Brain, Vol 120, Issue 8 1485-1508, Copyright © 1997 by Oxford University Press
HW Moser
The occasion of the presentation of the eighth Gordon Holmes Lecture left
me feeling both honoured and awed, as a result of my review of the Selected
Papers of Gordon Holmes (Phillips CG: Selected Papers of Gordon Holmes,
compiled and edited for the Guarantors of Brain. Oxford University Press,
1979), kindly presented to me by the sponsors of the meeting. This volume
lists 174 publications produced over a 55-year period, and contains
reprints of contributions to neuroanatomy, neuropathology, and to disorders
that affected the adrenal cortex, the spinal cord, the cerebellum and the
cerebral cortex. Yet I also feel a sense of sadness; the invitation to
present the lecture came from the late Anita Harding who, such a short time
before her illness, gave me personal guidance and encouragement. In this
lecture I endeavour to follow the example of Gordon Holmes, namely the
stepwise analysis of a clinical problem, first by observation of the
patient, followed by the application of techniques that can clarify it,
leading to new knowledge not only about the specific disorder, but also
about the nervous system and human biology in general and, it is to be
hoped, to more effective therapy.
REVIEWS
Adrenoleukodystrophy: phenotype, genetics, pathogenesis and therapy
Kennedy Krieger Institute, Baltimore, MD 21205, USA.
![]()
CiteULike
Connotea
Del.icio.us What's this?
This article has been cited by other articles:
![]() |
W. Furushima, M. Inagaki, A. Gunji, Y. Inoue, M. Kaga, and S. Mizutani Early Signs of Visual Perception and Evoked Potentials in Radiologically Asymptomatic Boys With X-linked Adrenoleukodystrophy J Child Neurol, August 1, 2009; 24(8): 927 - 935. [Abstract] [PDF] |
||||
![]() |
P. Brites, P. A. W. Mooyer, L. el Mrabet, H. R. Waterham, and R. J. A. Wanders Plasmalogens participate in very-long-chain fatty acid-induced pathology Brain, February 1, 2009; 132(2): 482 - 492. [Abstract] [Full Text] [PDF] |
||||
![]() |
S. Hein, P. Schonfeld, S. Kahlert, and G. Reiser Toxic effects of X-linked adrenoleukodystrophy-associated, very long chain fatty acids on glial cells and neurons from rat hippocampus in culture Hum. Mol. Genet., June 15, 2008; 17(12): 1750 - 1761. [Abstract] [Full Text] [PDF] |
||||
![]() |
V. A. Drover, D. V. Nguyen, C. C. Bastie, Y. F. Darlington, N. A. Abumrad, J. E. Pessin, E. London, D. Sahoo, and M. C. Phillips CD36 Mediates Both Cellular Uptake of Very Long Chain Fatty Acids and Their Intestinal Absorption in Mice J. Biol. Chem., May 9, 2008; 283(19): 13108 - 13115. [Abstract] [Full Text] [PDF] |
||||
![]() |
S H Wong, M Boggild, T P Enevoldson, and N A Fletcher Myelopathy but normal MRI: where next? Practical Neurology, April 1, 2008; 8(2): 90 - 102. [Abstract] [Full Text] [PDF] |
||||
![]() |
S. Marino, M. De Luca, M. T. Dotti, M. L. Stromillo, P. Formichi, P. Galluzzi, M. Mondelli, P. Bramanti, A. Federico, and N. De Stefano Prominent brain axonal damage and functional reorganization in "pure" adrenomyeloneuropathy Neurology, September 18, 2007; 69(12): 1261 - 1269. [Abstract] [Full Text] [PDF] |
||||
![]() |
H. H. Jung, I. Wimplinger, S. Jung, K. Landau, A. Gal, and F. L. Heppner Phenotypes of female adrenoleukodystrophy Neurology, March 20, 2007; 68(12): 960 - 961. [Full Text] [PDF] |
||||
![]() |
C. Lucke, D. L. Gantz, E. Klimtchuk, and J. A. Hamilton Interactions between fatty acids and {alpha}-synuclein J. Lipid Res., August 1, 2006; 47(8): 1714 - 1724. [Abstract] [Full Text] [PDF] |
||||
![]() |
J. D. Kingsley, M. Varman, A. Chatterjee, R. A. Kingsley, and K. S. Roth Immunizations for Patients With Metabolic Disorders Pediatrics, August 1, 2006; 118(2): e460 - e470. [Abstract] [Full Text] [PDF] |
||||
![]() |
How good at neurology are you? -- Answers Practical Neurology, June 1, 2006; 6(3): 201 - 201. [Full Text] [PDF] |
||||
![]() |
S. Mukherjee, E. Newby, and J. N Harvey Adrenomyeloneuropathy in patients with ;Addison's disease': genetic case analysis. J R Soc Med, May 1, 2006; 99(5): 245 - 249. [Abstract] [Full Text] [PDF] |
||||
![]() |
M. Linnebank, S. Kemp, R.J.A. Wanders, W. J. Kleijer, M. L.T. van der Sterre, J. Gartner, K. Fliessbach, A. Semmler, P. Sokolowski, W. Kohler, et al. Methionine metabolism and phenotypic variability in X-linked adrenoleukodystrophy Neurology, February 14, 2006; 66(3): 442 - 443. [Abstract] [Full Text] [PDF] |
||||
![]() |
S. Suda, Y. Komaba, T. Kumagai, M. Yamazaki, T. Katsumata, T. Kamiya, and Y. Katayama Progression of the olivopontocerebellar form of adrenoleukodystrophy as shown by MRI Neurology, January 10, 2006; 66(1): 144 - 145. [Full Text] [PDF] |
||||
![]() |
J. A. Hodapp, G. T. Carter, H. P. Lipe, S. J. Michelson, G. H. Kraft, and T. D. Bird Double Trouble in Hereditary Neuropathy: Concomitant Mutations in the PMP-22 Gene and Another Gene Produce Novel Phenotypes Arch Neurol, January 1, 2006; 63(1): 112 - 117. [Abstract] [Full Text] [PDF] |
||||
![]() |
J. H. Kim and H. J. Kim Childhood X-linked Adrenoleukodystrophy: Clinical-Pathologic Overview and MR Imaging Manifestations at Initial Evaluation and Follow-up RadioGraphics, May 1, 2005; 25(3): 619 - 631. [Abstract] [Full Text] [PDF] |
||||
![]() |
C. Peters, L. R. Charnas, Y. Tan, R. S. Ziegler, E. G. Shapiro, T. DeFor, S. S. Grewal, P. J. Orchard, S. L. Abel, A. I. Goldman, et al. Cerebral X-linked adrenoleukodystrophy: the international hematopoietic cell transplantation experience from 1982 to 1999 Blood, August 1, 2004; 104(3): 881 - 888. [Abstract] [Full Text] [PDF] |
||||
![]() |
A. Falorni, S. Laureti, A. De Bellis, R. Zanchetta, C. Tiberti, G. Arnaldi, V. Bini, P. Beck-Peccoz, A. Bizzarro, F. Dotta, et al. Italian Addison Network Study: Update of Diagnostic Criteria for the Etiological Classification of Primary Adrenal Insufficiency J. Clin. Endocrinol. Metab., April 1, 2004; 89(4): 1598 - 1604. [Abstract] [Full Text] [PDF] |
||||
![]() |
M. Dittmar and G. J. Kahaly Polyglandular Autoimmune Syndromes: Immunogenetics and Long-Term Follow-Up J. Clin. Endocrinol. Metab., July 1, 2003; 88(7): 2983 - 2992. [Abstract] [Full Text] [PDF] |
||||
![]() |
T. M. Burns, M. M. Ryan, B. Darras, and H. R. Jones Jr Current Therapeutic Strategies for Patients With Polyneuropathies Secondary to Inherited Metabolic Disorders Mayo Clin. Proc., July 1, 2003; 78(7): 858 - 868. [Abstract] [PDF] |
||||
![]() |
T Hitomi, T Mezaki, T Tsujii, M Kinoshita, H Tomimoto, A Ikeda, S Shimohama, T Okazaki, T Uchiyama, and H Shibasaki Improvement of central motor conduction after bone marrow transplantation in adrenoleukodystrophy J. Neurol. Neurosurg. Psychiatry, March 1, 2003; 74(3): 373 - 375. [Abstract] [Full Text] [PDF] |
||||
![]() |
C. Betterle, C. Dal Pra, F. Mantero, and R. Zanchetta Autoimmune Adrenal Insufficiency and Autoimmune Polyendocrine Syndromes: Autoantibodies, Autoantigens, and Their Applicability in Diagnosis and Disease Prediction Endocr. Rev., June 1, 2002; 23(3): 327 - 364. [Abstract] [Full Text] [PDF] |
||||
![]() |
J.-E. Cheon, I.-O. Kim, Y. S. Hwang, K. J. Kim, K.-C. Wang, B.-K. Cho, J. G. Chi, C. J. Kim, W. S. Kim, and K. M. Yeon Leukodystrophy in Children: A Pictorial Review of MR Imaging Features RadioGraphics, May 1, 2002; 22(3): 461 - 476. [Abstract] [Full Text] [PDF] |
||||
![]() |
G. N. O'Neill, M. Aoki, and R. H. Brown Jr. ABCD1 translation-initiator mutation demonstrates genotype-phenotype correlation for AMN Neurology, December 11, 2001; 57(11): 1956 - 1962. [Abstract] [Full Text] [PDF] |
||||
![]() |
M. Rantamaki, R. Krahe, A. Paetau, B. Cormand, I. Mononen, and B. Udd Adult-onset autosomal recessive ataxia with thalamic lesions in a Finnish family Neurology, September 25, 2001; 57(6): 1043 - 1049. [Abstract] [Full Text] [PDF] |
||||
![]() |
S. Ten, M. New, and N. Maclaren Addison's Disease 2001 J. Clin. Endocrinol. Metab., July 1, 2001; 86(7): 2909 - 2922. [Abstract] [Full Text] [PDF] |
||||
![]() |
R. Ito, E. R. Melhem, S. Mori, F. S. Eichler, G. V. Raymond, and H. W. Moser Diffusion tensor brain MR imaging in X-linked cerebral adrenoleukodystrophy Neurology, February 27, 2001; 56(4): 544 - 547. [Abstract] [Full Text] [PDF] |
||||
![]() |
G. Unterrainer, B. Molzer, S. Forss-Petter, and J. Berger Co-expression of mutated and normal adrenoleukodystrophy protein reduces protein function: implications for gene therapy of X-linked adrenoleukodystrophy Hum. Mol. Genet., November 1, 2000; 9(18): 2609 - 2616. [Abstract] [Full Text] [PDF] |
||||
![]() |
R G F Gray, M A Preece, S H Green, W Whitehouse, J Winer, and A Green Inborn errors of metabolism as a cause of neurological disease in adults: an approach to investigation J. Neurol. Neurosurg. Psychiatry, July 1, 2000; 69(1): 5 - 12. [Full Text] [PDF] |
||||
![]() |
E. R. Melhem, D. J. Loes, C. S. Georgiades, G. V. Raymond, and H. W. Moser X-linked Adrenoleukodystrophy: The Role of Contrast-enhanced MR Imaging in Predicting Disease Progression AJNR Am. J. Neuroradiol., May 1, 2000; 21(5): 839 - 844. [Abstract] [Full Text] |
||||
![]() |
D. Riva, S. M. Bova, and M. G. Bruzzone Neuropsychological testing may predict early progression of asymptomatic adrenoleukodystrophy Neurology, April 25, 2000; 54(8): 1651 - 1656. [Abstract] [Full Text] [PDF] |
||||
![]() |
G. Karpati and R. Hohlfeld Biologically stressed muscle fibers in sporadic IBM: A clue for the enigmatic etiology? Neurology, March 14, 2000; 54(5): 1020 - 1021. [Full Text] [PDF] |
||||
![]() |
D S FARRELL, M C LAWDEN, and N MESSIOS Radiologically selective visual pathway involvement in adult onset cerebral adrenoleukodystrophy J. Neurol. Neurosurg. Psychiatry, February 1, 2000; 68(2): 249a - 251. [Full Text] |
||||
![]() |
L. X. Liu, K. Janvier, V. Berteaux-Lecellier, N. Cartier, R. Benarous, and P. Aubourg Homo- and Heterodimerization of Peroxisomal ATP-binding Cassette Half-transporters J. Biol. Chem., November 12, 1999; 274(46): 32738 - 32743. [Abstract] [Full Text] [PDF] |
||||
![]() |
H W MOSER Treatment of X-linked adrenoleukodystrophy with Lorenzo's oil J. Neurol. Neurosurg. Psychiatry, September 1, 1999; 67(3): 279 - 280. [Full Text] |
||||
![]() |
H. W. Moser, S. Kemp, and K. D. Smith Mutational Analysis and the Pathogenesis of Variant X-linked Adrenoleukodystrophy Phenotypes Arch Neurol, March 1, 1999; 56(3): 273 - 275. [Full Text] [PDF] |
||||
![]() |
H. Takano, R. Koike, O. Onodera, R. Sasaki, and S. Tsuji Mutational Analysis and Genotype-Phenotype Correlation of 29 Unrelated Japanese Patients With X-linked Adrenoleukodystrophy Arch Neurol, March 1, 1999; 56(3): 295 - 300. [Abstract] [Full Text] [PDF] |
||||
![]() |
D. Restuccia, V. D. Lazzaro, M. Valeriani, A. Oliviero, D. L. Pera, C. Barba, M. Cappa, E. Bertini, M. D. Capua, and P. Tonali Neurophysiologic follow-up of long-term dietary treatment in adult-onset adrenoleukodystrophy Neurology, March 1, 1999; 52(4): 810 - 810. [Abstract] [Full Text] [PDF] |
||||
![]() |
T. Yamada, T. Taniwaki, N. Shinnoh, A. Uchiyama, N. Shimozawa, Y. Ohyagi, H. Asahara, and J. Kira Adrenoleukodystrophy protein enhances association of very long-chain acyl-coenzyme A synthetase with the peroxisome Neurology, February 1, 1999; 52(3): 614 - 614. [Abstract] [Full Text] [PDF] |
||||
![]() |
M. Wong, A. Shaibani, and B. C. P. Lee Transient hydrocephalus as a presenting sign associated with adrenoleukodystrophy Neurology, January 1, 1999; 52(2): 424 - 424. [Full Text] [PDF] |
||||
















