Different Forms of Cerebral Palsy

Because cerebral palsy is a collective term that combines the consequences of damage to the developing brain of various adverse factors, the clinical manifestations of cerebral palsy are varied. However, as a result of the vastly different nature of the damaging factors in a given period of brain development is largely formed by similar brain function disorders. There are several classifications of clinical forms of cerebral palsy. Adopted in Russia classification distinguishes five clinical forms of the disease (by A. Ford, 1952 modified by prof. K.A. Semenova, 1972):

1. Spastic diplegia

2. Double hemiplegia

3. Distonic form with choreoathetosis syndrom

4. Distonic form with ataxia

5. Spastic gemiplegia.

Spastic diplegia (Little’s disease). The most common form of cerebral palsy (up to 60% of all cases). First described in 1860 by the English surgeon, W.J. Little. It is characterized by spastic tetraparesis with a primary lesion of the legs (diplegia), hands suffer less. Muscle tone was changed according to the type: spasticity or spasticity-rigidity. Particularly pronounced increase in tone of the flexor muscles of the arms and in back of thigh muscle group and legs. Cognitive functions may be normal or reduced to a certain degree. There are speech disorders, such as dyslalia, dysarthria. Rehabilitation potential of patients with spastic diplegia depends on the severity of motor and mental disorders. The prognosis of spastic diplegia worsens, if you have epileptic seizures (symptomatic epilepsy). Systematic targeted rehabilitation treatment for mild to moderate forms of spastic diplegia can be very productive.

The typical posture of patients with spastic diplegia verticalization:

A triple-flexion posture (In according to prof. K.A. Semenova), where the head and body bent forward, legs bent at the hip and knee joints, support – in the forefoot;

A ballet dancer posture(In according to prof. K.A. Semenova), in which the head and body bent forward, legs bent at the hip and straighten the knee joints, support – in the forefoot.

With the high tone of the muscles and internal thigh rotators the crossing of legs are often formed when standing and performing stepper motions, the abnormal internal rotation of the lower extremities also takes place.

Most clearly the clinic of spastic diplegia is seen by the end of the first year of life. Children, have the delayed reduction (regression) of inborn postural reflexes and straightening reflexes formation. On this basis, the formation of pathological muscle interactions (synergies, synkinesis) occur, when the patient attempts to perform any active movement, disproportionate number of muscles are taking part in this process. If a child walks alone, his gait is altered (abnormal motor stereotype) and not stable: swaying of the torso when walking are being marked, bending forward, legs bent at the knees or on the contrary, there is knee joints recurvation during the stance period. During the swing period is often seen sagging of the forefoot. Reliance is carried out on the forefoot (ekvinus) internal-forefoot (ekvino-valgus) or external-forefoot (ekvino-varus). Stepping motions are small, the amplitude of the removal of the femur and shin forward is reduced. At each step the child as if jumps over the foothold in an attempt to shift the center of body weight forward. There is no so-called back push, meaning the child is not able to push off the foot of the support to take the next step effectively. There is shock setting of the foot on the support, when cushioning function is broken away, what means there is no slight flexion of the legs at the knee joint in the midstance phase. This increases the impact loading on the joints of the lower limbs, leading to early progression of arthrosis joints (dysplastic arthrosis) among the patients moving by their own or with somebody else support.

Double hemiplegia.

The most severe form of cerebral palsy. Manifested in the first months of life. Characterized by pronounced mental (mental retardation) and speech disorders, severe locomotor dysfunctions, arms suffer as well, and legs (spastic quadriplegia or pronounced tetraparesis), often with different disorders on the left and on the right. At the result of the high muscle tone (spasticity-rigidity or rigidity), arms are bent at the elbow and wrist joints are drown toward the torso, legs bent or straightened at the knee joints, bent at the hips, knee joints are rotated inward, the thighs are drown inward. Functions of holding a vertical posture among children with double hemiplegia are not formed. Sick children do not master the skills of sitting, standing, unassisted walking. Many of them are not able to hold their head, it is dropped to the chest or overturned back. Severe movement disorders are combined with early contractures of the joints and bone deformities. High muscle tone makes it difficult to care for a sick child. Rehabilitation potential of these children is low. These patients do not have an ability to take care of themselves. Most patients with double hemiplegia are suffering from epileptic attacks. However, there are cases when a severe form of spastic diplegia is mistakenly seen as a double hemiplegia. In such cases, persistent treatment can not only reduce the severity of movement disorders, but also improve speech and mental development.

Distonic form with choreoathetosis syndrom.

Intelligence in this form of cerebral palsy usually does not suffer. Involuntary movements (hyperkinesis) have a significant impact on posture and movement. They may be different: fast sweeping, abrupt called trochaic hyperkinesis, slow worm-like – athetosis. Choreoathetosis is frequently observed. Hyperkinesis in hands and facial muscles dominate over hyperkinesia in legs. Athetoid hyperkinesia predominates in fingers and hands, choreic – in the muscles of neck, trunk, proximal (closest to the body) parts of the limbs. The intensity of hyperkinesis increases with an attempt to run in active motion or with the emotional excitement. During the rest, it becomes much less uncontrolled movements; when the person is sleeping they almost disappear completely. There is a kind of muscle tone disorders – dystonia, characterized by variable muscle tone. The patients do not have normal muscle tone fixation, defining the position of the limbs and body in space. The congenital tonic reflexes, especially the neck ones, affect for a long time. Therefore the intensification of hyperkinesis during irritation of the neck proprioceptors (hyperkinesis trigger zone) takes place. With this form of cerebral palsy speech disorders (hyperkinetic dysarthria) are being observed, associated with involuntary movements and muscle tone disorder.

Because the energy consumption is significantly increasing during involuntary movements, many children suffering from distonic form of cerebral palsy have deficit of body weight. This also contributed to dysfunction of the autonomic nervous system.

In this form of cerebral palsy muscle tone in the first year of life changes in according to certain low. Among children in the age of 1 month it is reduced (so-called  floppy child). From the 2nd month of life, dystonic attacks take place, during which there is a sudden increase in muscle tone, and a rapid decline afterwards. Involuntary movements in severe cases first appeared in the age of 4 months, usually in the muscles of tongue, but among the majority of children with mild damage of nervous system hyperkinesis it occurs at the end of 1st – early 2nd year of life.

Epileptic attacks with distonic form of cerebral palsy are rare. Rehabilitation potential of these patients, is mainly determined by the feature and intensity of involuntary movements. Prognosis is much worse in the so-called double athetosis. In general, currently existing technologies of rehabilitation with mild distonic form of cerebral palsy gives the highest result.

Sometimes spastic diplegia is combined with hyperkinesia, mainly of athetoid and horeoatetosis, or ataxia (see below).

 Distonic form with ataxia.

In this form of the disease reduction in muscle tone (hypotonia), inhibition of innate postural (pose) reflexes is marked from the birth. The formation of rectifying (righting) reflex is delayed. Cerebellar disorders are common: ataxia (incoordination of movements in static and execution of movements), dismetriya (disparity motion disparity amplitude of voluntary movements of their purpose, breach of control over the distance), intention tremor (correction of motion with an excess of the amplitude when attempting to perform purposeful motor actions.) Children with a significant delay, compared to the physiological norm, begin to keep their head, rolling over from back to stomach and from stomach to back, to sit by themselves, stand, walk. Many children with this form of cerebral palsy have mental retardation and speech disorder. There may be seizures. Rehabilitation potential is generally low due to violations of the psyche.

 

Spastic gemiplegia

It is characterized by lesions of the limb of the same side (left or right hemiparesis), predominantly expressed in the hand. The asymmetry of spontaneous movements of the limbs of healthy and affected sides is noticeable from the birth. Congenital reflexes of movement are usually formed; righting reflexes are forming pathologically and with some delay. Because of this the child’s pose during the verticalization is also incorrect, for example, in the pose of standing, shoulder girdle of the affected side is omitted, the arm is bent at the elbow and adducted to the torso, leg is bent at the knee joint.

Asymmetry of posture leads to the formation of so-called paralytic scoliosis, with an arc of curvature of the spine bulge pointing in a healthy way. Paretic limbs are stunted, formed anatomical shortening of hands and feet of the affected side.

The gate of these children is changed: from a mismatch between the length of the legs of healthy and affected side and obliquity position of the pelvis, the healthy lower limb, is being “adjusted” to the other one. Therefore there is an excessive bending at the knee of the healthy leg and usually to much straightening of paretic leg in the knee joint during the support. In the paretic lower limb the flexion is absent; foot support is carried out on the forefoot. The hand of the affected side, usually bent at the elbow, is adducted to the body and is not involved in walking; it does not fulfill any mutual (reciprocal) movements during walking. The muscles of the paretic side are usually hypotrophic.

There are so-called imitation and coordination synkinesis (imitation – involuntary movement of limbs, which imitates the movement of any other extremity; coordination – a movement which the patient can not perform in isolation, but performs as part of integrated motor synergies). Partial (focal) convulsive paroxysms occur with spastic hemiplegia more frequently, there may be mental and speech disturbances.

The prognosis for this form of paralysis is usually determined by the severity of mental disorders and the presence of symptomatic epilepsy. Abnormal motor stereotype of sick children is resistant to treatment.

 

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