Congenital sensory neuropathy

Introduction

Introduction to congenital sensory neuropathy Congenital sensory neuropathy (congenital sensoryneuropathy) is a group of diseases with various clinical manifestations and confusing diseases. It is related to heredity and is autosomal dominant, and the pathogenesis is still inaccurate. Recurrent acromegaly ulcers that occur from birth, hand and foot ulcers are often caused by dystrophic and repeated injuries, and the pain in the extremities is reduced and the sense of touch is present. There is currently no special treatment. basic knowledge The proportion of illness: 0.001%-0.003% Susceptible population: newborn Mode of infection: non-infectious Complications: syringomyelia

Cause

Causes of congenital sensory neuropathy

(1) Causes of the disease

The disease is a group of diseases with diverse clinical manifestations and confusion. Related to heredity. Autosomal dominant inheritance. The current research suggests that the disease may have a certain correlation with environmental factors, genetic factors, dietary factors, and mood and nutrition during pregnancy. The degree of specific association is currently not finalized.

(two) pathogenesis

The pathogenesis is still inaccurate. It is an autosomal dominant inheritance and is a type of congenital sensory neuropathy with familial autonomic dysfunction and with no sweat.

Prevention

Congenital sensory neuropathy prevention

The disease cannot be prevented directly. However, regular examination should be carried out during pregnancy. If the child has a tendency to develop abnormalities, chromosome screening should be done in time, and abortion should be performed in time to avoid the birth of the diseased child.

Complication

Congenital sensory neuropathy complications Complications syringomyelia

Because the skin, muscles, and tissues have long lost the nutritional support of the nerves, it is easy to cause skin mucosa and muscle atrophy. Therefore, in the course of clinical treatment, attention should be paid to strengthening functional exercise to avoid further aggravation of muscle atrophy. Because the disease is a congenital dysplasia, patients with a clear diagnosis of this disease should be examined by head CT and other examinations to determine whether there is an intracranial abnormality.

Symptom

Congenital sensory neuropathy symptoms Common symptoms Acromegaly dystrophy phantom limb pain feeling inversion

From the time of birth, recurrent acromegaly ulcers, hand and foot ulcers are often caused by dystrophic and repeated injuries, and the pain in the extremities is reduced and the sense of touch is present. Some patients are completely sweatless, and the degree of temperature loss in the affected part is more than pain. Significant, but touch is rarely affected; lower limb performance is more serious than upper limbs, this disease is one of many diseases or syndromes associated with sensory neuropathy, common foot penetration, syringomyelia, Thevenard syndrome, familial autonomy Abnormal neurological function and congenital sensory neuropathy with no sweat.

Examine

Examination of congenital sensory neuropathy

Clinical skin examination: Recurrent acromegaly ulcers that occur at the time of birth. Hand and foot ulcers are often caused by nutritional disorders and repeated injuries. The pain in the extremities is diminished and the sense of touch exists. Some patients are completely sweat-free. The degree of temperature loss in the affected part is more pronounced than the pain, but the touch is rarely affected; the lower limbs are more severe than the upper limbs. Other examinations: Focus on electromyography to determine whether muscle function has occurred.

Diagnosis

Diagnosis and diagnosis of congenital sensory neuropathy

From the time of birth, recurrent acromegaly ulcers, hand and foot ulcers are often caused by dystrophic and repeated injuries, and the pain in the extremities is reduced and the sense of touch is present. Some patients are completely sweatless, and the degree of temperature loss in the affected part is more than pain. Significant, but the touch is rarely affected; if the lower limbs are more severe than the upper limbs, the disease can be considered.

The disease should be differentiated from other skin damage caused by nerve damage, such as peripheral neuritis, usually manifested as numbness, abnormal skin sensation and muscle contraction dyskinesia, after vitamin B1 and vitamin B12 can be relieved. Other nerve damage, such as bone marrow damage or osteomyelitis, can also cause neurological dysfunction, and even limb paralysis below the flat film.

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