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Textbook of Movement Disorders
Ashok Kumar
CHAPTER 1:
History of James Parkinson and Kinnier Wilson
JAMES PARKINSON (1755–1824)
SAMUEL ALEXANDER KINNIER WILSON (1878–1937)
CHAPTER 2:
Epidemiology of Parkinson's Disease
Descriptive epidemiology1
Incidence
USA
Europe
Asia10
Gender
Prevalence
Asia10
India22
Biological Differences
Mortality
Burden of Disease37
ANALYTICAL EPIDEMIOLOGY
Infective Risk Factor
Neurotoxic and Occupational Risk Factors
Lifestyle Risk Factors73
Physical Activity
Dietary Factors
Other Risk Factors
Anemia
Anti-inflammatory Drugs
Obesity
Serum Cholesterol126
Genetic Risk Factors
Proteins of Unknown Function
LRRK2
DJ-1
Stress-response Proteins
α-synuclein
PINK1
HtrA2
Proteins in the Ubiquitin-proteasome System (UPS)
Parkin
UCHL1
CHAPTER 3:
Basal Ganglia Circuit Related to Movement Disorders
INTRODUCTION
BASAL GANGLIA STRUCTURES
Striatum
Medium spiny neurons (MSNs)
Interneurons
Globus Pallidus
Subthalamic Nucleus
Substantia Nigra (SN)
BASAL GANGLIA CIRCUIT – THE MOTOR CIRCUIT
DISEASES OF THE BASAL GANGLIA
CHAPTER 4:
Pathophysiological Neuronal Oscillations: Invasive Recordings from the Basal Ganglia
INTRODUCTION
CURRENT TECHNOLOGIES FOR INVASIVE RECORDINGS
ABERRANT LFP OSCILLATIONS RECORDED FROM BASAL GANGLIA
ARE THERE MEDICATION / LESION / DBS EFFECTS?
SYNCHRONIZED NEURONAL ACTIVITY IN OTHER MOVEMENTS DISORDERS
OSCILLATIONS DURING MOTOR TASKS
OSCILLATIONS DURING COGNITIVE FUNCTIONS
CONCLUSION
CHAPTER 5:
Animal Models of Parkinson's Disease
ABSTRACT
INTRODUCTION
TOXIN MODELS
6-OHDA
MPTP
RESERPINE
ROTENONE
PARAQUAT AND MANEB
LPS
METHAMPHETAMINE
GENETIC MODELS
α-Synuclein (AS)
Parkin
LRRK2
UBIQUITIN C-TERMINAL HYDROLASE L1 (UCH-L1)
E3 Ligase Ubiquitin SKP1A
PINK1
DJ-1
COMBINATION MODELS (FUSION MODELS)
CONCLUSION
Conflict of Interest Statement
Acknowledgements
CHAPTER 6:
Etiology and Pathogenesis of Parkinson's Disease
GENETIC FACTORS
α-Synuclein (PARK 1)
Parkin (PARK 2)
UCH 1 (PARK 5)
PINK (PARK 6)
DJ1 (PARK 7)
LRRK 2 (PARK 8)
ENVIRONMENTAL FACTORS
PATHOGENESIS OF PARKINSON DISEASE
SUMMARY
CHAPTER 7:
New Insight into Parkinson's Disease Genetics
INTRODUCTION
α-Synuclein Gene AD
PARK 2 AR
PARK3 AD
PARK4 AD
PARK 5 AD
PARK6 AR
PARK7 AR
PARK8 AD
PARK9 AR
PARK10
PARK11 AD
PARK12 Xq
PARK13 AD
PARK14 AR
PARK15 AR
PARK16 AR
GBA AR
Tau Gene
Tyrosine Hydroxylase
LINGO1 and LINGO2
DISCUSSION
Genes’ Products Relations and Mechanisms Implicated in Mendelian's forms of PD
CHAPTER 8:
Nonmotor Symptoms of Parkinson's Disease
INTRODUCTION
DISCUSSION
The Major Nonmotor Symptoms in Parkinson Disease
Pain
Autonomic symptoms
Sleep dysfunction
Fatigue
Psychological symptoms
Cognitive Problems in Parkinson's Disease
CONCLUSION
CHAPTER 9:
Cognitive Dysfunction in Parkinson's Disease
INTRODUCTION
The Pathophysiological Basis of Cognitive Dysfunction in PD
NEUROPSYCHOLOGICAL CHANGES IN PARKINSON'S DISEASE
The Practical Cognitive Evaluation in Patients with PD
Treatment of Cognitive Dysfunction in PD
RECOMMENDATIONS
CHAPTER 10:
Psychosis in Parkinson's Disease
ABSTRACT
INTRODUCTION
What is Psychosis in PD?
PREVALENCE
Risk Factors
Concept of Different Phenotypes of PD
Phenomenology and Pattern of Psychosis in PD
Temporal Characteristics of Hallucinations
Insight
Delusions
Diagnostic Criteria5
A. Characteristic symptoms
B. Primary diagnosis
C. Chronology of the onset of symptoms of psychosis
D. Duration
E. Exclusion of other causes
F. Associated features: (specify if associated)
Assessment and Rating Scales of Psychosis
Pathophysiology of Psychosis in PD
Management of Psychosis in PD (Table 10.3)
INVESTIGATIONS
Drug Treatment
Clozapine
Quetiapine
Risperidone
Olanzapine
Aripiprazole
Ziprasidone
Acetylcholinesterase Inhibitors [ACI]
Ondansetron
CONCLUSION
CHAPTER 11:
Impact of the Parkinson's Disease on the Heart
INTRODUCTION
CARDIAC DENERVATION
EFFECT OF THE PD THERAPY
CHAPTER 12:
The Application of Motor Imagery in Patients with Parkinson's Disease: A State of the Art
INTRODUCTION
MOTOR IMAGERY: A NEW TECHNIQUE WITHIN NEUROLOGICAL REHABILITATION
EVALUATION OF MOTOR IMAGERY ABILITY
Evaluation of MI Ability by Means of Eye-movement Registration
A clinical assessment battery for patients with PD
FACILITATION OF MOTOR IMAGERY QUALITY
The Use of Cueing to Facilitate MI in Healthy Participants
Facilitation of MI Quality in Patients with PD
CONCLUSION
CHAPTER 13:
The Role of Tremor Analysis in Parkinson's Disease
INTRODUCTION AND AIMS
PHYSIOLOGICAL AND PATHOLOGICAL TREMORS
Classification of Tremors
Tremor Genesis
Mechanical oscillations of the extremity
Reflex activation of tremor
Central oscillator
Altered characteristics of feedforward and feedback loops
TREMOR ANALYSIS
Clinical Assessment
Rating Scales
Modified Fahn-Tolosa-Marin tremor rating scale (mFTMTRS)
Unified Parkinson's disease rating scale
Electrophysiological Tools
Movement detection techniques
Muscle activation detection techniques
Video Recording
The Technique of Signal Digitization and Processing
Digitization
Postprocessing
QUANTIFICATION OF TREMOR CHARAC-TERISTICS
Analysis of Accelerometric Recordings
Frequency
Intensity (total power, TP)
Frequency domain identification
Asymmetry-index (AI)
Irregularity
Number of peaks on power-spectra
Analysis of Surface Electromyography
Presence of tremor related burst activity
Pattern of activation of antagonistic muscles
Frequency
Intensity
Burst duration
TREMOR ANALYSIS FOR DIFFERENTIAL DIAGNOSIS
Suggested Clinical Protocol for Tremor Analysis
Electrophysiological Features of Essential Tremor
Electrophysiological Features of Psychogenic Tremor
Electrophysiological Features of Parkinsonian Tremors
TREMOR ANALYSIS FOR MEASURING EFFICACY OF PHARMACOLOGICAL AND SURGICAL THERAPIES
Effects of Deep Brain Stimulation on Tremor Characteristics
Impact of Ablative Procedures on Tremor Characteristics
Assessment of Efficacy of Various Pharmaco-logical Treatments on Tremor
TREMOR ANALYSIS IN CLINICAL RESEARCH
Better Understating of Tremor Genesis
Phenomenological studies
Intracranial recordings
Neuroimaging techniques and tremor analysis
Finding More Efficient Targets for Neuromo-dulation
Comparison the efficacy of zona incerta (ZI) stimulation with that of Vim stimulation
Bilateral effects of unilateral DBS
ACKNOWLEDGMENTS
CHAPTER 14:
Young Onset Parkinson's Disease
INTRODUCTION
Genetics and Clinical Features
Treatment
CHAPTER 15:
Management of Early Parkinson's Disease
INTRODUCTION
DIAGNOSIS
DRUGS
Anticholinergics
Amantadine
Levodopa
Dopamine Agonists
Ergot Alkaloids
Nonergot Alkaloids
Monoamine Oxidase B Inhibitors
Neuroprotection
SELECTION OF DRUG
Age
First-line Levodopa Treatment
First-line Dopamine Agonist Treatment
First-line MAO-B Inhibitors
TIMING OF TREATMENT
NONMOTOR SYMPTOMS
COMPLEMENTARY AND SUPPORTIVE THERAPIES
CONCLUSION
CHAPTER 16:
Levodopa in Movement Disorders
INTRODUCTION
HISTORY OF LEVODOPA (L-DOPA)
PHARMACOLOGICAL EFFECTS
PHARMACOKINETICS
THERAPEUTIC USES OF L-DOPA
1. Parkinson's Disease
Combination therapies
2. Restless Legs Syndrome (RLS)
3. L-dopa Responsive Dystonias (DRD)
IMPORTANT ISSUES WITH THE USE OF L-DOPA (Table 16.2)
1. GIT Effects
2. Cardiovascular Effects
3. Dyskinesias
4. Fluctuations
5. Behavioral Effects
6. Miscellaneous Adverse Effects
IMPORTANT DRUG INTERACTIONS2 (Table 16.5)
CONTRAINDICATIONS4 (Table 16.6)
CONCLUSION
CHAPTER 17:
Apomorphine, Duodopa or DBS– Last Line Treatment Options for Advanced Parkinson's Disease
INTRODUCTION
DUODENAL LEVODOPA INFUSION
CONTINUOUS SUBCUTANEOUS APOMORPHINE INFUSION
DEEP BRAIN STIMULATION
Apomorphine Infusion, Duodenal Infusion of Levodopa, or Deep Brain Stimulation–Which Treatment to Chose?
CHAPTER 18:
Vascular Parkinsonism
INTRODUCTION
HISTORICAL ASPECTS
EPIDEMIOLOGY
PATHOPHYSIOLOGY
PATHOLOGY
CLINICAL FEATURES
Onset and Course
Postural Instability and Gait Difficulty
Upper Limbs
Cranial Nerves
Upper Motor Neuron Signs
Muscle Tone in VP
Nonmotor Symptoms
IMAGING
DIAGNOSTIC CRITERIA
DIFFERENTIAL DIAGNOSIS
TREATMENT
CASE VIGNETTE
CHAPTER 19:
Progressive Supranuclear Palsy (PSP)
BACKGROUND
HISTORICAL PERSPECTIVE
GENETICS
EPIDEMIOLOGY
CLINICAL FEATURES
PSP—CLINICAL SUBTYPES
Richardson Syndrome
Progressive Supranuclear Palsy–Parkinsonism (PSP-P)
Progressive Supranuclear Palsy – Pure Akinesia with Gait Freezing (PSP – PAGF)
Progressive Supranuclear Palsy – Corticobasal Degeneration (PSP-CBD)
Progressive Supranuclear Palsy – Primary Non-Fluent Aphasia (PSP-PNFA)
DIAGNOSTIC CRITERIA
PATHOLOGY
Macroscopic Pathology57
Microscopic Pathology57
NEUROIMAGING
DIAGNOSTIC WORKUP
PROGNOSIS AND COMPLICATIONS
TREATMENT
CONCLUSION
CHAPTER 20:
Multiple System Atrophy
HISTORY
EPIDEMIOLOGY
CLINICAL FEATURES
DIAGNOSTIC CRITERIA
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
INVESTIGATIONS
PATHOGENESIS
TREATMENT
CLINICAL COURSE AND PROGNOSIS
CHAPTER 21:
Corticobasal Degeneration
BACKGROUND
NEUROPATHOLOGY
CLINICAL FEATURES
NEUROPSYCHOLOGY
NEUROIMAGING
PATHOGENESIS
BIOMARKERS
TREATMENT
Motor Symptoms
Cognitive and Neuropsychiatric Symptoms
FUTURE APPROACHES
SUMMARY
CHAPTER 22:
Neuropsychology of Parkinson-Plus Syndromes
PART ONE
Memory
Short-term Memory or Working Memory
Long-term Memory
Attention
Executive Functions
Language and Speech
Limb Apraxia
Constructional Apraxia
Alien Hand Syndrome
Visuospatial Deficits
PART TWO
Dementia with Lewy Bodies
Clinical Diagnostic Criteria
CENTRAL FEATURE: ESSENTIAL FOR POSSIBLE/PROBABLE DLB
PROGRESSIVE DISABLING MENTAL IMPAIRMENT
CORE FEATURES
SUGGESTIVE FEATURES
SUPPORTIVE FEATURES: Not proven to have a diagnostic specificity
EXCLUSION FEATURES
Corticobasal Syndrome
Progressive Supranuclear Palsy
Huntington's Disease
Multiple System Atrophy
CHAPTER 23:
Autonomic Functions in Parkinson's Disease and Other Movement Disorders
INTRODUCTION
PATHOPHYSIOLOGICAL INVOLVEMENT OF AUTONOMIC CENTERS IN PD
AUTONOMIC SYMPTOMS IN PD
GASTROINTESTINAL SYMPTOMS
Constipation
Dysphagia
Drooling/Hypersalivation
Nausea
Risk of Aspiration
CARDIOVASCULAR SYMPTOMS
Orthostatic Hypotension
UROGENITAL SYMPTOMS
Urinary Problems
Sexual Dysfunction
THERMOREGULATORY SYMPTOMS
Cold Limbs, Hyperhidrosis
Other Related Symptoms
Evaluation of Cardiac Autonomic Functions
Conventional Methods
Ewing's Tests
HRV Analysis
Time Domain Measures (Table 23.1)
Frequency Domain Measures of HRV (Table 23.2)
Blood Pressure Variability (BPV)
Baroreflex Sensitivity (BRS)
STUDIES INVESTIGATING AUTONOMIC INVOLVEMENT IN PD
CARDIAC AUTONOMIC INVOLVEMENT AS A MARKER OF PRE-PD
AUTONOMIC FUNCTIONS IN OTHER MOVEMENT DISORDERS
FUTURE DIRECTIONS
ACKNOWLEDGMENT
CHAPTER 24:
Tremors: Clinical Diagnosis and Treatment
INTRODUCTION
HISTORICAL ASPECTS
CLINICAL DEFINITION
CLASSIFICATION OF TREMOR
ETIOLOGY OF TREMORS
TREMOR ASSESSMENT SCALES
CLINICAL CHARACTERISTICS AND MANAGEMENT OF VARIOUS TYPES OF TREMORS
1. Physiological and Enhanced Physiological Tremor
Definition and characteristics
Epidemiology and causes
Management
2. Essential Tremors
Overview
Epidemiology
Genetics
Diagnosis
Clinical features
Pathology
Imaging
Management
3. Parkinsonian Tremors
Definition and characteristics
Epidemiology
Management
4. Orthostatic Tremors
Definition and characteristics
Management
5. Dystonic Tremors Syndromes
Definition and characteristics
Management
6. Primary Writing Tremor
Definition and characteristics
Management
7. Cerebellar Tremor Syndromes
Definition and characteristics
Management
8. Holmes Tremor
Definition and characteristics
Management
Tremor Syndromes in Peripheral Neuro-pathies
10. Palatal Tremor Syndromes
11. Psychogenic Tremors
12. Other Rare/Undetermined Syndromes
CONCLUSION
CHAPTER 25:
Essential Tremor
INTRODUCTION
DEFINITION OF A TREMOR
Criteria for Essential Tremor3
Core criteria
Secondary criteria
PATHOPHYSIOLOGY
Pathology
Etiology
Epidemiology
Clinical Features
History
Physical exam
Differential diagnosis
Investigations
Laboratory tests
Imaging
Surface EMG
Treatment
Medical management
Strength of Recommendation
Surgical management
Follow-up
CHAPTER 26:
MR Imaging in Essential Tremor
INTRODUCTION
Relationship Between ET and Parkinson's Disease (PD)
Role of Striatum/Thalamus in Tremor Genesis
CHAPTER 27:
Wilson's Disease
INTRODUCTION
COPPER METABOLISM
MOLECULAR BIOLOGY
CLINICAL FEATURES
Hepatic
Neurologic
Psychiatric
Ocular
Involvement of Other Systems
PATHOLOGIC FEATURES
Liver Pathology
Brain Pathology
STAGING
Diagnosis
Liver function tests
Ceruloplasmin
Copper
Urinary copper
Other biochemical tests
Hepatic copper concentration
Other copper tests Radiocopper
Cerebrospinal fluid (CSF) copper
Genetic analysis
Brain imaging
MRI brain
Approach to diagnosis
Family screening
Treatment
Diet
Drug therapy
Penicillamine
Trientine
Zinc
Ammonium tetrathiomolybdate
Antioxidants
Liver transplantation
Treatment in Special Situation Presymptomatic Patient
Maintenance therapy
Decompensated chronic liver disease
Pregnancy
Surgery
Prognosis
CHAPTER 28:
Dystonia
INTRODUCTION
CLINICAL FEATURES OF DYSTONIA IN GENERAL
CLASSIFICATION OF DYSTONIA
1. Classification by Age of Onset
2. Classification by Distribution
3. Etiological Classification
ETIOLOGY/CAUSES OF DYSTONIA
I. PRIMARY TORSION DYSTONIA (PTD)
1. Early Onset Autosomal Dominant PTD (DYT1)
2. Autosomal Recessive PTD (DYT2)
3. Non DYT1 PTD(DYT4)
4. ADPTD of Mixed Type
5. Adult Onset Focal PTD (DYT 7)
6. Early Onset Cranial – Cervical Segmental PTD (DYT 13)
7. Other PTD Genes and Susceptibility Loci
8. Paroxysmal Kinesigenic Dyskinesias (DYT10)
9. Paroxysmal Nonkinesigenic Dyskinesias (DYT 8)
II. DSYTONIA PLUS SYNDROMES
1. Dopa Responsive Dystonia
2. Myoclonus – Dystonia (DYT11)
3. Rapid Onset Dystonia- Parkinsonism (DYT12)
III. HEREDODEGENERATIVE DYSTONIAS
X- linked Dystonia-Parkinsonism (DYT3)
CLINICAL FEATURES OF SPECIFIC DYSTO-NIAS
1. PTD
i) Blepharospasm
ii) Oromandibular dystonia
iii) Spasmodic dysphonia
iv) Cervical dystonia: (Fig. 28.2)
v) Limb dystonia
Pathophysiology of Dystonia
DIAGNOSTIC APPROACH TO DYSTONIA
1. Primary Dystonia
2. Secondary Dystonia
Neuroimaging
CSF studies
Genetic testing
Electrophysiology
Tissue biopsy
TREATMENT OF DYSTONIA
I. MEDICATIONS
Treatment of status dystonicus
II. BOTULINUM TOXIN
III. SURGICAL TREATMENT
IV. PHYSICAL THERAPY
CHAPTER 29:
Dopa Responsive Dystonia
INTRODUCTION AND BRIEF HISTORY
CLINICAL FEATURES
AD- GTP cyclohydrolase I DRD (Classical DRD or DYT5)
AR- Tyrosine Hydroxylase DRD (AR-TH DRD)
AR- Sepiapterin Reductase DRD (AR- SPR DRD)
Genetic basis of DRD
Genetic- Biochemical- Clinical Correlations
Pathology and Histochemistry
Investigations
Biochemical investigations
Radiological investigations
Differential Diagnosis
Treatment
Classical DRD
AR-DRD
CONCLUSION
CHAPTER 30:
Writer's Cramp
INTRODUCTION
CLINICAL MANIFESTATIONS
Epidemiology
Risk Factors
Pathophysiology
Abnormal inhibition
Abnormal plasticity
Abnormal sensorimotor integration
Treatment
Pharmacological agents
Botulinum toxin injections
Behavioral and rehabilitative therapies
Surgical treatments
Acknowledgment
CHAPTER 31:
Meige's Syndrome
INTRODUCTION
HISTORICAL ASPECTS
EPIDEMIOLOGY AND RISK FACTORS
CLASSIFICATION
Primary Meige's Syndrome
Secondary Meige's syndrome
PATHOPHYSIOLOGY
CLINICAL FEATURES
Blepharospasm
Lower Facial and Oromandibular Spasms
Extracranial Spasms and Other Symptoms
CLINICAL COURSE
MANAGEMENT
CONCLUSION
CHAPTER 32:
Catatonia
INTRODUCTION
HISTORICAL BACKGROUND
NOSOLOGY
EPIDEMIOLOGY
ETIOLOGY
GENETIC FACTORS
BIOCHEMICAL FACTORS
Dopaminergic System
GABAergic System
Other Neurotransmitters
Neuropathology
Structural Imaging
Functional Imaging
Applied Electrophysiology
Neuropsychological Tests
PSYCHOSOCIAL AND PSYCHOANALYTICAL THEORIES
Emil Kraepelin (1856–1926)
Karl Jaspers (1883–1969)
Kleist, Wernicke and Leonhard
MODERN THEORISTS
CLINICAL FEATURES
Feature Description
DIAGNOSIS
Recognizing Catatonia
The Diagnostic Criteria
FORMS OF CATATONIA
Retarded Catatonia (Kahlbaum Syndrome)
Excited Catatonia (Manic Excitement)
Oneirophrenia
Malignant Catatonia
Other Conditions
DIFFERENTIAL DIAGNOSIS
MANAGEMENT
Assessment
Treatment
COURSE AND PROGNOSIS
PREVENTION
CHAPTER 33:
Hemifacial Spasm: Clinical Features and Management
INTRODUCTION
FUNCTIONAL ANATOMY OF THE FACIAL NERVE
CLINICAL AND EPIDEMIOLOGICAL FEATURES OF HEMIFACIAL SPASM (HFS)
DIFFERENTIAL DIAGNOSIS
ETIOLOGY OF HFS
PATHOPHYSIOLOGY OF HFS
QUALITY OF LIFE (Qol) MEASURES IN HFS
DIAGNOSIS OF HFS
PHARMACOLOGICAL TREATMENT OF HFS
NEUROSURGICAL TREATMENT OF HFS
EXTRACRANIAL PROCEDURES
INTRACRANIAL PROCEDURE
Neurostimulation procedures
Alcohol nerve injection at stylomastoidal foramen
Percutaneous selective thermal lesion of the facial nerve
Total or partial facial nerve section
Microvascular decompression of the facial nerve
CONCLUSION
CHAPTER 34:
Chorea: An Update
INTRODUCTION
CLINICAL FEATURES
PATHOPHYSIOLOGY
Classification
Relevant Diseases Presenting with Chorea
Diagnosis
Medical History
Physical Examination
Ancillary Tests
TREATMENT
Pharmacologic Treatment
Surgical Treatment
Other Therapies
CHAPTER 35:
Huntington's Disease
INTRODUCTION
CLINICAL FEATURES
Motor Signs and Symptoms
Behavioral and Psychiatric Symptoms
Dementia and Cognitive Symptoms
Juvenile HD
EPIDEMIOLOGY
DIFFERENTIAL DIAGNOSIS
Genetics
Genetic Testing
Imaging
TREATMENT
Tetrabenazine
Neuroleptics
Future Directions
CHAPTER 36:
Rheumatic Chorea
INTRODUCTION
EPIDEMIOLOGY
PATHOGENESIS
CLINICAL FEATURES
Chorea
Other Motor Manifestations
Behavioral Abnormalities
Other Neurological Manifestations
Nonneurological Manifestations
DIAGNOSIS
PROGNOSIS
TREATMENT
Prophylaxis of Streptococcus Infection
Treatment of Chorea
CHAPTER 37:
Athetosis
INTRODUCTION/DEFINITION
CLINICAL FEATURES
Diagnostic Criteria
Etiology and Pathogenesis
DRUGS
Neurodegenerative Disease
Huntington's Chorea
Wilson's Disease
Rare Causes
The Lesch–Nyhan syndrome and hyperuricemia
Calcification of vessels in the basal ganglia and cerebellum (hypothryoidism and Fahr's syndrome)
Metabolic disorders and associated choreoathe-tosis
Niemann-Pick disease (type C)
Glutaric acidemia (type 1)
Pseudoathetosis
Diagnostic Workup
MANAGEMENT
CHAPTER 38:
Hemiballismus
INTRODUCTION
DEFINITION
CLINICAL DESCRIPTION
Hemiballismus - Hemichorea - Athetosis
ANATOMICAL LOCALIZATION
Subthalamic Nucleus of Luys (STN)
Firing patterns
Etiology
Pathogenesis
Possible pathogenic mechanisms include
Prognosis
Diagnostic Approach
Treatment
Other Pharmacological Agents
CHAPTER 39:
PANDAS
INTRODUCTION
IMMUNE SYSTEM AND PSYCHIATRIC DIS-ORDERS
WORKING CRITERIA FOR DIAGNOSIS
1. OCD / Tics
Patient must meet lifetime strict criteria DSM III R DSM IV
2. Pediatric Onset
3. Episodic Course of Symptom Severity
4. Association with GABHS
5. Associated Neurological Abnormalities
PSYCHOLOGICAL COMORBIDITY
CELL MARKER
RELATIONSHIP BETWEEN SYDENHAM'S CHOREA AND PANDAS
MANAGEMENT
PROPHYLAXIS—ROLE IS CONTROVERSIAL
PANDAS—MYTH OR REALITY?
CHAPTER 40:
Tics
INTRODUCTION
Tourette Syndrome (TS)
Clinical Features
Diagnosis
Treatment
Pediatric Autoimmune Neuropsychiatric Disorder (PANDAS)
CHAPTER 41:
Myoclonus
INTRODUCTION
Neurologist's Journey with Myoclonus
Cortical Myoclonus / Epileptic Myoclonus
Epileptic Myoclonus can be Described as
CHAPTER 42:
Startle Syndrome
INTRODUCTION
Normal Startle Reflex and Hyperekplexia
Familial (Hereditary) Hyperekplexia
Clinical description
Genetics
GLRA1
SLC6A5
GLRB
GPHN
ARHGEF9
Clinical testing
Testing Strategy
Genetically Related (Allelic) Disorders
Genotype-phenotype correlations
Penetrance
Anticipation
Mode of inheritance
Parents of a proband
Sibs of a proband
Parents of a proband
Sibs of a proband
Carrier detection
Related genetic counseling issues
Family planning
Prenatal testing
History
Nomenclature
Prevalence
Differential diagnosis
Anxiety disorders
Startle epilepsy
Tic disorders
Paraneoplastic syndromes
Evaluations following initial diagnosis
Testing
Treatment of manifestations
Sporadic hyperekplexia
Course of the disease
Physical examination
Causes
Pathophysiology
Epidemiology
Race
Sex
Age
History
Associated diseases
Differential diagnosis
Laboratory studies
Special tests
Imaging studies
Other tests
Procedures
Medical care
Benzodiazepines
Skeletal muscle relaxants
Novel medications
Complications of SPS
Prognosis
Stiff baby syndrome
Stiff limb syndrome
CHAPTER 43:
Stereotypies
INTRODUCTION
HISTORICAL ASPECTS
DEFINITION
DIFFERENTIAL DIAGNOSIS
Mannerisms
Compulsions
Tics
Restless Legs Syndrome
Akathisia
Perseveration
Paroxysmal Dyskinesias
Tardive Dyskinesias
Seizure Activity
Secondary Causes of Head Nodding
CLASSIFICATION
PRIMARY STEREOTYPIES
SECONDARY STEREOTYPIES
ETIOPATHOLOGY
COMPLICATIONS
STRUCTURED ASSESSMENT OF STEREOTYPIES
INVESTIGATIONS
TREATMENT
Pharmacological Management
Behavior Therapy
CONCLUSION
CHAPTER 44:
Gait Disorders
INTRODUCTION
Normal Gait
THE GAIT CYCLE, PHYSIOLOGY AND EXAMINATION FOR CLUES TO DIAGNOSIS OF GAIT DISORDER
GAIT DISORDERS
PATHOPHYSIOLOGY OF GAIT DISORDERS
CLASSIFICATION OF GAIT DISORDERS
CAUSES OF GAIT DISORDERS
SPECIFIC GAIT DISORDERS
Hypokinetic Gait
Dyskinetic gait
ASSESSMENT AND SCORING OF GAIT
Self-assessed Measures
Performance-based Measures
Lab Assisted Measures
Domiciliary Gait Monitoring
TREATMENT OF GAIT DISORDERS
CHAPTER 45:
Hereditary Ataxias: An Overview and Indian Perspectives
INTRODUCTION
CLASSIFICATION OF HEREDITARY ATAXIAS
SPINOCEREBELLAR ATAXIA: OVERVIEW
Distinct and Overlapping Features
Genetic Etiologies of SCAs
Prevalence
Neuropathology: Histopathology, Postmortem Brain Studies
Molecular Pathophysiology: Nuclear (Transcription and DNA Repair), Aggregation (Nuclear and Cytoplasmic), Channelopathies (Direct and Indirect) and Mitochondrial (Direct and Indirect)
Protein Aggregation—A Failure of Cellular Clearance Machinery
Protein Context Drives Cellular Pathology in PolyQ Cell Models
Transcription Deregulation
Channelopathy
Neurological Spectrum of SCAs (Table 45.2 and Table 45.4) Clinicogenetic Features of PolyQ Associated SCAs (Coding-TRE)
SCA1
SCA2
SCA3
SCA6
SCA7
DRPLA
SCA17
Noncoding TRE SCAs
SCA8
SCA10
SCA12
SCA31
SCA36
NonTRE Mediated SCA
SCA5
SCA11
SCA13
SCA14
SCA15 and SCA16
SCA27
SCA28
Pathophysiology of ARCAs
Friedreich Ataxia
Introduction
Clinical features
Genotype-Phenotype Correlations in FRDA
Molecular Genetics
Indian Perspective
Management and Clinical Trial Studies
Ataxia Telangiectasia (AT) and AT-Like Diseases
Indian Perspective of Ataxia Telangiectasia
Ataxia with Vitamin E Deficiency (AVED)
Indian Scenario of AVED
Cerebrotendinous Xanthomatosis (CTX)
CTX and India
Abetalipoproteinemia
Late Onset Tay-Sachs Disease (LOTS)
Other Rare Forms of Recessive Cerebellar Ataxia
CONCLUSION
CHAPTER 46:
Paroxysmal Dyskinesias
INTRODUCTION
HISTORICAL ASPECTS
Clinical Description of the Main Types of Paroxysmal Dyskinesias Paroxysmal Kinesigenic Dyskinesia (PKD; DYT10)
Clinical Characteristics
Differential Diagnosis
Treatment
Paroxysmal Nonkinesigenic Dyskinesia (PNKD; DYT8)
Differential Diagnosis
Treatment
Exercise-induced Paroxysmal Dyskinesias (PED; DYT18)
Differential Diagnosis
Treatment
Secondary Causes of Paroxysmal Dyskinesias
Paroxysmal Dyskinesias Plus Syndromes
Infantile Convulsions and Choreoathetosis
Paroxysmal Choreoathetosis/Spasticity
Rolandic Epilepsy, Paroxysmal Exercise-induced Dystonia and Writer's Cramp Syndrome
Generalized Epilepsy and Paroxysmal Dyskinesia
Genetics of Idiopathic Dyskinesias
PKC: Genetic Heterogeneity
Paroxysmal Nonkynesigenic Dyskinesia
Genetic Heterogeneity in PNKD
PNKD Plus
Paroxysmal Choreoathetosis/Spasticity (CSE)
Generalized Epilepsy and Paroxysmal Dyskinesia
Paroxysmal Exercise-induced Dyskinesia
PED: Genetic Heterogeneity
CONCLUSION
CHAPTER 47:
Psychogenic Movement Disorders
INTRODUCTION
Diagnosis
Psychogenic Tremor
Psychogenic Gait Disorder
Psychogenic Dystonia
Psychogenic Parkinsonism
Psychogenic Myoclonus
History Taking
Neurophysiology
Psychogenic Movement Disorder in Children
Treatment
Behavioral Therapy
Relaxation Exercises
Drugs
Repeated Transcranial Magnetic Stimulation (TMS)
Prognosis
CONCLUSION
CHAPTER 48:
Movement Disorder and Epilepsy
ABSTRACT
INTRODUCTION
CLINICAL IMITATORS
Hyperekplexia
Paroxysmal Dyskinesia (PD)
Myoclonus
Nocturnal movements and seizures
Iatrogenic Conditions Causing Both
Genetic association
CONCLUSION
CHAPTER 49:
Structural MR Imaging in Movement Disorders
PARKINSONISM
Idiopathic Parkinson's Disease
Atypical Parkinsonism
Multiple System Atrophy
Progressive Supranuclear Palsy
Corticobasal Degeneration
Vascular Parkinsonism
Other Vascular Movement Disorders
Normal Pressure Hydrocephalus
Heredodegenerative Disorders
Huntington disease
Wilson disease
Neurodegeneration with Brain Iron Accumulation
Cerebellar Ataxias
Fahr's Disease
CHAPTER 50:
Emergencies in Movement Disorders
INTRODUCTION
Hypokinetic Disorders
Etiologies for Acute Parkinsonism
Infectious Parkinsonism
Neuroleptic Malignant Syndrome
Parkinsonism Hyperpyrexia Syndrome (PHS)
Catatonia
Dystonia
Supportive Care
Tardive and Neuroleptic-induced Syndromes
Hemiballism
Causes of Hemiballism
Management
DISORDERS OF SPINAL RIGIDITY IN MAN
Tetanus and Strychnine
Rigidity in the Stiff-person Syndrome
Drugs that may be Useful in Treatment of Spinal Rigidity and Spasms
SEROTONIN SYNDROME
Mechanisms of Action
Management
CHAPTER 51:
Movement Disorders in Children
INTRODUCTION
DESCRIPTION OF MOVEMENT DISORDERS
CLINICAL APPROACH TO A CHILD WITH ABNORMAL MOVEMENTS
In the Clinic
Home/Video
Neuropsychology/Physical (PT) and Occupational Therapy (OT)
Kinematic and Dynamic
A. Transient Developmental Movement Disorders
Benign Neonatal Myoclonus
Benign Myoclonus of Early Infancy
Shuddering (Shivering) Attacks
B. Paroxysmal Movement Disorders
Motor Stereotypies
Posturing During Masturbation (Infantile Masturbation)
Hyperekplexia (Hyperekplexia, Startle Disease)
Restless Legs Syndrome
Tics
Tourette Syndrome (TS)
Chorea and Choreoathetosis
Pediatric Autoimmune Neuropsychiatric Disorder associated with Streptococcus Infection (PANDAS)
Acute Disseminated Encephalomyelitis (ADEM)
Juvenile Huntington Disease (HD)
Steps in The Diagnosis of Juvenile HD
Kernicterus
Cerebral Palsy
Benign Hereditary Chorea
Other Choreas
Treatment
Bradykinesia
Treatment
Dystonia
Treatment
Myoclonus
Opsoclonus-myoclonus-ataxia (OMC)
Treatment
Tremor
Ataxia
Episodic Ataxia
Psychogenic Disorders
Summary
CHAPTER 52:
Idiopathic Normal Pressure Hydrocephalus
INTRODUCTION
BACKGROUND
HYPOTHESIS
EPIDEMIOLOGY –INCIDENCE RATE
CLINICAL SYMPTOMS
1. Characteristics, Severity and Assessment of Gait Disturbances
2. Characteristics, Severity and Assessment of Cognitive Function Disorders
3. Characteristics, Severity and Assessment of Urinary Disturbances (Urinary Incontinence)
4. Incidence of the Classical Triad
5. Other Clinical Symptoms
6. Radiological Findings
A. Computed tomography (CT), magnetic resonance imaging (MRI)
B. Cerebral blood flow (CBF)
C. Cisternography
7. Diagnosis
A. Flowchart of diagnosis
B. Flowchart for probable INPH
C. Flowchart for problems after shunt procedure
8. Comparison of International Guidelines
A. Characteristics of Japanese guidelines (Fig. 52.3)
B. Characteristics of the international guidelines on NPH (Fig. 52.7)
C. Comparisons of the two guidelines
D. Future issues
E. Differential diagnosis of iNPH
9. Treatment
Fixed Differential Pressure Valve
Flow-regulated Valves
Adjustable Valves or PVs
Antisiphon Devices
Regarding L-P Shunt
PERIOPERATIVE MANAGEMENT
POSTOPERATIVE MANAGEMENT AND COMPLICATION
PROGNOSIS AND OUTCOME
Outcome
ACKNOWLEDGMENTS
CHAPTER 53:
Botulinum Toxin in Movement Disorders
INTRODUCTION
HEMIFACIAL SPASM
BLEPHAROSPASM
OROMANDIBULAR DYSTONIA
WRITER'S CRAMP
SPASMODIC DYSTONIA
ADDUCTOR LARYNGEAL BREATHING DYSPHONIA
CERVICAL DYSTONIA (CD)
SPASTICITY
SIALORRHEA
ACKNOWLEDGEMENT AND DECLARATION
CHAPTER 54:
Deep Brain Stimulation in Movement Disorders
INTRODUCTION
DEEP BRAIN SURGERY FOR PARKINSON'S DISEASE
History and Introduction to Surgical Therapy for Parkinson's Disease
Selecting a Candidate with PD for DBS
Surgical Technique and Programming of the Stimulator
Target for Stimulation
Efficacy
Safety
Conclusions in Parkinson's Disease
DEEP BRAIN SURGERY FOR DYSTONIA
History and Introduction to Surgical Therapy for Dystonia
Selecting a Candidate with Dystonia for DBS
Target for DBS
Efficacy
Safety
Conclusions in Dystonia
DEEP BRAIN SURGERY FOR TREMORS
History and Introduction to Surgical Therapy for Tremors
Selecting a Candidate with Tremors for DBS
Target for DBS
Efficacy
DBS IN OTHER MOVEMENT DISORDERS
CONCLUSION
CHAPTER 55:
Role of Physiotherapy in Parkinson's Disease
INTRODUCTION
SYMPTOMS
1. Bradykinesia and Akinesia
2. Rigidity
3. Tremor
4. Postural Instability
5. Motor Control Deficits
6. Problems with Gait
7. Swallowing and Speech Problems
8. Respiratory Problems
EXAMINATION
TREATMENT GOALS
THERAPEUTIC INTERVENTIONS
1. Relaxation
2. Stress Management
3. Range of Motion and Flexibility Exercise
4. Strength and Endurance Training
5. Instability Training
6. Gait Training
7. Motor Learning Strategies
8. Respiratory Exercises
9. Aerobic Conditioning
10. Fall Prevention
11. Oromotor Training
CONCLUSION
INDEX
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