Absence of β-Amyloid Immunoreactivity in Mesial Temporal Lobe in Cockayne's Syndrome

Abstract

Cockayne's syndrome is associated with dementia and other physical signs of premature senescence. Death usually occurs in the first or second decade of life. Because previous neuropathologic descriptions have included neurofibrillary tangles and calcific and dystrophic cerebrovascular changes, we examined the mesial temporal lobes of three children with Cockayne's syndrome (confirmed by 254-nm ultraviolet light studies). Immunohistochemistry was used to determine if β-amyloid immunoreactivity was present in the parenchyma or cerebral blood vessels. Tissues from the mesial temporal lobe of patients with Alzheimer's disease and Down syndrome were used as controls. None of the three temporal lobes from patients with Cockayne's syndrome contained β-amyloid immunoreactive material in either the parenchyma or vessels; all of the Alzheimer's disease and Down syndrome controls had β-amyloid immunoreactivity. (J Child Neurol 1991;6:32-34).

Get full access to this article

View all access options for this article.

References

Brown WT : Premature aging syndromes. Curr Probl Dermatol 1987;17:152-165.

Brumback RA , Yoder FW , Andrews AD , et al: Normal pressure hydrocephalus: Recognition and relationship to neurological abnormalities in Cockayne's syndrome. Arch Neurol 1978;35:337- 345.

Schmickel RD , Chu EH , Trosko JE , et al: Cockayne's syndrome: A cellular sensitivity to ultraviolet light. Pediatrics 1977;60:135-139.

Otsuka F. , Tarone RE , Cayeux S. , et al: Use of lymphoblastoid cell lines to evaluate hypersensitivity to ultraviolet radiation in Cockayne's syndrome. J Invest Dermatol 1984;83:480-484.

Soffer D. , Grotsky HW , Rapin I. , et al: Cockayne's syndrome: Unusual neuropathological findings and a review of the literature. Ann Neurol 1979 ;6:340-348.

Masters C. : Neuropathological findings of aging, in Terry RD (ed): Aging and the Brain, vol 32. New York, Raven Press, 1988, pp 51-58.

Crome L. , Kanjilal GC : Cockayne's syndrome: Case report. J Neurol Neurosurg Psychiatry 1971;34:171-178.

Houston CS , Zaleski WA , Rozdilsky B. : Identical male twins and brother with Cockayne's syndrome. Am J Med Genet 1982;13:211-223.

Leech RW , Brumback RA , Miller RH , et al: Cockayne's syndrome: Clinicopathologic and tissue culture studies of affected siblings. J Neuropathol Exp Neurol 1985;44:507-519.

10.

Patton MA , Giannelli F. , Francis AJ , et al: Early onset Cockayne's syndrome: Case reports with neuropathological and fibroblast studies. J Med Genet 1989;26:154-159.

11.

Sternberger L. : Immunohistochemistry. New York, John Wiley, 1986, pp 90-209.

12.

Mann DM : The neuropathology of Alzheimer's disease: A review with pathogenetic, aetiological and theoretical considerations. Mech Ageing Dev 1985;31:213-255.

13.

Glenner GG : On causation theories in Alzheimer's disease. Hum Pathol 1985;16:433-435.

14.

Glenner GG , Wong CW : Alzheimer's disease and Down's syndrome. Sharing of a unique cerebrovascular amyloid fibril protein. Biochem Biophys Res Commun 1984;122:1131-1135.

15.

Masters CL , Simms G. , Weinmon DA , et al: Amyloid core protein in Alzheimer's disease and Down's syndrome . Proc Natl Acad Sci 1985;82:4245-4249.

16.

Woody RC , Griffin WST , Stanley LS , Roberts G. : Beta amyloid immunoreactivity in fetal, neonatal, infant and adult Down's syndrome hippocampus, abstract. Ann Neurol 1989;26:485.