Annals of Neurology 1994: Vol 36 Table of Contents

13 Pages·1994·3.1 MB·English

Annalsof
Neurology
Vol 36 July December 1994
Official Journal of the American Neurological Association and the Child Neurology Society
Editor Editorial Board
Robert A. Fishman, MD, M. Flint Beal, MD, Boston
University of California D. Frank Benson, MD, Los Angeles
San Francisco, CA
Thomas D. Bird, MD, Seattle
Founding Editor Robert C. Collins, MD, Los Angeles
Fred Plum, MD
Jasper R. Daube, MD, Rochester, MN
Editor, 1985-1992 Andrew G. Engel. MD, Rochester, MN
Arthur K. Asbury, MD
Marvin A. Fishman, MD, Houston
Associate Editors Richard S. J. Frackowiak, MD, London
Frank M. Longo, MD, PAD,
Myron D. Ginsberg, MD, Miami
San Francisco
Stephen M. Sagar, MD, David A. Greenberg, MD, PhD,
San Francisco Pittsburgh
Anita E. Harding, MD, London
Associate Editor for
Child Neurology Gregory L. Holmes, MD, Boston
Joseph J. Volpe, MD, Boston R. John Leigh, MD, Cleveland
Editorial Coordinator Howard L. Lipton, MD, Evanston, IL
Marilyn Stubblebine Robert P. Lisak, MD, Detroit
William R. Markesbery, MD,
Publisher
Little, Brown and Company, Boston, Lexington, KY
Massachusetts Richard P. Mayeux, MD, New York
Hugo W. Moser, MD, Baltimore
Publishing Staff John G. Nutt, MD, Portland, OR
Thomas A. Manning Peter C. O’Brien, PhD, Rochester, MN
Publisher Jonathan H. Pincus, MD, Washington, DC
David Dionne Gérard Said, MD, Paris, France
Executive Editor Stephen G. Waxman, MD, PhD,
Sherri Frank New Haven, CT
Managing Editor Huda Y. Zoghbi, MD, Houston
Tracey Solon Annals of Neurology is indexed in Index Medicus,
Assistant Managing Editor Current Contents/Clinical Medicine, Excerpta
Medica, Biological Abstracts, and Psychological
Anne Orens Abstracts.
Sales and Marketing Manager
Copyright © 1994 by the American Neurological
Fredda Purgalin Association. All rights reserved. Except as authorized in
the accompanying statement, no part of Annals of
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NO 1 JULY 1994
55 Pharmacological Evaluation of the Cholinergic
Notice System in Progressive Supranuclear Palsy
Thanks to Reviewers I. Litvan, MD, R. Blesa, MD, K. Clark, BA,
Robert A. Fishman, MD P. Nichelli, MD, J. R. Atack, PhD,
M. M. Mouradian, MD, J. Grafman, PhD,
and T. N. Chase, MD
Editorials
Cortical Control of Movement Correlation of Magnetization Transfer Ratio
Scott T. Grafton, MD with Clinical Disability in Multiple Sclerosis
A. Gass, MD, G. J. Barker, PhD, D. Kidd, MRCP,
Levodopa: Rational and Irrational J. W. Thorpe, MRCP, D. MacManus, DCR,
Pharmacology A. Brennan, DCR, P. S. Tofts, DPhil,
John G. Nutt, MD A. J. Thompson, MD, W. 1. McDonald, PhD,
and D. H. Miller, MD
Neurological Progress
Responsiveness of the Somatosensory System
Neuropeptides in the Sympathetic System: after Nerve Injury and Amputation in the
Presence, Plasticity, Modulation, and Human Hand
Implications W. Schady, FRCP, S. Braune, MD, S. Watson, FRCS,
Eduardo E. Benarroch, MD H. E. Torebjirk, MD, and R. Schmidt, MD
Use of Proton Magnetic Resonance Spectroscopy
Brief Review
for Monitoring Disease Progression in Multiple
Are Magnetic Resonance Findings Predictive Sclerosis
of Clinical Outcome in Therapeutic Trials in Douglas L. Arnold, MD, Gerald T. Riess, MD,
Muitiple Sclerosis? The Dilemma of Inferferon-B Paul M. Matthews, MD, DPhil,
W. I. McDonald, D. H. Miller, and A. J. Thompson Gordon S. Francis, MD, D. Louis Collins,
Christina Wolfson, PhD, and Jack P. Antel, MD
Original Articles
Pyruvate Dehydrogenase Deficiency: Molecular
Movement- and Task-related Activations of Basis for Intrafamilial Heterogeneity
Motor Cortical Areas: A Positron Emission Tatsuya Fujii, MD, DMedSci,
Tomographic Study Rudy N. Van Coster, MD, Susan E. Old, PhD,
Philippe Remy, MD, Monica Zilbovicius, MD, Rosella Medori, MD, Susan Winter, MD,
Anne Leroy-Willig, MD, PhD, Ruth M. Gubits, PhD,
André Syrota, MD, PhD, and Yves Samson, MD Paul M. Matthews, MD, DPhil, FRCPC,
Ruth M. Brown, MSc, Garry K. Brown, BM, PhD,
Wearing-off Fluctuations in Parkinson’s Disease:
Hans-Henrik M. Dahl, PhD,
Contribution of Postsynaptic Mechanisms
and Darryl C. De Vivo, MD
D. Bravi, MD, M. M. Mouradian, MD,
J. W. Roberts, MD, T. L. Davis, MD, Y. H. Sohn, MD, Suppression of Motor Cortical Excitability by
and T. N. Chase, MD Electrical Stimulation over the Cerebellum in
Ataxia
Cerebrospinal Fluid Human Immunodeficiency
Yoshikazu Ugawa, MD, Kieko Genba-Shimizu,
Virus Type 1 (HIV-1) p24 Antigen Levels in
John C. Rothwell, PhD, Makoto Iwata, MD,
HIV-1-related Dementia
and Ichiro Kanazawa, MD
W. Royal III, MD, O. A. Selnes, PhD,
M. Concha, MD, MHS, T. E. Nance-Sproson, MHA,
andJ . C. McArthur, MBBS, MPH
Brief Communications
Axonal Injury and Membrane Alterations in Are the Associations Between Alzheimer’s
Alzheimer’s Disease Suggested by In Vivo Disease and Polymorphisms in the
Proton Magnetic Resonance Spectroscopic Apolipoprotein E and the Apolipoprotein CII
Imaging Genes Due to Linkage Disequilibrium?
D. J. Meyerhoff, Dr rer nat, §. MacKay, MD, T. Tsuda, MD, PhD, R. Lopez, BSc,
J.-M. Constans, MD, D. Norman, MD, E. A. Rogaeva, PhD, M. Freedman, MD,
C. Van Dyke, MD, G. Fein, PhD, E. Rogaev, PhD, D. Drachman, MD,
and M. W. Weiner, MD D. Pollen, MD, J. Haines, PhD, Y. Liang, MD,
D. R. Crapper McLachlan, MD, R. Duara, MD,
Cognitive Functions and Quality of Life in and P. St. George-Hyslop, MD, FRCP(C)
Patients with Low-Grade Gliomas: The Impact
of Radiotherapy Parkinson’s Disease and Brain Levels of
M.J . B. Taphoorn, MD, A. Klein Schiphorst, MA, Organochlorine Pesticides
F. J. Snoek, PhD, J. Lindeboom, PhD, Lora Fleming, MD, MPH, John B. Mann, MS,
J. G. Wolbers, MD, A. B. M. F. Karim, MD, Judy Bean, PhD, Thomas Briggle, PhD,
P. C. Huijgens, MD, andJ .J . Heimans, MD and Juan R. Sanchez-Ramos, PhD, MD
Contents of Volume 36, July-December, 1994
103 Subacute Sclerosing Panencephalitis in an Reply
Infant: Diagnostic Role of Viral Genome John N. Whitaker, MD
Analysis
Tallie Z. Baram, MD, PhD, Twenty-One Patients with Strictly
Ignacio Gonzalez-Gomez, MD, Zong-De Xie, MD, Defined Postpoliomyelitis Syndrome: No
Dapeng Yao, Floyd H. Gilles, MD, Poliovirus-specific IgM Antibodies in the
Marvin D. Nelson, Jr, MD, Hahn T. Nguyen, Cerebrospinal Fluid
and Julius Peters, PhD Merja Roivainen, PhD, Esko Kinnunen, MD, PhD,
and Tapani Hovi, MD, PhD
Leber’s Hereditary Optic Neuropathy
Mitochondrial DNA Mutations in Multiple Efficacy of Lorenzo Oil in
Sclerosis Adrenomyeloneuropathy
H. Kellar-Wood, BSc, N. Robertson, MRCP, Ely Simon, MD
G. G. Govan, BSc, D. A. S. Compston, FRCP, Reply
and A. E. Harding, FRCP
Peter W. Kaplan, MD, MB, BS, FRCP,
Ronald Tusa, MD, PhD, Jan Shankroff, MAS,
JoEllen Heller, BA, and Hugo Moser, MD
4q35 Molecular Probes for the Diagnosis and
Letters Genetic Counseling of Facioscapulohumeral
Baclofen-induced Generalized Nonconvulsive Muscular Dystrophy
Status Epilepticus G. C. Deidda, BS, S. Cacurri, BS, 1. La Cesa, MD,
Rochelle Zak, MD, Gail Solomon, MD, C. Scoppetta, MD, and L. Felicetti, MD
Frank Petito, MD, and Douglas Labar, MD, PhD
Expanded Clinical Trials of Treatments for
Multiple Sclerosis: Copolymer 1 (COP-1)
Treatment Investigational New Drug (IND) Regular Departments
Program Message from the Editor
Yafit Stark, PhD Books
NO 2 AUGUST 1994
Editorials
Sequence Heterogeneity of HTLV-I Proviral
Will Ciliary Neurotrophic Factor Slow DNA in the Central Nervous System of Patients
Progression of Motor Neuron Disease? with HTLV-I—associated Myelopathy
Frank M. Longo, MD, PhD Jun-ichi Kira, MD, Yoshio Koyanagi, MD,
Takeshi Yamada, MD, Yasuto Itoyama, MD,
Mechanisms of Human T-Cell Lymphotropic
Jun Tateishi, MD, Shin-ichiro Akizuki, MD,
Virus Type I Viral—associated Myelopathy
Masao Kishikawa, MD, Eishi Baba, MD,
Howard L. Lipton, MD,
Minoru Nakamura, MD, Jun Suzuki, MD,
and Pradip Bandyopadhyay, PhD
Tatsufumi Nakamura, MD, Naomi Nakamura, PhD,
Naoki Yamamoto, MD, and Ikuo Goto, MD
Neurological Progress
Treatment of Abnormal Eye Movements That Biochemical Alterations in Multiple Sclerosis
Impair Vision: Strategies Based on Current Lesions and Normal-appearing White Matter
Concepts of Physiology and Pharmacology Detected by In Vivo *'P and 'H Spectroscopic
R. John Leigh, MD, Lea Averbuch-Heller, MD, Imaging
Robert L. Tomsak, MD, PhD, Bernd F. Remler, MD, C. A. Husted, PhD, D. S. Goodin, MD,
Stacy S. Yaniglos, OD, and Louis F. Dell’Osso, PhD J. W. Hugg, PhD, A. A. Maudsley, PhD,
J. 8. Tsuruda, MD, S. H. de Bie, MD, G. Fein, PhD,
G. B. Matson, PhD, and M. W. Weiner, MD
Original Articles
The Effects of Ciliary Neurotrophic Factor on Patterns of Cerebral Glucose Metabolism
Motor Dysfunction in Wobbler Mouse Motor Detected with Positron Emission Tomography
Neuron Disease Differ in Multiple System Atrophy and
Hiroshi Mitsumoto, MD, Ken Ikeda, MD, Olivopontocerebellar Atrophy
Tomas Holmlund, MD, Tom Greene, PhD, Sid Gilman, MD, Robert A. Koeppe, PhD,
Jesse M. Cedarbaum, MD, Vivien Wong, PhD, Larry Junck, MD, KarenJ . Kluin, MS,
and Ronald M. Lindsay, PhD Mary Lohman, BA, and Roy T. St Laurent, PhD
Contents of Volume 36, July-December, 1994 _ iti
Discordant Twins with Parkinson’s Disease: In Vivo Detection of Myelin Phospholipids in
Positron Emission Tomography and Early Signs Multiple Sclerosis with Phosphorus Magnetic
of Impaired Cognitive Circuits Resonance Spectroscopic Imaging
V. A. Holthoff, P. Vieregge, J. Kessler, U. Pietrzyk, C. A. Husted, PhD, G. B. Matson, PhD,
K. Herholz, J. Binner, R. Wagner, K. Wienhard, D. A. Adams, PhD, D. S. Goodin, MD,
G. Pawlik, and W.-D. Heiss and M. W. Weiner, MD
Repeated Treatment with Chimeric Anti-CD4 Positron Emission Tomographic Measurement of
Antibody in Multiple Sclerosis Cerebral Blood Flow and Temporal Lobectomy
J. W. Lindsey, MD, S. Hodgkinson, MD, William H. Theodore, William D. Gaillard,
R. Mehta, MD, D. Mitchell, MS, D. Enzmann, MD, Susumu Sato, Conrad Kufta, and Deborah Leiderman
and L. Steinman, MD
The Effect of Systemically Administered
Chromosomal Fragility Associated with Familial Recombinant Human Nerve Growth Factor in
Alzheimer’s Disease Healthy Human Subjects
Susan Ettinger, PhD, Marc E. Weksler, MD, Brent G. Petty, MD, David R. Cornblath, MD,
Xianting Zhou, PhD, John Blass, MD, Bruce T. Adornato, MD, Vinay Chaudhry, MD,
and Paul Szabo, PhD Charles Flexner, MD, Matthew Wachsman, MD, PhD,
Dominick Sinicropi, PhD, Louis E. Burton, PhD,
Autoantibodies Against the Hel-N1 and Stephen J. Peroutka, MD, PhD
RNA-binding Protein among Patients with
Lung Carcinoma: An Association with Type I
Anti—Neuronal Nuclear Antibodies
Edward J. Dropcho, MD, and Peter H. King, MD
Decreased Tyrosine Hydroxylase Content in the Special Article
Dopaminergic Neurons of MPTP-intoxicated The Denny-Brown Collection: A Research and
Monkeys: Effect of Levodopa and GM1 Teaching Resource
Ganglioside Therapy JoelA . Vilensky, PhD, Sid Gilman, MD,
A. Kastner, PhD, M. T. Herrero, MD, PhD, and Elaine M. Dec, MSW
E. C. Hirsch, PhD, J. Guillen, PhD,
M. R. Luquin, MD, PhD, F. Javoy-Agid, PhD,
J. A. Obeso, MD, PhD, and Y. Agid, MD, PhD
Decreased Levels of Soluble Amyloid B-Protein
Precursor Are Associated with Alzheimer’s Letters
Disease in Concordant and Discordant Andersen’s Syndrome? Or Klein-Lisak-Andersen
Monozygous Twin Pairs Syndrome?
Steven L. Wagner, PhD, Elaine R. Peskind, MD, Lewis P. Rowland, MD
David Nochlin, MD, Sally Provow, BS,
Reply
Jeffrey S. Farrow, BA, M. R. Pandian, PhD,
Rabi Tawil, MD, Louis J. Ptacek, MD,
Michelle Cleveland, BS, Ralph K. Ito, PhD,
Steven G. Pavlakis, MD, Darryl C. DeVivo, MD,
and Martin R. Farlow, MD
Audrey S. Penn, MD, Coskun Ozdemir, MD,
Role of Nerve Growth Factor in Suramin and Robert C. Griggs, MD
Neurotoxicity Studied In Vitro
Trial of Immunosuppression in Amyotrophic
James W. Russell, MD, Anthony J.W indebank, MD,
Lateral Sclerosis Using Total Lymphoid
and Jewel L. Podratz
Irradiation
LannyJ . Haverkamp, PhD,
R. Glenn Smith, MD, PhD, and Stanley H. Appel, MD
Reply
Brief Communications
Daniel B. Drachman, MD, Vinay Chaudhry, MD,
X-linked Pachygyria and Agenesis of the Corpus
David Cornblath, MD, Ralph W. Kuncl, MD, PhD,
Callosum: Evidence for an X Chromosome
Alan Pestronk, MD, Lora Clawson, RN, BSN,
Lissencephaly Locus
E. David Mellits, ScD, Shirley Quaskey, BS,
Elizabeth Berry-Kravis, MD, PhD,
Thomas Quinn, MD, Allison Calkins, MD,
and Jeanette Israel, MD
and Stanley Order, MD
Syncope: A Videometric Analysis of 56 Episodes
of Transient Cerebral Hypoxia
T. Lempert, MD, M. Bauer, MD, and D. Schmidt, MD
Decreased Dopamine Transporters with Age in Regular Departments
Healthy Human Subjects
119th Annual Meeting of the American
N. D. Volkow, MD,J . S. Fowler, PhD,
Neurological Association
G.J . Wang, MD, J. Logan, PhD, D. Schlyer, PhD,
R. MacGregor, PhD, R. Hitzemann, PhD, Program
and A. P. Wolf, PhD Abstracts
iv Contents of Volume 36, July-December, 1994
NO 3 SEPTEMBER 1994
387 Genetic Linkage Studies in Autosomal Dominant
Editorials
Parkinsonism: Evaluation of Seven Candidate
The Brain on Fire? Genes
Gerald Cohen, PhD T. Gasser, MD, Z. K. Wszolek, MD, J. Trofatter. PhD,
L. Ozelius, BS, R. J. Uitti, MD, C. S. Lee, MD,
Familial Alzheimer’s Disease
J. Gusella, PhD, R. F. Pfeiffer, MD, D. B. Calne, MD,
Thomas D. Bird, MD
and X. O. Breakefield, PhD
Voluntary Movement at the Elbow in Spastic
Hemiparesis
S.J. Fellows, PhD, C. Kaus, andA . F. Thilmann, MD
Neurological Progress
Ultrastructural Morphometric Abnormalities of
Vestibular Syndromes in the Roll Plane:
Sural Nerve Endoneurial Microvessels in
Topographic Diagnosis from Brainstem to
Diabetes Mellitus
Cortex
Caterina Giannini, MD, and Peter James Dyck, MD
Thomas Brandt, MD, and Marianne Dieterich, MD
Frequency and Clinical Correlates of
Anti—Neural IgM Antibodies in Neuropathy
Associated with IgM Monoclonal Gammopathy
E. Nobile-Orazio, MD, PhD, E. Manfredini, MD,
Original Articles M. Carpo, MD, N. Meucci, MD, S. Monaco, MD,
Alterations in Glutathione Levels in Parkinson’s S. Ferrari, MD, B. Bonetti, MD, G. Cavaletti, MD,
Disease and Other Neurodegenerative Disorders F. Gemignani, MD, L. Durelli, MD,
Affecting Basal Ganglia S. Barbieri, MD, PhD, S. Allaria, M. Sgarzi, MD,
Jeswinder Sian, BSc, David T. Dexter, PhD, and G. Scarlato, MD
AndrewJ . Lees, FRCP, Susan Daniel, MRCPath,
Yves Agid, MD, PhD, France Javoy-Agid, PhD, Phenobarbital Modifies Seizure-related Brain
Peter Jenner, DSc, and C. David Marsden, FRS Injury in the Developing Brain
Mohamad A. Mikati, MD, Gregory L. Holmes, MD,
Glutathione-related Enzymes in Brain in Antonia Chronopoulos, BA, Pamela Hyde, BS,
Parkinson’s Disease Samuel Thurber, BS, Arkadi Gatt,
J. Sian, BSc, D. T. Dexter, PhD, A. J. Lees, FRCP, Zhao Liu, MD, PhD, Suzanne Werner, BA,
S. Daniel, MRCPath, P. Jenner, DSc, and Carl E. Stafstrom, MD, PhD
and C. D. Marsden, FRS
Chromosome 14—encoded Alzheimer’s Disease:
Genetic and Clinicopathological Description
Matti Haltia, MD, Matti Viitanen, MD, Brief Communications
Raimo Sulkava, MD, Veli Ala-Hurula, MD, The Apolipoprotein E €4 Allele Does Not
Minna Poyhonen, MD, Lev Goldfarb, MD, Influence the Clinical Expression of the Amyloid
Paul Brown, PhD, Efrat Levy, PhD, Precursor Protein Gene Codon 693 or 692
Henry Houlden, MBBS, Richard Crook, BSc, Mutations
Alison Goate, PhD, Robert Clark, PhD, Joost Haan, MD, PAD,
Kevin Korenblat, BSc, Sunil Pandit, PhD, Christine Van Broeckhoven, PhD,
Helen Donis Keller, PhD, Lena Lilius, BSc, Li Liu, BSc, Cornelia M. van Duijn, PhD, Els Voorhoeve, MSc,
Karin Axelman, RN, Lotta Forsell, BSc, Frans van Harskamp, MD,
Bengt Winblad, PhD, MD, Lars Lannfelt, MD, John C. van Swieten, MD, PhD,
and John Hardy, PhD Marion L. C. Maat-Schieman, MD,
Raymund A. C. Roos, MD, PAD,
Phenotype of Chromosome 14—linked Familial and Egbert Bakker, PhD
Alzheimer’s Disease in a Large Kindred
Thomas H. Lampe, MD, Thomas D. Bird, MD, Immune Complex Deposition in the Choroid
David Nochlin, MD, Ellen Nemens, RN, MN, Plexus of Patients with Acquired
Steven C. Risse, MD, S. Mark Sumi, MD, Immunodeficiency Syndrome
Richard Koerker, MD, Brie Leaird, PA-C, M. F. Falangola, MD, B. G. Castro-Filho, MD,
Monna Wier, PhD, and Murray A. Raskind, MD and C. K. Petito, MD
Increased Transforming Growth Factor-B, Decreased Cerebrospinal Fluid Levels of
Interleukin-4, and Interferon-y in Multiple B-Endorphin in Japanese Patients with Joseph
Sclerosis Disease
Joanne Link, MD, PhD, Mats Séderstrim, MD, PhD, Toyojiro Matsuishi, MD, Tetsuo Sakai, MD,
Tomas Olsson, MD, PhD, Bo Héjeberg, MD, PhD, Shinichiro Nagamitsu, MD, Hironori Komori, MD,
Ake Ljungdahl, MD, PhD, and Hans Link, MD, PhD Hiroshi Iwashita, MD, and Hirohisa Kato, MD
Contents of Volume 36, July-December, 1994 v
Homozygous Inheritance of the Machado-Joseph Reply
Disease Gene Hiroo Yoshikawa, MD, Tomoya Nishimura, MD,
Anthony E. Lang, MD, E. A. Rogaeva, PhD, Saburo Sakoda, MD, and Takehiko Yanagihara, MD
T. Tsuda, MD, PhD, J. Hutterer, PhD,
and Peter St George-Hyslop, MD 3,4-Diaminopyridine in Demyelinating
Peripheral Neuropathies
Ryuji Tamura, MD, Yukio Mano, MD, PhD,
Letters
Tetsuya Takayanagi, MD, and Richard F. Mayer, MD
Emerging Ipsilateral Corticospinal Pathways
after Stroke? Reply
Antoinette Maassen van den Brink, Peter S. Bergin, FRACP, David H. Miller, FRACP,
Wim van der Kamp, MD, Nicholas P. Hirsch, FFARCS,
andJ . Gert van Dijk, MD, PhD and Nicholas M. F. Murray, FRCP
Reply The Use of Potassium Channel Blocking Agents
E. Palmer, MSc, P. Ashby, MD, and V. E. Hajek, MD in the Therapy of Demyelinating Diseases
Paul A. Felts, PhD, and Kenneth J. Smith, PhD
Mechanism of Action of Botulinum Toxin
Mark Hallett, MD, Franz X. Glocker, MD, Reply
and Giinther Deuschl, MD Nicholas M. F. Murray, FRCP
Utility of Suggestion-induced Spells in The Clinical Correlates of High-Titer IgG
Diagnosis of Pseudoseizures Anti-GM1 Antibodies
David N. Levine, MD Thomas A. Ala, MD, and William H. Frey II, PhD
Reply Reply
Marcelo E. Lancman, MD AndrewJ . Kornberg, MB, BS, FRACP,
and Alan Pestronk, MD
Predictive Value of Induction of Psychogenic
Seizures by Suggestion
DouglasJ . Lanska, MD, MS
Reply Regular Departments
Marcelo E. Lancman, MD Books
Correction
Elevated Expression of Messenger RNA for
Peripheral Myelin Protein 22 in Biopsied Conjoint Meeting of the Child Neurology
Peripheral Nerves of Patients with Society and International Child Neurology
Charcot-Marie-Tooth Disease Type 1A Association
John Kamholz, MD, PhD, Michael Shy, MD, 458 Program
and Steven Scherer, MD, PhD 486 CNS Abstracts
NO 4 OCTOBER 1994
577 Selective Vulnerability in Huntington’s Disease:
Editorials
Preferential Loss of Cannabinoid Receptors in
The Ischemic Penumbra, Injury Thresholds, and
Lateral Globus Pallidus
the Therapeutic Window for Acute Stroke
Eric K. Richfield, MD, PhD,
Myron D. Ginsberg, MD,
and Miles Herkenham, PhD
and William A. Pulsinelli, MD, PhD
The Sense of Antisense fos Oligonucleotides
Frank R. Sharp, MD
Effects of Edrophonium on Saccadic Velocity in
Normal Subjects and Myasthenic and
Neurological Progress Nonmyasthenic Ocular Palsies
Viability Thresholds and the Penumbra of Focal JasonJ . §. Barton, MD, FRCPC,
Ischemia Ana G. Huaman, MD,
K-A. Hossmann, MD, PhD and James A. Sharpe, MD, FRCPC
Original Articles
Suppression of Ischemia-induced Fos Expression Multislice Proton Magnetic Resonance
and AP-1 Activity by an Antisense Spectroscopic Imaging in X-linked
Oligodeoxynucleotide to c-fos mRNA Adrenoleukodystrophy
Philip K. Liu, PhD, Antero Salminen, PhD, Bernd Kruse, MD, Peter B. Barker, DPhil,
Ye Y. He, MD, Ming H.J iang, MD, J. J. Xue, MD, Peter C. M. van Zijl, PhD, Jeff H. Duyn, PhD,
Jia S. Liu, MD, and Chung Y. Hsu, MD, PhD Chrit T. W. Moonen, PhD, and Hugo W. Moser, MD
Contents of Volume 36, July-December, 1994
Band Heterotopia: Correlation of Outcome with Molecular Genetic Studies of Muscle Lactate
Magnetic Resonance Imaging Parameters Dehydrogenase Deficiency in White Patients
A. J. Barkovich, MD, R. Guerrini, MD, Seiichi Tsujino, MD, Sara Shanske, PhD,
G. Battaglia, MD, G. Kalifa, MD, T. N’Guyen, MD, A. Keith W. Brownell, MD, Ronald G. Haller, MD,
A. Parmeggiani, MD, M. Santucci, MD, and Salvatore DiMauro, MD
P. Giovanardi-Rossi, MD, T. Granata, MD,
and L. D'Incerti, MD Autoantibodies to Glutamic Acid Decarboxylase
in Palatal Myoclonus and Epilepsy
Magnetic Stimulation of Corticospinal Pathways R. Nemni, MD, S. Braghi, MD,
at the Foramen Magnum Level in Humans M. G. Natali-Sora, MD, V. Lampasona, BSc,
Yoshikazu Ugawa, MD, Yoshikazu Uesaka, MD, E. Bonifacio, PhD, G. Comi, MD, and N. Canal, MD
Yasuo Terao, MD, Ritsuko Hanajima, MD,
and Ichiro Kanazawa, MD Age-dependent Decline of Nigrostriatal
Dopaminergic Function: A Positron Emission
Neurological Complications of Peripheral and Tomographic Study of Grandparents and Their
Cutaneous T-Cell Lymphomas Grandchildren
David K. Kaufman, MD, Michael Cordes, Barry J. Snow, Sandra Cooper,
Thomas M. Habermann, MD, PaulJ . Kurtin, MD, Michael Schulzer, Brian D. Pate, Thomas J. Ruth,
and B. P. O’Neill, MD and Donald B. Calne
Trinucleotide Repeat Length and Rate of In Situ Polymerase Chain Reaction
Progression of Huntington’s Disease Demonstration of JC Virus in Progressive
Sergei N. Illarioshkin, MD, Shuichi Igarashi, MD, Multifocal Leukoencephalopathy, Including an
Osamu Onodera, MD, Elena D. Markova, MD, Index Case
Natalya N. Nikolskaya, MD, Hajime Tanaka, MD, Keisuke Ueki, MD, Edward P. Richardson,J r,M D,
Tina Z. Chabrashwili, MD, John W. Henson, MD, and David N. Louis, MD
Nina G. Insarova, MD, Kotaro Endo, MD,
Irina A. Ivanova-Smolenskaya, MD, PhD, Human Immunodeficiency Virus Encephalitis Is
and Shoji Tsuji, MD, PhD the Pathological Correlate of Dementia in
Acquired Immunodeficiency Syndrome
Red Nuclear and Cerebellar but No Olivary Clayton A. Wiley, MD, PhD,
Activation Associated with Essential Tremor: and Cristian Achim, MD, PhD
A Positron Emission Tomographic Study
A.J . Wills, MRCP, I. H. Jenkins, MRCP,
P. D. Thompson, PhD, FRACP,
L. J. Findley, MD, FRCP,
and D. J. Brooks, MD, FRCP Letters
Polymyositis in Patients Infected with Human Mechanism of Action of the Anticonvulsant
T-Cell Leukemia Virus Type I: The Role of the Felbamate: Opposing Effects on
Virus in the Cause of the Disease N-Methyl-p-Aspartate and y-Aminobutyric
Marta Leon-Monzon, PhD, Isabel Illa, MD, Acid, Receptors
and Marinos C. Dalakas, MD R. Duane Sofia, PhD
Prevalence of the 1.5-Mb 17p Deletion in Reply
Families with Hereditary Neuropathy with Jong M. Rho, MD, Sean D. Donevan, PhD,
Liability to Pressure Palsies Swaminathan Subramaniam, MD, PhD,
E. C. M. Mariman, PhD, and Michael A. Rogawski, MD, PhD
A. A. W. M. Gabreéls-Festen, MD, PhD,
Visual Grasp in Corticobasal Degeneration
S. E. C. van Beersum, L. J. Valentijn, F. Baas, PhD,
Daniel H. Jacobs, MD, John C. Adair, MD,
P. A. Bolhuis, PhD, P. J. H. Jongen, MD,
and Kenneth M. Heilman, MD
H. H. Ropers, MD, PAD,
and F. J. M. Gabreéls, MD, PhD Reply
M. Vidailhet, MD, S. Rivaud,
Brief Communications N. Gouider-Khouja, MD, B. Pillon, PhD,
A. M. Bonnet, MD, B. Gaymard, MD,
Dose-dependent Association of Apolipoprotein
Y. Agid, MD, PAD, and C. Pierrot-Deseilligny, MD
E Allele €4 with Late-Onset, Sporadic
Alzheimer’s Disease Muscle Carnitine Deficiency Associated with
Toshihiro Yoshizawa, MD, Zidovudine-induced Mitochondrial Myopathy
Kimiko Yamakawa-Kobayashi, PhD, Yolanda Campos, MSc, and Joaquin Arenas, PhD
Yasuko Komatsuzaki, MD, Tadao Arinami, MD,
E7’ichi Oguni, MD, Hidehiro Mizusawa, MD, Reply
Shin’ichi Shoji, MD, and Hideo Hamaguchi, MD Marinos C. Dalakas, MD
Increased Risk of Parkinson’s Disease in Parents Apolipoprotein E-e4 Allele Doses in Late-Onset
and Siblings of Patients Alzheimer’s Disease
Haydeh Payami, PhD, Kimberly Larsen, BA, Gérard Lucotte, PhD, Jean-Claude Turpin, MD,
Steven Bernard, MS, and John Nutt, MD and Paul Landais, MD
Contents of Volume 36, July-December, 1994 vii
NO 5 NOVEMBER 1994
759 Longitudinal Fluorodopa Positron Emission
Brief Review
Tomographic Studies of the Evolution of
Clinical Trials in a Changing Era Idiopathic Parkinsonism
Guy M. McKhann, MD F. J. G. Vingerhoets, MD,
B. J. Snow, MD, FRACP, FRCPC, C. S. Lee, MD,
M. Schulzer, PhD, MD, E. Mak, BASc,
and D. B. Calne, DM, FRCPC
Neurological Progress
Positron Emission Tomographic Evidence for
Neurological Complications of Organ Progression of Human MPTP-induced
Transplantation Dopaminergic Lesions
Roy A. Patchell, MD F. J. G. Vingerhoets, MD,
B.J .S now, MD, FRACP, FRCPC,J . W. Tetrud, MD,
J. W. Langston, MD, M. Schulzer, MD, PAD,
and D. B. Calne, DM, FRCPC
Original Articles
Dystonia in Ashkenazi Jews: Clinical
Oral Administration of Acetylcholine Receptor:
Characterization of a Founder Mutation
Effects on Experimental Myasthenia Gravis
Susan B. Bressman, MD, Deborah de Leon, MS,
Seiichi Okumura, MD, Kevin Mclntosh, PhD,
Patricia L. Kramer, PhD, Laurie J. Ozelius, BS,
and Daniel B. Drachman, MD
Mitchell F. Brin, MD, Paul E. Greene, MD,
The Retinoblastoma Gene Is Involved in Stanley Fahn, MD, Xandra O. Breakefield, PhD,
Malignant Progression of Astrocytomas and NeilJ . Risch, PhD
John W. Henson, MD, Bernd L. Schnitker, BS,
Induction of Nitric Oxide Synthase in
Katia M. Correa, BS, Andreas von Deimling, MD,
Demyelinating Regions of Multiple Sclerosis
Frank Fassbender, BS, Hong-Ji Xu, MD, PhD,
Brains
William F. Benedict, MD, David W. Yandell, PhD,
Lars Bé, MD, Ted M. Dawson, MD, PhD,
and David N. Louis, MD
Steven Wesselingh, BMBS, PhD, Sverre Mérk, MD,
Butyrylcholinesterase Reactivity Differentiates Steven Choi, BSc, Pei Ann Kong, BSc,
the Amyloid Plaques of Aging from Those of Daniel Hanley, MD, and Bruce D. Trapp, PhD
Dementia
M.-Marsel Mesulam, MD, and Changiz Geula, PhD
The Shoulder-Hand Syndrome after Stroke:
A Prospective Clinical Trial
Dieter F. Braus, MD, Joachim K. Krauss, MD,
Brief Communications
and Jorg Strobel, MD
Extracellular Human T-Cell Lymphotropic
Myelin Basic Protein Peptide Specificity and Virus Type I Tax Protein Induces Cytokine
T-Cell Receptor Gene Usage of HPRT Mutant Production in Adult Human Microglial Cells
T-Cell Clones in Patients with Multiple Sclerosis Suhayl Dhib-Jalbut, MD, Paul M. Hoffman, MD,
Patricia A. Lodge, PhD, Mark Allegretta, PhD, Toshio Yamabe, MSc, Di Sun, PhD, Jane Xia, MSc,
Lawrence Steinman, MD, Howard Eisenberg, MD, Gregory Bergey, MD,
and Subramaniam Sriram, MD and Francis W. Ruscetti, PhD
Very Long Chain Fatty Acids in X-linked Molecular Mimicry Between GQ, Ganglioside
Adrenoleukodystrophy Brain after Treatment and Lipopolysaccharides of Campylobacter jejuni
with Lorenzo’s Oil Isolated from Patients with Fisher’s Syndrome
Alfred Poulos, PhD, Robert Gibson, PhD, N. Yuki, MD, PhD, T. Taki, PhD, M. Takahashi,
Peter Sharp, BSc, Kaye Beckman, Med Tech, K. Saito, H. Yoshino, MD, T. Tai, MD, PAD,
and Padraic Grattan-Smith, MBBS, MRCP, FRACP S. Handa, MD, PhD, and T. Miyatake, MD, PhD
Oxidative Damage to Mitochondrial DNA Is Metabolic Recovery in Caudate Nucleus of
Increased in Alzheimer’s Disease Children Following Cerebral Hemispherectomy
Patrizia Mecocci, MD, Usha MacGarvey, MS, Harry T. Chugani, MD, and Bob Jacobs, PhD
and M. Flint Beal, MD
Apolipoprotein E ¢4 Allele Distribution in
Cytokine Expression in the Muscle of Alcoholic Dementia and in Alzheimer’s
HIV-infected Patients: Evidence for Disease in Japan
Interleukin-la Accumulation in Mitochondria Taro Muramatsu, MD, Susumu Higuchi, MD, PhD,
of AZT Fibers Hiroyuki Arai, MD, PhD,
R. K. Gherardi, MD, A. Florea-Strat, MD, Hidetada Sasaki, MD, PhD, Koichi Yamada, MD,
G. Fromont, MD, F. Poron, J-C. Sabourin, MD, Motoi Hayashida, MD, ScD, and
andJ . Authier, MD John Q. Trojanowski, MD, PhD
Vili Contents of Volume 36, July-December, 1994
Reply
Letters
Charles A. Thornton, MD, Robert C. Griggs, MD,
Basic Fibroblast Growth Factor and Muscular
and Richard T. Moxley Ill, MD
Dystrophy
J. P. Lefaucheur, MD, and A. Sébille, MD Carbamazepine-induced Atrioventricular Block
in a Child with Tuberous Sclerosis and Cardiac
Reply
Rhabdomyoma
Patricia A. D’Amore, PhD, and Eric P. Hoffman, MD
Timothy K. Knilans, MD
Reduced Glucose Transporter Concentrations in
Reply
Brains of Patients with Alzheimer’s Disease
Phyllis Pollack, MD, and Spencer G. Weig, MD
lan A. Simpson, PhD, and Peter Davies, PhD
Ready or Not
Reply
David Goldblatt, MD
William M. Pardridge, MD
No Structural or Biochemical Evidence for
Dopa-responsive Dystonia
Mitochondrial Cytopathy in a Case of
Guy V. Sawle, MRCP, DM,
Alternating Hemiplegia of Childhood
and C. David Marsden, FRS, DSc
Theodore Kyriakides, MB, ChB, MCRP(UK), and
Reply Anthi Drousiotou, PhD
Donald B. Calne, DM, FRCPC
Thornton-Griggs-Moxley Disease: Myotonic
Regular Departments
Dystrophy Type 2
Books
Lewis P. Rowland, MD
NO 6 DECEMBER 1994
846 The Role of Calcium-binding Proteins in
Editorials
Selective Motoneuron Vulnerability in
Polymerase Chain Reaction and the Diagnosis of
Amyotrophic Lateral Sclerosis
Viral Central Nervous System Diseases
Marta E. Alexianu, MD, Bao-Kuang Ho, MD,
Kenneth L. Tyler, MD
A. Habih Mohamed. PhD, Vincenzo La Bella, MD,
Oxygen Utilization and Delivery in Metabolic R. Glenn Smith, MD, PhD, and Stanley H. Appel, MD
Myopathies
Childhood Guillain-Barré Syndrome in
Ronald G. Haller, MD
Paraguay, 1990 to 1991
David E. Hart, MD, Laura A. Rojas. MD,
Joana A. Rosério,. MD, MPH, Humberto Recalde, MD,
Neurological Progress
and Gustavo C. Roman, MD
Trinucleotide Repeat Expansion in Neurological
Disease Evidence for Hypoxia-induced, Programmed
Albert R. La Spada, MD, PhD, Cell Death of Cultured Neurons
Henry L. Paulson, MD, PhD, Daniel M. Rosenbaum, MD, Marc Michaelson, BA,
and Kenneth H. Fischbeck, MD David K. Batter, PhD, Pooja Doshi, BA,
and John A. Kessler, MD
Original Articles Manganese Intoxication and Chronic Liver
Herpes Simplex Virus Genome in Human Failure
Nervous System Tissue Analyzed by Polymerase Robert A. Hauser, MD, Theresa A. Zesiewicz, MD,
Chain Reaction Alexander §. Rosemurgy, MD, Carlos Martinez, MD,
J. Richard Baringer, MD, and Pamela Pisani, BS and C. W. Olanow, MD, FRCP(C)
An Oxidative Defect in Metabolic Myopathies: Complex I, Iron, and Ferritin in Parkinson’s
Diagnosis by Noninvasive Tissue Oximetry Disease Substantia Nigra
William Bank, MD, and Britton Chance, PhD V. M. Mann, PhD,J . M. Cooper, PhD,
S. E. Daniel, FRCPath, K. Srai, PhD, P. Jenner, DSc,
A Plasma Exchange Versus Immune Globulin C. D. Marsden, FRS, and A. H. V. Schapira, MD
Infusion Trial in Chronic Inflammatory
Demyelinating Polyradiculoneuropathy Coenzyme Q,,. and Nicotinamide Block Striatal
Peter James Dyck, MD, William J. Litchy, MD, Lesions Produced by the Mitochondrial Toxin
Kay M. Kratz, Guillermo A. Suarez, MD, Malonate
Phillip A. Low, MD, Alvaro A. Pineda, MD, M. Flint Beal, MD, D. Ross Henshaw, BA,
AnthonyJ .W indebank, MD, Jeannine L. Karnes, MSc, Bruce G. Jenkins, PhD, Bruce R. Rosen, MD, PAD,
and Peter C. O’Brien, PhD and Jirg B. Schulz, MD
Contents of Volume 36, July-December, 1994 ix
Predictive Value of Apolipoprotein E High-Dose Dextromethorphan in Amyotrophic
Genotyping in Alzheimer’s Disease: Results of an Lateral Sclerosis: Phase I Safety and
Autopsy Series and an Analysis of Several Pharmacokinetic Studies
Combined Studies Dave Hollander, MD, FRCP(C), Jesus Pradas, MD,
Josephine Nalbantoglu, PhD, Richard Kaplan, PhD, Howard L. McLeod, PharmD,
Brian M. Gilfix, MDCM, PhD, William E. Evans, PharmD,
Philippe Bertrand, PhD, Yves Robitaille, MD, and Theodore L. Munsat, MD
Serge Gauthier, MD, David S. Rosenblatt, MD,
and Judes Peirer, PhD
Immunological and Pathological Study of
Brief Communications
Anti-Ri—associated Encephalopathy
Adilia Hormigo, MD, Josep Dalmau, MD, PhD, Hippocampal Neuronal Loss and Regional
Marc K. Rosenblum, MD, Mary E. River, MD, Hypometabolism in Temporal Lobe Epilepsy
and Jerome B. Posner, MD Thomas R. Henry, MD, Thomas L. Babb, PhD,
Jerome Engel, Jr, MD, PhD,
Amyloid B Protein Levels in Cerebrospinal Fluid John C. Mazziotta, MD, PhD,
Are Elevated in Early-Onset Alzheimer’s Disease Michael E. Phelps, PhD, and Paul H. Crandall, MD
Tamiko Nakamura, MD, Mikio Shojt, MD, PhD,
Yasuo Harigaya, MD, PhD, Neuropathologic Correlates of Persistent
Mitsunori Watanabe, MD, Kenji Hosoda, PhD, Neurologic Deficit in Lithium Intoxication
Tobun T. Cheung, PhD, Lillian M. Shaffer, PhD, Julie A. Schneider, MD, and Suzanne S. Mirra, MD
Todd E. Golde, PhD, Linda H. Younkin, MD, PhD,
Steven G. Younkin, MD, PhD,
and Shunsaku Hirai, MD, PhD Letters
Limitations of Magnetic Resonance
Phenotypic Heterogeneity of an Adult Form of
Spectroscopy in Patients with White Matter
Adrenoleukodystrophy in Monozygotic Twins
Disease
Gen Sobue, MD, Ikuko Ueno-Natsukari, MD, PhD,
David M. Yousem, MD,
Hideki Okamoto, MD, Thomas A. Connell, MD,
David H. Gutmann, MD, PhD,
Izumi Aizawa, MD, Koichi Mizoguchi, MD,
and Robert Lenkinski, PhD
Masamitsu Honma, PhD, Gen Ishikawa, MD, PhD,
Terunori Mitsuma, MD, Reply
and Naoki Natsukari, MD, PhD Jeffry R. Alger, PhD, Raphael Schiffman, MD,
and Norman W. Barton, MD, PhD
Pelizaeus-Merzbacher Disease Presenting as
Spinal Muscular Atrophy: Clinical and
Molecular Studies
Indexes to Volume 36
Edward M. Kaye, MD, Rhonda F. Doll, BS,
Marvin R. Natowicz, MD, PhD, Author Index
and Frances I. Smith, PhD Subject Index
SUPPLEMENTS TO VOLUME 36
Multiple Sclerosis: Approaches to Management
Introduction $12 Genetics and Multiple Sclerosis: An Overview
Donald H. Silberberg, MD George C. Ebers, MD, FRCPC
The Evolution of Multiple
Human Leukocyte Antigen Studies in Multiple
Sclerosis Sclerosis
The Evolution of Multiple Sclerosis Jan Hillert, MD
Epidemiology
Leonard T. Kurland, MD, DrPH
$18 Genetics of Multiple Sclerosis in British
Natural History of Multiple Sclerosis Columbia and Throughout Canada
Brian G. Weinshenker, MD A. Dessa Sadovnick, PhD
x Contents of Volume 36, July-December, 1994