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Management of Hemiplegic Children with Peripheral Sensory Loss
MANAGEMENT OF HEMIPLEGIC CHILDREN WITH PERIPIlERAL SENSORY LOSS MARGARET H. JONES, M.D.
Recently attention has been called to the frequency of peripheral sensory loss in brain-damaged children, especially those having hemiplegia. Management of such children, however, has continued to stress only motor training with the addition of bracing and surgery in case of orthopedic defects. Often, long periods of physical and occupational therapy have failed to develop functional use of the affected hand. In an adult, using multisensory stimulation rather than motor training, Forster has reported improved function in a hemiplegic patient whose deficit was mainly sensory. Whether emphasis on sensory stimulation in the management of the hemiplegic child with both sensory and motor deficits will lead to better functional use of the affected extremities is not known. Certainly, evaluation of both sensory and motor loss at as early an age as possible is important in understanding the child's total problem. The purpose of this article is to present reported data on the types and frequency of peripheral sensory loss in hemiplegic children and to add hitherto unpublished material from our laboratory. Findings will be discussed in relation to the management of these children. A preliminary report on the use of multisensory stimulation in the training of the young child with peripheral sensory loss is included. REPORTS IN THE LITERATURE OF TYPES AND FREQUENCY OF PERIPHERAL SENSORY LOSS
Tizard, Paine and Crothers,12 Tachdjian and Minearl l and Hohman, Baker and Reed 6 reported results of sensory testing in a total of 249 From the Department
of Pediatrics, University of California at Los Angeles.
766
HEMIPLEGIC CHILDREN WITH PERIPHERAL SENSORY LOSS
school-age and older cerebral-palsied children. Most were spastic hemiplegics, some triplegics or quadriplegics, with one side more involved than the other. Partial or complete sensory loss of one or more types ranged from 42 to 72 per cent, being 52 per cent for the group as a whole (Table 29). The most frequently found deficits were stereognosis or form discrimination, two-point discrimination, and passive movement or position sense, in decreasing frequency in that order (Table 30). Though partial or complete loss of light touch, pain, sharp-dull, hotcold, wet-dry, occurred in many children, none of these latter modalities was defective unless one of the three most commonly impaired was also deficient. Tachdjian and Minearl l demonstrated an inverse ratio between the extent of sensory loss and the functional use of the extremity, as noted in Table 31. They found that 87.5 per cent of hemiplegics who had no use of the affected hand had sensory loss. Conversely, patients with normal use of the hand showed no sensory deficit. Tizard, Paine and Crothers12 reported strong correlation between skeletal undergrowth and sensory deficit (Table 32). Of 16 patients TABLE
29. Frequency of Sensory Loss NUMBER OF PATIENTS
SENSORY DEFICIT
Number
106 .... 57 47 .... 34 96 .... 40 Totals 249 .... 131 TABLE
AUTHOR
Per Cent
53.8 72.3 41. 7
Tizard, Paine and Crothers12 Hohman, Baker and Reed 6 Tachdjian and Minearll
52.0
30. Types of Sensory Loss (3 Most Frequent) TYPE
NUMBER
TOTAL
T.C.P.12 H.B.R.o T.M.ll No. Stereognosis or form discrimination ...... 20 2-Point discrimination ...... 14 Passive movement or position sense ...... 15 Total number of patients in series ...... 106 TABLE
28 23
40 31
14
16
r;88T'36 68 28 "fi>\ 45 20
96
47
249
31. Functional Use of the Hand versus Sensory Lossl1 FUNCTIONAL USE OF HAND
%
PER CENT HAVING SENSORY LOSS
None .............. 87.5 Poor .............. 68.9 Fair .............. 30.0 Good.............. 6.6 Normal.............. 0.0
MARGARET H. JONES TABLE
32. Skeletal Undergrowth versus Sensory Deficit12 TOTAL
SENSORY LOSS
HEMIANOPSIA
14 2
14 2
Skeletal undergrowth ....... 16 No skeletal undergrowth ....... 12
TABLE
767
33. Severity of Sensory Deficit in Hemiplegia 7 Least severe .................. Face:
perioral lateral regions Trunk: posterior anterior Leg: upper lower foot Arm: upper lower Most severe ................... Hand
showing skeletal undergrowth, 14 had sensory loss and also hemianopsia. Of 12 not showing skeletal undergrowth, only 2 showed sensory loss and hemianopsia. Laine and Gros 7 discuss the degree of sensory loss in different parts of the affected side in hemiplegics, listing deficits in order of increasing severity (Table 33). Laine and Gros report that the affected arm and leg may show improvement in physical function and in sensory perception after operation in which contralateral frontal, parietal, temporal and occipital lobes are removed en masse (hemispherectomy). They note that voluntary motion returned simultaneously with the return of position sense. The reports presented above were obtained from tests of children of school age. In regard to the discriminatory abilities of normal young children, Haeusserman4 has stated that children of 4Y:! years can differentiate familiar objects by tactile discrimination of form and texture. Monfraiz, Tardieu and Tardieu9 report results with 12 geometric shapes used in the Grace Arthur Board with 218 children, ages 2y:! to 8y:! years. Those under 3y:! failed to identify geometric shapes, but could recognize familiar objects. Children between 3y:! and 6 could be graded by age by frequency of correct responses with the geometric shapes. MATERIALS AND METHODS USED IN OUR STUDY'
Our study was undertaken to assess methods of testing sensory deficits,
* Assistance was given in these studies by Paul Greenberg in the summer of 1956, supported by the Spastic Children's League, and by L. Huhn in the summer of 1957, supported by a fellowship grant from the National Foundation. Some of these tests on adults were administered by occupational therapists at United Cerebral Palsy As· sociation of Los Angeles County, in course of a research project supported by grant from United States Office of Vocational Rehabilitation.
768 TABLE
HEMIPLEGIC CHILDREN WITH PERIPHERAL SENSORY LOSS
34. Sensory Modalities Tested
Exteroceptive ........... Light touch Sharp-dull Pain Hot-cold Wet-dry Proprioceptive ........... Passive movement-position Pressure Vibration Cortico-senaory ........... Rough-smooth Texture Weight-length Size (thickness) Shape (2 dimensions) Form (3 dimensions) (object discrimination) Tickle or scrape (probably a combination of modalities)
particularly cortical-sensory modalities, in the cerebral-palsied child under six years of age. For comparison 27 adults, 13 children from 6 to 12 years and 14 children under 6 years (10 between 3 and 6 and 4 from 19 months to 3 years) were tested. Most were spastic hemiplegics, a few quadriplegic, athetoid or mixed, with one side more involved than the other. Modalities studied were essentially those tested in Hohman's5 series. They are listed in Table 34. For children under 6 years of age a special table with glass insert was developed to allow the tester to view the child's extremities without either blindfolding him or expecting him to keep his eyes closed (Fig. 61). The child was held by an adult or seated in a suitable chair opposite the examiner. In addition to the usual test objects, duplicates of those used for length, size, shape and form discrimination were made (Fig. 62). These were placed on top of the table and the child asked to pick out a matching object, rather than to answer verbally. Often several periods were required to secure satisfactory cooperation with the child between three and four years, but reproducible results were obtained in this group as well as those between four and six years. For the child under three years of age, in our study, the only modalities that were tested satisfactorily were stereognosis-using small familiar objects (ball, key, safety pin, pencil) and checking to see whether the child could name them when presented visually before testing by tactile discrimination; light touch-feather, cotton or small object (penny, dowel or pencil) carefully placed in hand; response to pin prick-speed and type of response and localization on the affected side as compared to the good side. McGraw8 described response to pin pick in a series of 75 normal children, ages a few minutes to 4 years, as outlined in Table 35. She differentiated two major features in the behavior pattern to pin prick,
MARGARET H. JONES MARGARET H. JONES
769 769
Onethe thesensorimotor, sensorimotor,the theother otherthe thecognitive cognitiveaspects. aspects.The Thesensorimotor sensorimotor one was based on the response of application of the pin to the skin,the the was based on the response of application of the pin to the skin, visual stimulus. stimulus.Only Onlythe thesensorimotor sensorimotorwas was cognitive, toto the thepin pinasas aavisual cognitive, considered in our study, for the pin was kept out of sight. considered in our study, for the pin was kept out of sight. Thefirst firstphase phaseofofresponse responsewas wasthe thenewborn newbornorordiffuse diffusephase, phase,which which The posconsisted of diffuse bodily movement accompanied by crying and consisted of diffuse bodily movement accompanied by crying and pos~ sibly local reflex withdrawal. The reaction was immediate. sibly local reflex withdrawal. The reaction was immediate.
Fig.61. 61.Table Tablewith withglass glassinsert insertfor fortesting testingsensory sensorydeficits deficitsininextremities. extremities. Fig.
Fig. 62. 62. Objects Objects used used for for testing testing sensory sensory deficits deficits and and form form discnmination. discIlmination. Fig.
770 TABLE
HEMIPLEGIC CHILDREN WITH PERIPHERAL SENSORY LOSS
35. Response to Pin Prick RESPONSE
AGE OF CHILD
Newborn or diffuse phase ........... Minutes to 1 month Inhibition of diffuse phase (cries only) ........... 1 month to 3 months General J'lcalization (deliberate withdrawal) ........... 6-13 months Specific localization ........... 8 months on
Inhibition of this phase occurred after a month or so, the response being delayed diffuse body movement with less reflex withdrawal. Later, about 6 months of age, the infant started to localize the area stimulated by deliberate withdrawal directed away from the source of irritation, as compared to reflex flexion type of withdrawal seen previously. Localization at this stage was mainly visual. Specific localization began by about 8 months, as indicated by the child's ability to touch or rub the area stimulated. In McGraw's series of 75 normal infants the phase of specific localization had occurred in all before 500 days. The affected side was regularly stimulated before the unaffected. RESULTS OF EVALUATION (54 PATIENTS)
Our findings as to the type and incidence of sensory deficits in hemiplegic children from 6 to 12 years and in adults were similar to those of other investigators, especially Hohman et al.: 6 namely, that about three-fourths had demonstrable deficits, mainly of the cortico-sensory type. One or more of the three following modalities was either lost or less well perceived on the affected side-stereognosis or form discrimination, two-point discrimination, position or passive movement. Comparing the results by age groups, it will be seen that those under six years of age showed approximately the same incidence of demonstrable cortico-sensory loss as did the older children and adults. In the children under three years of age the most frequent deficits demonTABLE
36. Sensory Deficit by Age Group* AGE
NUMBER OF PATIENTS
Over 16 years .............................. 6-12 years .............................. Under 6 years .............................. Total. .............................
27 13 14t 54
SENSORY DEFICIT
No.
%
20 10 9
75 77
39
65 74
* Los Angeles study: stereognosis or form, 2-point discrimination, position or passive mov~ment.
t In 3 of the 14 patients, tests oC this type were unsatisfactory.
MARGARET H. JONES
771
strated were light touch, paSSIve movement, abnormal response to pin prick. Figure 63 indicates a breakdown of the findings into exteroceptive, proprioceptive and cortico-sensory types of sensory loss. Light touch, indicated by the number "I" on the left-hand series of graphs, was the most reproducible test in the very young child and could be applied satisfactorily to all. Without calling attention to the fact that the affected arm might be touched with cotton or a feather, 75 per cent of the group under 6 years of age failed to appreciate the stimulus. In children from 6 to 12, only 58 per cent failed to respond, and in the SENSORY DEFICIT
L.A. STUDY BY AGE GROUP
0/0
80
'~\
60
40
x
20 <6 6-12 >16
<6 6-12 >16
<
6-12 >16
OL---~~~~~~~~~~~~~~~--
EXTEROCEPTIVE PROPRIOCEPTIVE CORTICOSENSORY
Fig. 63. Findings in exteroceptive, proprioceptive and cortico-sensory llPes of sensory
loss.
adult, only 26 per cent. Good response occurred on the other side in all. Similar findings were reported for pain or sharp-dull discrimination, indicated by number "2" on this same series of graphs. Number "3" on these graphs indicates the response to hot-cold stimulation, which was not satisfactory in many of the children under six, but showed no variation between older children and adults. The middle graph indicates the result of tests for perception of passive movement or position sense, and this also appeared to improve with age. On the right hand side of the chart the cortico-sensory loss, as noted by the shaded areas, representing loss of one or more of the fol-
772
HEMIPLEGIC CHn.DREN WITH PERIPHERAL SENSORY LOSS
lowing-form, shape, weight, length discrimination and object identification-remained at the same level in all ages. In this graph number "2" represents loss in two-point discrimination, number "3" loss in rough-smooth differentiation. The broken line "4" represents loss of localization to touch. As in Tizard, Paine and Crothers's12 series, no relation was found in our patients between intelligence, degree of atrophy of part, electroencephalographic abnormalities and incidence of sensory loss. We do not have data on length of the extremities. COMMENTS
The findings outlined above support DeJong's2 comment that "sensation and motion are interdependent and that severe motor disabilities may follow impairment of sensory functions." For a discussion of the current concepts of the physiology of motor learning, the reader is referred to Hellebrandt's5 review, which gives various hypotheses on the relation of sensory input to motor function, and Clark'sl lecture on sensory experience and brain structure. From the clinical point of view, Ogden, Robert and Carmichael'sl0 report of children with congenital deficits in sensory perception, but without motor abnormality, who show a delay in sitting, standing and walking, suggests a possible relation of sensory loss to motor development. Forster's3 success in the use of multisensory stimulation in retraining of an adult hemiplegic with a sensory loss due to cardiovascular accident is important in showing that, despite known central nervous system damage, improved sensory perception is possible with training and is followed by improved hand use. Current failures to achieve functional hand use with motor education alone in congenital or early acquired hemiplegics suggest the need of trial of another approach. Evaluation of sensory perception, particularly of the cortico-sensory type, should be attempted even in young children before a therapy program is outlined. Re-evaluation of sensory modalities as the child matures and particularly before any surgical procedure is done is essential. As illustrated in case reports by Tizard, Crothers and Paine,12 tendon transplant may be beneficial in patients with no sensory loss, but may not be helpful in those with sensory loss. These authors also found that the frequency of cortico-sensory loss is as great in the child whose hemiplegia was present from birth as in the patient who acquires the condition later, although the extent of involvement is usually greater in the latter children. Hohman et al. 6 found that "very small areas of the hand might be involved leaving a major portion with good sensory perception." They
MARGARET H. JONES
773
also stress the finding of impainnent of perception, as for example the ability to differentiate two points at % inch on one side and 1% inches on the other side. Sensations might be correctly, but more slowly, identified on the affected side as compared with the unaffected. Again emphasizing the correlation between recovery of sensory perception and development of motor function, is Twitchell'sls study in adults of return of finger reflexes which were deemed proprioceptive, as an index of final functional use of the hemiplegic hand. Without giving specific data, this author also states that "persistent defect of sensation of the affected limbs . . . particularly point localization, position sense, and other cortical discriminating functions is not conducive to good motor recovery." PRELIMINARY RESULTS OF TRAINING INCORPORATING SENSORY STIMULATION
Recently studies have been begun in our laboratory aimed at determining the effect of multisensory stimulation in the young hemiplegic child. We are interested in the effects of such training, both on tested sensory deficit and on spontaneous motor function. In the child under six years of age, sharply contrasting stimuli of temperature, texture, size and shape were presented first to the good hand and then the affected one. This was done in the fonn of a story told to the child, on an individual basis rather than in a group. Initial results are encouraging in that the frequency of spontaneous reach and grasp increased, first on the unaffected and then on the affected side. Graded series of motor perfonnances were also constructed for which rewards were given. The child was shown what was wanted, but was not helped in achieving it. These tasks involve eye-hand coordination, beginning with a simple motion. Progression from simple to more difficult motor tasks was possible even at age 2% years. In the older children, tactile discrimination with reward was set up, first calling attention to differences verbally by asking the child to feel and look at each of two disparate objects and finally to differentiate without vision. Gradually the children appeared to learn clues enough to make at least gross differentiations. In the past the unaffected ann and hand were sometimes immobilized to force use of the affected side. This resulted in stuttering, increased seizures and behavior problems. It is true that in the aboveoutlined training the child is asked to use the affected side, but the other side is only gently restrained and for short periods. The tasks required of the affected side are carefully graded, beginning with simple ones in which success is easily attainable. Thus far, none of the unde. sirable side effects have been noted.
774
HEMIPLEGIC CHILDREN WITH PERIPHERAL SENSORY LOSS
Training, using sensory stimulation, needs to be as carefully planned and carried out as does that for motor education. It needs to be done on an individual basis and by trained personnel and, as Forster notes, is time consuming. It needs to be combined with passive stretching if contractures are present. SUMMARY
An essential part of the evaluation of the hemiplegic patient is the sensory examination. Sensory deficit is often associated with poor functional use of the hemiplegic hand. It is possible, with some modifications of usual testing methods, to demonstrate sensory impairment or loss in children under six years of age. Table 37 reports major deficits found in the three age groups. TABLE
37. Maior Sensory Deficits Demonstrated in Hemiplegic Children of Different Ages
Over 6 years .............. Form or object discrimination 2-Point discrimination Position sense-passive movement 3-6 years .............. Identification of small familiar objects by name Match shapes and forms :I: Passive movement Under 3 years .............. Perception of light touch (penny or dowel in hand) Response to pin prick (degree oflocalization) Passive movement
Our findings are similar to those of other authors in respect to frequency of sensory impairment, particularly cortico-sensory deficits (40 to 65 per cent). It is also of interest that deficits in light touch were three times as frequent (75 per cent) in children under 6 years of age as in adults (26 per cent) . Current studies of the effect of multisensory stimulation training on the development of motor function in young hemiplegic children are encouraging. REFERENCES 1. Clark, W. LeGros: The Thirty·Second Maudsley Lecture; Sensory Experience and Brain Structure. J. Ment. Science, 104:434, 1958. 2. DeJong, R. D.: The Neurologic Examination. 2nd Ed. New York, Paul B. Hoeber Inc., 1958. 3. Forster, F. M., and Shields, C.: Cortical Sensory Defects Causing Disability. Arch. Phys. Med. 6 Rehabilitation, 40:56, 1959. 4. Haeusserman, E.: Developmental Potential of Preschool Children. New York, Grune & Stratton, Inc., 1958. 5. Hellebrandt, F. A.: The Physiology of Motor Learning. Cerebral Palsy Review, July-August, 1958.
MARGARET H. JONES
775
6. Hohman, L. B., Baker, L., and Reed, R.: Sensory Disturbance in Children with Infantile Hemiplegia, Triplegia and Quadriplegia. Am. 1. Phys. Med., 37: 1, 1958. 7. Laine, E., and Gros, C.: L'Hemispherectomy. Paris, Masson et Cie, 1956. 8. McGraw, M. B.: The Neuromuscular Maturation of the Human Infant. New York, Columbia University Press, 1943. 9. Monfraiz, Tardieu, G., and Tardieu, T. C.: Study of Tactile Sensation in the Normal Child. Presse Med., 67 (17), 18 April, 1959. 10. Ogden, T. E., Robert, F., and Carmichael, A. E.: Some Sensory Syndromes in Children: Indifference to Pain and Sensory Neuropathy. J. Neurol., Neurosurg. 6 Psychiat., 22:267, 1959. 11. Tachdjian, M. 0., and Minear, W.: Sensory Disturbances in the Hands of Cerebral Palsied Children. J. Bone 6 Joint Surg., 40A:85, 1958. 12. Tizard, J. P. M., Paine, R. S., and Crothers, C.: Disturbances in Sensation in Children with Hemiplegia. J.A.M.A., 155:628, 1954. 13. Twitchell, T. E.: The Prognosis of Motor Recovery in Hemiplegia. Bull. Tufts, N.E. Medical Center, 3:146, 1957. U.C.L.A. Medical Center Los Angeles 24, Calif.
Recently attention has been called to the frequency of peripheral sensory loss in brain-damaged children, especially those having hemiplegia. Management of such children, however, has continued to stress only motor training with the addition of bracing and surgery in case of orthopedic defects. Often, long periods of physical and occupational therapy have failed to develop functional use of the affected hand. In an adult, using multisensory stimulation rather than motor training, Forster has reported improved function in a hemiplegic patient whose deficit was mainly sensory. Whether emphasis on sensory stimulation in the management of the hemiplegic child with both sensory and motor deficits will lead to better functional use of the affected extremities is not known. Certainly, evaluation of both sensory and motor loss at as early an age as possible is important in understanding the child's total problem. The purpose of this article is to present reported data on the types and frequency of peripheral sensory loss in hemiplegic children and to add hitherto unpublished material from our laboratory. Findings will be discussed in relation to the management of these children. A preliminary report on the use of multisensory stimulation in the training of the young child with peripheral sensory loss is included. REPORTS IN THE LITERATURE OF TYPES AND FREQUENCY OF PERIPHERAL SENSORY LOSS
Tizard, Paine and Crothers,12 Tachdjian and Minearl l and Hohman, Baker and Reed 6 reported results of sensory testing in a total of 249 From the Department
of Pediatrics, University of California at Los Angeles.
766
HEMIPLEGIC CHILDREN WITH PERIPHERAL SENSORY LOSS
school-age and older cerebral-palsied children. Most were spastic hemiplegics, some triplegics or quadriplegics, with one side more involved than the other. Partial or complete sensory loss of one or more types ranged from 42 to 72 per cent, being 52 per cent for the group as a whole (Table 29). The most frequently found deficits were stereognosis or form discrimination, two-point discrimination, and passive movement or position sense, in decreasing frequency in that order (Table 30). Though partial or complete loss of light touch, pain, sharp-dull, hotcold, wet-dry, occurred in many children, none of these latter modalities was defective unless one of the three most commonly impaired was also deficient. Tachdjian and Minearl l demonstrated an inverse ratio between the extent of sensory loss and the functional use of the extremity, as noted in Table 31. They found that 87.5 per cent of hemiplegics who had no use of the affected hand had sensory loss. Conversely, patients with normal use of the hand showed no sensory deficit. Tizard, Paine and Crothers12 reported strong correlation between skeletal undergrowth and sensory deficit (Table 32). Of 16 patients TABLE
29. Frequency of Sensory Loss NUMBER OF PATIENTS
SENSORY DEFICIT
Number
106 .... 57 47 .... 34 96 .... 40 Totals 249 .... 131 TABLE
AUTHOR
Per Cent
53.8 72.3 41. 7
Tizard, Paine and Crothers12 Hohman, Baker and Reed 6 Tachdjian and Minearll
52.0
30. Types of Sensory Loss (3 Most Frequent) TYPE
NUMBER
TOTAL
T.C.P.12 H.B.R.o T.M.ll No. Stereognosis or form discrimination ...... 20 2-Point discrimination ...... 14 Passive movement or position sense ...... 15 Total number of patients in series ...... 106 TABLE
28 23
40 31
14
16
r;88T'36 68 28 "fi>\ 45 20
96
47
249
31. Functional Use of the Hand versus Sensory Lossl1 FUNCTIONAL USE OF HAND
%
PER CENT HAVING SENSORY LOSS
None .............. 87.5 Poor .............. 68.9 Fair .............. 30.0 Good.............. 6.6 Normal.............. 0.0
MARGARET H. JONES TABLE
32. Skeletal Undergrowth versus Sensory Deficit12 TOTAL
SENSORY LOSS
HEMIANOPSIA
14 2
14 2
Skeletal undergrowth ....... 16 No skeletal undergrowth ....... 12
TABLE
767
33. Severity of Sensory Deficit in Hemiplegia 7 Least severe .................. Face:
perioral lateral regions Trunk: posterior anterior Leg: upper lower foot Arm: upper lower Most severe ................... Hand
showing skeletal undergrowth, 14 had sensory loss and also hemianopsia. Of 12 not showing skeletal undergrowth, only 2 showed sensory loss and hemianopsia. Laine and Gros 7 discuss the degree of sensory loss in different parts of the affected side in hemiplegics, listing deficits in order of increasing severity (Table 33). Laine and Gros report that the affected arm and leg may show improvement in physical function and in sensory perception after operation in which contralateral frontal, parietal, temporal and occipital lobes are removed en masse (hemispherectomy). They note that voluntary motion returned simultaneously with the return of position sense. The reports presented above were obtained from tests of children of school age. In regard to the discriminatory abilities of normal young children, Haeusserman4 has stated that children of 4Y:! years can differentiate familiar objects by tactile discrimination of form and texture. Monfraiz, Tardieu and Tardieu9 report results with 12 geometric shapes used in the Grace Arthur Board with 218 children, ages 2y:! to 8y:! years. Those under 3y:! failed to identify geometric shapes, but could recognize familiar objects. Children between 3y:! and 6 could be graded by age by frequency of correct responses with the geometric shapes. MATERIALS AND METHODS USED IN OUR STUDY'
Our study was undertaken to assess methods of testing sensory deficits,
* Assistance was given in these studies by Paul Greenberg in the summer of 1956, supported by the Spastic Children's League, and by L. Huhn in the summer of 1957, supported by a fellowship grant from the National Foundation. Some of these tests on adults were administered by occupational therapists at United Cerebral Palsy As· sociation of Los Angeles County, in course of a research project supported by grant from United States Office of Vocational Rehabilitation.
768 TABLE
HEMIPLEGIC CHILDREN WITH PERIPHERAL SENSORY LOSS
34. Sensory Modalities Tested
Exteroceptive ........... Light touch Sharp-dull Pain Hot-cold Wet-dry Proprioceptive ........... Passive movement-position Pressure Vibration Cortico-senaory ........... Rough-smooth Texture Weight-length Size (thickness) Shape (2 dimensions) Form (3 dimensions) (object discrimination) Tickle or scrape (probably a combination of modalities)
particularly cortical-sensory modalities, in the cerebral-palsied child under six years of age. For comparison 27 adults, 13 children from 6 to 12 years and 14 children under 6 years (10 between 3 and 6 and 4 from 19 months to 3 years) were tested. Most were spastic hemiplegics, a few quadriplegic, athetoid or mixed, with one side more involved than the other. Modalities studied were essentially those tested in Hohman's5 series. They are listed in Table 34. For children under 6 years of age a special table with glass insert was developed to allow the tester to view the child's extremities without either blindfolding him or expecting him to keep his eyes closed (Fig. 61). The child was held by an adult or seated in a suitable chair opposite the examiner. In addition to the usual test objects, duplicates of those used for length, size, shape and form discrimination were made (Fig. 62). These were placed on top of the table and the child asked to pick out a matching object, rather than to answer verbally. Often several periods were required to secure satisfactory cooperation with the child between three and four years, but reproducible results were obtained in this group as well as those between four and six years. For the child under three years of age, in our study, the only modalities that were tested satisfactorily were stereognosis-using small familiar objects (ball, key, safety pin, pencil) and checking to see whether the child could name them when presented visually before testing by tactile discrimination; light touch-feather, cotton or small object (penny, dowel or pencil) carefully placed in hand; response to pin prick-speed and type of response and localization on the affected side as compared to the good side. McGraw8 described response to pin pick in a series of 75 normal children, ages a few minutes to 4 years, as outlined in Table 35. She differentiated two major features in the behavior pattern to pin prick,
MARGARET H. JONES MARGARET H. JONES
769 769
Onethe thesensorimotor, sensorimotor,the theother otherthe thecognitive cognitiveaspects. aspects.The Thesensorimotor sensorimotor one was based on the response of application of the pin to the skin,the the was based on the response of application of the pin to the skin, visual stimulus. stimulus.Only Onlythe thesensorimotor sensorimotorwas was cognitive, toto the thepin pinasas aavisual cognitive, considered in our study, for the pin was kept out of sight. considered in our study, for the pin was kept out of sight. Thefirst firstphase phaseofofresponse responsewas wasthe thenewborn newbornorordiffuse diffusephase, phase,which which The posconsisted of diffuse bodily movement accompanied by crying and consisted of diffuse bodily movement accompanied by crying and pos~ sibly local reflex withdrawal. The reaction was immediate. sibly local reflex withdrawal. The reaction was immediate.
Fig.61. 61.Table Tablewith withglass glassinsert insertfor fortesting testingsensory sensorydeficits deficitsininextremities. extremities. Fig.
Fig. 62. 62. Objects Objects used used for for testing testing sensory sensory deficits deficits and and form form discnmination. discIlmination. Fig.
770 TABLE
HEMIPLEGIC CHILDREN WITH PERIPHERAL SENSORY LOSS
35. Response to Pin Prick RESPONSE
AGE OF CHILD
Newborn or diffuse phase ........... Minutes to 1 month Inhibition of diffuse phase (cries only) ........... 1 month to 3 months General J'lcalization (deliberate withdrawal) ........... 6-13 months Specific localization ........... 8 months on
Inhibition of this phase occurred after a month or so, the response being delayed diffuse body movement with less reflex withdrawal. Later, about 6 months of age, the infant started to localize the area stimulated by deliberate withdrawal directed away from the source of irritation, as compared to reflex flexion type of withdrawal seen previously. Localization at this stage was mainly visual. Specific localization began by about 8 months, as indicated by the child's ability to touch or rub the area stimulated. In McGraw's series of 75 normal infants the phase of specific localization had occurred in all before 500 days. The affected side was regularly stimulated before the unaffected. RESULTS OF EVALUATION (54 PATIENTS)
Our findings as to the type and incidence of sensory deficits in hemiplegic children from 6 to 12 years and in adults were similar to those of other investigators, especially Hohman et al.: 6 namely, that about three-fourths had demonstrable deficits, mainly of the cortico-sensory type. One or more of the three following modalities was either lost or less well perceived on the affected side-stereognosis or form discrimination, two-point discrimination, position or passive movement. Comparing the results by age groups, it will be seen that those under six years of age showed approximately the same incidence of demonstrable cortico-sensory loss as did the older children and adults. In the children under three years of age the most frequent deficits demonTABLE
36. Sensory Deficit by Age Group* AGE
NUMBER OF PATIENTS
Over 16 years .............................. 6-12 years .............................. Under 6 years .............................. Total. .............................
27 13 14t 54
SENSORY DEFICIT
No.
%
20 10 9
75 77
39
65 74
* Los Angeles study: stereognosis or form, 2-point discrimination, position or passive mov~ment.
t In 3 of the 14 patients, tests oC this type were unsatisfactory.
MARGARET H. JONES
771
strated were light touch, paSSIve movement, abnormal response to pin prick. Figure 63 indicates a breakdown of the findings into exteroceptive, proprioceptive and cortico-sensory types of sensory loss. Light touch, indicated by the number "I" on the left-hand series of graphs, was the most reproducible test in the very young child and could be applied satisfactorily to all. Without calling attention to the fact that the affected arm might be touched with cotton or a feather, 75 per cent of the group under 6 years of age failed to appreciate the stimulus. In children from 6 to 12, only 58 per cent failed to respond, and in the SENSORY DEFICIT
L.A. STUDY BY AGE GROUP
0/0
80
'~\
60
40
x
20 <6 6-12 >16
<6 6-12 >16
<
6-12 >16
OL---~~~~~~~~~~~~~~~--
EXTEROCEPTIVE PROPRIOCEPTIVE CORTICOSENSORY
Fig. 63. Findings in exteroceptive, proprioceptive and cortico-sensory llPes of sensory
loss.
adult, only 26 per cent. Good response occurred on the other side in all. Similar findings were reported for pain or sharp-dull discrimination, indicated by number "2" on this same series of graphs. Number "3" on these graphs indicates the response to hot-cold stimulation, which was not satisfactory in many of the children under six, but showed no variation between older children and adults. The middle graph indicates the result of tests for perception of passive movement or position sense, and this also appeared to improve with age. On the right hand side of the chart the cortico-sensory loss, as noted by the shaded areas, representing loss of one or more of the fol-
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HEMIPLEGIC CHn.DREN WITH PERIPHERAL SENSORY LOSS
lowing-form, shape, weight, length discrimination and object identification-remained at the same level in all ages. In this graph number "2" represents loss in two-point discrimination, number "3" loss in rough-smooth differentiation. The broken line "4" represents loss of localization to touch. As in Tizard, Paine and Crothers's12 series, no relation was found in our patients between intelligence, degree of atrophy of part, electroencephalographic abnormalities and incidence of sensory loss. We do not have data on length of the extremities. COMMENTS
The findings outlined above support DeJong's2 comment that "sensation and motion are interdependent and that severe motor disabilities may follow impairment of sensory functions." For a discussion of the current concepts of the physiology of motor learning, the reader is referred to Hellebrandt's5 review, which gives various hypotheses on the relation of sensory input to motor function, and Clark'sl lecture on sensory experience and brain structure. From the clinical point of view, Ogden, Robert and Carmichael'sl0 report of children with congenital deficits in sensory perception, but without motor abnormality, who show a delay in sitting, standing and walking, suggests a possible relation of sensory loss to motor development. Forster's3 success in the use of multisensory stimulation in retraining of an adult hemiplegic with a sensory loss due to cardiovascular accident is important in showing that, despite known central nervous system damage, improved sensory perception is possible with training and is followed by improved hand use. Current failures to achieve functional hand use with motor education alone in congenital or early acquired hemiplegics suggest the need of trial of another approach. Evaluation of sensory perception, particularly of the cortico-sensory type, should be attempted even in young children before a therapy program is outlined. Re-evaluation of sensory modalities as the child matures and particularly before any surgical procedure is done is essential. As illustrated in case reports by Tizard, Crothers and Paine,12 tendon transplant may be beneficial in patients with no sensory loss, but may not be helpful in those with sensory loss. These authors also found that the frequency of cortico-sensory loss is as great in the child whose hemiplegia was present from birth as in the patient who acquires the condition later, although the extent of involvement is usually greater in the latter children. Hohman et al. 6 found that "very small areas of the hand might be involved leaving a major portion with good sensory perception." They
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also stress the finding of impainnent of perception, as for example the ability to differentiate two points at % inch on one side and 1% inches on the other side. Sensations might be correctly, but more slowly, identified on the affected side as compared with the unaffected. Again emphasizing the correlation between recovery of sensory perception and development of motor function, is Twitchell'sls study in adults of return of finger reflexes which were deemed proprioceptive, as an index of final functional use of the hemiplegic hand. Without giving specific data, this author also states that "persistent defect of sensation of the affected limbs . . . particularly point localization, position sense, and other cortical discriminating functions is not conducive to good motor recovery." PRELIMINARY RESULTS OF TRAINING INCORPORATING SENSORY STIMULATION
Recently studies have been begun in our laboratory aimed at determining the effect of multisensory stimulation in the young hemiplegic child. We are interested in the effects of such training, both on tested sensory deficit and on spontaneous motor function. In the child under six years of age, sharply contrasting stimuli of temperature, texture, size and shape were presented first to the good hand and then the affected one. This was done in the fonn of a story told to the child, on an individual basis rather than in a group. Initial results are encouraging in that the frequency of spontaneous reach and grasp increased, first on the unaffected and then on the affected side. Graded series of motor perfonnances were also constructed for which rewards were given. The child was shown what was wanted, but was not helped in achieving it. These tasks involve eye-hand coordination, beginning with a simple motion. Progression from simple to more difficult motor tasks was possible even at age 2% years. In the older children, tactile discrimination with reward was set up, first calling attention to differences verbally by asking the child to feel and look at each of two disparate objects and finally to differentiate without vision. Gradually the children appeared to learn clues enough to make at least gross differentiations. In the past the unaffected ann and hand were sometimes immobilized to force use of the affected side. This resulted in stuttering, increased seizures and behavior problems. It is true that in the aboveoutlined training the child is asked to use the affected side, but the other side is only gently restrained and for short periods. The tasks required of the affected side are carefully graded, beginning with simple ones in which success is easily attainable. Thus far, none of the unde. sirable side effects have been noted.
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HEMIPLEGIC CHILDREN WITH PERIPHERAL SENSORY LOSS
Training, using sensory stimulation, needs to be as carefully planned and carried out as does that for motor education. It needs to be done on an individual basis and by trained personnel and, as Forster notes, is time consuming. It needs to be combined with passive stretching if contractures are present. SUMMARY
An essential part of the evaluation of the hemiplegic patient is the sensory examination. Sensory deficit is often associated with poor functional use of the hemiplegic hand. It is possible, with some modifications of usual testing methods, to demonstrate sensory impairment or loss in children under six years of age. Table 37 reports major deficits found in the three age groups. TABLE
37. Maior Sensory Deficits Demonstrated in Hemiplegic Children of Different Ages
Over 6 years .............. Form or object discrimination 2-Point discrimination Position sense-passive movement 3-6 years .............. Identification of small familiar objects by name Match shapes and forms :I: Passive movement Under 3 years .............. Perception of light touch (penny or dowel in hand) Response to pin prick (degree oflocalization) Passive movement
Our findings are similar to those of other authors in respect to frequency of sensory impairment, particularly cortico-sensory deficits (40 to 65 per cent). It is also of interest that deficits in light touch were three times as frequent (75 per cent) in children under 6 years of age as in adults (26 per cent) . Current studies of the effect of multisensory stimulation training on the development of motor function in young hemiplegic children are encouraging. REFERENCES 1. Clark, W. LeGros: The Thirty·Second Maudsley Lecture; Sensory Experience and Brain Structure. J. Ment. Science, 104:434, 1958. 2. DeJong, R. D.: The Neurologic Examination. 2nd Ed. New York, Paul B. Hoeber Inc., 1958. 3. Forster, F. M., and Shields, C.: Cortical Sensory Defects Causing Disability. Arch. Phys. Med. 6 Rehabilitation, 40:56, 1959. 4. Haeusserman, E.: Developmental Potential of Preschool Children. New York, Grune & Stratton, Inc., 1958. 5. Hellebrandt, F. A.: The Physiology of Motor Learning. Cerebral Palsy Review, July-August, 1958.
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6. Hohman, L. B., Baker, L., and Reed, R.: Sensory Disturbance in Children with Infantile Hemiplegia, Triplegia and Quadriplegia. Am. 1. Phys. Med., 37: 1, 1958. 7. Laine, E., and Gros, C.: L'Hemispherectomy. Paris, Masson et Cie, 1956. 8. McGraw, M. B.: The Neuromuscular Maturation of the Human Infant. New York, Columbia University Press, 1943. 9. Monfraiz, Tardieu, G., and Tardieu, T. C.: Study of Tactile Sensation in the Normal Child. Presse Med., 67 (17), 18 April, 1959. 10. Ogden, T. E., Robert, F., and Carmichael, A. E.: Some Sensory Syndromes in Children: Indifference to Pain and Sensory Neuropathy. J. Neurol., Neurosurg. 6 Psychiat., 22:267, 1959. 11. Tachdjian, M. 0., and Minear, W.: Sensory Disturbances in the Hands of Cerebral Palsied Children. J. Bone 6 Joint Surg., 40A:85, 1958. 12. Tizard, J. P. M., Paine, R. S., and Crothers, C.: Disturbances in Sensation in Children with Hemiplegia. J.A.M.A., 155:628, 1954. 13. Twitchell, T. E.: The Prognosis of Motor Recovery in Hemiplegia. Bull. Tufts, N.E. Medical Center, 3:146, 1957. U.C.L.A. Medical Center Los Angeles 24, Calif.