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Infantile Huntington’s Disease

Published online by Cambridge University Press:  18 September 2015

R.H.A. Haslam*
Affiliation:
Departments of Pediatrics, Clinical Neurosciences, Pathology and Radiology, Faculty of Medicine, University of Calgary, Calgary, Alberta
B. Curry
Affiliation:
Departments of Pediatrics, Clinical Neurosciences, Pathology and Radiology, Faculty of Medicine, University of Calgary, Calgary, Alberta
R. Johns
Affiliation:
Departments of Pediatrics, Clinical Neurosciences, Pathology and Radiology, Faculty of Medicine, University of Calgary, Calgary, Alberta
*
Alberta Children’s Hospital, 1820 Richmond Road S.W., Calgary, Alberta, Canada T2T 5C7.
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Summary:

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A unique case of Huntington’s disease is reported because of the extremely early onset and death, and the atypical mode of presentation including severe behavioural problems and a negative family history. Although rare, Huntington’s disease must be considered along with the established degenerative disorders of white and gray matter peculiar to the pediatric population when one examines an infant or child with progressive motor deterioration, rigidity, mental retardation and behavioural abnormalities. Computed tomography is a reliable and non-invasive method of establishing the diagnosis during life.

Type
Original Articles
Copyright
Copyright © Canadian Neurological Sciences Federation 1983

References

Bell, J. (1934). Huntington’s chorea. In: Treasury of Human Inheritance. Vol. 4. Nervous Diseases and Muscular Dystrophies, (Bell, J. ed), p. 1, Cambridge University Press, London.Google Scholar
Byers, R.K. and Dodge, J.A. (1967). Huntington’s chorea in children: report of four cases. Neurology 17:587596.CrossRefGoogle ScholarPubMed
Byers, R.K., Gilles, F.H. and Fung, C. (1973). Huntington’s disease in children; neuropathologic study of four cases. Neurology 23:561569.CrossRefGoogle ScholarPubMed
Hansotia, P., Cleeland, C.S. and Chun, R.W.M. (1968). Juvenile Huntington’s chorea. Neurology 18:217224.CrossRefGoogle ScholarPubMed
Jervis, G.A. (1963). Huntington’s chorea in childhood. Arch. Neurol. 9:244257.CrossRefGoogle ScholarPubMed
Johns, R. and Haslam, R.H.A. (1982). CT measurements in 8 neurologically normal children. Unpublished data.Google Scholar
Markham, C.H. and Knox, J.W. (1965). Observations on Huntington’s chorea in childhood. J. Pediatr. 67:4657.CrossRefGoogle ScholarPubMed
McMenemey, W.H. (1969). Huntington’s disease. In: Greenfield’s Neuropathology2nd ed. (Blackwood, W., McMenemey, W.H., Meyer, A., Norman, R.M. and Russell, D.S. eds.) pp. 553558, Edward Arnold (Publishers) Ltd., London.Google Scholar
Shokeir, M.H. (1975). Investigations on Huntington’s disease in the Canadian Prairies, Clin. Genet. 7(4):345348.Google ScholarPubMed
Terrence, C.F., Delaney, J.F. and Alberts, M.C. (1976). Computed tomography for Huntington’s disease. Neuroradiology 13:173175.CrossRefGoogle Scholar