Yale Univ., New Haven, CT. Written to assist the clinician in the diagnosis and management of viral encephalitis. Rooted in neuropathological descriptions and pathogenesis concepts, text discusses encephalitides which are acute sporadic, acute epidemic, parainfectious immune mediated, and subacute or chronic. Halftone illustrations. DNLM: Encephalitis, Viral--diagnosis.

John Booss: National Director of Neurology, Department of Veterans Affairs, VA Connecticut, West Haven, and Professor of Neurology and of Laboratory Medicine, Yale University School of Medicine, New Haven, Connecticut Margaret M. Esiri: Professor of Neuropathology, Department of Clinical Neurology, University of Oxford, and Honorary Consultant Neuropathologist, Oxford Radcliffe NHS Trust, Oxford, United Kingdom

Preface

ix

Acknowledgments

xi

INTRODUCTION

1 Pathological Features of Encephalitis in Humans

3

(18)

Sources of Information on the Pathology of Encephalitis

3

(2)

Naked-Eye Examination of the Brain and Routine Light Microscopy

5

(1)

Immunocytochemistry

6

(1)

PCR

7

(1)

In Situ Hybridization

7

(1)

Electron Microscopy

8

(1)

Pathological Features Common to Most Forms of Viral Encephalitis

8

(4)

Pathological Features Restricted to Certain Forms of Encephalitis

12

(3)

Complications and Sequelae

15

(1)

The Need for Autopsies

16

(5)

2 Diagnostic Evaluation

21

(10)

Differentiation of Encephalitis from Other Conditions

21

(2)

Types of Encephalitis: Key Initial Questions

23

(1)

Clinical Classification of Encephalitis

24

(1)

Diagnostic Evaluation of Encephalitis

25

(2)

Diagnostic Virology and Serology

27

(2)

Encephalitis of Undetermined Etiology

29

(1)

Summary

29

(2)

3 Management Decisions in Acute Encephalitis

31

(10)

Common Management Issues

31

(1)

Antiviral and Immunomodulating Therapy

32

(1)

Therapy by Type of Encephalitis

32

(1)

Clinical Evaluation for Acute Management

33

(1)

Acute Management Decisions

34

(1)

Reduction of Raised ICP

35

(1)

Control of Seizures

35

(1)

Management in the Recovery Period

36

(1)

Appendix-Encephalitis Treatment Checklist Arranged by Clinical Classification

37

(4)

ACUTE SPORADIC ENCEPHALITIS

4 Herpes Simplex Encephalitis

41

(20)

Introduction and Classification of Acute Sporadic Encephalitis

41

(1)

HSV

42

(1)

Historical Considerations

42

(1)

Pathology of HSE

43

(4)

Pathology of Atypical HSV-1 Encephalitis and HSV-2 Encephalitis and Myelitis

47

(1)

Pathogenesis of HSE

47

(1)

Clinical Evaluation of HSE

48

(6)

Therapy, Course, and Outcomes

54

(7)

5 Systemic Viral Diseases and Encephalitis

61

(20)

Exanthematous Diseases

61

(4)

Mumps

65

(2)

EBV-IM-Glandular Fever

67

(3)

Adenovirus

70

(1)

Gastroenteritis-Rotaviruses

71

(1)

Hepatitis Viruses

72

(9)

6 Encephalitis Viruses Transmitted from Animals

81

(16)

Sporadic Arbovirus Infection

81

(1)

LCMV

82

(1)

Rabies

83

(6)

Herpes B Virus of Monkeys-Herpesvirus simiae Cercopithecine Herpesvirus

89

(8)

ACUTE PARAINFECTIOUS IMMUNE-MEDIATED ENCEPHALOMYELITIS

7 Pathology of Allergic Forms of Encephalomyelitis

97

(10)

Pathology of Human PVE (ADEM)

98

(1)

Pathology of AH LE

99

(1)

Acute Parainfectious Encephalopathy

100

(1)

Pathogenesis of Allergic Encephalitis

101

(6)

8 Clinical Syndromes Including Smallpox and Measles

107

(20)

ADEM

107

(3)

Acute Cerebellar Ataxia and Related Syndromes

110

(1)

Brain Stem ADEM Including Bickerstaff's Brain Stem Encephalitis

111

(1)

Miller Fisher Syndrome

112

(1)

ATM

112

(1)

Acute Hemorrhagic Leukoencephalopathy (Hurst)

113

(1)

Acute Encephalopathy, Including Reye's Syndrome

114

(1)

Postviral Stroke

115

(1)

Smallpox and Its Vaccination

116

(3)

Measles

119

(8)

9 Varicella-Zoster Virus: the Paradox of Immune Mediation and Immunocompromise

127

(16)

Chicken Pox

128

(1)

Zoster

129

(14)

ACUTE EPIDEMIC ENCEPHALITIS

10 Influenza Virus, Enteroviruses, and Other Epidemic Viruses

143

(20)

Human-To-Human Transmission

144

(10)

Animal-To-Human Transmission: Nipah Virus

154

(1)

Unknown Means of Transmission

155

(8)

11 The Arboviruses

163

(30)

Pathology

163

(1)

Pathogenesis

164

(1)

Diagnosis and Management

165

(1)

Mosquito-Borne Encephalitides

165

(15)

TBEs

180

(13)

SUBACUTE AND CHRONIC ENCEPHALITIS AND PRION DISEASES

12 HIV/AIDS

193

(28)

The Pathogenesis and Pathology of AIDS

194

(1)

Systemic Pathology of AIDS

195

(1)

Neuropathology of HIV Infection

196

(1)

Pre-AIDS Neuropathology in Untreated Individuals: Lymphocytic Meningitis

196

(1)

AIDS Neuropathology

196

(3)

Neuronal Pathology and Its Pathogenesis

199

(2)

Vascular Pathology

201

(1)

AIDS-Associated Myelopathy and HIV Myelitis

201

(1)

Influence of Antiretroviral Treatment on the Neuropathology of AIDS

201

(1)

Clinical Features of Early HIV Disease in the CNS

201

(4)

Antiretroviral Therapy

205

(1)

Clinical Features of AIDS in the CNS

205

(6)

Pediatric AIDS

211

(10)

13 Encephalitis in Immunocompromised Patients

221

(16)

PML

221

(4)

CMV Encephalitis and Radiculomyelitis

225

(3)

Enteroviral CNS Infections in Primary Hypogammaglobulinemie or Agammaglobulinemia

228

(1)

Immunosuppressive Measles Encephalitis (IME)

229

(1)

Encephalitis Associated with Organ Transplantation and Bone Marrow Allografting

230

(7)

14 Encephalitis in Immunocompetent Patients

237

(16)

SSPE

237

(5)

Progressive Rubella Panencephalitis (PRP)

242

(1)

HAM/TSP

243

(2)

Rasmussen's Encephalitis

245

(1)

Vilyuisk Encephalitis Bokhoror

246

(1)

Borna Disease

247

(6)

15 Creutzfeldt-Jakob Disease and Other Prion Disorders

253

(18)

Pathology

254

(4)

Pathogenesis of CJD

258

(2)

Diagnosis and Management

260

(6)

Infection Control

266

(5)

Index

271

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Viral Encephalitis in Humans

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