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Annals of Neurology Vol 50 July December 2001 Official Journal of the American Neurological Association and the Child Neurology Society Editor Editorial Board Richard T. Johnson, MD The Johns Hopkins University Yves Agid, MD, PhD, Paris Baltimore Rose-Mary Boustany, MD, Durham David R. Cornblath, MD, Baltimore Founding Editor, 1977-1984 Stefano Di Donato, MD, Milan Fred Plum, MD David J. Fink, MD, Pittsburgh Marc Fisher, MD, Worcester Editor, 1985-1992 Kirk A. Frey, MD, PhD, Ann Arbor Arthur K. Asbury, MD Christopher Gomez, MD, Minneapolis Scott T. Grafton, MD, Hanover, NH Editor, 1993-1997 James C. Grotta, MD, Houston Robert A. Fishman, MD Mark Hallett, MD, Bethesda Stephen L. Hauser, MD, San Francisco Associate Editors Bradley T. Hyman, MD, Boston Ralph W. Kuncl, MD, PhD George Karpati, MD, Montreal Baltimore Bruce R. Korf; MD, PhD, Boston Allan I. Levey, MD, PhD, Atlanta David Zee, MD Henry F. McFarland, MD, Bethesda Baltimore Solomon Moshé, MD, New York eter C. OBrien, PhD, Rochester, MN Associate Editor for Alan K. Percy, MD, Birmingham Child Neurology Ronald C. Petersen, MD, PhD, Michael V. Johnston, MD Rochester, MN Baltimore Louis J. Ptaéek II, MD, Salt Lake City Faye S. Silverstein, MD, Ann Arbor Managing Editor Douglass M. Turnbull, MD, Newcastle Sheila Rose Garrity, MS Upon Tyne Ronald J. Tusa, MD, PhD, Atlanta Editorial Assistant Andrea Greise Annals of Neurology is indexed in Index Medicus, Current Contents/Clinical Medicine, Excerpta Medica, Biological Publishing Staff Abstracts, Psychological Abstracts; and CINAHL. Shawn Morton Associate Publisher, Medical Sciences Address for subscription information, orders, or changes of address: John Wiley ¢ ” ustomer Service, 605 Third Avenue, New York, 1 212) 850-6000. Jennifer Nowak nt vet y i write to Caroline Senior Production Editor thaug, Subscrip ibution, John Wiley ~ Sons, In York, NY 10158-0012. James G. Pattis, Cunningham Associates Annual subscription rates: | $200.00 indivi $340.00 in tt $89.00 sp t Advertising Representative $200.00 in $340.00 *: $269.00 ind $469.00 instit Annals of Neurology (ISSN 0364-5134) is published r $39.00 (add $5.00 monthly for the can Neurological Associ Subscript Volumes 51 and $370.00 in l Liss, Inc. ¢ 2001 Wiley-Liss, Iné yutside North A reserved. No part of this publication may be reproduced in any form or by any means, except as permitted under section 107 or 108 of the 1976 United States Copyright Act, uc and print without either the prior written permission of th bliss t, and $284.00 authorization through the Copyright Clearance ( er, 2LZ element, ment must be made Rosewood Drive, Danvers, MA 01923, (978) 750-8400, fax us for undelivered 978) 750-4470. Requests to the publisher for permission ue has been should be addressed to the Permissions L Jepartment, John Wiley & Sons, Inc., 605 Third Avenue, New York, NY 10158-0012, 850-6011, fax (212) 850-6008, E-mail t S. and worldwide to Annals of Neurology [email protected]. Periodicals postage paid at New York, hn Wiley & Sons, NY, and at additional mailing offices NY 10158-0012. WILEY-LISS NO 1 JULY 2001 73 Potassium Channel Antibodies in Two Patients with Editorial Reversible Limbic Encephalitis Annals 25th Anniversary Camilla Buckley, MD, Joel Oger, MD, Fred Plum Linda Clover, BSc, Erdem Tiiziin, MD, Katherine Carpenter, Dip Psych, Matthew Jackson, MD, and Angela Vincent, FRCPath Notice Brain Magnetic Resonance Imaging in 23 Patients with Ad Hoc Reviewers Mucopolysaccharidoses and the Effect of Bone Marrow Transplantation Toshiyuki Seto, MD, Kinuko Kono, MD, Kyoko Morimoto, MD, Yuichi Inoue, MD, Neurological Progress Haruo Shintaku, MD, Hideji Hattori, MD, Neuroimmunophilins: Novel Neuroprotective and Osamu Matsuoka, MD, Neuroregenerative Targets Tsunekazu Yamano, MD, Xin Guo, PhD, James F. Dillman III, PhD, and Akemi Tanaka, MD Valina L. Dawson, PhD, and Ted M. Dawson, MD, PhD Microarray Analysis of Hippocampal Gene Expression in Global Cerebral Ischemia Kunlin Jin, MD, PhD, Xiao Ou Mao, MD, Original Articles Mark W. Eshoo, PhD, Tetsuya Nagayama, MD, Small Fibers Involvement in Friedreich’s Ataxia Manabu Minami, MD, Roger P. Simon, MD, Maria Nolano, MD, Vincenzo Provitera, MD, and David A. Greenberg, MD, PhD Claudio Crisci, MD, Anna Maria Saltalamacchia, TN, Gwen Wendelschafer-Crabb, MS, William Robert Kennedy, MD, Alessandro Filla, MD, Lucio Santoro, MD, and Giuseppe Caruso, MD Sodium Channel Inactivation in an Animal Model of Brief Communications Acute Quadriplegic Myopathy Progressive Mitochondrial Disease Resulting from a Mark M. Rich, MD, PhD, and Martin J. Pinter, PhD Novel Missense Mutation in the Mitochondrial DNA Subclinical Dopaminergic Dysfunction in Asymptomatic ND3 Gene Parkinson’s Disease Patients’ Relatives with a Decreased Robert W. Taylor, PhD, Rajinder Singh-Kler, PhD, Sense of Smell Christine M. Hayes, BSc, Philip E.M. Smith, FRCP, Henk W. Berendse, MD, PhD, Jan Booij, MD, PhD, and Douglass M. Turnbull, FRCP Chantal M. J. E. Francot, MSc, Paul L. M. Bergmans, MSc, Late-Onset Metachromatic Leukodystrophy Clinically Ron Hijman, PhD, Johannes C. Stoof; PhD, Presenting as Isolated Peripheral Neuropathy: and Erik Ch. Wolters, MD, PhD Compound Heterozygosity for the IVS2+1G—A Diagnosis of X-Linked Myotubular Myopathy by Mutation and a Newly Identified Missense Mutation Detection of Myotubularin (Thr408lle) in a Spanish Family Jocelyn Laporte, PhD, Wolfram Kress, MD, PhD, Manuel Comabella, MD, John S. Waye, PhD, and Jean-Louis Mandel, MD, PhD Nuria Raguer, MD, Barry Eng, BSc, Carmen Dominguez, PhD, Carmen Navarro, MD, PMP22 Transgenic Dorsal Root Ganglia Cultures Show Cecilia Borras, PhD, William Krivit, MD, PhD, Myelin Abnormalities Similar to Those of Human and Xavier Montalban, MD CMTIA Lucilla Nobbio, PhD, Gianluigi Mancardi, MD, Dichloroacetate Exerts Therapeutic Effects in Marina Grandis, MD, Giovanni Levi, PhD, Transgenic Mouse Models of Ueli Suter, PhD, Klaus-Armin Nave, PhD, Huntington’s Disease Anthony J. Windebank, MD, Michele Abbruzzese, MD, Ole A. Andreassen, MD, Robert J. Ferrante, PhD, and Angelo Schenone, MD Hsueh-Meei Huang, PhD, Alpaslan Dedeoglu, PhD, Larry Park, PhD, Kimberly L. Ferrante, BA, Prospective Study of Caffeine Consumption and Risk of Jennifer Kwon, BS, David R. Borchelt, PhD, Parkinson’s Disease in Men and Women ChristopherA . Ross, MD, PhD, Alberto Ascherio, MD, DrPH, Shumin M. Zhang, MD, ScD, Gary E. Gibson, PhD, and M. Flint Beal, MD Miguel A. Hernan, MD, DrPH, Ichiro Kawachi, MD, PhD, Graham A. Colditz, MD, DrPH, Frank E. Speizer, MD, Familial Frontotemporal Dementia and Parkinsonism and Walter C. Willett, MD, DrPH with a Novel Mutation at an Intron 10+11-Splice Site in the tau Gene Mature, Long-lived CD4* and CD8* T Cells Are Katsuichi Miyamoto, MD, PhD, Generated by the Thymoma in Myasthenia Gravis Anna Kowalska, PhD, Masato Hasegawa, PhD, Camilla Buckley, MRCP, Daniel Douek, PhD, Takeshi Tabira, MD, PhD, Keikichi Takahashi, PhD, John Newsom-Davis, FRS, Angela Vincent, FRCPath, Wataru Araki, MD, PhD, Ichiro Akiguchi, MD, PhD, and Nicholas Willcox, PhD and Akito Ikemoto, MD, PhD Contents of Volume 50, July-December 2001 Special Report Letters 121 Recommended Diagnostic Criteria Increased Hair Polyamine Levels in Patients with for Multiple Sclerosis: Guidelines from the Alzheimer’s Disease International Panel on the Diagnosis Man Ho Choi, MSc, Kyoung-Rae Kim, PhD, of Multiple Sclerosis In Seong Kim, MD, Dong Seok Lho, PhD, W. Ian McDonald, FRCP, and Bong Chul Chung, PhD Alistair Compston, FRCP, Gilles Edan, MD, Hiccups Start, Seizures Cease Donald Goodkin, Hans-Peter Hartung, MD, Akio Ikeda, MD, PhD, Keiko Matsuoka, MD, Fred D. Lublin, MD, Henry F. McFarland, MD, Yoshihumi Nakaya, MD, and Hiroshi Shibasaki, MD, PhD Donald W. Paty, MD, Chris H. Polman, MD, Stephen C. Reingold, PhD, Er ratum Magnhild Sandberg-Wollheim, MD, Reply William Sibley, MD, Alan Thompson, MD, Christophe Tzourio, MD, PhD Stanley van den Noort, MD, Brian Y. Weinshenker, MD, Regular Departments and Jerry S. Wolinsky, MD Books NO 2 AUGUST 2001 Original Articles 163 Presumed Pre- or Perinatal Arterial Ischemic Stroke: bi iat : “— ’ Risk Factors and Outcomes Pos e wn Seana sabre bes ogi Meredith R. Golomb, MD, Daune L. MacGregor, MD, oe ee — Trish Domi, Derek C. Armstrong, MD, Tanja Taivassalo, PhD, Eric A. Shoubridge, PhD, Brian » erase MD, Supriya Mayank, MD, Jacqueline Chen, M Eng, Nancy G. Kennaway, DPhil, and Gabrielle A. deVeber, MD Salvatore DiMauro, MD, Douglas L. Arnold, MD, ’ : ; anil Ronald G. Haller, MD Multiple Sclerosis: Altered Glutamate Homeostasis in Lesions Correlates with Oligodendrocyte and Axonal Metabolic Enzyme Expression in Dopaminergic Damage Neurons in Parkinson’s Disease: An In Situ Peter Werner, PhD, David Pitt, MD, and Cedric S. Raine, PhD Hybridization Study Ann E. Kingsbury, Mark Cooper, PhD, Blinded Positron Emission Tomography Study of Anthony H. V. Schapira, DSc, MD, FRCP, Dopamine Cell Implantation for Parkinson’s Disease and Oliver J. F. Foster, PhD, FRCP Toshitaka Nakamura, MD, Vijay Dhawan, PhD, Thomas Chaly, PhD, Masafumi Fukuda, MD, Large-Scale, Multicenter Study of Cerebrospinal Fluid Yilong Ma, PhD, Robert Breeze, MD, Paul Greene, MD, Tau Protein Phosphorylated at Serine 199 for the Stanley Fahn, MD, Curt Freed, MD, Antemortem Diagnosis of Alzheimer’s Disease and David Eidelberg, MD Nobuo Itoh, MD, Hiroyuki Arai, MD, PhD, Katsuya Urakami, MD, Koichi Ishiguro, PhD, Brain-Derived Neurotrophic Factor Enhances Fast Hideto Ohno, PhD, Harald Hampel, MD, Excitatory Synaptic Transmission in Human Epileptic Katharina Buerger, MD, Jens Wiltfang, MD, Dentate Gyrus Markus Otto, MD, Hans Kretzschmar, MD, Wei Jian Zhu, MD, PhD, and Steven N. Roper, MD Hans-Juergen Moeller, MD, Masaki Imagawa, MD, eeee eS eS a eee e Hideki Kohno, PhD, Kenji Nakashima, MD, Randomized Controlled Trial of Intravenous Shigeki Kuzuhara, MD, Hidetada Sasaki, MD, PhD, Immunoglobulin Versus Oral Prednisolone in Chronic cel Ri tte POD Inflammatory Demyelinating Polyradiculoneuropathy — - —__—_—_- ——— Richard Hughes, MD, FMedSci, Sian Bensa, BSc, Increased Frequency of the Tumor Necrosis Factor-c- Hugh Willison, PhD, Peter Van den Bergh, MD, PhD, 308 A Allele in Adults with Human Immunodeficiency Giancarlo Comi, PhD, Isabel Illa) MD, PhD, Virus Dementia Eduardo Nobile-Orazio, PhD, Pieter van Doorn, PhD, Michael W. Quasney, MD, PhD, Qing Zhang, BS, Marinos Dalakas, MD, Martin Bojar, CSc, Susie Sargent, MD, Margaret Mynatt, BS, Anthony Swan, PhD, and the Inflammatory Neuropathy Jonathan Glass, MD, and Justin McArthur, MD Cause and Treatment (INCAT) Group Contents of Volume 50, July-December 2001 _ iii B-Enolase Deficiency, a New Metabolic Myopathy of Sporadic Creutzfeldt-Jakob Disease in a Young Dutch Distal Glycolysis Valine Homozygote: Atypical Molecular Phenotype Giacomo P. Comi, MD, Francesco Fortunato, BS, Mark W. Head, PhD, Gerrit Tissingh, MD, Sabrina Lucchiari, PhD, Andreina Bordoni, BS, Bernard M. J. Uitdehaag, MD, PhD, Alessandro Prelle, MD, Stefano Jann, MD, Frederik Barkhof, MD, PhD, Tristan J. R. Bunn, MSc, Angélica Keller, PhD, Patrizia Ciscato, BS, James W. Ironside, FRCP, Wouter Kamphorst, MD, PhD, Sara Galbiati, MD, Luca Chiveri, MD, Yvan Torrente, MD, and Philip Scheltens, MD, PhD Guglielmo Scarlato, MD, and Nereo Bresolin, MD Frameshift Mutation in the Collagen VI Gene Causes Imaging Appearance of the Symptomatic Perforating Ullrich’s Disease Artery in Patients with Lacunar Infarction: Occlusion Itsuro Higuchi, MD, Tadafumi Shiraishi, MD, or Other Vascular Pathology? Teruto Hashiguchi, MD, Masahito Suehara, MD, Joanna M. Wardlaw, FRCR, FRCP, MD, Takahito Niiyama, MD, Masanori Nakagawa, MD, Martin S. Dennis, FRCP, MD, Kimiyoshi Arimura, MD, Ikuro Maruyama, MD, Charles P. Warlow, FRCP, MD, and Mitsuhiro Osame, MD and Peter A. Sandercock, FRCP, MD Proton Magnetic Resonance Spectroscopy of Brain Chromogranins as Markers of Altered Hippocampal Metabolites in Galactosemia Circuitry in Temporal Lobe Epilepsy Zhiyue J. Wang, PhD, Gerard T. Berry, MD, Susanne Pirker, MD, Thomas Czech, MD, Steffi F. Dreha, MD, Huaging Zhao, MA, Christoph Baumgartner, MD, Hans Maier, MD, Stanton Segal, MD, and Robert A. Zimmerman, MD Klaus Novak, MD, Sabine Fiirtinger, MSc, Reiner Fischer-Colbrie, PhD, and Giinther Sperk, PhD Adult Neuronal Ceroid Lipofuscinosis with Palmitoyl- Protein Thioesterase Deficiency: First Adult-Onset Increased Incidence of Sporadic Creutzfeldt-Jakob Patients of a Childhood Disease Disease on the Island of Crete Associated with a High Orto P. van Diggelen, PhD, Stephane Thobois, MD, Rate of PRNP 129-Methionine Homozygosity in the Caroline Tilikete, MD, Marie-Thérése Zabot, PhD, Local Population Joke L. M. Keulemans, BS, Patrick A. van Bunderen, BS, Andreas Plaitakis, MD, Anna K. Viskadouraki, MD, Peter E. M. Taschner, PhD, Monique Losekoot, PhD, Minas Tzagournissakis, MD, Ioannis Zaganas, MD, and Yakov V. Voznyi, PhD Susan Verghese-Nikolakaki, PhD, Vasilis Karagiorgis, Toannis Panagiotides, MD, Constantine Kilindireas, MD, Eustratios Patsouris, MD, Christine Haberler, MD, Letters Herbert Budka, MD, Survival in a Transgenic Model of FALS Is Independent and Theodoros Sklaviadis, PhD of iNOS Expression Marjatta Son, PhD, Hassan M. Fathallah-Shaykh, MD, Clinical and Genetic Analysis of Four Mexican Families and Jeffrey L. Elliott, MD With Spinocerebellar Ataxia Type 10 Astrid Rasmussen, MD, Tohru Matsuura, MD, Herpes Simplex Virus Type 1-Induced Acute Retinal Luis Ruano, MD, Petra Yescas, MSc, Adriana Ochoa, BA, Necrosis Tetsuo Ashizawa, MD, and Elisa Alonso, MD Kei Numazaki, MD, PhD, and Norihiko Itoh, DVM Reorganization of Human Motor Cortex After Hand Reply Replantation Georges M. G. M. Verjans, PhD, Simone Réricht, MD, Jochen Machetanz, MD, and Albert D. M. E. Osterhaus, DVM, PhD Kerstin Irlbacher, MD, Ludwig Niehaus, MD, Edgar Biemer, MD, and Bernd-Ulrich Meyer, MD Interleukin-12p40 Genotype Plays a Role in the Susceptibility to Multiple Sclerosis Brief Communications Tineke van Veen, MSc, J. Bart A. Crusius, MSc, Hans M. Schrijver, MD, Gerd Bouma, MD, PhD, Nonprogressive Autosomal Recessive Ataxia Maps to Joep Killestein, MD, Lisa van Winsen, MD, Chromosome 9q34-9qter in a Large Consanguineous A. Salvador Pena, MD, PhD, Lebanese Family Chris H. Polman, MD, PhD, Valérie Delague, PhD, Corinne Bareil, PhD, and Bernard M.]. Uitdehaag, MD, PhD Patrice Bouvagnet, MD, PhD, Nabiha Salem, MA, Eliane Chouery, MA, Jacques Loiselet, MD, PhD, Polymorphism of the Interleukin-1 Gene Complex in André Mégarbané, MD, PhD, Localization-Related Epilepsy and Mireille Claustres, MD, PhD Jukka Peltola, MD, Tapani Keriinen, MD, PhD, Sirpa Rainesalo, MD, and Mikko Hurme, MD, PhD Levodopa Induces Dyskinesias in Normal Squirrel Monkeys Daniel M. Togasaki, MD, PhD, Louis Tan, MRCP, Peter Protell, BA, Donato A. Di Monte, MD, Regular Departments Maryka Quik, PhD, and J. William Langston, MD Books iv Contents of Volume 50, July-December 2001 NO 3 SEPTEMBER 2001 358 Alzheimer’s Disease versus Dementia with Lewy Bodies: Editorials Cerebral Metabolic Distinction with Autopsy Chronic Inflammatory Demyelinating Confirmation Polyradiculoneuropathy Satoshi Minoshima, MD, PhD, Norman L. Foster, MD, Richard A. C. Hughes, MD, FRCP, FMedSci Anders A. F. Sima, MD, PhD, Kirk A. Frey, MD, PhD, Roger L. Albin, MD, and David E. Kuhl, MD Parkin and Parkinson’s: More than Homonymy? Olga Corti, PhD, and Alexis Brice, MD Topiramate Blocks Perinatal Hypoxia-Induced Seizures in Rat Pups Sookyong Koh, MD, PhD, and Frances E. Jensen, MD Original Articles PO Protein Is a Target Antigen in Chronic A Disorder Similar to Huntington’s Disease Is Inflammatory Demyelinating Polyradiculoneuropathy Associated with a Novel CAG Repeat Expansion Wei Xing Yan, MD, Juan J. Archelos, MD, Russell L. Margolis, MD, Elizabeth O’Hearn, MD, Hans-Peter Hartung, MD, Adam Rosenblatt, MD, Virginia Willour, PhD, and John D. Pollard, BSc, MD, PhD Susan E. Holmes, PhD, Mary L. Franz, MSW, Colleen Callahan, BA, Hyon S. Hwang, BA, Lewy Bodies and Parkinsonism in Families with Parkin Juan C. Troncoso, MD, and Christopher A. Ross, MD, PhD Mutations Matt Farrer, PhD, Piu Chan, MD, Rong Chen, PhD, Low Cerebrospinal Fluid Hypocretin (Orexin) and Louis Tan, MD, Sarah Lincoln, BS, Dena Hernandez, BS, Altered Energy Homeostasis in Human Narcolepsy Lysia Forno, MD, Katrina Gwinn-Hardy, MD, Seiji Nishino, MD, PhD, Beth Ripley, BS, Leonard Petrucelli, PhD, Jennifer Hussey, BS, Sebastiaan Overeem, MSc, Sona Nevsimalova, MD, PhD, Andrew Singleton, PhD, Caroline Tanner, MD, PhD, Gert Jan Lammers, MD, PhD, Jitka Vankova, MD, John Hardy, PhD, and J. William Langston, MD Michele Okun, MA, William Rogers, BS, Stephen Brooks, MD, and Emmanuel Mignot, MD, PhD ANNA-3 Anti-Neuronal Nuclear Antibody: Marker of Lung Cancer-Related Autoimmunity Transected Neurites, Apoptotic Neurons, and Reduced Koon Ho Chan, MD, Steven Vernino, MD, PhD, Inflammation in Cortical Multiple Sclerosis Lesions and Vanda A. Lennon, MD, PhD John W. Peterson, BS, Lars Bé, MD, PhD, Sverre Mérk, MD, PhD, Ansi Chang, MD, Nemaline Myopathy: A Clinical Study of 143 Cases and Bruce D. Trapp, PhD Monique M. Ryan, MB, BS, Christina Schnell, MS, Corinne D. Strickland, MS, Lloyd K. Shield, MB, BS, Graeme Morgan, MB, BS, Susan T. Iannaccone, MD, Nigel G. Laing, PhD, Alan H. Beggs, PhD, Brief Communications and Kathryn N. North, MD, BS Pure Representational Neglect After Right Thalamic Nigrostriatal Collaterals to Thalamus Degenerate in Lesion Parkinsonian Animal Models Stéphanie Ortigue, MS, Isabelle Viaud-Delmon, PhD, Amanda Freeman, BS, Brian Ciliax, PhD, Roy Bakay, MD, Jean-Marie Annoni, MD, Theodor Landis, MD, Joseph Daley, BS, R. Daniel Miller, MS, Christoph Michel, PhD, Olaf Blanke, MD, Glenda Keating, PhD, Allan Levey, MD, PhD, atrik Vuilleumier, MD, and Eugéne Mayer, PhD and David Rye, MD, PhD Elevated Corticotropin Releasing Hormone/ Central and Peripheral Nervous System Infection, Corticotropin Releasing Hormone-R1 Expression in Immunity, and Inflammation in the NHP Model of Postmortem Brain Obtained from Children with Lyme Borreliosis Generalized Epilepsy Andrew R. Pachner, MD, Diego Cadavid, MD, Wei Wang, MD, Kimberly E. Dow, MD, Gale Shu, BS, Donna Dail, MS, Sarah Pachner, and Douglas D. Fraser, MD, PhD, Emir Hodzic, DVM, and Stephen W. Barthold, DVM, PhD Early-Onset Multisystem Mitochondrial Disorder Molecular and Clinical Diversity in Paraneoplastic Caused by a Nonsense Mutation in the Mitochondrial Immunity to Ma Proteins DNA Cytochrome C Oxidase II Gene Myrna R. Rosenfeld, MD, PhD, Joseph G. Eichen, MS, Yolanda Campos, MSc, Alberto Garcia-Redondo, MSc, Deborly F. Wade, BS, Jerome B. Posner, MD, Miguel A. Ferndndez-Moreno, PhD, and Josep Dalmau, MD, PhD Mercedes Martinez-Pardo, MD, Guillermo Goda, Pharm B, Juan C. Rubio, MSc, Miguel A. Martin, Pharm B, Complex Immunomodulatory Effects of Interferon-B in Pilar del Hoyo, Pharm B, Ana Cabello, MD, Multiple Sclerosis Include the Upregulation of T Belen Bornstein, MD, Rafael Garesse, PhD, Helper 1-Associated Marker Genes and Joaquin Arenas, PhD Klaus-Peter Wandinger, MD, Claus-Steffen Stiirzebecher, MD, Bibiana Bielekova, MD, Ocular Flutter Associated with a Localized Lesion in Greg Detore, Andreas Rosenwald, MD, the Paramedian Pontine Reticular Formation Louis M. Staudt, MD, PhD, Henry F. McFarland, MD, Fred Schon, FRCP, Timothy L. Hodgson, PhD, and Roland Martin, MD Dominic Mort, MRCP, and Christopher Kennard, FRCP Contents of Volume 50, July-December 2001 v 422 Reply Expedited Publication Marco Rovaris, MD, Giancarlo Comi, MD, Sodium Channel Inactivation Defects Are Associated Maria Pia Sormani, PhD, with Acetazolamide-Exacerbated Hypokalemic Periodic Jerry S. Wolinsky, MD, David Ladkani, PhD, Paralysis and Massimo Filippi, MD Said Bendahhou, PhD, Theodore R. Cummins, PhD, Robert C. Griggs, MD, Ying-Hui Fu, PhD, 423 Borna Disease Virus RNA Is Absent in Chronic and Louis ]. Ptacek, MD Multiple Sclerosis Claus G. Haase, MD, Sergei Viazov, MD, Melanie Fiedler, MD, Nikolaus Koenig, MD, Letters Pedro M. Faustmann, MD, Alteration of the Serotoninergic System in Fatal and Michael Roggendorf, MD Familial Insomnia Pietro Cortelli, Ronald Polinsky, Pasquale Montagna, Monozygotic Twins with X-Linked and Elio Lugaresi Adrenoleukodystrophy and Different Phenotypes Reply Maja Di Rocco, MD, Laura Doria-Lamba, MD, and Julia Wanschitz, MD, Marin Guentchev, MD, Ubaldo Caruso, PhD and Herbert Budka, MD Effects of Seasons on Magnetic Resonance Imaging- Measured Disease Activity in Patients with Multiple Regular Departments Sclerosis Helen Tremlett, BPharm Hons, PhD 425. Books NO 3 SEPTEMBER 2001 SUPPLEMENT 1 126th Annual Meeting of the 30th Annual Meeting of the American Neurological Child Neurology Society $80 Program Association $92 Abstracts Program Abstracts Neurology Outcomes Research: Current Science and Future Directions Program Abstracts NO 4 OCTOBER 2001 443 Matrix Metalloproteinases and Their Tissue Inhibitors Editorials as Markers of Disease Subtype and Response to Endogenous Retroviruses and Multiple Sclerosis Interferon-B Therapy in Relapsing and Secondary- Dennis L. Kolson, MD, PhD, Progressive Multiple Sclerosis Patients and Francisco Gonzdlez-Scarano, MD Yanina Galboiz, MSc, Sarah Shapiro, PhD, Matrix Metalloproteinases in Multiple Sclerosis: Is It Nitza Lahat, PhD, Hannah Rawashdeh, MD, Time for a Treatment Trial? and Ariel Miller, MD, PhD Gary A. Rosenberg, MD 452 Hereditary Motor and Sensory Neuropathy-Russe: New Autosomal Recessive Neuropathy in Balkan Gypsies P. K. Thomas, DSc, MD, Luba Kalaydjieva, PhD, MD, Original Articles Brian Youl, MD, Tamara Rogers, PhD, Monocyte Activation and Differentiation Augment Dora Angelicheva, PhD, Rosalind H. M. King, PhD, Human Endogenous Retrovirus Expression: Implications Velina Guergueltcheva, MD, Jaume Colomer, MD, for Inflammatory Brain Diseases Constantin Lupu, MD, Axinia Corches, MD, James B. Johnston, PhD, Claudia Silva, MSc, Gabriela Popa, MD, Luciano Merlini, MD, Janet Holden, MD, Kenneth G. Warren, MD, Alex Shmarov, PhD, MD, John R. Muddle, Arthur W. Clark, MD, and Christopher Power, MD Michelle Nourallah, and Ivailo Tournev, PhD, MD vi Contents of Volume 50, July-December 2001 458 Are Certain Diuretics Also Anticonvulsants? 521 Human Brain Mapping in Dystonia Reveals Both Dale C. Hesdorffer, PhD, James P. Stables, MSA, Endophenotypic Traits and Adaptive Reorganization W. Allen Hauser, MD, John F. Annegers, PhD, Sabine Meunier, MD, PhD, Line Garnero, PhD, and Gregory Cascino, MD Antoine Ducorps, BSc, Leonor Maziéres, MD, PhD, Stéphane Lehéricy, MD, PhD, Individual Blood—Brain Barrier Phenylalanine Transport Sophie Tézenas du Montcel, MD, Bernard Renault, PhD, and Marie Vidailhet, MD, PhD Determines Clinical Outcome in Phenylketonuria Josef Weglage, MD, PhD, Dirk Wiedermann, PhD, Jonas Denecke, MD, Reinhold Feldmann, PhD, Hans-Georg Koch, MD, Kurt Ullrich, MD, Erik Harms, MD, and Harald E. Méller, PhD Brief Communications Neuronal Activity in the Globus Pallidus in Chorea Human Apolipoprotein E4 Accelerates B-Amyloid Caused by Striatal Lacunar Infarction Deposition in APPsw Transgenic Mouse Brain Takao Hashimoto, MD, PhD, Hiroshi Morita, MD, PhD, Donald B. Carter, PhD, Edwige Dunn, MS, Tsuyoshi Tada, MD, PhD, Tetsuhiro Maruyama, MD, PhD, Denise D. McKinley, BS, Nancy C. Stratman, MS, Yuzo Yamada, MD, PhD, and Shu-ichi Ikeda, MD, PhD Timothy P. Boyle, BS, Susan L. Kuiper, MS, Myelinopathia Centralis Diffusa (Vanishing White Jo A. Oostveen, MS, Royal J. Weaver, PhD, Matter Disease): Evidence of Apoptotic Oligodendrocyte Jennifer A. Boller, BS, and Mark E. Gurney, PhD Degeneration in Early Lesion Development Wolfgang Briick, MD, Jochen Herms, MD, Autosomal Dominant Glut-1 Deficiency Syndrome Knut Brockmann, MD, Walter Schulz-Schaeffer, MD, and Familial Epilepsy and Folker Hanefeld, MD Knut Brockmann, MD, Dong Wang, MD, Christoph G. Korenke, MD, A Novel Deafness/Dystonia Peptide Gene Mutation that Arpad von Moers, MD, Causes Dystonia in Female Carriers of Mohr- Yuan-Yuan Ho, PhD, Tranebjaerg Syndrome Juan M. Pascual, MD, PhD, Russell H. Swerdlow, MD, and G. Frederick Wooten, MD Kunyan Kuang, MD, Multisystem Disorder Associated with a Missense Hong Yang, MD, Li Ma, Mutation in the Mitochondrial Cytochrome 5 Gene Pamela Kranz-Eble, BS Flemming Wibrand, PhD, Kirstine Ravn, MSc, Jorge Fischbarg, MD, PhD, Marianne Schwartz, PhD, Thomas Rosenberg, MD, Folker Hanefeld, MD, Nina Horn, PhD, and John Vissing, MD, PhD and Darryl C. De Vivo, MD Dextran Reduces Embolic Signals After Carotid Elevated Serum and Cerebrospinal Fluid Levels Endarterectomy of Soluble Human Herpesvirus Type 6 Cellular Christopher R. Levi, MBBS, FRACP, Receptor, Membrane Cofactor Protein, in Patients with Jacinda L. Stork, BA, BAppSci (Hons), Multiple Sclerosis Brian R. Chambers, MD, FRACP, Samantha S. Soldan, MS, Anna Fogdell-Hahn, PhD, Anne L. Abbott, MBBS, FRACP, Heather M. Cameron, RN, Meghan B. Brennan, BA, Barbara B. Mittleman, MD, Anna Peeters, BSc (Hons), PhD, John P. Royle, FRACS, FACS, Clara Ballerini, PhD, Luca Massacesi, MD, Andrew K. Roberts, FRACS, FACS, Gary Fell, MBBS, FRACS, Tsukasa Seya, MD, PhD, Henry F. McFarland, MD, Michael C. Hoare, MBBS, FRACS, and Steven Jacobson, PhD Anthony T. W. Chan, Mmed (Surg), FRACS, Relationship of the Extended Tau Haplotype to Tau and Geoffrey A. Donnan, MD, FRACP Biochemistry and Neuropathology in Progressive Mutation of the Doublecortin Gene in Male Patients Supranuclear Palsy with Double Cortex Syndrome: Somatic Mosaicism Wan-Kyng Liu, PhD, Tien V. Le, BS, Jennifer Adamson, BS, Detected by Hair Root Analysis Matthew Baker, BSc, Natalie Cookson, BS, Mitsuhiro Kato, MD, Masayo Kanai, MD, John Hardy, PhD, Michael Hutton, PhD, Osamu Soma, MD, Yuichi Takusa, MD, Shu-Hui Yen, PhD, and Dennis W. Dickson, MD Toshiyuki Kimura, MD, Chikahiko Numakura, MD, Pick’s Disease Associated with the Novel Tau Gene Takasumi Matsuki, MD, Shigeki Nakamura, PhD, Mutation K369I and Kiyoshi Hayasaka, MD Manuela Neumann, MD, Walter Schulz-Schaeffer, MD, R. Anthony Crowther, PhD, Michael J. Smith, Maria Grazia Spillantini, PhD, Michel Goedert, MD, PhD, and Hans A. Kretzschmar, MD Letters Metabolic Changes Following Subthalamotomy for Conjugal Multiple Sclerosis Advanced Parkinson’s Disease Christopher Hawkes, MD, FRCP Philip C. Su, MD, Yilong Ma, PhD, Masafumi Fukuda, MD, Marc J. Mentis, MD, Reply Ham-Min Tseng, MD, Ruoh-Fang Yen, MD, George C. Ebers, MD, A. D. Sadovnick, PhD, FRCP(c), Hon-Man Liu, MD, J. R. Moeller, PhD, and Pierre Duquette, FRCP(c), for the Canadian and David Eidelberg, MD Collaborative Study Contents of Volume 50, July-December 2001 vii 553 Compound Heterozygosity and Variable 554 Electroencephalographic and Clinical Correlation of Penetrance in SOD1 Amyotrophic Lateral Hyponatremia Induced During Transurethral Resection Sclerosis Pedigrees of the Prostate Venkata R. Reddy, MD, and Sreenivasa S. Moorthy, MD Matthew J. Parton, MRCP, Peter M. Andersen, MD, DMSc, Wendy J. Broom, BSc, and Christopher E. Shaw, MD Obituary Clarence Joseph Gibbs, Jr, 1924-2001 Reply Richard T. Johnson, MD Collette K. Hand, PhD, Veronique Mayeux-Portas, PhD, Jawad Khoris, MD, Valerie Briolotti, Pierre Clavelou, MD, PhD, Regular Departments William Camu, MD, PhD, and Guy A. Rouleau, MD, PhD 557 Books NO 5 NOVEMBER 2001 602 A Nitric Oxide Donor Induces Neurogenesis and Editorial Reduces Functional Deficits After Stroke in Rats Behavioral Neurology in the Emergency Room: Ruilan Zhang, MD, Li Zhang, MD, Language Testing, Brain Imaging, and Acute Stroke Zhenggang Zhang, MD, PhD, Ying Wang, MD, Therapy Mei Lu, PhD, Margot LaPointe, PhD, and Howard S. Kirshner, MD Michael Chopp, PhD Human Herpesvirus 6 Limbic Encephalitis After Stem Original Articles Cell Transplantation Hypoperfusion of Wernicke’s Area Predicts Severity of Mark S. Wainwright, MD, PhD, Semantic Deficit in Acute Stroke Paul L. Martin, MD, PhD, Richard P. Morse, MD, Argye E. Hillis, MD, MS, Robert J. Wityk, MD, Mary Lacaze, MD, James M. Provenzale, MD, Elizabeth Tuffiash, Norman J. Beauchamp, MD, MSH, R. Edward Coleman, MD, Marcello A. Morgan, Michael A. Jacobs, PhD, Peter B. Barker, DPhil, Christine Hulette, MD, Joanne Kurtzberg, MD, and Ola A. Selnes, PhD Cheryl Bushnell, MD, Leon Epstein, MD, and Darrell V. Lewis, MD Attention and Cognition in Bradykinetic—Rigid Syndromes: An Event-Related Potential Study Plasticity of Language Networks in Patients with Brain Zvezdan Pirtosek, MD, Marjan Jahanshahi, PhD, Tumors: A Positron Emission Tomography Activation Geoff Barrett, PhD, and Andrew J. Lees, MD, FRCP Study Alexander Thiel, MD, Karl Herholz, MD, Adem Koyuncu, Molecular Heterogeneity of Myophosphorylase Mehran Ghaemi, MD, Lutz W. Kracht, MD, Deficiency (McArdle’s Disease): A Genotype-Phenotype Birgit Habedank, and Wolf-Dieter Heiss, MD Correlation Study Miguel A. Martin, Pharm B, Juan C. Rubio, MSc, Induction of Proinflammatory Molecules in Mice with Jenny Buchbinder, PhD, Roberto Fernandez-Hojas, PhD, Amyotrophic Lateral Sclerosis: No Requirement for Pilar del Hoyo, Pharm B, Susana Teijeira, PhD, Proapoptotic Interleukin-1B in Neurodegeneration Josep Gamez, MD, Carmen Navarro, MD, Minh Dang Nguyen, BSc, Jean-Pierre Julien, PhD, José M. Fernandez, MD, Ana Cabello, MD, and Serge Rivest, PhD Yolanda Campos, MSc, Carlos Cervera, MD, José M. Culebras, PhD, Antoni L. Andreu, PhD, Depletion of Corticotrophin-Releasing Factor Neurons Robert Fletterick, PhD, and Joaquin Arenas, PhD in the Pontine Micturition Area in Multiple System Atrophy Magnetoencephalographic Fields from Patients with Eduardo E. Benarroch, MD, DSci, Spontaneous and Induced Migraine Aura and Ann M. Schmeichel, AS Susan M. Bowyer, PhD, Sheena K. Aurora, MD, John E. Moran, PhD, Norman Tepley, PhD, Immunopathology of Secondary-Progressive Multiple and K. M. A. Welch, MD Sclerosis John W. Prineas, MB, BS, Eunice E. Kwon, MSc, Poststreptococcal Acute Disseminated Encephalomyelitis Eun-Sook Cho, MD, Leroy R. Sharer, MD, with Basal Ganglia Involvement and Auto-Reactive Michael H. Barnett, MB, BS, Emilia L. Oleszak, PhD, Antibasal Ganglia Antibodies Brad Hoffman, BS, and Bryan P. Morgan, PhD Russell C. Dale, MRCP, Andrew J. Church, BSc, Francisco Cardoso, PhD, Elizabeth Goddard, Tim C. Cox, Brief Communications W. K. “Kling” Chong, Amanda Williams, MRCP, Nigel J. Klein, PhD, Brian G. Neville, FRCP, Case-Control Study of the Extended Tau Gene Edward J. Thompson, DSc, and Gavin Giovannoni, PhD Haplotype in Parkinson’s Disease Demetrius M. Maraganore, MD, Dena G. Hernandez, BS, Sexual Dimorphism and Developmental Regulation of Andrew B. Singleton, PhD, Matthew J. Farrer, PhD, Substantia Nigra Function Shannon K. McDonnell, MS, Michael L. Hutton, PhD, Jana Veliskova, MD, PhD, and Solomon L. Moshé, MD John A. Hardy, PhD, and Walter A. Rocca, MD, MPH Vili Contents of Volume 50, July-December 2001 661 Endocytic Disturbances Distinguish Among Subtypes of Letters Alzheimer’s Disease and Related Disorders Anne Cataldo, PhD, Helicobacter Pylori-Induced Reduction of Acute Levodopa Absorption in G. William Rebeck, PhD, Parkinson’s Disease Patients Bernadino Ghetti, MD, Mariangela Pierantozzi, PhD, Antonio Pietroiusti, MD, Christine Hulette, MD, Carol Lippa, MD, Alberto Galante, MD, Giuseppe Sancesario, MD, Christine Van Broeckhoven, PhD, DrSc, Gianluigi Lunardi, PhD, Ernesto Fedele, PhD, Cornelia van Duijn, PhD, Patrick Cras, MD, PhD, Patrizia Giacomini, MD, Nenad Bogdanovic, MD, Thomas Bird, MD, and Paolo Stanzione, MD Corrinne Peterhoff,; BS, and Ralph Nixon, MD, PhD Pharmacodynamic Modeling of Oral Levodopa in Adult-Onset Leukoencephalopathy with Vanishing Parkinson’s Disease White Matter Presenting with Dementia Manuela Contin, PharmD, Roberto Riva, MD, Fiorenzo Albani, PharmD, Paolo Martinelli, MD, Konstantin Prass, MD, Wolfgang Briick, MD, and Agostino Baruzzi, MD Nicolas W. J. Schréder, Andreas Bender, MD, Maria Prass, MD, Tilo Wolf, MD, Reply Marjo S. Van der Knaap, MD, PhD, Raul de la Fuente-Fernandez, MD, and Rolf Zschenderlein, MD and A. Jon Stoessl, MD, FRCPC Relapsing—Remitting Autoimmune Agrypnia Are the “Newly Discovered” Paraneoplastic Anna Paola Batocchi, MD, Giacomo Della Marca, MD, Anti-Collapsin Response-Mediator Massimiliano Mirabella, MD, Marcella Caggiula, MD, Protein 5 Antibodies Simply Giovanni Frisullo, MD, Gioacchino Francesco Mennuni, MD, Anti-CV2 Antibodies? and Pietro Attilio Tonali, MD Jéréme Honnorat, MD, PhD, Jean-Christophe Antoine, MD, Functional Activation of Microgyric Visual Cortex in a and Marie-Francoise Belin, PhD Human Giorgio M. Innocenti, MD, Philippe Maeder, MD, Reply Maria G. Knyazeva, PhD, Eleonora Fornari, Vanda A. Lennon, MD, PhD, Zhiya Yu, MD, PhD, and Thierry Deonna, MD Thomas J. Kryzer, AS, and Guy E. Griesmann, MS Convergence Retraction Nystagmus: A Disorder of Classic Rett Syndrome in a Boy as Vergence? a Result of Somatic Mosaicism for Holger Rambold, MD, Detlef Kémpf, MD, a MECP2 Mutation and Christoph Helmchen, MD Judith Armstrong, MsC, Pilar Péo, MD, PhD, Mercé Pineda, MD, PhD, Elena Aibar, MsC, Dysautonomia in Human T-Cell Lymphotrophic Virus Esther Gean, MsC, MD, Type I-Associated Myelopathy/Tropical Spastic Viceng Catala, MsC, PhD, Paraparesis and Eugenia Monrés, MsC, PhD Alexandre H. Alamy, MD, MSc, Fernando B. Menezes, MD, MSc, Ana C. B. Leite, MD, PAD, Regular Departments Osvaldo M. Nascimento, MD, PhD, and Abelardo Q-C. Aratijo, MD, PhD 693 Books DECEMBER 2001 708 Long-Term Hyperexcitability in the Hippocampus After Editorials Experimental Head Trauma Microarrays, Markers of Disease, and the Myth of Vijayalakshmi Santhakumar, BS, Anna D. H. Ratzliff, BS, “Nonhypothesis-Driven” Research Jade Jeng, BS, Zsolt Toth, MD, PhD, and Ivan Soltesz, PhD David A. Greenberg, MD, PhD Epilepsy After Head Injury: The Impact of Impact Fluoxetine Modulates Motor Performance and Cerebral Daniel Lowenstein, MD Activation of Patients Recovering from Stroke Jérémie Pariente, MD, Isabelle Loubinoux, PhD, Imaging Stroke Recovery: Lessons from Prozac Christophe Carel, MD, Jean-Francois Albucher, MD, John A. Detre, MD Anne Leger, MD, Claude Manelfe, MD, PhD, Olivier Rascol, MD, PhD, and Francois Chollet, MD Original Articles Disease Mechanisms Revealed by Transcription Blood Genomic Responses Differ After Stroke, Seizures, Profiling in SOD1-G93A Transgenic Mouse Spinal Hypoglycemia, and Hypoxia: Blood Genomic Cord Fingerprints of Disease Mary K. Olsen, BA, Steven L. Roberds, PhD, Yang Tang, MD, Aigang Lu, MD, Bruce J. Aronow, PhD, Brenda R. Ellerbrock, BA, Timothy J. Fleck, BA, and Frank R. Sharp, MD Denise K. McKinley, BA, and Mark E. Gurney, PhD Contents of Volume 50, July-December 2001 ix 741 Dementia and Neurodevelopmental Predisposition: Reorganization of Somatic Sensory Function in the Cognitive Dysfunction in Presymptomatic Subjects Human Thalamus After Stroke Precedes Dementia by Decades in Frontotemporal Shinji Ohara, MD, PhD, and Frederick A. Lenz, MD, PhD Dementia Daniel H. Geschwind, MD, PhD, Janik Robidoux, PhD, Evidence for Postjunctional Serotonin (5-HT,) Maricela Alarcon, PhD, Bruce L. Miller, MD, Receptors in the Trigeminocervical Complex Kirk C. Wilhelmsen, MD, PhD, Jeffrey L. Cummings, MD, Peter J. Goadsby, MD, DSc, Simon Akerman, MSc, and Ziad S. Nasreddine, MD and R. James Storer, PhD Compensating for Central Nervous System Increased HTLV-I Proviral Load and Preferential Dysmyelination: Females with a Proteolipid Protein Expansion of HTLV-I Tax-Specific CD8 T Cells in Gene Duplication and Sustained Clinical Improvement Cerebrospinal Fluid from Patients with HAM/TSP Ken Inoue, MD, PhD, Hajime Tanaka, MD, PhD, Masahiro Nagai, MD, PhD, Yoshihisa Yamano, MD, PhD, Fernando Scaglia, MD, Akiko Araki, MD, Meghan B. Brennan, BA, Carlos A. Mora, MD, Lisa G. Shaffer, PhD, and James R. Lupski, MD, PhD and Steven Jacobson, PhD McLeod Neuroacanthocytosis: Genotype and Phenotype Spinocerebellar Ataxia Type 2 Presenting as Familial Adrian Danek, MD, PhD, Justin P. Rubio, PhD, Levodopa-Responsive Parkinsonism Luca Rampoldi, PhD, Mengfatt Ho, D Phil (Oxon), Din-E Shan, MD, PhD, Bing-Wen Soong, MD, PhD, Carol Dobson-Stone, MBiochem, Frangois Tison, MD, PhD, Chen-Ming Sun, MD, Shwn-Jen Lee, PhD, PT, William A. Symmans, MD, Matthias Oechsner, MD, Kwong-Kum Liao, MD, and Ren-Shyan Liu, MD Wolfgang Kalckreuth, MD, Julie M. Watt, BSc, Complete Allele Information in the Diagnosis of Alastair ]. Corbett, MD, FRACP, Facioscapulohumeral Muscular Dystrophy by Triple Hisham H. M. Hamdalla, MD, MRCP (UK), DNA Analysis Andrew G. Marshall, MD, MRCP (UK), Richard J. L. F. Lemmers, BSc, Peggy de Kievit, Ian Sutton, MD, MRCP (UK), Maria Teresa Dotti, MD, Michel van Geel, PhD, Michiel J.R. van der Wielen, Alessandro Malandrini, MD, Ruth H. Walker, MD, PhD, Egbert Bakker, PhD, George W. Padberg, MD, PhD, Geoff Daniels, PhD, and Anthony P. Monaco, MD, PhD Rune R. Frants, PhD, and Silvére M. van der Maarel, PhD Dementia in Hereditary Cerebral Hemorrhage with So Near Yet So Far: Neglect in Far or Near Space Amyloidosis-Dutch Type Is Associated with Cerebral Depends on Tool Use Amyloid Angiopathy but Is Independent of Plaques and Alan J. Pegna, PhD, Leila Petit, MA, Neurofibrillary Tangles Anne-Sarah Caldara-Schnetzer, MA, Asaid Khateb, PhD, Remco Natté, MD, Jean-Marie Annoni, MD, Roman Sztajzel, MD, Marion L. C. Maat-Schieman, MD, PhD, and Theodor Landis, MD Joost Haan, MD, PhD, Marjolijn Bornebroek, MD, PhD, Raymund A.C. Roos, MD, PhD, and Sjoerd G. van Duinen, MD, PhD The Wid* Protein Protects Against Axonal Degeneration: A Model of Gene Therapy for Peripheral Letters Neuropathy N-Acetylaspartate—A Marker of Neuronal Integrity Min Sheng Wang, PhD, Guofu Fang, MS, Edith V. Sullivan, PhD, Elfar Adalsteinsson, PhD, Deborah G. Culver, MS, Albert A. Davis, Daniel M. Spielman, PhD, Ralph E. Hurd, PhD, Mark M. Rich, PhD, MD, and Jonathan D. Glass, MD and Adolf Pfefferbaum, MD Cigarette Smoking and the Incidence of Parkinson’s N-Acetylaspartate: Usefulness as an Indicator of Viable Disease in Two Prospective Studies Neuronal Tissue Miguel A. Hernan, MD, Shumin M. Zhang, MD, Douglas L. Arnold, Nicola de Stefano, Paul ,M. Matthews, Ana M. Rueda-deCastro, BS, Graham A. Colditz, MD, and Bruce D. Trapp Frank E. Speizer, MD, and Alberto Ascherio, MD Reply Effect of Therapeutic Ionizing Radiation on the Human Ernst Martin, MD, Thorsten Thiel, PhD, Brain Andrea Capone, MD, Juergen Henning, PhD, and R. Grant Steen, PhD, David Spence, BS, Shengie Wu, MS, Jacques Schneider, MD Xiaoping Xiong, PhD, Larry E. Kun, MD, and Thomas E. Merchant, DO, PhD Bilateral High-Frequency Synchronous Discharges Associated with Posterior Fossa Disorders Mario-Ubaldo Manto, MD, and John Moore, BE Effectiveness of Intravenous Immunoglobin Therapy in Cerebellar Ataxia Associated with Gluten Sensitivity Brief Communications Katrin Biirk, MD, Arthur Melms, MD, Jorg B. Schulz, MD, Molecular and Clinical Correlation in Five Indian and Johannes Dichgans, MD Families with Spinocerebellar Ataxia 12 Achal K. Srivastava, DM, Shweta Choudhry, MSc, Cerebrospinal Fluid Interleukin-6 Levels: A Possible Musuwadi S. Gopinath, MD, Sanghamitra Roy, MSc, Marker of Disease Activity Manjari Tripathi, DM, Samir K. Brahmachari, PhD, Kurt A. Jellinger, MD, PhD, Norbert Résler, MD, and Satish Jain, DM and Ildiko Wichart, PhD x Contents of Volume 50, July-December 2001

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