Chapter 10 Spina bifida cystica

1. The family histories of 722 infants who were born with spina bifida cystica were studied. 2. The index cases were referred for surgical treatment and were not selected in any way from the genetic point of view. 3. Intensive inquiries were made to obtain a complete family pedigree, including a prospective follow-up of siblings born after the index case. 4. Of 1,256 siblings 85 or 6.8% had gross malformation of the central nervous system: spina bifida cystica in 54, anencephaly in 22, and uncomplicated hydrocephalus in 9. 5. Of 306 children born after the index case 25 (8%) or 1 in 12 were affected. 6. There was a progressive increase in multiple cases in the family with increasing family size. In sibships of five or more, multiple cases occurred in 24.1%. 7. In 118 families cases of gross malformation of the central nervous system were known to have occurred among members of the family other than siblings. Cases occurred in three generations. 8. It is possible that spina bifida cystica might be a recessively inherited condition.

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Spina bifida cystica in northern Nigeria

Child s Nervous System ◽

10.1007/bf00307931 ◽

1990 ◽

Vol 6 (2) ◽

pp. 103-106 ◽

Cited By ~ 8

Author(s):

O. A. Mabogunje

Keyword(s):

Spina Bifida ◽

Northern Nigeria ◽

Spina Bifida Cystica

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Selection for Treatment in Spina Bifida Cystica

BMJ ◽

10.1136/bmj.4.5886.189 ◽

1973 ◽

Vol 4 (5886) ◽

pp. 189-197 ◽

Cited By ~ 43

Author(s):

G. K. Smith ◽

E. D. Smith

Keyword(s):

Spina Bifida ◽

Spina Bifida Cystica ◽

Selection For

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THE RELATIONSHIP OF NON-PROGRESSIVE HYDROCEPHALUS TO INTELLECTUAL FUNCTIONING IN CHILDREN WITH SPINA BIFIDA CYSTICA

PEDIATRICS ◽

10.1542/peds.37.5.787 ◽

1966 ◽

Vol 37 (5) ◽

pp. 787-793

Author(s):

Angeles Badell-Ribera ◽

Kenneth Shulman ◽

Nancy Paddock

Keyword(s):

Spinal Cord ◽

Spina Bifida ◽

Psychosocial Adjustment ◽

Functional Disability ◽

Psychological Status ◽

Significant Discrepancy ◽

Essentially Normal ◽

Intellectual Capacity ◽

Spina Bifida Cystica ◽

Spinal Cord Dysfunction

Seventy-five patients with variable degrees of spinal cord dysfunction secondary to spina bifida cystica have been studied with special reference to their neurological and psychological status. Their ages ranged from 5 to 21 years. The patients were divided into five different groups on the basis of functional disability secondary to spinal cord dysfunction. Those patients with a history of rapid head enlargement and who had not had neurosurgical treatment for this problem comprised 62% of the population studied (total seventy-five). These patients were considered to have non-progressive hydrocephalus. The hydrocephalic subgroup scored lower in psychological testing and presented a significant discrepancy between verbal and performance scores which could be considered a characteristic sign of brain damage. The psychological test scores of the patient with the same severe paralytic defect but without hydrocephalus were essentially normal. The physical rehabilitation potential of these patients is dependent upon the degree of physical disability, but their over-all rehabilitation achievement is largely determined by their intellectual capacity and psychosocial adjustment. We believe that if we are to improve the long-range prognosis of such children, one of the objectives of neurosurgery should be early and effective treatment of the hydrocephalus.

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