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Hypomelanosis of Ito. Neurological Complications in 34 Cases

Published online by Cambridge University Press:  18 September 2015

Ignacio Pascual-Castroviejo ,
Luisa López-Rodriguez ,
María de la Cruz Medina ,
Cipriano Salamanca-Maesso  and
Carmen Roche Herrero
Ignacio Pascual-Castroviejo*
Affiliation:
Paediatric Neurology Service, Hospital Infantil «La Paz», and La Universidad Autónoma, Madrid, Spain
Luisa López-Rodriguez
Affiliation:
Paediatric Neurology Service, Hospital Infantil «La Paz», and La Universidad Autónoma, Madrid, Spain
María de la Cruz Medina
Affiliation:
Paediatric Neurology Service, Hospital Infantil «La Paz», and La Universidad Autónoma, Madrid, Spain
Cipriano Salamanca-Maesso
Affiliation:
Paediatric Neurology Service, Hospital Infantil «La Paz», and La Universidad Autónoma, Madrid, Spain
Carmen Roche Herrero
Affiliation:
Paediatric Neurology Service, Hospital Infantil «La Paz», and La Universidad Autónoma, Madrid, Spain
*
Servicio de Neurología Infantil, Hospital de la S.S. «La Paz», Paseo de la Castellana 261, 28046 Madrid, Spain
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Abstract:

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We studied 34 Spanish children with hypomelanosis of Ito. This disease has an incidence of 1 per 1000 new patients consulting a paediatric neurological service, or 1 per 8000-10,000 unselected patients in a children's hospital. About 94% of our patients show noncutaneous abnormalities. Mental retardation (IQ below 70) was present in 64.7%; another 14.7% had an IQ between 70 and 90, usually associated with poor school performance. Four children exhibited autistic behaviour. Seizures of various types were present in 53% of cases. Other skin alterations in addition to the typical hypomelanosis were observed in 38% of our cases: caf6-au-lait spots, angiomatous nevi, nevus marmorata, nevus of Ota, Mongolian blue spot, heterochromia of the iris or hair, and other nonspecific pigmentations. Other associated disorders occur inconsistently and include macrocephaly, microcephaly, h6mihypertrophy, kyphoscoliosis, coarse facial features, genital anomalies, inguinal hernia, congenital heart disease, hypertelorism, and abnormalities of the teeth, feet and eyes. Autosomal dominant inheritance is demonstrated in some but not all cases.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 15 , Issue 2 , May 1988 , pp. 124 - 129
Copyright
Copyright © Canadian Neurological Sciences Federation 1988

References

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