Major central nervous system malformations notified in Northern Ireland, 1969 to 1973. 1976

J H Elwood

In Northern Ireland, during the years 1969 to 1973, there were 485 notifications of anencephalus, 525 of spina bifida and 328 of hydrocephalus in a population of 157,725 (live and still) births. The data were based on the voluntary notification system for congenital malformations which has been operating in Northern Ireland and in England and Wales sine 1964. The malformation rates per 1000 (live and still) births were 3-1 for anencephalus, 3-3 for spina bifida and 2-1 for hydrocephalus. Secular trends in these data indicate a decrease in the incidence of all three major central nervous system malformations, but this is significant only in relation to anencephalus. Spina bifida was more common in female births than in male births, and 79 per cent of affected infants were liveborn. Indirect evidence suggests that some 40 per cent of the total number of live and stillborn infants having spina bifida at birth will survive to one year of age.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D009660 Northern Ireland The northern one-sixth of the island of Ireland - between the North Atlantic Ocean and the North Sea; northwest of France. The capital is Belfast.
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006849 Hydrocephalus Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, INTRACRANIAL HYPERTENSION; HEADACHE; lethargy; URINARY INCONTINENCE; and ATAXIA. Communicating Hydrocephalus,Congenital Hydrocephalus,Obstructive Hydrocephalus,Post-Traumatic Hydrocephalus,Aqueductal Stenosis,Cerebral Ventriculomegaly,Fetal Cerebral Ventriculomegaly,Hydrocephalus Ex-Vacuo,Hydrocephaly,Aqueductal Stenoses,Cerebral Ventriculomegalies,Cerebral Ventriculomegalies, Fetal,Cerebral Ventriculomegaly, Fetal,Fetal Cerebral Ventriculomegalies,Hydrocephalus Ex Vacuo,Hydrocephalus Ex-Vacuos,Hydrocephalus, Communicating,Hydrocephalus, Congenital,Hydrocephalus, Obstructive,Hydrocephalus, Post-Traumatic,Post Traumatic Hydrocephalus,Stenoses, Aqueductal,Stenosis, Aqueductal,Ventriculomegalies, Cerebral,Ventriculomegalies, Fetal Cerebral,Ventriculomegaly, Cerebral,Ventriculomegaly, Fetal Cerebral
D000757 Anencephaly A malformation of the nervous system caused by failure of the anterior neuropore to close. Infants are born with intact spinal cords, cerebellums, and brainstems, but lack formation of neural structures above this level. The skull is only partially formed but the eyes are usually normal. This condition may be associated with folate deficiency. Affected infants are only capable of primitive (brain stem) reflexes and usually do not survive for more than two weeks. (From Menkes, Textbook of Child Neurology, 5th ed, p247) Aprosencephaly,Absence of Brain, Congenital,Anencephalia,Anencephalus,Congenital Absence of Brain,Hemicranial Anencephaly,Incomplete Anencephaly,Partial Anencephaly,Anencephalias,Anencephalies, Partial,Anencephaly, Hemicranial,Anencephaly, Incomplete,Anencephaly, Partial,Aprosencephalies,Brain Congenital Absence,Partial Anencephalies
D012621 Seasons Divisions of the year according to some regularly recurrent phenomena usually astronomical or climatic. (From McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed) Seasonal Variation,Season,Seasonal Variations,Variation, Seasonal,Variations, Seasonal
D012737 Sex Factors Maleness or femaleness as a constituent element or influence contributing to the production of a result. It may be applicable to the cause or effect of a circumstance. It is used with human or animal concepts but should be differentiated from SEX CHARACTERISTICS, anatomical or physiological manifestations of sex, and from SEX DISTRIBUTION, the number of males and females in given circumstances. Factor, Sex,Factors, Sex,Sex Factor
D016135 Spinal Dysraphism Congenital defects of closure of one or more vertebral arches, which may be associated with malformations of the spinal cord, nerve roots, congenital fibrous bands, lipomas, and congenital cysts. These malformations range from mild (e.g., SPINA BIFIDA OCCULTA) to severe, including rachischisis where there is complete failure of neural tube and spinal cord fusion, resulting in exposure of the spinal cord at the surface. Spinal dysraphism includes all forms of spina bifida. The open form is called SPINA BIFIDA CYSTICA and the closed form is SPINA BIFIDA OCCULTA. (From Joynt, Clinical Neurology, 1992, Ch55, p34) Rachischisis,Spina Bifida,Status Dysraphicus,Cleft Spine,Open Spine,Schistorrhachis,Spinal Dysraphia,Bifida, Spina,Cleft Spines,Dysraphia, Spinal,Dysraphicus, Status,Dysraphism, Spinal,Dysraphisms, Spinal,Open Spines,Rachischises,Spina Bifidas,Spinal Dysraphias,Spinal Dysraphisms,Spine, Cleft,Spine, Open

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