Case-based Approach to Common Neurological Disorders

Contents
About the Editors
Part I: CNS Infections
	1: Japanese Encephalitis
		Case Scenario
		1.1	 Introduction
		1.2	 Epidemiology
		1.3	 Pathogenesis
		1.4	 Pathology
		1.5	 Clinical Manifestation
		1.6	 Diagnosis
		1.7	 Differential Diagnosis
			1.7.1	 Herpes Simplex Encephalitis
			1.7.2	 Acute Disseminated Encephalomyelitis
		1.8	 Treatment
		1.9	 Prognosis
		References
	2: Herpes Encephalitis
		2.1	 Introduction
		2.2	 Epidemiology
		2.3	 Pathology
		2.4	 Pathogenesis
		2.5	 Clinical Manifestation
		2.6	 Diagnosis
		2.7	 Differential Diagnosis
			2.7.1	 Japanese Encephalitis
			2.7.2	 Brain Abscess
			2.7.3	 Treatment and Prognosis
		References
	3: Acute Bacterial Meningitis
		3.1	 Introduction
		3.2	 Epidemiology
		3.3	 Pathogenesis
			3.3.1	 Clinical Features
		3.4	 Differential Diagnosis
		3.5	 Diagnosis
		3.6	 Treatment
		3.7	 Prognosis
		References
	4: Cryptococcal Meningoencephalitis
		4.1	 Introduction
		4.2	 Epidemiology
		4.3	 Pathophysiology
		4.4	 Clinical Manifestations
		4.5	 Diagnosis
		4.6	 Differential Diagnosis
		4.7	 Treatment
		4.8	 Prognosis
		References
	5: Neurocysticercosis
		5.1	 Introduction
		5.2	 Epidemiology
		5.3	 Lifecycle of the Parasite
		5.4	 Clinical Manifestations
			5.4.1	 Epilepsy/Seizures
			5.4.2	 Focal Neurological Deficits
			5.4.3	 Raised Intracranial Pressure
			5.4.4	 Cognitive Decline
		5.5	 Diagnosis
		5.6	 Neuroimaging
		5.7	 Laboratory and Immunological Diagnosis
		5.8	 Diagnostic Criteria
		5.9	 Degrees of Diagnostic Certainty
		5.10	 Treatment
		5.11	 Treatment of Extraparenchymal Neurocysticercosis
		5.12	 Prevention and Control of Parasites
		References
	6: Tetanus
		6.1	 Introduction
		6.2	 Epidemiology
		6.3	 Microbiology
		6.4	 Natural History of Disease
		6.5	 Pathophysiology
		6.6	 Clinical Manifestation
			6.6.1	 Severity Grading
		6.7	 Diagnosis
		6.8	 Differential Diagnosis
		6.9	 Treatment
			6.9.1	 Intensive Care
			6.9.2	 Other Adjunct Therapies
			6.9.3	 Other Supportive Care
		6.10	 Vaccination
		6.11	 Complications
			6.11.1	 Prognosis
		References
	7: Tubercular Meningitis
		7.1	 Introduction
		7.2	 Epidemiology
		7.3	 Pathophysiology
		7.4	 Clinical Manifestations
		7.5	 Neurological Complications in TB Meningitis
		7.6	 Diagnosis
		7.7	 Treatment and Prognosis
		References
	8: Subacute Sclerosing Panencephalitis
		8.1	 Introduction
		8.2	 History
		8.3	 Epidemiology of SSPE Relative to Measles
		8.4	 Pathogenesis
		8.5	 Pathology
		8.6	 Clinical Manifestations
			8.6.1	 Classical SSPE
		8.7	 Criteria for Diagnosis of SSPE
		8.8	 Non-classical or Atypical SSPE
		8.9	 Fulminant SSPE
		8.10	 Protracted Course in SSPE
		8.11	 SSPE in Adults
		8.12	 HIV and Measles
		8.13	 Investigations
		8.14	 Differential Diagnosis
		8.15	 Missed Diagnosis of SSPE
		8.16	 Treatment
		References
	9: Brain Abscess
		9.1	 Introduction
		9.2	 Etiology
		9.3	 Bacterial Brain Abscess
		9.4	 Fungal Brain Abscess
		9.5	 Protozoal and Helminthic Brain Abscess
		9.6	 Mycobacterial Abscesses
		9.7	 Pathogenesis and Pathophysiology
		9.8	 Clinical Presentation
		9.9	 Diagnosis
		9.10	 Differential Diagnosis
		9.11	 Treatment
		References
Part II: Cardiovascular Disorders
	10: Acute Ischemic Stroke
		10.1	 Introduction
		10.2	 Epidemiology
		10.3	 Risk Factors
		10.4	 Clinical Features
		10.5	 Diagnosis
		10.6	 Treatment
		10.7	 Exclusion Criteria for Treatment with t-PA [21*]
		10.8	 Special Considerations in a Select Group of  Patients [21]
		10.9	 Secondary Prevention of Acute Ischemic Stroke
		References
	11: Subarachnoid Hemorrhage
		11.1	 Introduction
		11.2	 Epidemiology
		11.3	 Pathophysiology
		11.4	 Etiology of SAH
			11.4.1	 Traumatic SAH
			11.4.2	 Spontaneous SAH
		11.5	 Clinical Manifestation
		11.6	 Grading of SAH
		11.7	 Diagnosis
			11.7.1	 Diagnostic Tools
				11.7.1.1	 Computed Tomography
				11.7.1.2	 Lumbar Puncture
			11.7.2	 Angiographic Studies
				11.7.2.1	 CT Cerebral Angiography
				11.7.2.2	 Digital Subtraction Angiography
				11.7.2.3	 Magnetic Resonance Imaging and MR Angiography
			11.7.3	 Differential Diagnosis
		11.8	 Management
			11.8.1	 Acute Phase
				11.8.1.1	 Initial Stabilization
				11.8.1.2	 Prevention of Rebleeding
				11.8.1.3	 Management of Intracranial Hypertension
				11.8.1.4	 Management of Hydrocephalus
				11.8.1.5	 Management of Seizure
				11.8.1.6	 Management of Cardiopulmonary Dysfunction
			11.8.2	 Subacute Phase
			11.8.3	 Chronic/Delayed Phase
				11.8.3.1	 Prevention of Vasospasm and DCI
					Nimodipine
					Magnesium
					Statins
					Clazosentan
		11.9	 Other Treatments
			11.9.1	 Treatment for Vasospasm and Delayed Cerebral Ischemia
		11.10	 Recent Advances in the Management of Ruptured Aneurysm
			11.10.1	 Endovascular Flow Diversion
		11.11	 Prognosis
		References
	12: Intracerebral Hemorrhage
		12.1	 Introduction
		12.2	 Epidemiology
		12.3	 Diagnosis
			12.3.1	 Interpreting the CT Scan
		12.4	 Management of BP
			12.4.1	 Correction of Coagulopathy
			12.4.2	 Surgical Management
			12.4.3	 Ancillary Management
		12.5	 Prognosis
		References
	13: Cerebral Sinus and Venous Thrombosis
		13.1	 Introduction
		13.2	 Relevant Anatomy
		13.3	 Pathophysiology
		13.4	 Etiology
		13.5	 Clinical Features
		13.6	 Diagnosis
		13.7	 Management
			13.7.1	 Anticoagulations
			13.7.2	 Management of Early Complications
		13.8	 Prognosis
		References
	14: Large Hemispheric Stroke
		14.1	 Introduction
		14.2	 Pathophysiology
		14.3	 Clinical Features
		14.4	 Imaging
		14.5	 Management
		14.6	 Prognosis
		14.7	 Future Directions
		References
Part III: Epilepsy
	15: Childhood Absence Epilepsy
		15.1	 Introduction
		15.2	 Epidemiology
		15.3	 Pathophysiology
		15.4	 Genetics
		15.5	 Diagnosis
		15.6	 Treatment
		15.7	 Outcomes
		References
	16: Status Epilepticus
		16.1	 Introduction
		16.2	 Etiology and Pathophysiology
		16.3	 Definition/Classification
		16.4	 Diagnosis
			16.4.1	 Continuous EEG Monitoring
		16.5	 Treatment
		16.6	 Outcome
		References
Part IV: Neuromuscular Diseases
	17: Myasthenia Gravis
		17.1	 Introduction
		17.2	 Pathophysiology
		17.3	 Clinical Presentation
		17.4	 MuSK Myasthenia
		17.5	 MGFA Classification of Myasthenia Gravis
		17.6	 Investigations
		17.7	 Electrophysiological Test
		17.8	 Thymus and Myasthenia
		17.9	 Management
			17.9.1	 Pharmacological Treatment
			17.9.2	 Autoimmune Treatment
			17.9.3	 Plasma Exchange and Intravenous Immunoglobulin
			17.9.4	 Newer Treatments
		17.10	 Thymectomy
		References
	18: Amyotrophic Lateral Sclerosis
		18.1	 Introduction
		18.2	 Epidemiology
		18.3	 Phenotypes and Clinical Features
		18.4	 Pathogenesis
		18.5	 Diagnostic Criteria of ALS
		18.6	 Investigations
			18.6.1	 Nerve Conduction Studies (NCS)
			18.6.2	 Electromyography (EMG)
			18.6.3	 Repetitive Nerve Stimulation and Single-Fiber EMG
			18.6.4	 Neuroimaging Findings in ALS
			18.6.5	 Other Investigations
		18.7	 Familial ALS (FALS)
		18.8	 ALS Management and Multidisciplinary Approach
			18.8.1	 Dietary Supplements
		References
	19: Myotonic Dystrophy
		19.1	 Introduction
		19.2	 Epidemiology
		19.3	 Pathogenesis
		19.4	 Clinical Manifestations
			19.4.1	 Diagnosis
			19.4.2	 EMG
			19.4.3	 Differential Diagnosis
			19.4.4	 Treatment and Prognosis
		References
	20: Guillain-Barré Syndrome
		20.1	 Introduction
		20.2	 Epidemiology
		20.3	 Pathology
		20.4	 Pathogenesis
		20.5	 Clinical Manifestation
		20.6	 Diagnosis
		20.7	 Differential Diagnosis
			20.7.1	 Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
			20.7.2	 Myasthenia Gravis
		20.8	 Treatment
		20.9	 Prognosis
		References
	21: Inflammatory Muscle Diseases
		21.1	 Introduction
		21.2	 Incidence
		21.3	 Dermatomyositis (DM)
		21.4	 Polymyositis (PM)
			21.4.1	 Muscle Pathology
		21.5	 Necrotizing Myopathy (NM)
			21.5.1	 Muscle Pathology
		21.6	 Overlap Myositis (OM)
			21.6.1	 Muscle Pathology
		21.7	 Inclusion Body Myositis (IBM)
			21.7.1	 Muscle Pathology
			21.7.2	 Pathogenesis
		21.8	 Laboratory Investigations
		21.9	 MRI Findings in Inflammatory Muscle Diseases
			21.9.1	 Course of Illness
			21.9.2	 Risk of Malignancy and Extramuscular Manifestations in Myositis
			21.9.3	 Treatment
		21.10	 Management of Inclusion Body Myositis
		References
Part V: Neuroimmunology
	22: Optic Neuritis
		22.1	 Introduction
		22.2	 Anatomy
		22.3	 Epidemiology
		22.4	 Pathophysiology
		22.5	 Symptoms and Signs
		22.6	 Investigations and Laboratory Workup
		22.7	 Differential Diagnosis
			22.7.1	 Anterior Ischemic Optic Neuropathy (AION)
			22.7.2	 Compressive and Infiltrative Optic Neuropathy
			22.7.3	 Infectious Optic Neuropathy
			22.7.4	 Hereditary Optic Neuropathy
		22.8	 Management
		22.9	 Prognosis
		References
	23: Autoimmune Encephalitis
		23.1	 Introduction
		23.2	 Epidemiology
		23.3	 Pathophysiology
		23.4	 Clinical Manifestations
		23.5	 Diagnosis
			23.5.1	 Electroencephalography (EEG)
			23.5.2	 Imaging
			23.5.3	 Cerebrospinal Fluid (CSF) Analysis
			23.5.4	 Autoantibody Test
			23.5.5	 Investigating for Malignancy
		23.6	 Differential Diagnosis
		23.7	 Treatment and Prognosis
		References
Part VI: Movement Disorders
	24: Progressive Supranuclear Palsy
		24.1	 Introduction
		24.2	 Aetiopathogenesis
			24.2.1	 Aetiology
				24.2.1.1	 Genetics
				24.2.1.2	 Environmental Factors and Mitochondrial Dysfunction
			24.2.2	 Neuropathology
		24.3	 Clinical Features
			24.3.1	 Motor Symptoms
				24.3.1.1	 Postural Instability and Falls
				24.3.1.2	 Extrapyramidal Signs
			24.3.2	 Ocular Motor Abnormalities
				24.3.2.1	 Early Stage
				24.3.2.2	 Middle Stage
				24.3.2.3	 Late Stage
			24.3.3	 Behavioural and Cognitive Features
			24.3.4	 Speech, Swallowing and Other Neurological Signs
		24.4	 Diagnostic Criteria
		24.5	 Differential Diagnosis
		24.6	 Investigations
		24.7	 Management
			24.7.1	 Medication
			24.7.2	 Physical Therapy and Other Supportive Measures
		24.8	Conclusion
		References
	25: Multiple System Atrophy
		25.1	 Introduction
		25.2	 Epidemiology
		25.3	 Pathology
			25.3.1	 Macroscopy
			25.3.2	 Histopathology
		25.4	 Pathogenesis
		25.5	 Clinical Manifestation
		25.6	 Autonomic Dysfunction
			25.6.1	 Pyramidal Signs
			25.6.2	 Cognitive Dysfunction and Behavioral Disturbances
			25.6.3	 Sleep Disturbances
			25.6.4	 Red Flags
		25.7	 Diagnosis
			25.7.1	 Clinical Diagnostic Criteria
			25.7.2	 Imaging
			25.7.3	 Cardiac Autonomic Function Tests
			25.7.4	 Sudomotor Testing
			25.7.5	 Urological Evaluation
			25.7.6	 Olfactory Tests
			25.7.7	 Other Investigations
			25.7.8	 Unified MSA Rating Scale (UMSARS)
		25.8	 Differential Diagnosis
		25.9	 Treatment and Prognosis
			25.9.1	 Parkinsonism
			25.9.2	 Camptocormia
			25.9.3	 Focal Dystonia
			25.9.4	 Cerebellar Dysfunction
			25.9.5	 Orthostatic Hypotension
			25.9.6	 Supine Hypertension/Nocturnal Hypertension
			25.9.7	 Postprandial Hypotension (PPH)
			25.9.8	 Urological Dysfunction
			25.9.9	 Sexual Dysfunction
			25.9.10 Respiratory Dysfunction
			25.9.11	 Gastrointestinal Dysfunction
			25.9.12 REM Behavior Disorder
			25.9.13 Depression
		25.10	 Prognosis of MSA
		References
	26: Lower-Body Parkinsonism
		26.1	 Introduction
			26.1.1	 Vascular (Arteriosclerotic) Parkinsonism (VP)
				26.1.1.1	 Epidemiology
				26.1.1.2	 Clinical Features
				26.1.1.3	 Aetiology
				26.1.1.4	 Pathology
				26.1.1.5	 Mechanism
				26.1.1.6	 Diagnostic Criteria
				26.1.1.7	 Imaging in VP
				26.1.1.8	 Treatment of VP
			26.1.2	 Normal Pressure Hydrocephalus (NPH)
				26.1.2.1	 Epidemiology
				26.1.2.2	 Clinical Presentation
				26.1.2.3	 Imaging
				26.1.2.4	 Comorbidities
				26.1.2.5	 CSF Tap Test/Lumbar Drainage
				26.1.2.6	 CSF Infusion Test
				26.1.2.7	 Treatment of NPH
		References
	27: Childhood Dystonia
		27.1	 Introduction
		27.2	 Common Causes of Acquired Dystonia
		27.3	 Dystonia Mimics
			27.3.1	 Spasmus Nutans
			27.3.2	 Sandifer Syndrome
			27.3.3	 Satoyoshi Syndrome
		27.4	 Genetic Dystonia
		27.5	 Approach to Childhood Dystonia
		27.6	 Treatment
		References
	28: Neurodegeneration with Brain Iron Accumulation
		28.1	 Introduction
		28.2	 Epidemiology
		28.3	 Etiology
		28.4	 Pathogenesis
		28.5	 Clinical Features
		28.6	 Pantothenate Kinase-Associated Neurodegeneration (PKAN)
		28.7	 Phospholipase A2-Associated Neurodegeneration (PLAN)
		28.8	 Mitochondrial Membrane Protein-Associated Neurodegeneration (MPAN)
		28.9	 Beta-Propeller Protein-Associated Neurodegeneration (BPAN)
		28.10	 Fatty Acid Hydroxylase-Associated Neurodegeneration (FAHN)
		28.11	 Coenzyme A Synthase Protein-Associated Neurodegeneration (CoPAN)
		28.12	 Kufor-Rakeb Syndrome
		28.13	 Neuroferritinopathy
		28.14	 Aceruloplasminemia
		28.15	 Woodhouse-Sakati Syndrome
		28.16	 Idiopathic NBIA
		28.17	 Diagnosis
		28.18	 Differential Diagnosis
		28.19	 Treatment
		References
	29: Frontotemporal Dementia
		29.1	 Introduction
		29.2	 Epidemiology of Frontotemporal Dementia
		29.3	 Clinical Features of FTD
		29.4	 Behavioural-Variant Frontotemporal Dementia (bvFTD)
		29.5	 Diagnostic Criteria
		29.6	 Molecular Pathology
		29.7	 Genetics in FTD
		29.8	 Clinical, Pathological, and Genetic Correlations in FTD
		29.9	 Imaging in bvFTD
		29.10	 Patient Management and Future Challenges
		References
	30: Parkinson’s Disease
		30.1	 Introduction
		30.2	 Classification
		30.3	 Clinical Features
			30.3.1	 Motor
			30.3.2	 Non-Motor
		30.4	 Causes
			30.4.1	 Environmental Factors and Genetics
		30.5	 Epidemiology
		30.6	 Pathophysiology
		30.7	 Diagnosis
		30.8	 Imaging
		30.9	 Management
		30.10	 Gene Therapy
		30.11	 Prognosis
		References
Part VII: Others
	31: Trigeminal Neuralgia
		31.1	 Introduction
		31.2	 Clinical Picture
		31.3	 Epidemiology
		31.4	 Pathophysiology
		31.5	 Diagnostic Criteria and Sub-classification as per International Association for the Study of Pain (IASP)
		31.6	 Differential Diagnosis of TN
		31.7	 Management
		References
	32: Hypoxic Ischemic Encephalopathy
		32.1	 Introduction
		32.2	 Epidemiology
		32.3	 Pathophysiology
		32.4	 Clinical Manifestations
		32.5	 Management and Prognostication
		References
	33: Coma and Vegetative State
		33.1	 Introduction
		33.2	 Anatomy and Pathophysiological Basis of Coma
		33.3	 Etiology of Coma
		33.4	 Immediate Assessment of the Comatose Patients
		33.5	 Fundoscopy in a Comatose Patient
		33.6	 Neurological Assessment in Coma
		33.7	 Pupils in Comatose Patient
		33.8	 Ocular Movements in a Comatose Patient
		33.9	 Coma and Respiration
		33.10	 Assessment of the Motor Function
		33.11	 Rapid Management of the Comatose Patients
		33.12	 Prognosis of Coma
		References
	34: Metabolic Syndromes in Neurology
		34.1	 Introduction
		34.2	 Small-Molecule Disorders
		34.3	 Large-Molecule Disorders
		References
	35: Traumatic Brain Injury
		35.1	 Introduction
		35.2	 Epidemiology
		35.3	 Pathophysiology
		35.4	 Classification
			35.4.1	 Mild TBI/Concussion
			35.4.2	 Moderate and Severe TBI
		35.5	 Types of Injury
		35.6	 Management of TBI
			35.6.1	 ICP Monitoring and Management
			35.6.2	 Cerebral Perfusion Pressure
			35.6.3	 Post-traumatic Seizures
			35.6.4	 Nutrition
		35.7	 Rehabilitation and Outcome
		References
	36: Spinal Cord Injury
		36.1	 Introduction
			36.1.1	 Classification
			36.1.2	 Initial Stabilization
			36.1.3	 Evaluation
			36.1.4	 Instability
			36.1.5	 Neurological Classification
			36.1.6	 Imaging
			36.1.7	 Airway Management
			36.1.8	 Cardiovascular Management
		36.2	 Decompressive Surgery
		36.3	 The Surgical Timing
			36.3.1	 Intravenous Methylprednisolone
			36.3.2	 Neuroprotection
			36.3.3	 Neurodegeneration
		References
	37: Alzheimer’s Disease
		37.1	 Introduction
		37.2	 Epidemiology
		37.3	 Clinical Features
			37.3.1	 Diagnostic Criteria
			37.3.2	 Role of Biomarkers in the Diagnosis of AD
		37.4	 Genetics
			37.4.1	 Pathology
			37.4.2	 Differential Diagnosis
			37.4.3	 Treatment
		References
	38: Intracranial Hypertension
		38.1	 Pathophysiology of Intracranial Hypertension
		38.2	 Clinical Features
		38.3	 Monitoring of ICP
		38.4	 Management of Raised ICP
		References