Indian J Pediatr 1992; 59 : 159-164
Assessment of Cerebral Palsy Suresh Rao Aroor
National b2stitute of Mental Health and Neurosciences, Bangalore Great changes take place during a baby's growth and maturation in abnormal as well as in normal motor development. Normal motor development means the gradual unfolding of a child's latent abilities. The baby's early and fairly simple movements change and become more varied and complex. Stage by stage, former achievements are modified, elaborated and adapted to suit finer and more selective movement patterns and skills. This process continues for many years, but the greatest and fastest changes take place in the first 18 months, during which time the most fundamental and important milestones are reached. By this time, the child is able to get up against gravity, walk with some balance and use his hands for manipulation though still rather clumsily. There are many things he still cannot do, which will develop in the future and change his activities further. Up to about 3 years of age, the improvement in balance and hand skills continues at a fairly quick rate. At about the age of 5 years, he is ready for school. He now has good control of his balance and can co-ordinate selective and precise movements of his hands for manual skills. From now on, development slows down, although co-ordination and skills Reprint reqaests : Dr. S.R. Aroor, Pediatric Neurologist, National Institute of Mental Healthand Neurosciences, Bangalore-560 029.
continue to improve during the rest of his school life. The child with cerebral palsy also develops, but at a slower rate. His development, however, is not only retarded, but follows an abnormal course. In severe cases, that is to say, in children whose whole body is affected, there may be but little change for a long time or development may become arrested altogether at a very early stage. Furthermore, whereas the changes in the development of a normal child's motor patterns are most rapid and significant up to about 5 years of age, changes in the activities of a child with cerebral palsy arc slowed down but may continue into adolescence or even adult life. This is especially so in the case of some athetoids and in ataxic children, who remain mobile and do not easily develop contractures and deformities. Indeed, some athetoid children do not acquire walking until they are 14 or 15 years of age. Many of the essential and fundamental patterns of motor development which emerge in a normal child at certain stages of growth, in preparation for future, more complex activity, are missing in children with CP. The child's development is therefore not only delayed but disordered and disturbed as a result of the lesion. The sequence of this development in different types of CP have been carefully observed on a great number of children over many years. It is important to know the sequences of 159
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abnormal development in order to detect the first signs of deteriorating postural behaviour and to reecognise its implications for future activities and the possible development of typical contractures and deformities. Development is a continuous process from conception to maturity. The sequence of development is the same in all children in the cephalocaudal direction. The first step towards walking is the development of head, hand and trunk control that is crawl, creep, sit before he can stand and walk. Certain primitive reflexes such as the grasp and walking reflex, have to be lost before the corresponding voluntary movement is acquired. The rate of development in children are different. W e c a n all say what the average level of development is for a child of a given age, but none can say what the normal is, for it is impossible to draw the dividing line between normal and abnormal. Further away from the normal, more likely he is to be abnormal. Hence in the assessment of CP it is essential to know the normal sequence of development in different fields. Diagnosis and Assessment of Cerebral Palsy The diagnosis of CP in the first year is regarded by some as a matter of great difficulty and is not apparent usually before 1218 months of age. In fact CP of the spastic type, except in mild cases, can be readily diagnosed in the first few days of life. The rigid form can be readily diagnosed in the earliest infancy. The athetoid form cannot usually be diagnosed early, because one cannot be sure of the diagnosis until athetoid movements develop which may not
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be for 1 or 2 years after birth. Congenital ataxia cannot be diagnosed until about 6 months, because it is dependent on certain purposive movements not found before then: but tremor can be diagnosed early certainly by the time the baby is able to sit. It would be profitable to begin by enumerating the main difficulties in early diagnosis. These are : 1. There are grades of severity of cerebral palsy, from the severe form diagnosed readily in the newborn period, to the mildest form which is first brought to the doctor's attention at 9 or 10 years. It can be extremely difficult to diagnose mild degrees of Spasticity in early infancy. Signs may be equivocal for several months before it finally becomes clear that disease is present. Brisk knee jerk s may be thought to be within normal limits, but with the passage of time it becomes clear that they are pathological. It is impossible to draw the line between normal and abnormal, and to say for instance whether brisk tendon jerks or slight hypertonia are normal or otherwise. Sometimes one has to be prepared to wait and see, in order to determine whether a child is affected or not. In the majority of cases, the diagnosis is obvious in the early days or weeks of life. 2. There are several types of cerebral palsy, each with its own clinical presentation and findings. 3. The diagnosis is greatly complicated by the wide range of levels of intelligence, and particularly by the frequency with which mental subnormality is found. Mental subnormality alone has a profound effect on the developmental pattern. 4. The delayed appearance of signs of cerebral palsy, particularly signs of atheto-
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sis. As babies grow older, certain signs become more obvious. 5. The occasional disappearance of signs of cerebral palsy. One sometimes detects signs of the spastic form of cerebral palsy in early infancy and f'mds that these signs gradually disappear. Andre-Thomas described several examples of the disappearance of signs of cortical injury especially hemiplegia, and emphasised that on that account prognsosis must always be guarded and that examinations must always be repeated. Minkowski using the Andre-Thomas method of examination, divided 74 newborn babies into three groups, (a) normal (25 infants), (b) minor neurological abnormalities (43 infants), (c) gross neurological abnormalities (6 infants). On re-examination, 2 years or more later of the 25 who were normal in the newborn period, 19 were 9 subsequently and 6 had minor but temporary problems (such as ocular defects and delayed walking): of 43 in Group B (showing minor neurological signs in the newborn period), 22 were normal subsequently, 18 had minor neurological handicaps but three had serious sequelae. Of 6 who showed serious neurological signs in the newborn period, 3 continued to show severe sequelae on follow-up, while the remainder showed trivial and temporary neurological signs. One pays much less attention to single sign than to a combination of signs. For instance, one would pay little attention to some degree of hypertonia alone but one would pay much more attention to a combination of hypertonia and delayed motor development, or an unusually small head circumference. One pays much less attention to delayed motor development alone, than one does to delayed motor
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development combined with delayed social responsiveness (late smiling) or a small head circumference. Cerebral palsy is not diagnosed merely on the basis of exaggerated knee jerks, or ankle clonus or hypertonia without other abnormal signs. 9 The diagnosis must be made, as always, on thebasis of the history, the examination, and the interpretation of one's findings. The history includes the 'risk factors' (namely, family history of cerebral palsy, prematurity, especially extreme prematurity multiple pregnancy, low birthweight in relation to the duration of gestation, mental subnormality and severe anoxia, convulsions, hyperbilirubinaemia or cerebral haemorrhage in the newborn period). The mother may herself have noticed that the baby feels stiff or is stiff On one side, or keeps one hand clenched when the other is open, or does not kick the legs properly. The baby may kick both legs together instead of reciprocal kicking. The mother may have 9 that when the baby creeps, one leg trails after the other. She may notice that the child consistently refuses to use one hand. She may give a clear history of 'dissociation' meaning in this context that there is severe retardation in gross motor development such as sitting, while the baby is more advanced in other fields of development.
Spastic Form After taking the history, the 9 is made on the developmental examination. In summary the following are the essential points. First 3 months
1. If newborn, note the quality and quantity of his movement for the spastic child tends to be relatively immobile. If he has spastic quadriplegia he may lie with his limbs
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unduly extended, and his hands unusually tightly closed. After about 3 months the hands should be predominantly loosely open. A hemiplegic child would be likely to have ,one hand tightly closed and the other open and there wilt be asymmetry of movement. 2. Observe the child, his head size and shape, his facial expression his alertness and interest in his surroundings. 3. Hold the child up with your hands in his axilla. There may be abnormal extension of the hip and knees (or asymmetry) and the legs may cross. 4. Hold in ventral suspension. There is usually delayed motor development and so there will be excessive head lag. The arms and legs commonly hang down lifelessly without the flexion of the elbows and knees and slight hip extension seen in the normal child. 5. Place him in the prone position, in order to assess maturation. The spastic infant commonly assumes an immature position owing to the mental subnormality but may show excessive extensor tone, as above. 6. Place him in the supine position. Note the symmetry or asymmetry of the kick. Note whether the hands are equally open or closed. Assess muscle tone by feeling the muscles, assessing the resistance to a passive movement, assessing the range of movement and shaking the limbs (for passivity). Assess the range of movement especially in the hips (after flexing them to a right angle) and in- dorsiflexion of the ankle. When doing this, test for ankle clonus. Test the knee jerks-beginning to tap over the dorsum of the ankle. Test the bicep jerks beginning to tap over the shoulder. 7. Pull him to the sitting position in order to
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assess head lag. Sway him gently from side to side in order to determine the degree of head control (passivity). When he is being pulled up into the sitting position, have the hand in the popliteal space in order to detect spasm of the hamstrings. When leaning forward he repeatedly falls back, because of spasm of the erector spinae, gluetei and hamstrings, when he is pulled up to the sitting position, he may rise to his feet, because of excessive extensor tone and give the wrong impression of advanced weight bearing. The true diagnosis is revealed by the other signs of excessive tone mentioned above, the exaggerated knee jerks, the ankle clonus and reduced abduction of the hip and ankle dorsiflexion with head lag when he is pulled up. 8. Measure his head circumference and relate this to his weight. At the end of this period persistence of the Moro reflex, grasp reflex and symmetrical tonic reflex point to the diagnosis. They should have disappeared by 2 to 3 months. Four to 8 months
1. Observe the child as in the case of the younger infant, noting the quality, quantity and symmetry of movement. 2. Give the child a 1 inch cube to go for. This may reveal the typical spastic approach, unilateral if he has a hemiplegia, with the slow characteristic dorsiflexion of the wrist with splaying out of the fingers as he approaches the objects often with ataxia and tremor. 3. Hold the child up with your hands in his axiUa in order to determine whether there is excessive extension of the legs. Test in ventral suspension and the prone position, supine position and pulled to sitting position as above.
AROOR: ASSESSMENTOF CEREBRAL PALSY 4. Note signs of general retardation. 5. Measure the head circumference. 6. Check the hearing. These are the basic signs of the spastic form of cerebral palsy. In a busy clinic, it would take perhaps 2 or 3 minutes to carry out the tests described.
Athetosis It is virtually impossible to make a definite diagnosis of athetosis, until the athetoid movements are seen and these may be delayed for some years. The condition may be suspected because of one of the conditions known to place the child at risk of athetosisparticularly severe anoxia or hyperbilirubinaemia. When those 'risk' conditions occurred, it is easy to imagine that the child has athetoid movements, when the movements are the normal arm and leg movements. The early clinical signs of athetosis are developmental delay, excessive extensor tone, rhythmical tongue thrusting, sucking and swallowing difficulties and ataxia on reaching for objects. These are followed latter by development of athetoid movements.
Rigidity The rigid form is diagnosed by the extreme rigidity of all limbs,in the absence of signs of disease of the pyramidal tract, such as increased tendon jerks, ankle clonus, positive stretch reflex and extensor plantar response. It is almost always associated with a severe degree of mental deficiency. Ataxia The ataxic form is diagnosed by the ataxia in the child's approach to an object, and ataxia in sitting and walking.
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Hypotonic Form of Cerebral Palsy This is a very rare form of cerebral palsy, which can readily be confused with the hypotonias. Almost all infants with this condition are mentally subnormal. The circumference of the skull is likely to be small. There is an increased range of movement. Fits occur in a third. The plantar responses are extensor, and the knee jerks are exaggerated, so that benign congenital hypotonia and the Werdnig-Hoffmann syndrome can be readily excluded.
Abnormal Signs in Cerebral Palsy These are : 1. Shrill or high pitched cry 2. Apathy, excessive somnolence, irritability, hyperalertness 3. Pithed frog position in supine, opisthotonus, extension of legs, little spontaneous movementts, asymmetry of movements 4. Tremors, convulsions, apnoeic attacks 5. Head size small, buldging fontanelle 6. Eyes: roving nystagmus, pupils fixed or pin point 7. Poor orientation to sound 8. Reflexes: Increased, absent, asymmetric, abnormal moro reflexes especially without flexion or adduction, absent oral reflexes. 9. Poor sucking and feeding 10. Tone: Hypo or hypertonia, asymmetry 11. Gross under development: Defective in prone, ventral suspension, pulled to sitting position.
Conditions Wrongly Diagnosed as Cerebral Palsy Some of these are : Mental retardation; Isolated motor retardation; Voluntary resis-
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tance to passive movement; Abnormality of joints; U n s t e a d i n e s s of gait; N o r m a l movements; Causes of toe-walking; Congenital shortening of muscles; Lower m o t o r neurone diseases; Involuntary movementsother causes; Degenerative conditions; and O t h e r syndromes.
3. Kershaw JD. Ilandicapped Children. London : William Heinemann Medical Bb0ks Ltd.
REFERENCES
5. Eidinova MB, Vinarskaya MP. Cerebral Palsy in Children and its Treatment. London: Pergamon Pre~.
1. Bronson C, Paine RS. The Natural History of Cerebral Pal.~y. Harvard, London : Harvard University Press. 2. Woods GE. The Handicapped Child-Assessment and Management. London: Blackwell Scientific Publications.
4. Jouwen BCL, Preehth I-IFR. Neurological examination of the child with minor nervous dysfunction. In: Levin KA, Cerebral Palsy, New York : E & S. Livingstone Ltd.
6. Holt KS. Assessment of Cerebral Palsy. New York: Llyod-Luke Medical Books Ltd. 7. Bobath B, Bobath K. Motor Development in Different Types of Cerebral Palsy.