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X-Linked Hydrocephalus

Published online by Cambridge University Press:  18 September 2015

Ruben I. Kuzniecky
Affiliation:
Departments of Neurology and Pathology, Montreal Children's Hospital, McGill University
Gordon V. Watters*
Affiliation:
Departments of Neurology and Pathology, Montreal Children's Hospital, McGill University
Lise Watters
Affiliation:
Departments of Neurology and Pathology, Montreal Children's Hospital, McGill University
Kathleen Meagher-Villemure
Affiliation:
Departments of Neurology and Pathology, Montreal Children's Hospital, McGill University
*
Department of Neurology, Montreal Children's Hospital, 2300 Tupper Street, Montreal, Quebec, Canada H3H 1P3
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Abstract:

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Two French-Canadian families with seven cases of hydrocephalus in two generations are presented. The pattern of inheritance is consistent with an X-linked recessive transmission. The clinical and pathologic characteristics of this entity are reviewed. The anomaly of adducted thumbs was present in one patient and its cause is considered. The hypothesis of primary hydrocephalus and secondary compression of the aqueduct as the mechanism for aqueductal stenosis is discussed.

Type
Original Articles
Copyright
Copyright © Canadian Neurological Sciences Federation 1986

References

1.Shannon, MH, Nadler, HL. X-Linked hydrocephalus. J Med Genet, 1968; 5: 326328.CrossRefGoogle ScholarPubMed
2.Ricciardi, VM, Marcus, ES. Congenital hydrocephalus and cerebellar agenesis. Clin Genet, 1978; 13: 433447.Google Scholar
3.McMillan, P, Williams, B. Aqueductal stenosis: case review and discussion. J Neur, Neurosurg, Psych. 1977; 40: 521532.CrossRefGoogle ScholarPubMed
4.Bickers, DS, Adams, RD. Hereditary stenosis of the aqueduct of sylvius as a cause of congenital hydrocephalus. Brain, 1949: 72: 246262.CrossRefGoogle ScholarPubMed
5.Edwards, JH. The syndrome of x-linked hydrocephalus. Arch Dis Child. 1961; 36: 486493.CrossRefGoogle Scholar
6.Jansen, J.Sex linked hydrocephalus. Devel Med and Child Neurology. 1975; 17: 633640.CrossRefGoogle ScholarPubMed
7.Weckesser, E, Reed, S, Heiple, K. Congenital clasped thumbs — a syndrome, not a specific entity. Journal of Bone and Joint Surgery. 1968; 50A: 1417.CrossRefGoogle Scholar
8.Yakovlev, P. Paraplegias of hydrocephalics. American Journal of Mental deficiency. 1947; 51: 561.Google ScholarPubMed
9.Landrieu, P, Ninane, J, Ferriere, Get al. Aqueductal stenosis in x-linked hydrocephalus: a secondary phenomen? Devel Med and Child Neurology, 1979; 21: 637641.CrossRefGoogle Scholar
10.Sovik, O, Van Der Hagen, C. Loken, A. X-Linked aqueductal stenosis. Clinical Genetics, 1977; 11: 416420.Google Scholar
11.Borit, A, Sidman, RL. New mutant mouse with communicating hydrocephalus and secondary aqueductal stenosis. Acta Neuro-pathol, 1972; 21: 316331.Google ScholarPubMed
12.Raimondi, AJ, Clark, SJ, McLone, DG. Pathogenesis of aqueductal occlusion in congenital murine hydrocephalus. J Neurosurgery, 1976; 45: 6667.CrossRefGoogle ScholarPubMed
13.Williams, B. Is aqueductal stenosis a result of hydrocephalus? Brain 1977, 96: 399412.CrossRefGoogle Scholar
14.Burton, B. Recurrence risks for congenital hydrocephalus. Clinical Genetics, 1979; 16: 4753.CrossRefGoogle ScholarPubMed
15.Hobbins, JC, Grannum, PA. Ultrasound in the diagnosis of congenital anomalies. Am J Obstet Gynecol 1979; 134: 331.CrossRefGoogle ScholarPubMed
16.Lorber, J. The family history of simple congenital hydrocephalus; An epidemiologist study based on 270 probands. Z. Kinderchir 1984, Supplement 11, 9495.Google Scholar