Neurobiology of Disease

by Johnston, Michael V; Adams, Harold P; Fatemi, Ali

• ISBN13: 9780199937837
• ISBN10: 0199937834
• Edition: 2nd
• Format: Hardcover
• Copyright: 2016-09-15
• Publisher: Oxford University Press

Table of Contents

SECTION I: Movement Disorders (Joel Perlmutter, Washington University)
1. History of Parkinsonism
Michael Silver, MD and Stewart A. Factor, DO
2. Parkinson Disease: Pathophysiology, Clinical Manifestations and Course including Diagnosis and Differential Diagnosis
Michael J. Soileau, MD and Kelvin L. Chou, MD
3. Parkinson Disease: Treatment, Current Clinical Trials
Thien Thien Lim, MD and Hubert H. Fernandez, MD
4. Parkinson Syndromes: Multiple System Atrophy
Melvin W. Kong, MD and Ejaz A. Shamim MD, MS
5. Parkinson Syndromes: Progressive Supranuclear Palsy
Vikas Kotagal, MD, MS and Praveen Dayalu, MD
6. Parkinson Syndromes: Corticobasal Degeneration
Sami Barmada, MD, PhD
7. Huntington Disease: Etiology, Genetics, and Clinical Manifestations
Roger L. Albin, MD and Henry L. Paulson, MD, PhD
8. Huntington Disease: Treatment, Current Clinical Trials
Charles S. Venuto, PharmD and Karl Kieburtz, MD, MPH
9. Essential Tremors
Arif Dalvi, MD, MBA, Kelly E. Lyons, PhD, and Rajesh Pahwa, MD
10. Dystonia: History and Classification
H. A. Jinnah, MD, Cecilia N. Prudente, MD, Samuel J. Rose, MD and Ellen J. Hess, MD
11. Dystonia: Pathophysiology, Clinical Manifestations and Course Including Diagnosis, and Differential Diagnosis, Treatment, Current Clinical Trials
Jeri Yvonne Williams, MD and David G. Standaert, MD, PhD
12. Paroxysmal Dyskinesias
Olga Waln, MD and Joseph Jankovic, MD.
13. The Expanded Polyglutamine Tract Spinocerebellar Ataxias
Elsdon Storey, MD
14. Ataxias: Autosomal Recessive and other Ataxias
Vikram G. Shakkottai, MD, PhD

SECTION II: Dementias (David Knopman, Mayo Clinic)
15. Alzheimer's Disease: Pathology and Pathogenesis
Bradley T Hyman, MD, Beatriz G Perez-Nievas, MD, Isabel Barroeta-Espar, MD, Alberto Serrano-Pozo, MD, Matthew Frosch, MD, and Teresa Gomez-Isla, MD
16. Alzheimer's Disease: Clinical Manifestations and Diagnostic/Differential Diagnosis
Michael S. Rafii, MD, PhD
17. Alzheimer's Disease: Management and Treatment
Gregory A. Jicha, MD, PhD and Frederick A. Schmitt, PhD
18. Frontotemporal Degenerations
Jonathan Graff-Radford, MD, and Keith A. Josephs, MD, MST, MSc
19. Diagnosis and Therapy for Lewy Body Dementia
Bela R. Turk, MD and Ali Fatemi, MD
20. Pathogenesis of Lewy Body Dementia
Jagan A. Pillai, MD, PhD and James B. Leverenz, MD
21. Cerebrovascular Dementia
Rahul Karamchandani, MD and Nancy R. Barbas, MD, MSW

SECTION III: Motorneuron Diseases (Merit Cudkowicz, Massachusetts General Hospital, Harvard University)
22. Types of MNDS
Nimish Thakore, MD and Erik P. Pioro, MD, PhD
23. Clinical Presentations, Diagnostic Criteria, Lab Testing
Nimish Thakore, MD and Erik P. Pioro, MD, PhD
24. Symptomatic Care
Matthew Gladman, MD and Lorne Zinman, MD, MSc
25. ALS: Epidemiology
Éilis J. O'Reilly, ScD
26. ALS: Pathogenesis
Laura Ferraiuolo, MD and Stephen J. Kolb, MD
27. ALS: Insights from Genetics
Matthew B. Harms, MD and Timothy M. Miller, MD, PhD
28. ALS: In Vitro and In Vivo Disease Models
Brian J. Wainger, MD, PhD
29. ALS Pathology
Brent T. Harris, MD, PhD, Galam A. Khan, MD, and Saed Sadeghi, MD
30. ALS: Clinical Trials Past Reflections Informing New Directions
Katharine A. Nicholson, MD and James D. Berry, MD
31. Upper Motor Neuron Disorders: Hereditary Spastic Paraplegia and Primary Lateral Sclerosis
John K. Fink, MD
32. UMN Disorders (HSP and PLS): Pathogenesis, Insights from Genetics, In Vitro and In Vivo Disease Models, Pathology and Clinical Trials
Sabrina Paganoni, MD, PhD and Nazem Atassi, MD, MMSc
33. SMA: Pathogenesis
Umrao R. Monani, PhD and Darryl C. De Vivo, MD
34. SMA: Insights from Genetics
Arthur H.M. Burghes, MD and Vicki L. McGovern, MD
35. SMA: In Vitro and In Vivo Models of Spinal Muscular Atrophy
W. David Arnold, MD and Arthur H.M. Burghes, MD

SECTION IV: Paroxysmal Disorders (Solomon Moshe, Albert Einstein College of Medicine)
36. Biological Basis of Primary Generalized Epilepsies-Genetics
Carla Marini, MD and Renzo Guerrini, MD
37. Biological Basis of Primary Generalized Epilepsies - Pathophysiology
Mark W. Youngblood, MD and Hal Blumenfeld, MD
38. Temporal Lobe Epilepsy
Edward H. Bertram, MD
39. Focal Cortical Dysplasias
Ali Mahta, MD and Peter B. Crino, MD, PhD
40. Biological Bases of Symptomatic Generalized Epilepsies in Children
Dragos A. Nita, MD, Miguel A. Cortez, MD, Jose Luis Perez Velazquez, MD, and O. Carter Snead 3rd, MD
41. Epilepsies that Occur Predominantly in Girls
Lynette G Sadleir, MD, Jozef Gecz, MD, and Ingrid E Scheffer, MD
42. Epidemiology of Epilepsy: Incidence, Prevalence, and Risk Factors
Dale C Hesdorffer, PhD, MPH
43. Clinical Presentation, Diagnosis, and Course- Epilepsy Syndromes with Focal Seizures
Alex Boro, MD and Jerome Engel, Jr., MD
44. Medical Management of Epilepsy
Jules C. Beal, MD and Emilio Perucca, MD
45. Neuroimaging in Epilepsy
William A. Gomes, MD, PhD
47. Alternative Therapies for Epilepsy: Dietary Treatments
Carl E. Stafstrom, MD, PhD
48. Migraine and Other Headache Disorders
Robert W. Charlson, MD and Matthew S. Robbins, MD
49. Neurobiology of Migraine
Victor Rosenfeld, MD and John Stern, MD

SECTION V: Pediatric Neurology and Developmental Disorders (Tanjala Gipson and Deepa Menon, Kennedy Krieger Institute and Johns Hopkins University)
50. Autism and Intellectual Disabilities: Overview of Autism and Intellectual Disabilities
Tanjala T. Gipson, MD and Michael V. Johnston, MD
51. Tuberous Sclerosis Complex: Pediatric Aspects
Tanjala T. Gipson, MD, Andrea Poretti, MD, Rebecca McClellan, MGC, CGC, and Michael V. Johnston, MD
52. Neurofibromatosis Type 1
David S. Wolf, MD, PhD
53. Neurobiology of Autism and Intellectual Disability: Fragile X Syndrome
Dejan B. Budimirovic, MD and Megha Subramanian, BA
54. Autism and Intellectual Disabilities: PTEN Gene mutation
Deepa U. Menon, MBBS, MD
55. Rett Syndrome
C.L. Smith-Hicks, MD and SakkuBai Naidu, MD
56. Angelman Syndrome
Kristin W. Barañano, MD, PhD
57. Down Syndrome
George T. Capone, MD
58. Coffin-Lowry Syndrome
Michael V. Johnston, MD
59. Congenital Hypothyroidism
Mary Lee Gregory, MD, PhD
60. Pediatric Neurotransmitter Disorders
Phillip L. Pearl, MD and William P. Welch, MD
61. Attention Deficit-Hyperactivity Disorder
Mihee J. Bay, MD and Bruce K. Shapiro, MD
62. Diseases of Mitochondrial Energy Metabolism
Jacqueline Weissman, MD, MS and Lisa Emrick, MD
63. Genetic Metabolic Disorders: MethylmalonicAciduria
Siddharth Srivastava, MD and Jeffrey Chinsky, MD PhD
64. The Urea Cycle Disorders
Andrea L. Gropman, MD, Belen Pappa, MD, and Nicholas Ah Mew, MD
65. Phenylketonuria
Hilary Vernon, MD, PhD
66. Cerebral Creatine Deficiency Disorders
Peter W. Schutz, MD and Sylvia Stockler, MD
67. Congenial Disorders of Glycosylation
Marc C. Patterson, MD, FRACP
68. Glutaric Aciduria Type I
Hilary Vernon, MD, PhD
69. Lysosomal Storage Disroders
Gustavo H.B. Maegawa, MD, PhD, FACMG
70. Peroxisomal Disorders
Gerald V. Raymond, MD, Mohamed Y. Jefri, MD, Kristin W. Baranano, MD, and Ali Fatemi, MD
71. Leukodystrophies
Ulrike Schrifl, MD, SakkuBai Naidu, MD, and Ali Fatemi, MD
72. Cerebral Palsy
Joan M. Jasien, MD, Bruce K. Shapiro, MD and Alexander H. Hoon, Jr., MD
73. Neonatal Brain Injuries
Katharina Goeral, MD, Ali Fatemi, MD, and Michael V. Johnston, MD
74. The Neurobiology of Encephalopathy of Prematurity
Gwendolyn J. Gerner, MD and Vera Joanna Burton, MD
75. Epilepsy Syndromes in Childhood
Verena Staedtke, MD and Eric H. Kossoff, MD
76. Migraine and Headaches in Children
Jessica Klein, MD and Christopher Oakley, MD
77. Congenital Hydrocephalus
Vera Joanna Burton, MD and Edward Ahn, MD
78. Spina Bifida and Related Conditions
Stephen L. Kinsman, MD
79. Smith-Lemli-Opitz Syndrome
Ryan W. Lee, MD
80. Sickle Cell Anemia
Eboni I. Lance, MD

SECTION VI: Neuroimmunological Diseases (Carlos Pardo-Villamizar, Johns Hopkins University)
81. The Epidemiology of Multiple Sclerosis: An Historical Perspective
Kassandra L. Munger, ScD
82. Multiple Sclerosis: Pathology
Bogdan Florin Gh. Popescu, MD, Yong Guo, MD, and Claudia Francesca Lucchinetti, MD
83. Multiple Sclerosis: MRI and Other Imaging Approaches in MS
Martina Absinta, MD and Daniel S. Reich, MD, PhD
84. Multiple Sclerosis: White Matter vs Grey Matter Involvment (The Cause of Disability in MS)
Massimo Filippi, MD, and Maria A. Rocca, MD.
85. Multiple Sclerosis: Monitoring disease activity and progression
Pavan Bhargava, MD and Shiv Saidha, MBBCh, MD, MRCPI
86. Symptomatic Treatment of Multiple Sclerosis
Kathleen Costello, RN, BSN, MS
87. Exercise and Multiple Sclerosis
Jeffrey R Hebert, PT, PhD
88. Multiple Sclerosis: Disease Modifying Therapies
Pavan Bhargava, MD and Peter A. Calabresi, MD
89. Neuromyelitis Optica
Teri L. Schreiner, MD, MPH and Jeffrey L. Bennett, MD, PhD
90. Acute Disseminated Encephalomyelitis
Benjamin M. Greenberg, MD, MHS and Allen Desena, MD
93. Paraneoplastic Neurological Disorders
John C. Probasco, MD
94. Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis
Elizabeth M. Wells, MD, MHS
95. Other Proven and Putative Autoimmune Disorders of the CNS: Anti-GAD Associated Neurological Disorders; Stiff-Person syndrome, Cerebellar Ataxia, Progressive Encephalopathy with Rigidity and Myoclonus (PERM), and Encephalitis.
Scott D. Newsome, DO
96. Other Proven and Putative Autoimmune Disorders of the CNS: Hashimoto's Encephalopathy
Eoin P. Flanagan, MBBCh and Richard J. Caselli, MD
97. Other Proven and Putative Autoimmune Disorders of the CNS: Neuro-Bechet's Syndrome
Afshin Borhani Haghighi M.D and Bernadette Kalman, MD, PhD, DSc
98. Post-Streptococcal Movement Disorders
Davide Martino, MD and Gavin Giovannoni, MD
99. Rasmussen Encephalitis
Jan Bauer, MD and Christian G. Bien, MD
100. Autoimmune Encephalitis
Andrew McKeon, MD
101. Other Proven and Putative Autoimmune Disorders of the PNS: Myasthenia gravis
Doris G. Leung, MD
102. Vasculitis of the central nervous system
Christian Pagnoux, MD and Richard H. Swartz, MD

SECTION VII: Cerebrovascular Diseases (Harold Adams, University of Iowa)
103. Stroke Epidemiology and Impact: Mortality, Incidence, Prevalence, Stroke Subtypes, Sex, Race/Ethnicity, Geography U.S. and World
Virginia J Howard, PhD
104. Acute Ischemic Stroke
Harold P. Adams, Jr., MD
105. Neuroimaging of Acute Stroke
Chelsea S. Kidwell, MD and Kambiz Nael, MD
106. Common Risk Factors and Medical Prevention Therapies
George Khouri, MD, Shelly Ozark, MD, and Bruce Ovbiagele, MD, MSc, MAS
107. Surgical and Radiologic Intervention for Prevention of Ischemic Stroke
Andrew Ringer, MD
108. Unusual Causes of Stroke
Enrique C. Leira, MD, MS
109. Pediatric Stroke
Melissa Chung, MD and Warren Lo, MD
110. Vascular Biology of Cerebral Ischemia
Jason A. Ellis, MD and E. Sander Connolly, Jr., MD
111. Intracerebral Hemorrhage
Craig Anderson, MD
112. Subarachnoid Hemorrhage
David Hasan, MD
113. Genetics of Cerebral Ischemia including Genetic Syndromes
Andrea Porretti, MD and Michael V. Johnston, MD
114. Molecular Targets of Cerebral Ischemia
Ryan J. Felling, MD, PhD
115. Sturge-Weber Syndrome and related Cerebrovascular Malformation Syndromes.
Angela Quain, MS and Anne M. Comi, MD

SECTION VIII: Peripheral and Autonomic Nervous System Disorders and Pain (Nicholas Maragakis, Johns Hopkins University)
116. Neuropathies Associated with Infection or Toxic Exposure
Doris Leung, MD
117. Mechanisms Contributing to the Development and Progression of Diabetic Polyneuropathy
Lucy M. Hinder, PhD, Kelli A. Sullivan, PhD, Stacey A. Sakowski, PhD, and Eva L. Feldman, MD, PhD
118. Diabetic and PreDiabetic Neuropathy
Lindsay Zilliox, MD and James W. Russell, MD, MS, FRCP
119. Anatomy, Localization, and Pattern Recognition of Peripheral Neuropathy
Noah Kolb, MD and A. Gordon Smith, MD
120. Drug Discovery of Neuropathic Pain
Brian J. Wainger, MD, PhD
121. Neuropathies, Autonomic
Phillip A. Low, MD
122. Animal Models of Peripheral Neuropathy and Neuropathic Pain
Katelyn Donaldson, MD and Ahmet Höke, MD, PhD
123. Diagnostic Tests and Clinical Scales for Peripheral Neuropathy
John D. Markman, MD
124. Complex Regional Pain Syndrome
Frank S. Pidcock, MD
125. Entrapment Neuropathies
James W. Teener, MD
126. Demyelinating Neuropathies
John A. Goodfellow, MD, Amy I. Davidson, MD, and Hugh J. Willison, MD
127. Immune Mediated Neuropathies
John A. Goodfellow, MD, Amy I. Davidson, MD, and Hugh J. Willison, MD
128. Radiculopathy and Plexopathy
Michael Wheaton, MD, Dustin Nowacek, MD, and Zachary London, MD

SECTION IX: Neoplastic and Paraneoplastic Diseases (Lisa DeAngelis, Memorial Slaon-Kettering Cancer Center)
129. Infiltrative Astrocytomas: WHO grade II - Diffuse Astrocytoma; WHO grade III - Anaplastic Astrocytoma
Aaron Mammoser, MD, MS
130. Glioblastomas
Aaron Mammoser, MD, MS
131. Oligodendrogliomas
Thomas J. Kaley, MD
132. Primary CNS Tumores: Ependymomas
Brett J. Theeler, MD and Mark R. Gilbert, MD
133. Meningiomas
Jennie Taylor, MD and Patrick Y. Wen, MD
134. Pineal Region Neoplasms
Neil Haranhalli, MD and Jerome J. Graber, MD, MPH
135. Pituitary Tumors
Toral R. Patel, MD and Viviane S. Tabar, MD
136. Primary Central Nervous System Lymphoma
Lisa M. DeAngelis, MD
137. Familial CNS Tumor Syndromes: Neurofibromatosis Type 1
Ibrahim Hussain, BA and David H. Gutmann, MD, PhD
138. Familial CNS Tumor Syndromes: Neurofibromatosis Type-2
Jennie W. Taylor, MD and Scott R. Plotkin, MD, PhD
139. Familial CNS Tumor Syndromes: Tuberous Sclerosis
Darcy A. Krueger, MD, PhD
140. Familial CNS Tumor Syndromes: Von Hippel-Lindau Disease
Gurvinder Kaur, MD, Leonel Ampie, MD, Joseph Weiner, MD, and Aruna Ganju, MD
141. Pediatric Tumors: Pontine Glioma
Kristin Schroeder, MD, MPH and Oren Becher, MD
142. Pediatric Tumors: Medulloblastoma
Kevin C De Braganca, MD and Roger J. Packer, MD
143. Pediatric Tumors: Cerebellar Astrocytoma
Vijay Ramaswamy, MD, FRCPC, Jason T. Huse, MD, PhD, and Yasmin Khakoo, MD
144. Brain Metastases
Jasmin Jo, MD and David Schiff, MD
145. Overview: Other Proven and Putative Autoimmune Disorders of the CNS
Myrna R. Rosenfeld, MD, PhD, Maarten J. Titulaer, MD, PhD, and Josep Dalmau, MD, PhD
146. Chemotherapy and Radiation Therapy
Mary R. Welch, MD and Craig Nolan, MD

SECTION X: Infectious Diseases of the Nervous System (Karen Roos, Indiana University)
147. Acute Bacterial Meningitis
Pratibha Singhi, MD, PhD, Naveen Sankhyan, MD, PhD, and Sunit Singhi, MD, PhD
148. Cerebrospinal Fluid
John E. Greenlee, MD
149. History of Human Immunodeficiency Virus Infection and the Nervous System
Avindra Nath, MD
150. HIV-Associated Neurocognitive Disorders
Joshua Alexander, DO, MPH, David J Croteau, MD, FRCPC, and Ronald J Ellis, MD, PhD
151. HIV Infection: Opportunistic Infections in HIV-Infected Individuals
William Tyor, MD
152. Varicella Zoster Virus Infection of the Nervous System
Don Gilden, MD, Randall J. Cohrs, PhD, Ravi Mahalingam, PhD, and Maria A. Nagel, MD
153. Brain Abcess
Bela R. Turk, MD, Ali Fatemi, MD, and Michael V. Johnston, MD
154. Epidural Abscess and Sudural Empyema
Tyler R West, DO and Kelly J Baldwin, MD
155. Spinal Epidural Abcess
Minal A. Shah, MD and Rabih O. Darouiche, MD
156. Transverse Myelitis
Ivana Vodopivec, MD, PhD and Tracey A. Cho, MD
157. CNS Tuberculosis
Andrea T. Cruz, MD, MPH, and Jeffrey R. Starke, MD
158. Rabies
Alan C. Jackson, MD
159. Disorders Caused by Botulinum Toxin and Tetanus Toxin
Carl E. Stafstrom, MD, PhD
160. Rickettsial Diseases
Lisa Sun, MD and Michael V. Johnston, MD
161. Ehrlichial Diseases
Nicholas S. Havens, MD and William E. Roland, MD, FACP
162. Nervous System Lyme Disease
John J. Halperin, MD
163. Congenital and Perinatal Viral Infections
James F. Bale, Jr., MD
164. Neurologic Manifestations of HTLV-1
Abelardo Q-C Araujo, MD, MSc, PhD, FAAN
165. Fungal Infections of the Central Nervous System
Pratibha Singhi,MD, Karthi Nallasamy, MD, and Sunit Singhi, MD
166. Malaria
Kiran Thakur, MD
167. Babesiosis
Kristyn E. Feldman, MD and Joshua Biddle, MD
168. Amebic Encephalitis
Pratibha Singhi, MD and Arushi G. Saini, MD
169. Prions
James A. Mastrianni, MD, PhD and Joshuae G. Gallardo, MD
170. Neurological Complications of Infective Endocarditis
Jonah Grossman, MD, Tanzila Shams, MD, and Cathy Sila, MD
171. Post-Infectious Encephalitis
Agusto A. Miravalle, MD
172. Central Nervous System Whipple Disease
Arun Venkatesan, MD, PhD
173. Progressive Multifocal Leukoencephalopathy
Arun Swaminathan, MBBS and Joseph R. Berger, MD

SECTION XI: Sleep Disturbances (Mark Dyken, University of Iowa)
174. REM Sleep Behavior Disorder
Shannon S. Sullivan, MD
175. Restless Leg Syndrome and Periodic Limb Movements in Sleep
Jennifer Accardo, MD, MSCE
176. Insomnia
Wilfred R. Pigeon, MD and Henry J. Orff, MD
177. Sleep Apnea
Jennifer Accardo, MD, MSCE
178. Neurobiology of Circadian Rhythm Disorders
Christopher R. Jones, MD, PhD

SECTION XII: Substance Abuse and Toxicological Disorders (Barry Kosofsky, Weil-Cornell University Medical Centers)
179. Neurologic Manifestations of Organic Chemicals
John L. O'Donoghue, VMD, PhD, DABT
180. Neurobiology of Drug Addiction and Clinical Aspects of Drug Abuse
Leslie R. Whitaker, MD and Bruce T. Hope, MD
181. Neurological Effects of Marine Toxins
Kazuma Nakagawa, MD
182. Fetal Alcohol Syndrome
Carmen Lopez-Arvizu, MD, Carmel Bogle, MD, and Harolyn M.E. Belcher, MD, MHS
183. Fetal Exposure to Tobacco and Cannabis
Edore C. Onigu-Otite, MD
184. Neurobiology of Child Maltreatment and Psychological Trauma
Nikeea Copeland-Linder, PhD, MPH, Edore Onigu-Otite, MD, Jennifer Serico, PhD, Mariflor Jamora, MD, and Harolyn M.E. Belcher, MD, MHS
185. Neurobiology of Intrauterine Opiate and Cocaine Exposure
Harolyn M.E. Belcher, MD, MHS and Samantha Hutchison, MD

SECTION XIII: Neurologic Manifestations of Medical Disorders (John Probasco, Johns Hopkins University)
186. Electrolyte Disturbances
Mark N. Rubin, MD and Alejandro A. Rabinstein, MD
187. Nutritional Deficiencies
Deanna Saylor, MD, MHS and John C. Probasco, MD
188. Alcohol Abuse
Killian A. Welch, MD
189. Thyroid Disease
Makoto Ishii, MD, PhD
190. Parathyroid, Adrenal, Ovarian, Testicular and Pituitary Disease
Gary M. Abrams, MD
191. Cardiac Disease
Roger E. Kelley, MD
192. Neurobiology of Brain Injury after Cardiac Arrest
Lucia Rivera-Lara, MD and Romergryko G. Geocadin, MD
193. Gastrointestinal Disease
Brian P. Bosworth, MD, Brian R. Landzberg, MD, and Elisa McEachern, MD
194. Neurological Manifestations of Renal Disease
Joshua S. Hundert, MD, Rashmi Verma, MD, Ritika Suri, MD, Anika T. Singh, MD, and Ajay Singh, MD
195. Systemic Lupus Erythematosis
Richard B. Rosenbaum, MD
196. Neurologic Manifestations of Hematological Disease
Elham Bayat, MD