Neurogenic muscle contractures: causes, symptoms, diagnosis, treatment

The term "contracture" is applicable to all cases of persistent, fixed muscle shortening. At the same time EMG looks "silent" in contrast to the transient forms of muscle contraction (cramp, tetanus, tetany) accompanied by high-voltage high-frequency discharges on the EMG.

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

[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12]

The main causes of neurogenic contractures:

1. Consequences of long-existing flaccid or spastic paresis (including early and late hemiplegic contracture).
2. Late stages of focal and generalized torsion dystonia.
3. Congenital fibrotic contractures and joint deformities.
4. Congenital multiple arthrogryposis.
5. Myopathy.
6. Syndrome of a rigid spine.
7. Glycogenoses (insufficiency of phosphorylase and other phosphorus-containing enzymes.
8. Late stages of untreated Parkinson's disease.
9. Contractures of mixed nature (cerebral palsy, hepato-lenticular degeneration, etc.).
10. Post-paralytic contracture of facial muscles.
11. Ischemic contracture of Volkmann (rather vascular than neurogenic origin).
12. Psychogenic (conversion) contractures.

Long-existing deep flaccid paresis or limb plexy 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 extremities (radiculopathy, plexopathy, isolated motor neuropathies and polyneuropathies of various origins) easily leads to persistent contracture in one or more extremities.

Deep central paresis (especially plegia) in the absence of recovery, or with incomplete recovery, usually leads to contractures of varying degrees, 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, volumetric processes, Stryumpel's disease and other degenerative diseases. "Early contracture" in stroke is called early (the first hours or days of a stroke) transient increase in muscle tone mainly in the proximal parts of the paralyzed arm; in the leg, the extensor tone predominates. "Early contracture" has a duration of several hours to several days. A particular case of early contracture is the hormometonia in hemorrhagic strokes (paroxysmal muscle tone increase observed in paralyzed, less often in the non-paralyzed limbs). Late contracture is formed during the recovery phase of the course (from 3 weeks to several months) of stroke and is characterized by a persistent limitation of mobility in the joint (ve).

The late stages of focal and generalized torsion dystonia (long-existing tonic form of spastic torticollis, brachial or dystonic dystonia, contractures in generalized dystonia) can lead to persistent contractures in the joints (especially in the area of the hands and feet) of the limbs and spine.

Congenital fibrous contractures and deformities of the joints are manifested already from the birth or in the first months of the child's life in the form of fixed pathological postures due to insufficient muscle development (shortening) or their destruction (fibrosis): congenital clubfoot, congenital corticollis (congenital retraction of the nodding muscle), congenital hip dislocation, etc. Differential diagnosis is performed with dystonia: dystonic spasms are characterized by typical dynamics. With congenital contractures, a mechanical cause of limitation of mobility is revealed. The role of the nervous system in their genesis is not entirely clear.

Congenital multiple arthrogryposis is characterized by congenital multiple contractures of the joints due to a sharp underdevelopment (aplasia) of the muscles. The spine and muscles of the trunk usually do not suffer. Often there is a status of disraphicus.

Some forms of myopathy: congenital muscular dystrophy of type I and type II; other rare forms of myopathy (for example, Rothauf-Mortier-Beyer's muscular dystrophy, or Betlem's myodystrophy, congenital myodystrophies) that occur with the defeat of the limb muscles, eventually lead to contractures in the proximal joints.

The syndrome of the rigid spine is a rare form of myodystrophy, which begins in preschool or early school age and is manifested by the limitation of movements of the head and thoracic spine with the formation of flexural contractures in the elbow, hip and knee joints. A diffuse but grossly pronounced predominantly proximal hypotrophy and muscle weakness are characteristic. Tendon reflexes are absent. Scoliosis is characteristic. The EMG is a muscle type lesion. The course of the disease is stationary or poorly progressive.

Glycogenoses sometimes cause muscle spasms induced by physical exertion, and resemble transient contractures.

Late stages of untreated Parkinson's disease are often manifested by contractures mainly in the distal parts of the hands ("parkinsonic hand").

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

Post-paralytic contracture of facial muscles develops after flaccid paralysis of the muscles innervated by the facial nerve (neuropathy of the facial nerve of different etiology), but differs from other post-paralytic contractures (in the extremities) in the possibility of hyperkinesis development in the same zone ("post-paralytic facial hemispasm").

Folkmann's ischemic contracture is caused by fibrosis of the muscles and surrounding tissues due to their ischemia (muscular lodge syndrome) and is characteristic mainly for fractures in the elbow joint region, but can be observed in the shin area as well as in other parts of the body.

Psychogenic contractures can develop after prolonged mono- and, especially, paraparesis (pseudo-paralysis) that can not be treated. A positive diagnosis of psychogenic disease is necessary. A reliable confirmation of the diagnosis is the psychotherapeutic elimination of paralysis and contracture.