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CP Forms

Disorders now known under the general term of ‘cerebral palsy’ puzzled physicians from time immemorial, and their descriptions can be found in the works of Hippocrates and Galen, as well as in some ancient works of art and literature. It was not until the 19th centuries, however, that first attempts at their clinical systematization had been made. Among those who early concerned themselves with the treatment and classification of cerebral palsy disorders were William Little, considered to be the founder of cerebral palsy studies, Osler, Charcot and Freud.

It has been found extremely difficult to systematize cerebral palsy comprehensively—because its causes and forms are many and varied, the ways in which each of them may proceed are starkly different, and there is no distinct correlation between their clinical manifestations and their case history, morphological and laboratory data. Attempts to pigeonhole its forms into categories, subcategories, groups and subgroups have entangled the classification to such an extent that it often makes no sense at all when applied in clinical practice. However all-embracing a classification may look, it cannot pinpoint every essential and concomitant syndrome that may arise in the course of a disease, as well as the degree of compensation, probable etiology, etc. Especially so because within an established form there might be variants and aberrations.

From the practical point of view, the classifications based on incidence of motor disorders and type of abnormal muscle tone seem more acceptable than any others.

All Adeli Method materials use the standard Russian classification of 1978, according to which all cerebral palsy conditions are divided into the following forms:

1. Spastic diplegia. 2Double hemiplegia. 3. Hyperkinetic form. 4. Atonic-astatic form. 5. Hemiplegic form and 6. Ataxic form.

Spastic diplegia, otherwise known as Little's disease, is the most common form. Both halves of the body are involved, and the legs are affected more severely than the arms, varying in manipulating ability from pronounced pareses to slight awkwardness. Early contractures, deformations of the spinal cord and joints, and equinovarus and valgus deformations of the feet are characteristic. A pathology of the cranial nerves, such as convergent squint, optic nerve atrophy or impairment of hearing, occurs in 30-40 % of the children. Speech disturbances in the form of delayed speech development or pseudobulbar dysarthria are found in 70-80 % of the children. Mental impairment in some or other degree is found in 30-40 % of the children. The prognosis for movement abilities is worse than with hemiparesis. Only 20-25 % of the patients can walk without mechanical aids, and 40-50% can move when supported by any means or in a wheelchair. Children with spastic diplegia may adapt socially nearly as well as healthy ones when they are mentally normal and their arms function well.  

Double hemiplegia (tetraplegia) is one of the most severe forms, in which both the arms and the legs are equally affected with movement impairments, or the arms are affected more severely than the legs. Patients with tetraplegia find it exceedingly difficult to learn to sit. They are unable to stand and walk. Any attempt at movement results in concomitant reactions that usually manifest themselves in higher muscle tone, so that the child gets fixed in a pathological pose. Early contractures and deformations of the trunk and limbs are characteristic. In 35-45% of the cases, motor impairments are accompanied with a pathology of the cranial nerves, such as squint, optic nerve atrophy, impaired hearing, or pseudobulbar disturbances. Microcephaly, usually of secondary nature, is found in 50-75% of the patients. Intellectual retardation is found in 90%, and seizures in 45-60% of the patients. The outlook as far as movement abilities are concerned is bad. Severe mental impairments prevent social adaptation and learning even in those rare cases when the patients have acquired habits of sitting and standing. No self-care or simple work is possible for lack of motivation and because of severe motor incapacity of the arms.

Hyperkinetic form (dystonic form, athetoid form and athetoid tetraplegia in early age) manifests itself in various types of hyperkinesia, such as athetosis, choreoathetosis, chorea-formed movements, or torsional dystonia. Hyperkinesia and muscle dystonia result in an unstable posture. Neither the trunk nor the limbs can be positioned correctly. Voluntary movements are uncoordinated, jerky and sprawly. Pseudoluxations of the hip, shoulder, jaw and other joints are not uncommon. Patients learn to stand and walk very late. Generally they cannot do so unless the legs are affected in less degree than the trunk and arms and control over the head and balancing reactions are developed enough. Flexional deformations in the hip and knee joints resulted from the patient spending most of the time sitting usually make it difficult to stand and walk. As the body rests more on the interior surfaces of the feet in standing, this generally results in valgus (when the feet are turned outwards). Besides motor disturbances, impaired hearing is found in 30-45% of patients with hyperkinetic form of cerebral palsy, paresis of the upward movement of the eyes in 30-35%, pseudobulbar disturbances (salivation, mastication and swallowing problems) in 60-7-% and seizures in 10-15%. Speech impairment similar to extra-pyramidal dysarthria occurs in most patients. Between 60 and 73 % of children with hyperkinetic cerebral palsy can be taught to walk independently, but voluntary motor activity, especially fine motor control, remains disturbed to a significant degree. This makes learning impossible in some cases. Where motor disturbances are moderate, the children may be taught to write and draw. Those with sound intellect can finish school, secondary vocational and even higher learning institutions and adapt themselves to some kinds of work.

Atonic-astatic form (hypotonic form in early age) manifests itself in the patient being unable to keep a vertical position because of the defective postural control mechanisms. Control of the head and sitting, standing and walking functions either do not develop at all (astasia and abasia) or develop very slowly. It is not until the age of 1.5 or 2 years that children with this form of cerebral palsy begin to keep a sitting posture. For a long time their sitting posture remains unstable, with the trunk swaying. Prehension is usually with pronation of the hand, dysmetria and intention tremor. Because of low level of motivation and for fear of losing balance, it usually takes a long time for the hands to have developed manipulating movements. Standing and walking becomes possible between 4 and 8 years of age. At first the child can stand for a short time either when being supported by an adult or steadying himself by the wall. If the child is left unsupported, he falls by the force of gravity, as neither the arms can react protectively nor the trunk can make compensating movements to keep the balance. Children with this form of cerebral palsy either are unable to walk independently at all or begin walking after the age of 7 to 9 years. Their gait is unsteady and erratic. The head and trunk make too many swaying movements. Children often drop down. The arms are little or not involved in balancing reactions. The child cannot walk for a long distance, and normally moves about the apartment. When he finds himself in an unfamiliar setting, his motor reactions get inhibited. Marked intellectual retardation, along with negativism, lack of emotion and aggressiveness, is found in 87-90% of the children. General gross speech underdevelopment, with elements of cerebellar dysarthria, is characteristic. Seizures are found in 40-50% of the children. Cranial nerve pathology manifests itself in optical nerve atrophy, squint or nystagmus. The prognosis for movement abilities and social adaptation is bad.

Hemiplegic form (spastic hemiplegia, hemiparesis) is a type of cerebral palsy in which only the right or left part of the body is affected. Usually the arm is affected more severely than the leg. Children with hemiparesis acquire age-related habits later than healthy ones. Over time, a steady pathological pattern of the movement of the limbs and trunk develops, such as adduction of the shoulder, flexion and pronation of the forearm, flexion and ulnar deviation of the hand, adduction of the thumb, scoliosis of the spinal cord, torsion of the pelvis, and equinovarus or valgus deformation of the foot involving the shortening of the heel tendon (tendon of Achilles). The paretic extremities—the arms more than the legs—get atrophied and lag in development, especially in severe forms of damage. Motor disturbances go along with cranial nerve pathology, such as unilateral or bilateral atrophy of the optical nerve, hemianopsia, convergent squint, and weakness of the facial muscles. Sensory disturbances usually manifest themselves in asteriognosis or impaired discriminating sense; there are fewer cases of impaired sensitivity to pain, temperature sense, and tactile sensation. Convulsive attacks are found in 30-40% of the patients, and speech pathology in the form of pseudobulbar or cortical dysarthria or dyslalia in 35-40%. The degree of mental retardation found in approximately in 40% of the patients is varied from slight mental underdevelopment to gross intellectual deficiency, not always correlated with severity of motor impairments. Perceptual disturbances make learning more difficult even when the intellect is normal, as the child has impaired perception of images and it is hard for him to read, write and do sums. The prognosis for motor development depends on severity of the hemiparesis, but is good in most cases when treatment begins in time and is adequate. Almost all patients can walk independently. Self-care depends on how far the arms are affected, but even when its possibilities are markedly limited, children with unaffected intellect can be taught to attend themselves more efficiently. Social adaptation, therefore, is largely a factor of the child’s intellectual abilities rather than the degree of his motor deficiency.

Ataxic form (hypotonic form in early age) almost always shows such clinical symptoms as inability to coordinate one’s voluntary movements and keep one's balance. In the first year of life, however, it manifests itself only in muscular hypotonia and in a delay in the age-related rate of motor and mental development. It is not until static and locomotor functions develop and the arms can make more voluntary movements that ataxia becomes more pronounced. Static ataxia is less discernable than locomotor ataxia. The ability to keep an upright posture when sitting or standing is rather late to develop. Children with the ataxic form of cerebral palsy begin to walk by the age of two or three. Ataxia shows itself mostly in swinging movements when walking. Lack of coordination of voluntary movements takes the shape of dysmetria, asynergia, intention tremor, and unsteadiness in standing and walking. Muscular tone is reduced. Cranial nerve pathology is rare. Speech impairment is either moderately delayed speech development or cortical dysarthria. The prognosis for motor development and social adaptation is mostly good. Children can study at a special boarding school or even ordinary school and later learn a vocation not involving fine differentiated hand movements.

Mixed forms, in which two or more of the previous forms of cerebral palsy are combined, such as spastic-ataxic, spastic-hyperkinetic and ataxic-hyperkinetic forms, have also occurred in clinical practice.  Usually they develop in older age on the basis of spastic, hyperkinetic, ataxic or atonic-astatic forms.

Source: Detskiye tserebrialnye paralichi (Cerebral Palsy Forms) by L.Badalian, L.Zhurba, O.Timonina. Kiev, Zdorovie, 1988.

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Last updated by BP: 10.19.2005 14:05 (GMT-05:00).