Category: 2019

The Causes of Epilepsy: Common and Uncommon Causes in Adults and Children

The Causes of Epilepsy: Common and Uncommon Causes in Adults and Children

Shorvon, S., Guerrini, R., Schachter, S., & Trinka, E. (Eds.).
Cambridge: Cambridge University Press, 2019
Pages 989
ISBN: 978-1-108-42075-4 Hardback


The identification of the cause of an epileptic seizure is a key element in the clinical management of all patients. In recent decades, advances in theory, neuroimaging, molecular genetics and molecular chemistry have revolutionised our ability to investigate and identify the underlying cause. The definitive and unrivalled textbook on the causes of epilepsy, this second edition is extensively revised and expanded. It provides concise descriptions of all the major genetic and acquired conditions that cause epilepsy in adults and children, and the provoking factors for epileptic seizures and of the causes of status epilepticus. A new section considers clinical approaches to diagnosing causes, to guide and assist clinicians in investigations. With 128 chapters written by leading figures from around the world, this comprehensive and authoritative resource is indispensable to senior and junior clinicians and trainees working in the field of epilepsy, including specialists in neurology, paediatrics, neurophysiology, psychiatry and neurosurgery.


Part I: Introduction
1. Concept of Causation in Epilepsy
2. Epileptogenesis in Idiopathic Epilepsy
3. An introduction to Epilepsy Genetics
4. Epileptogenesis in Symptomatic Epilepsy
5. Animal Models of Epilepsy

Part II: Approaches to the Clinical Investigation and Diagnosis of Cause
6. Approach to the Diagnosis of Neonatal Seizures
7. Approach to the Genetic Diagnosis of Epileptic Encephalopathies and Developmental Encephalopathies with Epilepsy of Early Childhood
8. Approach to the Diagnosis of Childhood-onset Epilepsy Associated with Developmental Delay
9. Approach to the diagnosis of cortical developmental disorders and their clinical genetics
10. Approach to the diagnosis of the Inborn Errors of Metabolism Associated with Epilepsy and their Clinical genetics
11. Approach to the Diagnosis of Epilepsy Presenting with Myoclonus
12. Approach to theDiagnosis of Epilepsy Syndromes with Multiple Causes
13. Approach to the Diagnosis of Causation in Epilepsy in Adults

Part III: Idiopathic Epilepsies: Pure Epilepsies without Known Cause
14. Idiopathic Generalized Epilepsies
15. Benign Partial Epilepsies of Childhood

Part IV (a): Symptomatic Epilepsies of Genetic or Developmental Origin: Single Gene Disorders and Inborn Errors of Metabolism
16. Benign Familial Neonatal Epilepsy (BFNE)
17. Sleep-related Hypermotor Epilepsy (SHE)
18. Genetic Epilepsy with Febrile Seizures Plus (GEFS+)
19. Dravet Syndrome and Other SCN1A Disorders
20. Familial Lateral Temporal Lobe Epilepsy
21. Familial Focal Epilepsy with Variable Foci
22. PCDH19 Mutations Related Epilepsy: phenotype and genotype
23. CDKL5 Encephalopathy
24. KCNQ2 Encephalopathy
25. FOXG1 Encephalopathy
26. STXBP1 Encephalopathy
27. Rett Syndrome
28. GLUT1 Deficiency Syndrome
29. Other Rare Single-gene Disorders Causing Epileptic Encephalopathy
30. Mitochondrial Epilepsies
31. Lysosomal Disorders and Epilepsy
32. Peroxisomal disorders and epilepsy
33. Menkes’ Disease
34. Neuroacanthocytosis
35. Organic acid and Amino Acid Metabolism Disorders
36. Porphyrias
37. Pyridoxine-Dependent Epilepsy
38. Fatty Acid Oxidation Disorders
39. GABA Metabolism Deficiency Syndromes
40. Disorders of Creatine Metabolism and Epilepsy
41. Epilepsy Caused by Congenital Disorders of Glycosylation
42. Urea Cycle Disorders
43. Hyperinsulinism-hyperammonaemia and Biotin Pathway Defects
44. Other Single Gene Disorders

Part IV (b): Symptomatic Epilepsies of Genetic or Developmental Origin: Progressive Myoclonic Epilepsies
45. Unverricht–Lundborg disease (or progressive myoclonus epilepsy type 1)
46. Dentatorubral-pallidoluysian atrophy; DRPLA
47. Lafora Body Disease
48. Epileptic Syndromes in Mitochondrial Disease
49. Neuronal Ceroid Lipofuscinoses
50. Sialidosis
51. Progressive Myoclonic Epilepsies: other rarer causes

Part IV (c): Symptomatic Epilepsies of Genetic or Developmental Origin: Neurocutaneous Syndromes
52. Tuberous Sclerosis Complex
53. Neurofibromatosis
54. Sturge-Weber Syndrome
55. Other Neurocutaneous Syndromes

Part IV (d): Symptomatic Epilepsies of Genetic or Developmental Origin: Epilepsies Associated with Chromosomal Abnormalities
56. Copy Number Variations Causing Epilepsy
57. MECP2 Duplication Syndrome
58. Down Syndrome
59. Fragile X Syndrome
60. 4p Deletion (Wolf–Hirschhorn) Syndrome
61. Inverted Duplicated Chromosome 15 (Isodicentric Chromosome 15)
62. Ring Chromosome 20
63. Ring Chromosome 14 and Other Rare Ring Chromosomal Disorders
64. Angelman Syndrome

Part IV (e): Symptomatic Epilepsies of Genetic or Developmental Origin: Epilepsies Associated with Developmental Anomalies of Cerebral Structure
65. Hemimegalencephaly
66. Focal Cortical Dysplasia
67. Agyria–pachygyria Band spectrum
68. Corpus Callosum and Epilepsies
69. Polymicrogyria and Schizencephaly
70. Periventricular Nodular Heterotopia
71. Microcephaly
72. Arachoid Cysts
73. Disorders Associated with Tubulinopathies and motoropathies

Part V (a): Symptomatic Epilepsies of Acquired Origin: Epilepsies Associated with Cerebral Trauma
74. Epilepsy Associated with Head Injury
75. ‘De Novo’ Epilepsy after Neurosurgery
76. Epilepsy after Epilepsy Surgery
77. Epilepsy after Abusive Head Trauma

Part V (b): Symptomatic Epilepsies of Acquired Origin: Epilepsies Associated with Cerebral Tumours
78. Epilepsy Associated with Glioma
79. Epilepsy Associated with Ganglioglioma, Dysembryoplastic Neuroepithelial Tumor, and Related Tumors
80. Hypothalamic Hamartoma and Gelastic Epilepsy
81. Epilepsy Associated with Meningioma
82. Metastatic Disease

Part V (c): Symptomatic Epilepsies of Acquired Origin: Epilepsies Associated with Cerebral Infection
83. Epilepsy Associated with Viral Encephalitis
84. Bacterial Meningitis and Focal Suppurative Intracranial Infections in Children
85. Bacterial Meningitis and Pyogenic Abscess in Adults
86. Epilepsy Associated with Malaria
87. Epilepsy Associated with Neurocysticercosis
88. Other Parasitic Diseases
89. Epilepsy Associated with Tuberculosis
90. HIV and Seizures
91. Emerging and Less Common Central Nervous system Viral Encephalitides

Part V (d): Symptomatic Epilepsies of Acquired Origin: Epilepsies Associated with Cerebrovascular Disease
92. Epilepsy Associated with Intracerebral Hemorrhage
93. Epilepsy Associated with Subarachnoid Hemorrhage
94. Epilepsy and Cerebrovascular Disease
95. Epilepsy Associated with Arteriovenous Malformations
96. Epilepsy Associated with Cavernous Malformations
97. Epilepsy Associated with Other Vascular Disorders

Part V (e): Symptomatic Epilepsies of Acquired Origin: Epilepsies Associated with Cerebral Immunological Disorders
98. Rasmussen’s Encephalitis and Related Conditions
99. Epilepsy Associated with Systemic Lupus Erythematosus and Other Collagen Vascular Diseases
100. Epilepsy Associated with Inflammatory and Immunological Diseases of the Central Nervous System
101. Epilepsy in Multiple Sclerosis and Other Acquired Demyelinating Diseases
102. Immune-Mediated Epilepsy

Part V (f): Symptomatic Epilepsies of Acquired Origin: Epilepsies Associated with Other Cerebral Disorders
103. Hippocampal Sclerosis
104. Epilepsy Associated with Psychiatric Disorders
105. Hydrocephalus and Porencephaly
106. Epilepsy Associated with Alzheimer’s Disease and Other Adult Neurodegenrative
107. Epilepsy Associated with Eclampsia and the Posterior Reversible Encephalopathy
108. Cerebral Palsy
109. Seizures and Epilepsy Associated with Pertussis and Other Vaccinations

Part VI: Provoking Factors and Provoked Epilepsies: Reflex Seizures
110. Fever as a Precipitating Factor for Epileptic Seizures
111. The Menstrual Cycle and Catamenial Epilepsy
112. Sleep and Epilepsy
113. Electrolyte and Sugar Disturbances
114. Drug-Induced Seizures
115. Recreational and illicit drugs causing seizures and epilepsy
116. Alcohol- and Toxin-Induced Seizures
117. Visual Stimuli, Photosensitivity and Photosensitive Epilepsy
118. Startle-Induced and Other Sensory-Induced Epilepsy
119. Primary Reading Epilepsy
120. Auditory-Induced Epilepsy
121. Seizures Induced by Eating, a Rare but Special Form of Reflex Epilepsy
122. Hot Water Epilepsy
123. Reflex Epilepsy with Higher-Level Processing

Part VII: Status Epilepticus
124. The Causes of Status Epilepticus in Children
125. The Common Causes of Convulsive Status Epilepticus in Adults
126. Uncommon Causes of Status Epilepticus
127. Causes of Nonconvulsive Status Epilepticus in Adults
128. The Causes of Epilepsia Partialis Continua

Queen Square: A history of the National Hospital and its Institute of Neurology

Queen Square: A history of the National Hospital and its Institute of Neurology

Simon Shorvon, Alastair Compston
Cambridge University Press, 2019
Pages: 561
ISBN: 978 1 107 10082 4 (Hardback)
Online ISBN: 781316181430


As the first neurological hospital in the world, founded in 1859, the National Hospital, Queen Square, and its affiliated Institute of Neurology remain leading neurological centres providing exceptional clinical services, teaching and research. Illustrated by over 100 historical images and much unpublished archival material, this book provides a comprehensive history of the National Hospital, the Institute, and their staff. It relates the ups and downs of the Hospital and Institute in war and peacetime, their financial struggles, many personality conflicts, efforts to remain independent and to maintain neurological dominance, academic and clinical contributions, issues relating to specialisation and sub-specialisation and relations between disciplines, and the changing roles of the Hospital and Institute. The history is told from varying perspectives against the backdrop of the evolution of British clinical neuroscience, the special position of London medicine, and the influence of world wars, and is set in the context of modern British social history.

Table of contents


Chapter 1: Foundation and Making of the National Hospital

Chapter 2: Queen Square, the Salmon Pink and Other Hospital Buildings

Chapter 3: Queen Square and Neurology 1860–1902

Chapter 4: National Hospital Quadrumvirate

Chapter 5: Roller-coaster Ride and the National Hospital Rubs Along 1902–1945

Chapter 6: Five Dominant Physicians

Chapter 7: The NHS Arrives and the Hospital Celebrates its Centenary 1946–1962

Chapter 8: Beyond the Walls: British Neurology Outside Queen Square

Chapter 9: Neurosurgery and War Neurology at Queen Square

Chapter 10: Other Clinical Specialties at Queen Square

Chapter 11: The Investigatory Specialties at Queen Square

Chapter 12: The Medical School and the Institute of Neurology

Chapter 13:  The Rise of Academic Neurology at Queen Square 1962– 1997

Chapter 14:  Never, Never, Ever –  Change and Integration 1962– 1997