arms, neck, chest and abdomen. The normal skin of the face disappears, the face is rigid, the mouth is difficult, the hardened part is pigmented, and the spots are removed.

(2) Face-shoulder-type 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 lips of the sacral muscles appear 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.

(C) Progressive lipodystrophy (lipodystrophy) Women are more common, more than 5 to 10 years old before and after onset, often symmetric distribution, slow progress. It is characterized by progressive subcutaneous fat loss or weight loss, onset on the face, cheeks and ankles, skin relaxation, loss of normal elasticity, deep eyelids, 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 fat tissue disappeared.

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