Journal of Pediatric Ophthalmology and Strabismus

Cerebral Palsy and Mental Retardation : Ocular Findings

L Landau, MD; D Berson, MD

Abstract

The term cerebral palsy designates a group of nonprogressive disorders which result from malfunction of the motor centers and pathways of the brain, and whose origin may be perinatal, before the central nervous system has reached relative maturity.' Cerebral palsy is characterized by paralysis, weakness, incoordination, or other aberrations of motor function, with or without mental retardation. This study presents the ocular findings in a group of children suffering from cerebral palsy, relates them to the grade of mental retardation, and emphasizes the frequency of ocular findings in families with children suffering from cerebral palsy and mental retardation.

Material and Methods

Seventy-two children, aged 8 to 16 years, with cerebral palsy and mental retardation, hospitalized in Kfar Shwedi (Institute for Retarded Children, Jerusalem) were examined; 29 were female and 43 male. All the children underwent an eye examination including visual acuity (when possible), eye movements, slitlamp, and fundoscopy.

Results

Intelligence quotients (I.Q.) of the children were as follows: (I) I.Q. of 1-25: 14 children; (2) I.Q. of 26-35: 24 children; (3) I.Q. of 3655: 33 children; and (4) I.Q. of 56-70: 1 child.

As in other observations,2"4 squint was the most common finding (Table I). Twenty-six of the children (37.5 per cent) showed squint. The ophthalmoscopic findings in our group of children are also noted in Table I. Five children showed bilateral macular degeneration. In three of them, retinitis pigmentosa was also present. One of the three showed a macular picture of Spiel mayer- Vogt degeneration and had a blind and mentally retarded brother. The other two had a tapetoretinal degeneration. One of these had two blind and mentally retarded brothers. All three children were blind. Two other children had obvious macular changes and myopia. The occurrence of cataract is impressive. Among the 72 children examined, 13 children from seven families had siblings with cerebral palsy. Parents were first cousins in three of the seven families. Our examinations did not include all the siblings of these 13 children. It is of interest that in some families all the children presented ophthalmoscopic changes, while in others none did (Table II).

Discussion

Guibor examined 142 children and was impressed by the fact that motor defects of the eyes occurred in 75 per cent of them.2 There is no indication in his work of the children's I.Q., but some of them were able to undergo pleoptic and orthoptic treatment. Breakey examined 100 unselected children with cerebral palsy who were under ambulant care in a hospital clinic.3 Schacht et al 4 examined 98 children who were from special public schools and who had an I.Q. of 70 or better upon serial psychometric testing.

Table

Our group from Kfar Shwedi was somewhat different. The children were hospitalized for care and treatment of severe physical and mental retardation (I.Q. below 55).

In comparing our ocular findings to those of Guibor, Breakey, and Schacht, it is rioted that macular degeneration appeared only once in the Guibor series and not at all in the two others, while in our series, five children (6.9 per cent) presented this finding. Retinitis pigmentosa is not mentioned at all by them, while in our group three children (4.1 per cent) suffered from this disease. Our single child with coloboma of the optic nerve points to a disturbance in primitive epithelial papilla s during embryonal development. Other findings, such as optic atrophy and cataract were found in our group as well as in the previously mentioned works, but the percentage in our work is higher (Table I).

There are little data in the literature on the relationship between cerebral palsy and mental retardation.…

The term cerebral palsy designates a group of nonprogressive disorders which result from malfunction of the motor centers and pathways of the brain, and whose origin may be perinatal, before the central nervous system has reached relative maturity.' Cerebral palsy is characterized by paralysis, weakness, incoordination, or other aberrations of motor function, with or without mental retardation. This study presents the ocular findings in a group of children suffering from cerebral palsy, relates them to the grade of mental retardation, and emphasizes the frequency of ocular findings in families with children suffering from cerebral palsy and mental retardation.

Material and Methods

Seventy-two children, aged 8 to 16 years, with cerebral palsy and mental retardation, hospitalized in Kfar Shwedi (Institute for Retarded Children, Jerusalem) were examined; 29 were female and 43 male. All the children underwent an eye examination including visual acuity (when possible), eye movements, slitlamp, and fundoscopy.

Results

Intelligence quotients (I.Q.) of the children were as follows: (I) I.Q. of 1-25: 14 children; (2) I.Q. of 26-35: 24 children; (3) I.Q. of 3655: 33 children; and (4) I.Q. of 56-70: 1 child.

As in other observations,2"4 squint was the most common finding (Table I). Twenty-six of the children (37.5 per cent) showed squint. The ophthalmoscopic findings in our group of children are also noted in Table I. Five children showed bilateral macular degeneration. In three of them, retinitis pigmentosa was also present. One of the three showed a macular picture of Spiel mayer- Vogt degeneration and had a blind and mentally retarded brother. The other two had a tapetoretinal degeneration. One of these had two blind and mentally retarded brothers. All three children were blind. Two other children had obvious macular changes and myopia. The occurrence of cataract is impressive. Among the 72 children examined, 13 children from seven families had siblings with cerebral palsy. Parents were first cousins in three of the seven families. Our examinations did not include all the siblings of these 13 children. It is of interest that in some families all the children presented ophthalmoscopic changes, while in others none did (Table II).

Discussion

Guibor examined 142 children and was impressed by the fact that motor defects of the eyes occurred in 75 per cent of them.2 There is no indication in his work of the children's I.Q., but some of them were able to undergo pleoptic and orthoptic treatment. Breakey examined 100 unselected children with cerebral palsy who were under ambulant care in a hospital clinic.3 Schacht et al 4 examined 98 children who were from special public schools and who had an I.Q. of 70 or better upon serial psychometric testing.

Table

TABLE IOCULAR FINDINGS WITH CEREBRAL PALSY

TABLE I

OCULAR FINDINGS WITH CEREBRAL PALSY

Table

TABLE IIFAMILIES WITH MORE THAN ONE CHILD WITH CEREBRAL PALSY

TABLE II

FAMILIES WITH MORE THAN ONE CHILD WITH CEREBRAL PALSY

Table

TABLE IIIOCULAR FINDINGS IN RELATIONSHIPTO I.Q

TABLE III

OCULAR FINDINGS IN RELATIONSHIPTO I.Q

Our group from Kfar Shwedi was somewhat different. The children were hospitalized for care and treatment of severe physical and mental retardation (I.Q. below 55).

In comparing our ocular findings to those of Guibor, Breakey, and Schacht, it is rioted that macular degeneration appeared only once in the Guibor series and not at all in the two others, while in our series, five children (6.9 per cent) presented this finding. Retinitis pigmentosa is not mentioned at all by them, while in our group three children (4.1 per cent) suffered from this disease. Our single child with coloboma of the optic nerve points to a disturbance in primitive epithelial papilla s during embryonal development. Other findings, such as optic atrophy and cataract were found in our group as well as in the previously mentioned works, but the percentage in our work is higher (Table I).

There are little data in the literature on the relationship between cerebral palsy and mental retardation. Asher in a survey of 400 cases of cerebral palsy in childhood, found 160 children with an I.Q. below 70, 79 of whom had an I.Q. below 50 (19.7 per cent).6 One of her conclusions was: "The mean I.Q. of the various groups varied inversely with the severity of the physical handicap." In our group 14 children had an I.Q. below 25 (19.4 per cent). Six of these had ocular disease, and of these four were totally blind and one nearly so. Fifty-seven children had an I.Q. between 26 and 55 (79 per cent); six of these had ocular findings. The relationship between the gravity of cerebral palsy, mental retardation, and seriousness of the eye involvement is obvious (Table III). It is possible to add to the conclusion of Asher that the more severe the physical handicap and the lower the I.Q., the more severe the eye findings.

Ocular Findings in Children from Families with More than One Child Suffering from Cerebral Palsy and Mental Retardation

Cooper7 reviewed the family history of a large series of patients who had been seen in a clinic in New York. His findings suggested that familial factors were of greater importance in cerebral palsy than had previously been considered. Regarding this disease, Ford has written: "Morbid inheritance is, undoubtedly, responsible in some cases, for studies by Book and Hanhart, as well as many others, have revealed that spastic diplegia with mental retardation is sometimes inherited as a simple recessive characteristic and several cases in identical twins have been described."8

The literature concerning ocular findings in familial cerebral palsy is limited. Hotting and Passweg described cerebral palsy and tapetoretinal degeneration in four children out of seven in one family.* Porencephaly, optic nerve atrophy, and nystagmus were found in two other families. Blumel et al described a combination of congenital cataract and cerebral palsy in a brother and sister.10 They concluded that the striking similarity of the characteristic findings and generally strong resemblance of the children suggest a recessive autosomal type of inheritance. In another article discussing the same children, the authors reached the conclusion that, since both parents were phenotypically normal, a transmutation of the x-chromosome in the mother must be considered a possible mechanism responsible for the condition of the children." From our findings (Table II), we were impressed with the frequency of familial cases, although no conclusions could be drawn.

Treatment

The eye treatment of children suffering from cerebral palsy and mental retardation poses some problems. According to Uba, people whose I.Q. is below 25 cannot care for their own needs and achieve a maximum mental age of about three years." The 25 to 55 I.Q. range includes persons who can learn simple tasks. Individuals with an I.Q. between 55 and 70 can learn to care for themselves and may be able to have an occupation. Their final mental age is in the 8-to-12 year range.

In our children with severe mental retardation conservative treatment of squint is impossible. In such cases only operation for cataract and optical correction can be attempted.

Summary

1. Among 72 children suffering from cerebral palsy and mental retardation, 53 ocular findings were noted including 27 not connected with strabismus.

2. Ocular findings in cerebral palsy, excluding strabismus, are more frequent as the mental retardation is more severe.

3. The number of families with more than one child suffering from cerebral palsy and mental retardation is impressive.

4. The possibilities of treatment of ocular findings are limited.

Acknowledgment: We are grateful to Dr. Henriette Finkler from Kfar Shwedi Institute for retarded children in Jerusalem for her kind help.

References

1. Nelson-Vaughan-McKay: Textbook of Pediatrics. Philadelphia, WB Saunders, 1969, p 1311.

2. Guibor GP: Some eye defects seen in cerebral palsy with some statistics. Am J Phys Med 32:342-347, 1953.

3. Breakey AS: Ocular findings in cerebral palsy. AMA Arch Ophtholmol 53:852, 1 955.

4. Schacht W, Wallace H, Palmer M et al: Ophthalmologic findings in children with cerebral palsy. Pediatrics 19:623 (April Pt 1), 1957.

5. Mann Ida: Developmental Abnormalities of the Eye. 1957 p 113.

6. Asher P, Schoneil FE: A survey of 400 cases of cerebral palsy in childhood. Arch Dis Child, 25:360, 1950.

7. Cooper W: Familial incidence of cerebral palsy. Dev Med Child Neurol 10:250, 1968.

8. Ford FR: Diseases of the Nervous System in Infancy, Childhood and Adolescence. 1966, p 16.

9. Hottinger A, Passweg E: Über familiares Auftreten cerebraler spasticher Lähmungen in Kombination mit Augenveränderungen. Schweiz Med Wschr 87:601, 1957.

10. Blumel J, Evans EB, Eggers GWN: A combination of congenital cataract and cerebral palsy in a brother and a sister. Arch Ophthalmol 63:246-253, 1960.

11. Blumel J, Ohnuki Y, Awa A: Chromosome anomaly in two cases of cerebral palsy, a brother and a sister. Nature (Lond) 189:154, 1961.

12. Uba A: Mental Retardation Abstracts. 2:1180, 1969.

TABLE I

OCULAR FINDINGS WITH CEREBRAL PALSY

TABLE II

FAMILIES WITH MORE THAN ONE CHILD WITH CEREBRAL PALSY

TABLE III

OCULAR FINDINGS IN RELATIONSHIPTO I.Q

10.3928/0191-3913-19711101-08

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