Annals of Neurology 1997: Vol 41 Table of Contents

9 Pages·1997·2.5 MB·English

Annals of
Neurology
Vol 41 January ¥ June 1997
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 Thomas D. Bird, MD, Seattle
San Francisco, CA
Robert C. Collins, MD, Los Angeles
Founding Editor, 1977-1984 David R. Cornblath, MD, Baltimore
Fred Plum, MD Jasper R. Daube, MD, Rochester, MN
Editor, 1985-1992 Kenneth H. Fischbeck, MD,
Arthur K. Asbury, MD Philadelphia, PA
Richard S. J. Frackowiak, MD, London
Associate Editors
Frank M. Longo, MD, PhD, Kirk A. Frey, MD, PhD, Ann Arbor
San Francisco David A. Greenberg, MD, PhD,
Stephen M. Sagar, MD, Pittsburgh
San Francisco Stephen L. Hauser, MD, San Francisco
Associate Editor for Gregory L. Holmes, MD, Boston
Child Neurology Edward H. Koo, MD, Boston
Joseph J. Volpe, MD, Boston Bruce R. Korf; MD, PhD, Boston
Richard P. Mayeux, MD, New York
Editorial Coordinator
Marilyn Stubblebine Henry F. McFarland, MD, Bethesda
Jerry R. Mendell, MD, Columbus, OH
Publisher
John G. Nutt, MD, Portland, OR
Lippincott-Raven Publishers
Peter C. O’Brien, PhD, Rochester, MN
Philadelphia, Pennsylvania
John B. Penney, Jr, MD, Boston
Publishing Staff Alan K. Percy, MD, Birmingham
Ronald C. Petersen, MD, PhD,
Marcia Serepy
Rochester, MN
Executive Publisher
Louis J. Ptacek II, MD, Salt Lake City
Grace R. Caputo Takehiko Yanagihara, MD, Osaka,
Senior Production Editor
Japan
Nicholas Minicucci Jr.
Publishing Co., Inc
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P U B i S&S W FE 8 Ss
NO 1 JANUARY 1997
74 Preton Magnetic Resonance Spectroscopic Imaging for
Message from the Editor Discrimination of Absence and Complex Partial
Robert A. Fishman, MD Seizures
Fernando Cendes, MD, Jeff A. Stanley, PhD,
Francois Dubeau, MD, Frederick Andermann, MD, and
Douglas L. Arnold, MD
Editorials
Reversible Schwann Cell Hyperplasia and Sprouting of
Recent Advances in Heritable Ataxias
Sensory and Sympathetic Neurites after Intraventricular
Orest Hurko, MD
Administration of Nerve Growth Factor
The Pathology of Alzheimer’s Disease: Numbers Count Jiirgen Winkler, MD, Gilbert A. Ramirez, MS,
Robert D. Terry, MD H. Georg Kuhn, PhD, Daniel A. Peterson, PhD,
Patricia A. Day-Lollini, PhD, Gregory R. Stewart, PhD,
Mark A. Tuszynski, MD, PhD, Fred H. Gage, PhD, and
Leon J. Thal, MD
Original Articles Skew Deviation Following Vestibular Nerve Surgery
Familial Episodic Ataxia: Clinical Heterogeneity in Four P. Riordan-Eva, FRCOphth, J. P. Harcourt, FRCS,
Families Linked to Chromosome 19p M. Faldon, PhD, G. B. Brookes, FRCS, and
Robert W. Baloh, MD, Qing Yue, MD, M. A. Gresty, PhD
Joseph M. Furman, MD, PhD, and Stanley F. Nelson, MD
Neuronal Loss Correlates with but Exceeds
Neurofibrillary Tangles in Alzheimer’s Disease
Teresa Gémez-Isla, MD, Richard Hollister, BA,
Brief Communications
Howard West, MD, Stina Mui, BA, John H. Growdon, MD,
Ronald C. Petersen, MD, PhD, Joseph E. Parisi, MD, and Inclusion Body Myositis and Paraproteinemia:
Bradley T. Hyman, MD, PhD Incidence andI pathologic Correlations
Marinos C. Dalakas, MD, Isabel Illa, MD,
A Rapidly Progressive Adolescent-onset Eduard Gallardo, PhD, and Candido Juarez, PhD
Oculopharyngeal Somatic Syndrome with Rimmed
Vacuoles in Two Siblings Patients Homozygous for the 17p11.2 Duplication in
Michael R. Rose, MD, David N. Landon, MB, BS, Charcot-Marie-Tooth Type 1A Disease
Alex Papadimitriou, MD, and John A. Morgan-Hughes, MD E. LeGuern, MD, PhD, R. Gouider, MD, D. Mabin, MD,
S. Tardieu, BS, N. Birouk, MD, P. Parent, MD,
Activation Mapping in Essential Tremor with P. Bouche, MD, and A. Brice, MD
Functional Magnetic Resonance Imaging
Stefan F. Bucher, PhD, Klaus C. Seelos, MD, Stereotactic Thalamotomy in Tremor-dominant
Richard C. Dodel, MD, Maximilian Reiser, MD, and Parkinson’s Disease: An H,'°O PET Motor Activation
Wolfgang H. Oertel, MD Study
Henning Boecker, MD, Adrian J. Wills, MRCP,
The Spectrum of Hippocampal Sclerosis: A Quantitative Andres Ceballos-Baumann, MD, Michael Samuel, MRCP,
Magnetic Resonance Imaging Study David G. T. Thomas, FRCS, C. David Marsden, FRS, and
W. Van Paesschen, MRCP, A. Connelly, PhD, David J. Brooks, MD, FRCP
M. D. King, PhD, G. D. Jackson, FRACP, and
J. S. Duncan, FRCP Adult-onset Krabbe’s Disease in Siblings with Novel
Mutations in the Galactocerebrosidase Gene
Amyloid (A&) Deposition in Chromosome 1-linked Gary L. Bernardini, MD, PhD,
Alzheimer’s Disease: The Volga German Families Daniel G. Herrera, MD, PhD, Debbie Carson, RN,
D. M. A. Mann, T. Iwatsubo, D. Nochlin, S. M. Sumi, Rita DeGasperi, PhD, Miguel A. Gama Sosa, PhD,
E. Levy-Lahad, and T. D. Bird Edwin H. Kolodny, MD, and Rosario Trifiletti, MD, PhD
Which Clinical Sign of Parkinson’s Disease Best Mitochondrial Function in Muscle from Elderly
Reflects the Nigrostriatal Lesion? Athletes
Francois J. G. Vingerhoets, MD, Elizabeth J. Brierley, MB BS, MRCP,
Michael Schulzer, MD, PhD, Margaret A. Johnson, BSc, PhD,
Donald B. Calne, DM, FRCPC, and Andrew Bowman, MB ChB, MRCP,
Barry J. Snow, MD, FRACP, FRCPC Gary A. Ford, MB BChir, FRCP, Feisal Subhan, BSc,
James W. Reed, BSc, PhD, Oliver F. W. James, BCh, FRCP,
Bilateral Parasagittal Parietooccipital Polymicrogyria and Douglass M. Turnbull, MB BS, MD, PhD, FRCP
and Epilepsy
R. Guerrini, MD, F. Dubeau, MD, O. Dulac, MD, X-Linked Vacuolated Myopathy: Membrane Attack
A. J. Barkovich, MD, R. Kuzniecky, MD, C. Fett, MD, Complex Deposition on Muscle Fiber Membranes with
M. Jones-Gotman, PhD, R. Canapicchi, MD, H. Cross, MD, Calcium Accumulation on Sarcolemma
D. Fish, MD, P. Bonanni, MD, I. Jambaqué, MD, and Jean-Pierre Louboutin, MD, PhD, Marcello Villanova, MD,
F, Andermann, MD Brigitte Lucas-Héron, PhD, and Michel Fardeau, MD
Contents of Volume 41, January-June 1997
Expedited Publication Letters
121 Collection and Normal Levels of the Amyloid Precursor Trihexyphenidyl Withdrawal Encephalopathy
Protein in Plasma Ken Johkura, MD, Shunsake Matsumoto, MD,
Scott Whyte, FRACP, Neil Wilson, BAppSci, Osamu Hasegawa, MD, and Yoshiyuki Kuroiwa, MD
Jon Currie, FRACP, Paul Maruff; PhD,
Valencia Malone, RN, Ruxsana Shafig-Antonacci, BAppSci, Early Copper Histidine Therapy in Classic Menkes
Peter Tyler, BAppSci, Kathryn L. Derry, BSc, Disease
John Underwood, PhD, Qiao-Xin Li, PhD, Bibudhendra Sarkar, PhD
Konrad Beyreuther, PhD, and Colin L. Masters, MD
Reply
Stephen G. Kaler, MD
Special Report
The Role of MRI in Clinical Trials of Multiple
Sclerosis: Comparison of Image Processing Techniques
A. C. Evans, PhD, J. A. Frank, MD, J. Antel, MD, and Regular Departments
D. H. Miller, MD Books
NO 2 FEBRUARY 1997
181 Botulinum Toxin Treatment of Muscle Cramps: A
Editorials
Clinical and Neurophysiological Study
Frontotemporal Dementia Is on the MAPt Laura Bertolasi, MD, Alberto Priori, MD, PhD,
Kirk C. Wilhelmsen, MD, PhD
Giuliano Tomelleri, MD, Luigi G. Bongiovanni, MD,
Mitochondrial Function in Huntington’s Disease: Clues Emiliana Fincati, MD, Alessandro Simonati, MD,
for Pathogenesis and Prospects for ‘ireatment Domenico De Grandis, MD, and Nicolo’ Rizzuto, MD
A. H. V. Schapira, MD
Acute and Chronic Effects of Hypoxia on the
Developing Hippocampus
James Owens, Jr, PhD, Carol A. Robbins, BS,
H. Jurgen Wenzel, MD, DSc, and
Special Report
Philip A. Schwartzkroin, PhD
Optic Pathway Gliomas in Children with
Neurofibromatosis 1: Consensus Statement from the Sequestration of RNA in Alzheimer’s Disease
NF1 Optic Pathway Glioma Task Force
Neurofibrillary Tangles and Senile Plaques
Robert Listernick, MD, David N. Louis, MD,
Stephen D. Ginsberg, PhD, Peter B. Crino, MD, PhD,
Roger J. Packer, MD, and David H. Gutmann, MD, PhD
Virginia M.-Y. Lee, PhD, James H. Eberwine, PhD, and
John Q. Trojanowski, PhD
Epidemiology of Mutations in Superoxide Dismutase in
Original Articles Amyotrophic Lateral Sclerosis
Hereditary Frontotemporal Dementia Is Linked to M. E. Cudkowicz, MD, D. McKenna-Yasek, RN,
Chromosome 17q21-q22: A Genetic and P. E. Sapp, BS, W. Chin, BS, B. Geller, BS,
Clinicopathological Study of Three Dutch Families D. L. Hayden, MS, D. A. Schoenfeld, PhD,
P. Heutink, PhD, M. Stevens, MD, P. Rizzu, MSc, B. A. Hosler, PhD, H. R. Horvitz, PhD, and
E. Bakker, PhD, J. M. Kros, MD, PhD, A. Tibben, PhD, R. H. Brown, PhD
M. F. Niermeijer, MD, PhD, C. M. van Duijn, PhD,
B. A. Oostra, PhD, and J. C. van Swieten, MD, PhD Dopaminergic Function in Familial Parkinson’s Disease:
A Clinical and '*F-Dopa Positron Emission
Energy Metabolism Defects in Huntington’s Disease
Tomography Study
and Effects of Coenzyme Q,,
Walter J. Koroshetz, MD, Bruce G. Jenkins, PhD, P. Piccini, MD, P. K. Morrish, MRCP, N. Turjanski, MD,
Bruce R. Rosen, MD, PhD, and M. Flint Beal, MD G. V. Sawle, DM, D. J. Burn, MD, R. A. Weeks, MD,
M. H. Mark, MD, D. M. Maraganore, MD,
Familial Risk of Migraine: A Population-based Study A. J. Lees, MD, and D. J. Brooks, MD
Walter F. Stewart, PhD, Judy Staffa, BS,
Richard B. Lipton, MD, and Ruth Ottman, PhD Deletion and Conversion in Spinal Muscular Atrophy
Patients: Is There a Relationship to Severity?
Muscle-Eye-Brain Disease: A Neuropathological Study
Christine J. DiDonato, PhD, Susan E. Ingraham, BS,
M. Haltia, MD, I. Leivo, MD, H. Somer, MD,
Jerry R. Mendell, MD, Thomas W. Prior, PhD,
H. Pihko, MD, A. Paetau, MD, T. Kivelé, MD,
A. Tarkkanen, MD, F. Tomé, MD, E. Engvall, MD, and Sharon Lenard, MS, Richard T. Moxley III, MD,
P. Santavuori, MD Julaine Florence, MHS, and Arthur H. M. Burghes, PhD
Contents of Volume 41, January—June 1997 _ iii
Autoantibodies Detected to Expressed K* Channels Are 273 Pupil-involving Third-nerve Palsy and Carotid Stenosis:
Implicated in Neuromyotonia Rapid Recovery Following Endarterectomy
Tan K. Hart, MRCP, Caroline Waters, BSc, L. J. Balcer, MD, S. L. Galetta, MD, D. M. Yousem, MD,
Angela Vincent, MRCPath, Claire Newland, PhD, M. A. Golden, MD, and A. K. Asbury, MD
David Beeson, PhD, Olaf Pongs, PhD, Christine Morris,
PhD, and John Newsom-Davis, FRS
Expedited Publication
Involvement of the Ipsilateral Motor Cortex in Finger
Genetic Evidence for the Involvement of 7 in
Movements of Different Complexities
Progressive Supranuclear Palsy
Robert Chen, MBBChir, FRCPC, Christian Gerloff; MD,
Chris Conrad, BS, Athena Andreadis, PhD,
Mark Hallett, MD, and Leonardo G. Cohen, MD
John Q. Trojanowski, MD, PhD, Dennis W. Dickson, MD,
Motion Direction Discrimination in Blind Hemifields David Kang, MS, Xiaohua Chen, MD,
Jason J. S. Barton, MD, PhD, FRCPC, and Wigbert Wiederholt, MD, Larry Hansen, MD,
James A. Sharpe, MD, FRCPC Eliezer Masliah, MD, Leon J. Thal, MD,
Robert Katzman, MD, Yu Xia, MS, and
Tsunao Saitoh, PhD
Brief Communications
Maternal Inheritance in Parkinson’s Disease
G. Frederick Wooten, MD, Lillian J. Currie, PhD, RN,
Letters
James P. Bennett, MD, PhD, Madaline B. Harrison, MD,
Selegiline and Mortality in Parkinson’s Disease:
Joel M. Trugman, MD, and W. Davis Parker, Jr, MD
Another View
Molecular Analysis of Cytochrome c Oxidase Deficiency A. J. Lees, J. Head, and Y. B. Shlomo
in Leigh’s Syndrome
Financial Influences on Career Choices in Neurology
Paula L. Adams, BSc, Robert N. Lightowlers, PhD, and
David M. Feinberg, MD
Douglas M. Turnbull, MD
Reply
Plasma Levels of Amyloid B Proteins AB1—40 and
Susan S. Spencer, MD
AB 1-42(43) Are Elevated in Down’s Syndrome
Takahiko Tokuda, MD, PhD, Tetsuro Fukushima, MS,
Shu-ich Ikeda, MD, PhD, Yoshiki Sekijima, MD,
Shin’ichi Shoji, MD, PhD, Nobuo Yanagisawa, MD, PhD, Regular Departments
and Akira Tamaoka, MD, PhD Books
NO 3 MARCH 1997
314 Oculomotor Nerve and Muscle Abnormalities in
Editorial Congenital Fibrosis of the Extraocular Muscles
Changing Views of Guillain-Barré Syndrome Elizabeth C. Engle, MD, Boyan C. Goumnerov, MD,
Arthur K. Asbury, MD, and Guy M. McKhann, MD Craig A. McKeown, MD, Martha Schatz, MD,
Donald R. Johns, MD, John D. Porter, PhD, and
Alan H. Beggs, PhD
Soluble Adhesion Molecules (sVCAM-1 and sICAM-1)
Neurological Progress in Cerebrospinal Fluid and Serum Correlate with MRI
Understanding Circadian Clocks: From c-Fos to Activity in Multiple Sclerosis
Fly Balls Peter Rieckmann, MD, Bernd Altenhofen, MD, Antje Riegel,
William J. Schwartz, MD Jiirgen Baudewig, MSc, and Klaus Felgenhauer, MD
Differential Effects of Levodopa on Dopaminergic
Function in Early and Advanced Parkinson’s Disease
Richard Torstenson, MSc, Per Hartvig, PharmD, PhD,
Bengt Langstrém, PhD, Géran Westerberg, PhD, and
Original Articles Joakim Tedroff; MD, PhD
Appropriate Number of Plasma Exchanges in Guillain-
Barré Syndrome Genetic Predisposition to Multiple Sclerosis as Revealed
The French Cooperative Group on Plasma Exchange in by Immunoprinting
Guillain-Barré Syndrome Cornelia Epplen, MD, Silke Jickel,
Eduardo J. M. Santos, MSc, Marcus DSouza,
Transthyretin Amyloidosis: A New Mutation Associated Dieter Poehlau, MD, Bernd Dotzauer, Eckhart Sindern, MD,
with Dementia Michael Haupts, MD, Klaus-Peter Riide, MD,
R. B. Petersen, PhD, H. Goren, MD, M. Cohen, MD, Frank Weber, MD, Jens Stéver, Sigrid Poser, MD,
S. L. Richardson, MS, N. Tresser, MD, A. Lynn, MS, Walter Gehler, MD, Jean-Pierre Malin, MD,
M. Gali, BS, M. Estes, MD, and P. Gambetti, MD Horst Przuntek, MD, and Jorg T. Epplen, MD
iv Contents of Volume 41, January-June 1997
Treatment of Ramsay Hunt Syndrome with Acyclovir- Increased Apolipoprotein E €4 in Epilepsy with
Prednisone: Significance of Early Diagnosis and Senile Plaques
Treatment Gunnar K. Gouras, MD, Norman R. Relkin, MD, PhD,
Shingo Murakami, MD, Naohito Hato, MD, David Sweeney, BA, David G. Munoz, MD,
Joji Horiuchi, MD, Nobumitsu Honda, MD, Tan R. Mackenzie, MD, and Sam Gandy, MD, PhD
Kiyofumi Gyo, MD, and Naoaki Yanagihara, MD
Use of Desferrioxamine in the Treatment of
Imaging of 5- and p-Opioid Receptors in Temporal Acer | pl
Lobe Epilepsy by Positron Emission Tomography Hiroaki Miyajima, MD, Yoshitomo Takahashi, MD,
Igal Madar, PhD, Ronald P. Lesser, MD, Tadashi Kamata, MD, Hideaki Shimizu, MD,
Gregory Krauss, MD, Jon Kar Zubieta, MD, PhD, Naoki Sakai, MD, and Jonathan D. Gitlin, MD
John R. Lever, PhD, Chris M. Kinter, PhD,
Hayden T. Ravert, PhD, John L. Musachio, PhD, A Novel Mitochondrial tRNA Phenylalanine Mutation
William B. Mathews, PhD, Robert F. Dannals, PhD, Presenting with Acute Rhabdomyolysis
and J. James Frost, MD, PhD P. F. Chinnery, MRCP, M. A. Johnson, PhD,
R. W. Taylor, PhD, R. N. Lightowlers, PhD, and
Pathological Correlates of Extrapyramidal Signs in D. M. Turnbull, FRCP
Alzheimer’s Disease
Yan Liu, MD, MS, Yaakov Stern, PhD,
Michael R. Chun, MD, Diane M. Jacobs, PhD,
Letters
Patricia Yau, and James E. Goldman, MD, PhD
Delayed OEF Elevation is an Unreliable Index of Tissue
Mononeuropathy Multiplex in Rhesus Monkeys with at Risk
Chronic Lyme Disease Myron D. Ginsberg, MD
John D. England, MD, Rudolf P. Bohm, Jr, DVM,
E. Donald Roberts, DVM, PhD, and Mario T. Philipp, PhD Reply
J. C. Baron, MD, M. Furlan, MD, and
Imaging of Axonal Damage in Multiple Sclerosis: G. Marchal, MD, PhD
Spatial Distribution of Magnetic Resonance
Imaging Lesions Prediction for Unimpaired Subjects Is Different from
S. Narayanan, MSc, L. Fu, MSc, E. Pioro, MD, DPhil, Diagnosis of Demented Patients
Allen D. Roses, MD, and Ann M. Saunders, PhD
N. De Stefano, MD, D. L. Collins, PhD, G. S. Francis, MD,
J. P. Antel, MD, P. M. Matthews, MD, DPhil, and
Reply
D. L. Arnold, MD
Bradley T. Hyman, MD, PhD, and Robert B. Wallace, MD
Correlation of Clinical, Magnetic Resonance Imaging,
Early American Professorships in Neurology
and Cerebrospinal Fluid Findings in Optic Neuritis
Richard F. Mayer, MD, and Gregory K. Bergey, MD
Lawrence D. Jacobs, MD, Samer E. Kaba, MD,
Colleen M. Miller, RN, CNS, Roger L. Priore, ScD, and Reply
Carol M. Brownscheidle, PhD Christopher G. Goetz, MD, and Eric J. Pappert, MD
Lack of Association of Apolipoprotein E €4 Allele with
Brief Communications
Bulbar-onset Motor Neuron Disease
A Japanese Family Carrying a Novel Mutation in the Rainer Bachus, MD, Sebastian Bader,
Emery-Dreifuss Muscular Dystrophy Gene Reinhard Gefsner, MD, PhD, and Albert C. Ludolph, MD
Yaeko Ichikawa, MD, Masahiko Watanabe, MD,
Hisatomo Kowa, MD, Shigeo Murayama, MD,
Takehiko Mizuno, MD, Issei Komuro, MD,
Ryouji Ishiki, MD, Jun Goto, MD, and Regular Departments
Ichiro Kanazawa, MD Books
NO 4 APRIL 1997
Editorials Original Articles
Ocular Muscle Involvement by Myasthenia Gravis
Henry J. Kaminski, MD, and Robert L. Ruff, MD, PhD 423 Acetylcholine Receptor Expression in Human
Extraocular Muscles and Their Susceptibility to
New Developments in Hereditary Inclusion Body Myasthenia Gravis
Myopathies C. MacLennan, BA, D. Beeson, PhD, A.-M. Buijs, BA,
Valerie Askanas, MD, PhD A. Vincent, MRCPath, and J. Newsom-Davis, FRS
Contents of Volume 41, January—June 1997 v
Gene Locus for Autosomal Recessive Distal Myopathy Brain Regional Differences in the Expansion of a CAG
with Rimmed Vacuoles Maps to Chromosome 9 Repeat in the Spinocerebellar Ataxias: Dentatorubral-
Takeshi Ikeuchi, MD, Tomoya Asaka, MD, Pallidoluysian Atrophy, Machado-Joseph Disease, and
Masaaki Saito, MD, Hajime Tanaka, MD, Spinocerebellar Ataxia Type 1
Sari Higuchi, MD, Keiko Tanaka, MD, Kyoko Saida, MD, Hideji Hashida, Jun Goto, Hiroshi Kurisaki,
Eiichiro Uyama, MD, Hidenobu Mizusawa, MD, Hidehiro Mizusawa, and Ichiro Kanazawa
Nobuyoshi Fukuhara, MD, Ikuya Nonaka, MD,
A Familial Syndrome of Congenital Cataract, Mental
Masaharu Takamori, MD, and Shoji Tsuji, MD Impairment, and Dentate Gyrus Atrophy
Arthur J. Hudson, MD, FRCP(C), and
Peripheral Neuropathy in Human I deficiency
David G. Munoz, MD, FRCP(C)
Virus—infected Patients with the Diffuse Infiltrative
Lymphocytosis Syndrome Marked Age-dependent Neuroprotection by Brain-
Antoine Moulignier, MD, Francois-Jéréme Authier, MD, derived Neurotrophic Factor Against Neonatal Hypoxic—
Marielle Baudrimont, MD, PhD, Gilles Pialoux, MD, Ischemic Brain Injury
Laurent Belec, MD, PhD, Marc Polivka, MD, Yu Cheng, MD, Jeffrey M. Gidday, PhD, Qiao Yan, PhD,
Bernard Clair, MD, Francoise Gray, MD, PhD, Aarti R. Shah, MS, and David M. Holtzman, MD
Jacqueline Mikol, MD, and Romain K. Gherardi, MD
Immunochemical Analysis of Dopamine Transporter
Machado-Joseph Disease: Clinical, Molecular, and Protein in Parkinson’s Disease
Metabolic Characterization in Chinese Kindreds Gary W. Miller, PhD, Julie K. Staley, PhD,
Craig J. Heilman, BS, Jeffrey T. Perez, BS,
Bing-wen Soong, MD, PhD, Chung-hui Cheng, BS,
Deborah C. Mash, PhD, David B. Rye, MD, PhD,
Ren-shyan Liu, MD, and Din-e Shan, MD, PhD
and Allan I. Levey, PhD
Machado-Joseph Disease Gene Product Is a
Cortical Peptide Changes in Huntington’s Disease May
Cytoplasmic Protein Widely Expressed in Brain
Be Independent of Striatal Degeneration
Henry L. Paulson, MD, PhD, Sonal S. Das, BA,
Michael F. Mazurek, MD, FRCP(C), Sarah Garside, MD,
Peter B. Crino, MD, PhD, Matthew K. Perez, MS, and M. Flint Beal, MD
Sonal C. Patel, Denise Gotsdiner, MD,
Kenneth H. Fischbeck, MD, and Randall N. Pittman, PhD
Clinical and Pathological Phenotype of the Original
Brief Communications
Family with Charcot-Marie-Tooth Type 1B: A 20-Year
Various Types of Hereditary Inclusion Body
Study
Myopathies Map to Chromosome 9p1-ql
Thomas D. Bird, MD, George H. Kraft, MD,
Zohar Argov, MD, Efrat Tiram, BSc, Iris Eisenberg, BSc,
Hillary P. Lipe, RN, Kimbra L. Kenney, MD, and
Menahem Sadeh, MD, Christine E. Seidman, MD,
S. Mark Sumi, MD
J. G. Seidman, PhD, George Karpati, MD, and
Stella Mitrani-Rosenbaum, PhD
"H-Magnetic Resonance Spectroscopy-Determined
Cerebral Lactate and Poor Neurological Outcomes in Expression of the Copper—Zinc Superoxide Dismutase
Children with Central Nervous System Disease Gene in Amyotrophic Lateral Sclerosis
Stephen Ashwal, MD, Barbara A. Holshouser, PhD, Kazutoshi Nishiyama, MD, PhD,
Lawrence G. Tomasi, MD, Stanford Shu, MD, Shigeo Murayama, MD, PhD, Shin Kwak, MD, PhD, and
Ronald M. Perkin, MD, Gerald A. Nystrom, MD, and Ichiro Kanazawa, MD, PhD
David B. Hinshaw, Jr, MD
Complex I Function in Familial and Sporadic Dystonia
Health-related Quality of Life and Seizure Control in A. H. V. Schapira, T. Warner, M. T. Gash,
Temporal Lobe Epilepsy M. W. J. Cleeter, C. F. M. Marinho, and J. M. Cooper
Richard S. McLachlan, MD, Kristopher J. Rose, BA,
Paul A. Derry, PhD, Casey Bonnar, BScN,
Warren T. Blume, MD, and John P. Girvin, MD
Letters
Correlation of Widespread Preoperative Magnetic Identity of Interferon-Bla Product in Multiple Sclerosis
Resonance Imaging Changes with Unsuccessful Surgery Study
for Hippocampal Sclerosis Wieland W. Wolf, PhD
S. M. Sisodiya, MRCP, PhD, N. Moran, MRCP,
S. L. Free, PhD, N. D. Kitchen, MD, FRCS(SN), Reply
J. M. Stevens, FRCR, W. F. J. Harkness, FRCS, Lawrence Jacobs, MD
D. R. Fish, MD, FRCP, and S. D. Shorvon, MD, FRCP
Central Nervous System Whipple’s Disease
Wim I. M. Verhagen, MD, PhD,
Midbrain Dopaminergic Neuronal Degeneration in a
Patrick L. M. Huygen, PhD, and Johanna E. Dalman, MD
Transgenic Mouse Model of Familial Amyotrophic
Lateral Sclerosis Treatment Guidelines in Central Nervous System
V. Kostic, MD, PhD, M. E. Gurney, PhD, Whipple’s Disease
H.-X. Deng, PhD, T. Siddique, MD, C. J. Epstein, MD, and P. J. Schnider, MD, E. C. Reisinger, MD, T. Berger, MD,
S. Przedborski, MD, PhD G. J. Krejs, MD, and E. Auff; MD
Contents of Volume 41, January-June 1997
Reply 563 Reply
E. D. Louis, MD, MS, T. Lynch, MRCPI, S. Fahn, MD, Dennis W. Choi, MD, PhD, Mark D. Goldberg, MD, and
and J. Odel, MD John W. McDonald, MD, PhD
Long-range Planning of Neurology
Walter G. Bradley, DM, FRCP
The Vehicle for Cyclosporine Is Neurotoxic In Vitro Regular Departments
Anthony J. Windebank, MD 564 Correction
NO 5 MAY 1997
615 The Absence of an Apolipoprotein €4 Allele Is
Editorials
Associated with a More Aggressive Form of
Diffusion-weighted MRI: An ECG for “Brain Attack”? Alzheimer’s Disease
Walter J. Koroshetz, MD, and Gilberto Gonzalez, MD, PhD Yaakov Stern, PhD, Jason Brandt, PhD,
Marilyn Albert, PhD, Diane M. Jacobs, PhD,
Diffusion-weighted MRI for Diagnosis and Treatment
Xinhua Liu, PhD, Karen Bell, MD, Karen Marder, MD,
of Ischemic Stroke
Mary Sano, PhD, Steven Albert, PhD,
Justin A. Zivin, MD, PhD
Caridad Del-Castillo Castenada, BA, Fred Bylsma, PhD,
Ben Tycko, MD, and Richard Mayeux, MD
Cortical Reorganization in Patients with Facial Palsy
Special Report Michel Rijntjes, Martin Tegenthoff, MD,
The Quest for Unbiased Research: Randomized Clinical Joachim Liepert, MD, Georg Leonhardt, MD,
Trials and the CONSORT Reporting Guidelines Sylvia Kotterba, MD, Stephan Miiller, MD,
Kenneth F. Schulz, PhD, MBA Stephan Kiebel, Jean-Pierre Malin, MD,
Hans-Christoph Diener, MD, and Cornelius Weiller, MD
Juvenile-onset Spinal Muscular Atrophy Caused by
Original Articles Compound Heterozygosity for Mutations in the
Clinical Utility of Diffusion-weighted Magnetic HEXA Gene
Resonance Imaging in the Assessment of Ischemic Ruth Navon, PhD, Rami Khosravi, Judith Melki, MD, PhD,
Stroke Liat Drucker, MSc, Bertrand Fontaine, MD, PhD,
H. L. Lutsep, MD, G. W. Albers, MD, A. DeCrespigny, PhD, Jean Claude Turpin, MD, PhD, Bathien N’Guyen, MD,
G. N. Kamat, MD, M. P. Marks, MD, and Michel Fardeau, MD, Pierre Rondot, MD, and
M. E. Moseley, PhD Nicole Baumann, MD, PhD
Enlargement of Human Cerebral Ischemic Lesion Cerebral Generators Involved in the Pathogenesis of the
Volumes Measured by Diffusion-weighted Magnetic Restless Legs Syndrome
Resonance Imaging Stefan F. Bucher, PhD, Klaus C. Seelos, MD,
Alison E. Baird, FRACP, PhD, Andrew Benfield, BS, Wolfgang H. Oertel, MD, Maximilian Reiser, MD, and
Gottfried Schlaug, MD, Bettina Siewert, MD, Claudia Trenkwalder, MD
Karl-Olof Lévblad, MD, Robert R. Edelman, MD, and
Oxidative Damage and Metabolic Dysfunction in
Steven Warach, MD, PhD
Huntington’s Disease: Selective Vulnerability of the
Brain Impairment in Well-nourished Chronic Alcoholics Basal Ganglia
Is Related to Ethanol Intake Susan E. Browne, PhD, Allen C. Bowling, MD, PhD,
Josep M. Nicolés, MD, Ramén Estruch, MD, Usha MacGarvey, MSc, M. Jay Baik, BS,
Manel Salamero, MD, Nuria Orteu, MD, Stephanie C. Berger, BS, Miratul M. K. Mugit, BSc,
Joaquim Fernandez-Sola, MD, Emilio Sacanella, MD, and Edward D. Bird, MD, and M. Flint Beal, MD
Alvaro Urbano-Marquez, MD
Leukodystrophy in Patients with Ovarian Dysgenesis
Regional and Cellular Expression of the Dentatorubral- Raphael Schiffmann, MD, Gioacchino Tedeschi, MD,
Pallidoluysian Atrophy Gene in Brains of Normal and R. Philip Kinkel, MD, Bruce D. Trapp, PhD,
Affected Individuals Joseph A. Frank, PhD, Christine R. Kaneski, MS,
K. Nishiyama, MD, PhD, K. Nakamura, MD, PhD, Roscoe O. Brady, MD, Norman W. Barton, MD, PhD,
S. Murayama, MD, PhD, M. Yamada, PhD, and Lawrence Nelson, MD, and Jack A. Yanovski, MD, PhD
I. Kanazawa, MD, PhD
Periventricular Nodular Heterotopia and Intractable
Atrophy of the Corpus Callosum, Cognitive Temporal Lobe Epilepsy: Poor Outcome after Temporal
Impairment, and Cortical Hypometabolism in Lobe Resection
Progressive Supranuclear Palsy L. M. Li, MD, F. Dubeau, MD, F. Andermann, MD,
Hiroshi Yamauchi, MD, PhD, D. R. Fish, MD, C. Watson, MD, PhD,
Hidenao Fukuyama, MD, PhD, Yasuhiro Nagahama, MD, G. D. Cascino, MD, S. F. Berkovic, MD, N. Moran, MD,
Yukinori Katsumi, MD, Yun Dong, MD, J. S. Duncan, MD, A. Olivier, MD, PhD, R. Leblanc, MD,
Junji Konishi, MD, PhD, and Jun Kimura, MD and W. Harkness, MD
Contents of Volume 41, January-June 1997 vii
669 Increases in Soluble VCAM-1 Correlate with a Decrease Allelic Heterogeneity of Mediterranean Myoclonus and
in MRI Lesions in Multiple Sclerosis Treated with the Cystatin B Gene
Interferon B-1b Pierre Labauge, MD, Réda Ouazzani, MD,
Peter A. Calabresi, MD, Laura R. Tranquill, MS, Amel M’Rabet, MD, Djamel Grid, MD, Pierre Genton, MD,
James M. Dambrosia, PhD, Lael A. Stone, MD, Charlotte Dravet, MD, Taieb Chkili, MD,
Heidi Maloni, RN, Craig N. Bash, MD, Corrine Beck, PhD, Catherine Buresi, MD,
Joseph A. Frank, MD, and Henry F. McFarland, MD Michel Baldy-Moulinier, MD, and Alain Malafosse, MD
Phenotypic Variability in Friedreich Ataxia: Role of the CAG Repeat Number Governs the Development Rate of
Associated GAA Triplet Repeat Expansion Pathology in Huntington’s Disease
Laura Montermini, PhD, Andrea Richter, PhD, John B. Penney, Jr, MD, Jean-Paul Vonsattel, MD,
Kenneth Morgan, PhD, Cristina M. Justice, BS, Marcy E. MacDonald, PhD, James F. Gusella, PhD, and
Dominique Julien, BS, Barbara Castellotti, MS, Richard H. Myers, PhD
Jocelyne Mercier, BS, Josée Poirier, BS,
Fiorentino Capozzoli, MD, Jean-Pierre Bouchard, MD, A Subset of Senile Dementia with High Incidence of
Bernard Lemieux, MD, Jean Mathieu, MD, the Apolipoprotein E €2 Allele
Michel Vanasse, MD, Marie-Heélene Seni, RT, Kenji Ikeda, MD, PhD, Haruhiko Akiyama, MD, PhD,
Gail Graham, MD, Frederick Andermann, MD, Tetsuaki Arai, MD, Naruhiko Sahara, MA,
Eva Andermann, MD, PhD, Serge B. Melangon, MD, Hiroshi Mori, PhD, Mihoko Usami, PhD,
Bronya J. B. Keats, PhD, Stefano Di Donato, MD, Masuhiro Sakata, MD, Toshio Mizutani, MD, PhD,
and Massimo Pandolfo, MD Koichi Wakabayashi, MD, PhD, and
Hitoshi Takahashi, MD, PhD
Dutch Hereditary Cerebral Amyloid Angiopathy:
Structural Lesions and Apolipoprotein E Genotype
M. Bornebroek, MD, J. Haan, MD, PhD,
S. G. Van Duinen, MD, PhD,
Brief Communications
M. L. C. Maat-Schieman, MD,
Endoglin Gene Polymorphism as a Risk Factor for M. A. Van Buchem, MD, PhD, E. Bakker, PhD,
Sporadic Intracerebral Hemorrhage C. Van Broeckhoven, PhD, and R. A. C. Roos, MD, PhD
M. J. Alberts, MD, J. P. Davis, BA, C. Graffagnino, MD,
C. McClenny, RN, D. Delong, PhD, C. Granger, MD,
M. H. Herbstreith, BS, K. Boteva, MD, Regular Departments
D. A. Marchuk, PhD, and A. D. Roses, MD 698 Correction
NO 6 JUNE 1997
Message from the Editor Special Report
Robert A. Fishman, MD 706 Frontotemporal Dementia and Parkinsonism Linked to
Chromosome 17: A Consensus Conference
Norman L. Foster, MD, Kirk Wilhelmsen, MD, PhD,
Anders A. F. Sima, MD, PhD, Margaret Z. Jones, MD,
Constance J. D'Amato, BS, Sid Gilman, MD,
The New Editor of the Annals and Conference Participants
Arthur K. Asbury, MD
Original Articles
High Frequency of Apolipoprotein E 2 Allele in
Hemorrhage Due to Cerebral Amyloid Angiopathy
James A. R. Nicoll, MD, Clair Burnett, Seth Love, PhD,
Editorial David I. Graham, MB, Debbie Dewar, PhD,
Cerebral Amyloid Angiopathy and Apolipoprotein E: James W. Ironside, MB, Janice Stewart,
Bad News for the Good Allele? and Harry V. Vinters, MD
Steven M. Greenberg, MD, PhD, and Bradley T. Hyman,
MD, PhD Motor Neuron Syndromes in Cancer Patients
P. A. Forsyth, MD, J. Dalmau, MD, PhD, F. Graus, MD,
V. Cwik, MD, M. K. Rosenblum, MD,
and J. B. Posner, MD
Altered Intrathymic T-Cell Repertoire in Human
Point of View Myasthenia Gravis
Paraneoplastic Primary Lateral Sclerosis and Frédérique Truffault, MSci, Sylvia Cohen-Kaminsky, PhD,
Amyotrophic Lateral Sclerosis Iman Khalil, PhD, Philippe Levasseur, MD,
Lewis P. Rowland, MD and Sonia Berrih-Aknin, PhD
Contents of Volume 41, January—June 1997
Presenilin-1 Protein Expression in Familial and 813 Is There a Genetic Susceptibility Locus for Parkinson’s
Sporadic Alzheimer’s Disease Disease on Chromosome 22q13?
Allan I. Levey, MD, PhD, Craig J. Heilman, BS, K. Wilhelmsen, D. Mirel, K. Marder, M. Bernstein,
James J. Lah, MD, PhD, Norman R. Nash, BS, A. Naini, S. M. Leal, L. J. Cote, M.-X. Tang, G. Freyer,
Howard D. Rees, PhD, Masakazu Wakai, MD, J. Graziano, and R. Mayeux
Suzanne S. Mirra, MD, David B. Rye, MD, PhD,
David Nochlin, MD, Thomas D. Bird, MD,
and Elliott J. Mufson, PhD
Magnetic Resonance Imaging of Periventricular
Leukomalacia and Its Clinical Correlation in Children Expedited Publications
Paivi Olsén, MD, Eija Padékké, MD, A Double-blind Controlled Study of Gabapentin and
Leena Vainionpad, MD, Juhani Pyhtinen, MD, Baclofen as Treatment for Acquired Nystagmus
and Marjo-Riitta Jérvelin, MD L. Averbuch-Heller, MD, R. J. Tusa, MD, PhD,
L. Fuhry, MD, K. G. Rottach, MD, G. L. Ganser, MD,
Painful Proximal Diabetic Neuropathy: Inflammatory W. Heide, MD, U. Biittner, MD, R. J. Leigh, MD
Nerve Lesions and Spontaneous Favorable Outcome
Gérard Said, Fabienne Elgrably, Catherine Lacroix, Friedreich-like Ataxia with Retinitis Pigmentosa Caused
Violaine Planté, Céline Talamon, David Adams, by the His’®’Gln Mutation of the a-Tocopherol
Michel Tager, and Gérard Slama Transfer Protein Gene
Takanori Yokota, Toshiaki Shiojiri, Takanari Gotoda,
A New Type of Hereditary Motor and Sensory Makoto Arita, Hiroyuki Arai, Tatsuhide Ohga,
Neuropathy Linked to Chromosome 3 Takashi Kanda, Junichi Suzuki, Tomihiro Imai,
Hiroshi Takashima, MD, Masanori Nakagawa, MD, Hiroyuki Matsumoto, Seiyo Harino, Motohiro Kiyosawa,
Keiichi Nakahara, MD, Masahito Suehara, MD, Hidehiro Mizusawa, and Keizo Inoue
Toshio Matsuzaki, MD, Itsuro Higuchi, MD,
Hidemasa Higa, MD, Kimiyoshi Arimura, MD,
Teruo Iwamasa, MD, Shuji Izumo, MD,
and Mitsuhiro Osame, MD
A Mismatch Between Kinesthetic and Visual Perception Letters
in Parkinson’s Disease Treatment of Parkinson’s Disease: Disagreements
Mehmet Demirci, MD, Stephen Grill, MD, PhD, Michael Schulzer, MD, PhD
Lisa McShane, PhD, Mark Hallett, MD
Reply
Clinical Trial of Plasma Exchange and High-dose J. Godbold, PhD, J. Mandeli, PhD, and
Intravenous Immunoglobulin in Myasthenia Gravis C. W. Olanow, MD, FRCP(C)
Philippe Gajdos, MD, Sylvie Chevret, MD, PhD,
Bernard Clair, MD, Christine Tranchant, MD, Neuropsychological and Behavioral Changes and
and Claude Chastang, MD, PhD, for the Myasthenia Gravis Weight Gain after Medial Pallidotomy
Clinical Study Group Anthony E. Lang, Andres Lozano, Ronald Tasker, Jan Duff,
Jean Saint-Cyr, and Lisa Trépanier
Multiple Sclerosis: Re-expression of a Developmental
Gene in Chronic Lesions Correlates with Remyelination Reply
Elisabetta Capello, MD, Rhonda R. Voskuhl, MD, Mark S. Baron, MD, Jerrold L. Vitek, MD, PhD,
Henry F. McFarland, MD, and Roy A. E. Bakay, MD, Joanne Green, PhD,
Cedric S. Raine, PhD, DSc, FRCPath Yoshiki Kaneoke, MD, Takao Hashimoto, MD,
Robert S. Turner, PhD, John L. Woodard, PhD,
Steven A. Cole, MD, William M. McDonald, MD,
Brief Communications
and Mahlon R. DeLong, MD
Human T-Cell Lymphotropic Virus Type I—Associated
Facial Nerve Palsy in Trinidad and Tobago
Courtenay Bartholomew, MD, Farley Cleghorn, MBBS,
Noreen Jack, MBBS, Jeffrey Edwards, MBBS,
and William Blattner, MD
Regular Departments
A Novel Presenilin-1 Mutation: Increased B-Amyloid 837 Books
and Neurofibrillary Changes
T. Gomez-Isla, MD, PhD, W. Wasco, PhD,
W. P. Pettingell, BS, S. Gurubhagavatula, MS,
S. D. Schmidt, BS, P. D. Jondro, BA,
M. McNamara, BA, L. A. Rodes, BA, T. DiBlasi,
Indexes to Volume 41
W. B. Growdon, P. Seubert, PhD, D. Schenk, PhD,
J. H. Growdon, MD, B. T. Hyman, MD, PhD, 838 Author Index
and R. E. Tanzi, PhD 842 Subject Index
Contents of Volume 41, January-June 1997 ix