Automatic Babinske sign positive
Introduction
Introduction The Babinske sign is a special spinal reflex that occurs when the spinal cord loses control of the cerebral cortex. When the blunt object is used to scribe the foot, the big toe is bent back, and the four toes are fan-shaped outward. The spinal cord of the positive adult is active under the control of the cerebral cortex motor area. Normally, this reflection is suppressed and cannot be manifested. When the cone system or the extrapyramidal system is damaged and the inhibition is lost, the Babinski sign appears. The Babinski's sign can be examined clinically to determine the function of the pyramidal or extrapyramidal system. Babinski's sign is also positive before the infant's pyramidal tract is not well developed, and when the adult is deeply asleep or anesthetized. Common in dopa-responsive dystonia, it is a rare hereditary disease that occurs in children or adolescents with dystonia or gait abnormalities as the first symptom.
Cause
Cause
Half of the disease is sporadic, and half of them are autosomal dominant. It is currently believed that the lack of isoenzyme GCHI of GTP cyclohydrolase leads to a decrease in dopamine synthesis, which is the main cause of DRD. Reactive dystonia is common in childhood, with more women than men. The age of onset is generally 4-8 years old, but it can be as early as infancy and as late as adulthood.
Infants are less common in infants and are often misdiagnosed as cerebral palsy or spastic paraplegia. The symptoms of adult onset are similar to Parkinson's disease. The initial symptoms are often horseshoe varus and gait abnormalities due to muscle weakness in the lower extremities. Later, the condition is progressively aggravated, and the limbs may be stiff, the movement is slow, and the face is expressionless. Half of the patients developed a positional, intentional tremor of 8-10 Hz (different from 4-5 Hz resting tremor of Parkinson's disease), which is generally stable in adulthood. Some mild patients have difficulty walking and fatigue in the afternoon, and writing pens have a long time to write.
In some cases, the sputum and Babinski signs are positive in some children. 75% of children with dystonia have characteristic intercondyal changes, that is, when the muscles are incomplete in the morning, the muscle tension is less, and gradually becomes more serious, which is most obvious at dusk. After the day break, it can be improved slightly, but it is intensified after activities and sports.
Examine
an examination
Related inspection
Neurological examination muscle tone examination
1 has clinical manifestations of dystonia, such as limb stiffness. Gait abnormalities, etc.
2 Symptoms of severity and obvious changes in the daytime, morning light and heavy, and increased after the event.
3 nervous system examination: normal muscle strength, some children with increased muscle tone, sputum sputum positive, Babinski sign positive.
4 low-dose levodopa treatment response is rapid, long-lasting effect.
Diagnosis is based primarily on clinical presentation and responsiveness to low-dose dopa formulations. Infants or adults with onset, unexplained limb muscle abnormalities, tremors and gait strange as the first symptom, morning light weight as the main clinical features, especially those with family history should be highly suspected of this disease. Suspicious patients given oral low-dose dopa preparations, most of the symptoms are relieved in 1-3 days, if appropriate, increase the dose. Foreign reports (Torbjoerna1991) carbidopa / levodopa dose increased to 25/100 (containing levodopa 100mg and Carbidopa 25mg), 3 times / d is still invalid, can rule out the diagnosis of DRD.
Diagnosis
Differential diagnosis
The disease should be differentiated from cerebral palsy, juvenile Parkinson's disease, torsion sputum, hepatolenticular degeneration, and spastic paraplegia.
1, cerebral palsy often with abnormal increase in muscle tone and sputum as the main features but often accompanied by mental retardation, convulsions and mood disorders without fluctuations do not respond to dopa preparations.
2, juvenile Parkinson's disease rarely occurs in children under 8 years of age, PET test shows that 18F-dopa intake decreased, long-term application of dopa preparations need to gradually increase the dose, and prone to side effects, deterioration of the end of the drug and other side effects.
3, hepatolenticular degeneration often accompanied by liver damage and mental and mental abnormalities, KF ring visible in the cornea.
4, a very small number of patients with initial symptoms and signs resemble spastic paraplegia, the dramatic response of low-dose dopa may be the most important identification point.
1 has clinical manifestations of dystonia, such as limb stiffness. Gait abnormalities, etc.
2 Symptoms of severity and obvious changes in the daytime, morning light and heavy, and increased after the event.
3 nervous system examination: normal muscle strength, some children with increased muscle tone, sputum sputum positive, Babinski sign positive.
4 low-dose levodopa treatment response is rapid, long-lasting effect.
Diagnosis is based primarily on clinical presentation and responsiveness to low-dose dopa formulations. Infants or adults with onset, unexplained limb muscle abnormalities, tremors and gait strange as the first symptom, morning light weight as the main clinical features, especially those with family history should be highly suspected of this disease. Suspicious patients given oral low-dose dopa preparations, most of the symptoms are relieved in 1-3 days, if appropriate, increase the dose. Foreign reports (Torbjoerna1991) carbidopa / levodopa dose increased to 25/100 (containing levodopa 100mg and Carbidopa 25mg), 3 times / d is still invalid, can rule out the diagnosis of DRD.
