Hostname: page-component-cd9895bd7-jkksz Total loading time: 0 Render date: 2024-12-27T09:39:23.989Z Has data issue: false hasContentIssue false

1990 Richardson Lecture — Challenges for Neurology in the Nineties: Will We Survive?

Published online by Cambridge University Press:  18 September 2015

Rights & Permissions [Opens in a new window]

Extract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

It is a great honor for me to present the Richardson Lecture this year. Although I was not personally acquainted with Dr. Richardson, his contributions to Canadian neurology are legendary, not the least of which is immortalized in the disease he and his colleagues first described in 1964: Steele-Richardson-Olszewski syndrome.

I have entitled my talk “Challenges for Neurology in the 90s: Will We Survive?”, to highlight some of the issues that I believe are important for us to consider as we embark upon our academic enterprise during the last decade of the 20th century. There are a number of opportunities as well as, potentially, major difficulties that we face, and I wish to focus attention on some of them.

Type
Special Feature
Copyright
Copyright © Canadian Neurological Sciences Federation 1991

References

1.Gusella, JF, Tanzi, RE, Anderson, MA, et al. DNA markers for nervous system disease. Science 1984; 225: 13201328.CrossRefGoogle Scholar
2.Martin, JB. Molecular genetic studies in the neuropsychiatric disorders. TINS 1989; 12: 130137.Google ScholarPubMed
3.Friedmann, T. Progress toward human gene therapy. Science 1989; 244: 12751281.CrossRefGoogle ScholarPubMed
4.Gusella, JF, Wexler, NS, Conneally, PM, et al. A polymorphic DNA marker linked to Huntington’s disease. Nature 1983; 306: 234238.Google ScholarPubMed
5.Martin, JB. Huntington’s disease: New approaches to an old problem. Neurology 1984; 34: 10591072.Google Scholar
6.Gilliam, TC, Bucan, M, MacDonald, ME, et al. A DNA segment encoding two genes very tightly linked to Huntington’s disease. Science 1987; 238: 950952.CrossRefGoogle ScholarPubMed
7.Hayden, MR, Hewitt, J, Wasmuth, JJ, et al. AA polymorphic DNA marker that represents a conserved expressed sequence in the region of the Huntington disease gene. Am J Hum Genet 1988; 42: 125131.Google Scholar
8.Wasmuth, JJ, Hewitt, J, Smith, D, et al. A highly polymorphic locus very tightly linked to the Huntington’s disease gene. Nature 1988; 332: 734736.CrossRefGoogle Scholar
9.Robbins, C, Theilmann, J, Youngman, S, et al. AEvidence from family studies that the gene causing Huntington disease is telomeric to D4S95 and D4S90. Am J Hum Genet 1989; 44: 422425.Google Scholar
10.Theilmann, J, Kanani, S, Shiang, R, et al. Non-random association between alleles detected at D4S95 and D4S98 and the Huntington’s disease gene. J Med Genet 1989; 26: 676682.CrossRefGoogle ScholarPubMed
11.Snell, RG, Lazarou, LP, Youngman, S, et al. Linkage disequilibrium in Huntington’s disease: An improved localization for the gene. J Med Genet 1989; 26: 673675.CrossRefGoogle ScholarPubMed
12.Bucan, M, Zimmer, M, Whaley, WL, et al. Physical maps of 4p16.3, the area expected to contain the Huntington disease mutation. Genomics 1990; 6: 115.Google ScholarPubMed
13.Conneally, PM, Haines, JL, Tanzi, RE, et al. Huntington disease: No evidence for locus heterogeneity. Genomics 1989; 5: 304308.CrossRefGoogle ScholarPubMed
14.Martin, JB. Molecular genetics: Applications to the clinical neuro sciences. Science 1987; 238: 765772.CrossRefGoogle Scholar
15.St. George-Hyslop, PH, Tanzi, JL, Tanzi, RE, et al. HThe genetic defect causing familial Alzheimer’s disease maps on chromosome 21. Science 1987; 235: 885887.CrossRefGoogle Scholar
16.Gusella, JF. Location cloning strategy for characterizing genetic defects in Huntington’s disease and Alzheimer’s disease. FASEB J 1989; 3: 20362041.Google ScholarPubMed
17.Goate, AM, Owen, MJ, James, LA, et al. Predisposing locus for Alzheimer’s disease on chromosome 21. Lancet 1989; 1: 352355.Google ScholarPubMed
18.Schellenberg, GD, Bird, TD, Wijsman, EM, et al. Absence of linkage of chromosome 21q21 markers to familial Alzheimer’s disease. Science 1988; 241: 1507–10.Google ScholarPubMed
19.Pericak-Vance, MA, Bebout, JL, Haynes, CAet al. ALinkage studies in familial Alzheimer’s disease: evidence for chromosome 19 linkage. Am J Hum Gen 1990; 47: A194.Google Scholar
20.St. George-Hyslop, PH, Haines, JL, Farrer, LAet al. Genetic linkage studies suggest that Alzheimer’s disease is not a single homogeneous disorder. Nature 1990; 347(6289): 194197.CrossRefGoogle Scholar
21.Abraham, CR, Potter, H. Alzheimer’s disease: Recent advances in understanding the brain amyloid deposits. Biotechnology 1989; 7: 147153.Google Scholar
22.Selkoe, DJ. Deciphering Alzheimer’s disease: The amyloid precursor protein yields new clues. Science 1990; 248: 1058–60.CrossRefGoogle ScholarPubMed
23.Whitson, JS, Selkoe, DJ, Cotman, CW. myloid beta protein enhances the survival of hippocampal neurons in vitro. Science 1989; 243: 1488–90.CrossRefGoogle Scholar
24.Yankner, BA, Dawes, LR, Fisher, S,et al. Neurotoxicity of a fragment of the amyloid precursor associated with Alzheimer’s disease. Science 1989: 245: 417420.CrossRefGoogle ScholarPubMed
25.Van Nostrand, WE, Wagner, SL, Suzuki, M,et al. Protease nexin-Il, a potent antichymotrypsin, shows identity to amyloid β-protein precursor. Nature 1989: 341: 546549.CrossRefGoogle ScholarPubMed
26.Van Broeckhoven, C, Haan, J, Bakker, E,et al. Amyloid β-protein precursor gene and hereditary cerebral hemorrhage with amyloidosis (Dutch). Science 1990: 248: 11201123.Google ScholarPubMed
27.Levy, E, Carman, MD, Fernandez-Madrid, U,et al. AMutation of the Alzheimer’s disease amyloid gene in hereditary cerebral hemorrhage, Dutch type. Science 1990: 248: 11241127.CrossRefGoogle Scholar
28.Prusiner, SB. Scrapie prion. Ann Rev Microbiol 1989; 43: 345374.CrossRefGoogle Scholar
29.Cohen, DH, Fiener, H, Jensson, O, Frangione, B. Amyloid fibril in hereditary cerebral hemorrhage with amyloidosis (HCHW A) is related to the gastro-enteropancreatic neuroendocrine protein, gamma trace. J Exp Med 1983: 158: 623628.Google Scholar
30.Klein, G. The approaching era of the tumor suppressor genes. Science 1987; 238: 15391545.Google ScholarPubMed
31.Cavenee, WK,et al. Expression of recessive alleles by chromosomal mechanisms in retinoblastoma. Nature 1983; 305: 779784.CrossRefGoogle ScholarPubMed
32.Friend, SH, Dryja, TP, Weinberg, RA. Oncogenes and tumor-suppressing genes. New Engl J Med 1988; 318: 618622.Google ScholarPubMed
33.Huang, HJ, Yee, YK, Shew, JY, et al. Suppression of the neoplastic phenotype by replacement of the RB gene in human cancer cells. Science 1988; 242: 15631566.CrossRefGoogle ScholarPubMed
34.Hong, FD, Huang, HJ, et al. Structure of the human retinoblastoma gene. Proc Natl Acad of Sci of USA 1989; 86: 55025506.CrossRefGoogle ScholarPubMed
35.Seizinger, BR, Martuza, RL, Gusella, JF. Loss of genes on chromosome 22 in tumor genesis of acoustic neuroma. Nature 1986; 322: 644647.Google Scholar
36.Seizinger, BR, Rouleau, GA, Ozelius, LJ. Common pathogenetic mechanism for three tumor types in bilateral acoustic neurofibro-matosis. Science 1987; 236(4799): 317319.Google Scholar
37.Seizinger, BR, de la Monte, S, Atkins, L,et al. Molecular genetic approach to human meningioma: Loss of genes on chromosome 22. Proc Natl Acad Sci (USA) 1987; 84: 54195423.CrossRefGoogle ScholarPubMed
38.Seizinger, BR, Rouleau, GA, Ozelius, LJ, et al. von Hippel-Lindau disease maps to the region of chromosome 3 associated with renal cell carcinoma. Nature 1988; 332: 268269.CrossRefGoogle Scholar
39.Freymann, JG. he public’s health care paradigm is shifting: Medicine must swing with it. Gen Int Med 1989; 4: 313319.CrossRefGoogle Scholar
40.Bloom, A. The closing of the American mind: How higher education has failed democracy and impoverished the souls of today’s stu-dents.New York: Simon and Schuster, 1987.Google Scholar
41.Bishop, JM. Chauncey Leake Lecture. UCSF. May 1990.Google Scholar
42. Editorial UCSF. 1989; 246: 1541.CrossRefGoogle Scholar
43.Chalfie, M, Wolinsky, E. The identification and suppression of inher-ited neurodegeneration in caenorhabditis elegans. Nature. 1990; 345: 410416.Google ScholarPubMed
44.Jan, LY, Jan, YN. A superfamily of ion channels (letter). Nature. 1990; 345(6277): 672.CrossRefGoogle Scholar
45.Meissen, GJ, Meyers, RH, Mastromauro, CA, et al. A Predictive testing for Huntington’s disease with use of a linked DNA marker. N Engl J Med. 1988; 318: 535542.Google ScholarPubMed
46.Huggins, M, Block, M, Karani, S, et al. Ethical and legal dilemmas arising during predictive testing for adult onset disease: The expe-rience of Huntington’s disease. Am J Hum Gen. 1990; 47: 412.Google Scholar