Neurogenic muscle contractures: causes, symptoms, diagnosis, treatment

The term "contracture" is applicable to all cases of persistent fixed muscle shortening. In this case, the EMG appears "silent" in contrast to transient forms of muscle contraction (cramps, tetanus, tetany), accompanied by high-voltage high-frequency discharges on the EMG.

Syndromic neurogenic muscle contractures should be differentiated from other (non-neurogenic) contractures, which can be dermatogenic, desmogenic, tendogenic, myogenic and arthrogenic.

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The main causes of neurogenic contractures:

1. Consequences of long-term flaccid or spastic paresis (including early and late hemiplegic contracture).
2. Late stages of focal and generalized torsion dystonia.
3. Congenital fibrous contractures and joint deformities.
4. Congenital multiple arthrogryposis.
5. Myopathies.
6. Rigid spine syndrome.
7. Glycogenosis (deficiency of phosphorylase and other phosphorus-containing enzymes.
8. Late stages of untreated Parkinson's disease.
9. Contractures of mixed nature (cerebral palsy, hepatolenticular degeneration, etc.).
10. Post-paralytic contracture of facial muscles.
11. Volkmann's ischemic contracture (of vascular rather than neurogenic origin).
12. Psychogenic (conversion) contractures.

Long-term deep flaccid paresis or plegia of a limb due to diseases or damage to the cells of the anterior horn of the spinal cord (spinal stroke, progressive spinal amyotrophy, amyotrophic lateral sclerosis, etc.), anterior roots, plexuses and peripheral nerves of the limbs (radiculopathy, plexopathy, isolated motor neuropathy and polyneuropathies of various origins) easily leads to persistent contracture in one or more limbs.

Deep central paresis (especially plegia) in the absence of recovery or incomplete recovery, as a rule, leads to contractures of varying degrees of severity, unless special measures are taken to prevent them. Such contractures can develop with spastic mono-, para-, tri- and tetraparesis and have both cerebral and spinal origin.

The most common causes are strokes, craniocerebral and spinal injuries, encephalitis, space-occupying processes, Strumpell's disease and other degenerative diseases. "Early contracture" in stroke is an early (first hours or days of stroke) transient increase in muscle tone mainly in the proximal parts of the paralyzed arm; in the leg, extensor tone predominates. "Early contracture" lasts from several hours to several days. A special case of early contracture is hormetonia in hemorrhagic strokes (paroxysmal increases in muscle tone observed in paralyzed, less often - in non-paralyzed limbs). Late contracture develops at the recovery stage of the course (from 3 weeks to several months) of stroke and is characterized by persistent limitation of mobility in the joints(es).

Late stages of focal and generalized torsion dystonia (long-standing tonic form of spasmodic torticollis, brachial or crural dystonia, contractures in generalized dystonia) can lead to persistent contractures in the joints (especially in the area of the hands and feet), limbs and spine.

Congenital fibrous contractures and joint deformities manifest themselves already from birth or in the first months of a child's life in the form of fixed pathological postures due to insufficient muscle development (shortening) or their destruction (fibrosis): congenital clubfoot, congenital torticollis (congenital retraction of the sternocleidomastoid muscle), congenital hip dislocation, etc. Differential diagnosis is carried out with dystonia: dystonic spasms are characterized by typical dynamism. In congenital contractures, a mechanical cause of limited mobility is revealed. The role of the nervous system in their genesis is not entirely clear.

Congenital multiple arthrogryposis is characterized by congenital multiple joint contractures due to severe underdevelopment (aplasia) of muscles. The spine and trunk muscles are usually not affected. Status dysraphicus is often present.

Some forms of myopathy: congenital muscular dystrophy type I and type II; other rare forms of myopathy (for example, Rottauf-Mortier-Beyer muscular dystrophy, or Bethlem muscular dystrophy, congenital muscular dystrophies), occurring with damage to the muscles of the limbs, eventually also lead to contractures in the proximal joints.

Rigid spine syndrome is a rare form of muscular dystrophy that begins in preschool or early school age and is manifested by limited movements of the head and thoracic spine with the formation of flexion contractures in the elbow, hip and knee joints. Diffuse but not severely expressed predominantly proximal muscle hypotrophy and weakness is characteristic. Tendon reflexes are absent. Scoliosis is characteristic. The EMG shows a muscular type of lesion. The course of the disease is stationary or slightly progressive.

Glycogen storage diseases sometimes cause exercise-induced muscle spasms that resemble transient contractures.

Late stages of untreated Parkinson's disease often present with contractures predominantly in the distal parts of the hands ("Parkinsonian hand").

Contractures of mixed nature are observed in the picture of combined (pyramidal, extrapyramidal, anterior corneal) disorders, for example, in cerebral palsy, hepatolenticular degeneration and other diseases.

Post-paralytic contracture of the facial muscles develops after flaccid paralysis of the muscles innervated by the facial nerve (facial nerve neuropathy of various etiologies), but differs from other post-paralytic contractures (in the limbs) by the possibility of developing hyperkinesis in the same area (“post-paralytic facial hemispasm”).

Volkmann's ischemic contracture is caused by fibrosis of muscles and surrounding tissues due to their ischemia (muscle bed syndrome) and is characteristic mainly of fractures in the elbow area, but can also be observed in the shin area, as well as in other parts of the body.

Psychogenic contractures may develop after long-term mono- and, especially, paraparesis (pseudoparalysis), which are not amenable to therapy. A positive diagnosis of psychogenic disease is necessary. A reliable confirmation of the diagnosis is psychotherapeutic elimination of paralysis and contracture.