generalized dystonia
Introduction
Introduction Dystonia refers to a dyskinesia characterized by abnormal movements and postures of muscle tension caused by incoordination or excessive contraction of the agonist muscles. Systemic dystonia refers to three or more dystonias of the head and neck, limbs, and trunk muscle groups, such as torsion spasms.
Cause
Cause
Dystonia can be divided into primary and secondary depending on the cause.
The etiology of primary dystonia is unknown to date, and may be related to heredity, is autosomal recessive, or is autosomal dominant and sexually linked. Most of the primary torsion mites of autosomal dominant inheritance are caused by mutations in the DYT1 gene, which is located in the 9q32-34 region of the autosomal 9th long arm. Environmental factors such as trauma or overwork can cause the onset of genes in primary dystonia. For example, oral-mandibular dystonia can have a history of facial or dental damage; overwork of one limb can also induce dystonia. Such as writing cymbals, typists, instrumentalists and athletes. The etiology may jeopardize dysfunction of the basal ganglia with reorganization of the motor circuit or changes in sensory connections above the level of the spinal cord.
Secondary dystonia is caused by lesions such as striatum, thalamus, blue spot, and brainstem reticular formation, such as hepatolenticular degeneration, nuclear jaundice, ganglioside deposition, globus pallidus, substantia nigra and Red nuclear pigmentation, progressive supranuclear palsy, familial basal ganglia calcification, hypothyroidism, poisoning, cerebrovascular disease, brain trauma, encephalitis, drugs (levodopa, phenothiazines, butyrylbenzenes, Metoclopramide) induced. Non-specific pathological changes were seen in primary torsion sputum, including degeneration of small neurons in the putamen, thalamus and caudate nucleus, and increased basal ganglia lipids and lipipidations. The pathological features of secondary torsion spasm vary with the primary disease; there is no pathologically specific change in dystonia such as spastic torticollis, writing sputum, Meige syndrome, and occupational spasm.
Examine
an examination
Related inspection
CT examination, biochemical examination, blood electrolyte examination, muscle tone examination
It is usually not difficult to diagnose according to medical history, physical involuntary movements and abnormal postures. It is important to diagnose idiopathic torsion dystonia and to exclude other causes of dystonia. Normal development history before the onset, lack of other neurological signs and normal laboratory tests are also important for diagnosis. Patients with idiopathic torsade dystonia who are onset before the age of 30 should be tested for genetics such as DYT1.
Diagnosis
Differential diagnosis
Hemidystonia: refers to the muscles involved in the ipsilateral upper and lower limbs, mostly caused by secondary causes. Blood electrolytes, drugs, trace elements and biochemical tests help to diagnose and classify the cause. 1. CT or MRI examination is meaningful for differential diagnosis. 2. Positron emission tomography (PET) or single photon emission tomography (SPECT) can show some biochemical metabolism in the brain, which is meaningful for diagnosis. 3. Genetic analysis is important for the diagnosis of certain hereditary dystonia.
Segmental dystonia: characterized by segmental distribution, such as cranial-neck dystonia, upper or lower limbs with or without the central axis, dystonia of the head and neck, lower limbs with or without trunk dystonia, trunk - The neck (does not affect the head and face) dystonia and so on. Segmental dystonia is a type of dystonia syndrome.
Localized dystonia: dystonic syndrome is called dystonia, which is an inconsistency or excessive contraction of active muscles and antagonistic muscle contractions, which leads to the synthesis of dyskinesia characterized by abnormal muscle movements and postures. The sign is characterized by involuntaryness and continuity. Although it is called dystonia syndrome, the changes in muscle tone are not noticeable, but the abnormal posture posture and involuntary transformation action are noticeable. Dystonia can affect the normal range of motion, extent, speed, and muscle stiffness of the affected limb.
It is usually not difficult to diagnose according to medical history, physical involuntary movements and abnormal postures. It is important to diagnose idiopathic torsion dystonia and to exclude other causes of dystonia. Normal development history before the onset, lack of other neurological signs and normal laboratory tests are also important for diagnosis. Patients with idiopathic torsade dystonia who are onset before the age of 30 should be tested for genetics such as DYT1.
