Movement Disorder Emergencies: Diagnosis and Treatment

Preface
Series Editor Introduction
Contents
Contributors
List of Videos
Part I: Practical Approach to the Patient with a Movement Disorder Emergency
	1: Practical Approach to Management of the Movement Disorders Patient in the Hospital and Intensive Care Unit
		Patient Vignette
			Discussion
		Introduction
		Principles of the Management of the Patient in the Hospital and Intensive Care Unit
		Identification of Etiologies and/or Precipitating Factors
		Principles of Management
			Acute Management (<24 Hours)
			Management After the First 24 Hours
		Conclusion
		References
	2: Neuro-Ophthalmologic Emergencies in Movement Disorders
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Acute/Subacute-Onset Supranuclear Saccadic Gaze Palsies
			Description
			Mechanism
			Clinical Evaluation
			Etiologies
		Ocular Flutter and Opsoclonus
			Description
			Mechanism
			Clinical Evaluation
			Etiologies
		Nystagmus
			Downbeat Nystagmus
				Description
				Etiologies
				Mechanism
				Treatment
			Upbeat Nystagmus
				Description
				Etiologies
				Mechanism
				Treatment
		Oculogyric Crisis
			Description
			Etiologies
			Mechanism
			Treatment
		Conclusion
		References
	3: Movement Disorder Emergencies of the Upper Aerodigestive Tract
		Patient Vignette
		Introduction
		Acute Airway Assessment and Management
		Breathing Disturbances from Primary Disorders
			Spasmodic Dysphonia
			Adductor Laryngeal Breathing Dystonia
			Multiple System Atrophy: Abductor Weakness
		Iatrogenic Causes of Breathing Disturbances
			Spasmodic Dysphonia: Airway Obstruction Secondary to Botulinum Toxin Treatment
			Drug-Induced Tardive Dystonia
			Parkinson’s Disease: Airway Compromise Related to Deep Brain Stimulator
		Swallowing Emergencies
			Swallowing Assessment
			Treatment of Swallowing Disorders
		Specific Disorders Related to Swallowing Emergencies
			Oromandibulolingual Dystonia
			Multiple System Atrophy
			Multiple Sclerosis
			Amyotrophic Lateral Sclerosis
			Parkinson’s Disease
			Huntington’s Disease
			Palatal Myoclonus
		Conclusion
		References
Part II: Hyperacute Movement Disorder Emergencies
	4: Acute Parkinsonism
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Noninfectious Acute Parkinsonism
			Structural Lesions
			Toxic/Metabolic
			Autoimmune/Paraneoplastic
			Psychiatric
		Infectious Parkinsonism
			Classification and Clinical Features
			Neuropathology and Imaging
			Evaluation
			Treatment
				Comments on Patient 1
				Comments on Patient 2
		Conclusion
		References
	5: Parkinsonism-Hyperpyrexia Syndrome in Parkinson’s Disease
		Patient Vignette
		Introduction
		Clinical Features
		PHS and Deep Brain Stimulation
		PHS Epidemiology
		PHS Clinical Syndrome
		Illustrative Cases from the Literature
			Case 1 [17]
			Case 2 [16]
			Case 3 [24]
			Case 4 [45]
		Risk Factors and Pathogenesis
		Treatment
		Dyskinesia-Hyperpyrexia Syndrome
		Conclusion
		References
	6: Neuroleptic Malignant Syndrome
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Epidemiology
			Incidence
			Risk Factors
		Pathophysiology
		Clinical Characteristics
			Prodromal Signs
			Signs and Symptoms
			Laboratory Evaluation
			Diagnostic Criteria
			Clinical Course and Outcome
			Evaluation and Differential Diagnosis
		Treatment
		Conclusion
		References
	7: Malignant Catatonia
		Patient Vignette
		Introduction
		Clinical Presentation: Pre-antipsychotic Drug Era
		Contemporary Presentation
		The Malignant Catatonia Syndrome
		Pathogenesis
		Management
		Conclusion
		References
	8: Serotonin Syndrome
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Criteria for Diagnosis of Serotonin Syndrome
		Neurochemistry
		Neuroanatomy
			The Role of Serotonin
		Epidemiology
		Drugs Associated with Serotonin Syndrome
		Selected Patient Profiles
			The Patient with Psychiatric Disease
			The Patient with Parkinson’s Disease
			The Patient with a Severe Infection
			The Patient with Pain
			The Recreational Drug Abuser
			Genetic Polymorphisms and Drug Interactions with CYP-450
			The Patient with Human Immunodeficiency Virus
			The Patient with Cough
			The Patient with Migraine Headaches
			Methylene Blue and the Surgical Patient
			The Pediatric Patient
			The Patient Who Uses Herbal Remedies
		Differential Diagnosis
		A Common Mistake: Misdiagnosis of Serotonin Syndrome as Neuroleptic Malignant Syndrome
		Laboratory Studies
		Management of the Patient
			An Overview
		The Role of Benzodiazepines
		The Role of Serotonin Receptor Blockers
			Oral Cyproheptadine
			Intravenous Chlorpromazine
			Induction of Paralysis
			Treatment of Hyperthermia
			Treatment of Autonomic Instability
			Treatment of the Pediatric Patient
		The Prognosis
		Prevention
		Conclusion
		Authors’ Statements
		References
	9: Acute Spinal Rigidity
		Patient Vignettes
			Patient 1
			Patient 2
		The Differential Diagnosis of Rigidity
			Rigidity and Basal Ganglia Disease
			Increased Tone in Spasticity and the Upper Motor Neuron Syndrome
			Frontal Lobe Rigidity
			Muscle Stiffness and Peripheral Nerve Hyperexcitability
			Primary Muscle Disease and Muscle Stiffness
			Clinical Features of Spinal Rigidity
		Causes of Spinal Rigidity in Man
			Structural Lesions of the Spinal Cord
			Necrotizing Myelopathy
			Tetanus and Strychnine
			Spinal Segmental Rigidity and Myoclonus
			Spinal Interneuronitis and the Stiff Leg Syndrome
			Rigidity in the Stiff Person Syndrome
			Progressive Encephalomyelitis with Rigidity and Myoclonus
			Other Immune Associations of Rigidity
		Management
		Conclusion
		References
	10: Status Dystonicus
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Terminology and Definition
		Prevalence and Phenomenology
		Differential Diagnosis
		Triggers
		Etiology
		SD in Inherited Neurological Syndromes
		SD in Acquired Neurological Syndromes
		Mechanisms
		Management and Outcome
		Course and Outcome
		Conclusion
		References
	11: Posthypoxic Myoclonus and Its Management
		Clinical Vignette
		Clinical Features and Diagnostic Assessments
		Pathogenic Mechanisms
		Therapeutic Approaches
		Prognosis
		Conclusion
		References
Part III: Acute Movement Disorder Emergencies
	12: Tic Emergencies
		Patient Vignettes
			Patient 1
			Patient 2
			Patient 3
			Patient 4
			Patient 5
		Introduction
		Tic Exacerbations
		Neurological Complications from Tics
		Pain Related to Tics
		Abrupt Onset of Tics Secondary to Central Nervous System Disorders
		New Involuntary Movements from Tic Drugs
		Mood Disturbance Caused by Pharmacotherapy for Tics
		References
	13: Coprolalia and Malignant Phonic Tics
		Introduction
		Clinical Symptoms
		Treatment
		Conclusion
		References
	14: Hemiballism-Hemichorea
		Patient Vignettes
			Patient 1
			Patient 2
			Patient 3
		Clinical Description and Etiology
		Pathophysiology
		Prognosis
		Management
		Conclusion
		References
	15: Sydenham’s Chorea, PANDAS, and Other Post-streptococcal Neurological Disorders
		Patient Vignette
		Introduction
		Clinical Features and Diagnosis
			Sydenham’s Chorea
			Pandas
			Post-streptococcal Acute Disseminated Encephalomyelitis
			Post-streptococcal Acute Myoclonus
			Post-streptococcal Autoimmune Dystonia with Isolated Striatal Necrosis
			Post-Streptococcal Paroxysmal Dystonic Choreoathetosis
			Post-streptococcal Parkinsonism
			Post-streptococcal Opsoclonus-Myoclonus Syndrome
		Pathophysiology
		Treatment
			Sydenham’s Chorea
				Symptomatic Treatment
				Antibiotic Treatment
				Immunotherapy
			Pandas
				Symptomatic Treatment
				Antibiotic Treatment
			Immunotherapy
			Post-streptococcal Acute Disseminated Encephalomyelitis
			Post-streptococcal Acute Myoclonus
			Post-streptococcal Autoimmune Dystonia with Isolated Striatal Necrosis
			Post-streptococcal Paroxysmal Dystonic Choreoathetosis
			Post-streptococcal Parkinsonism
			Post-streptococcal Opsoclonus-Myoclonus Syndrome
		Conclusion
		References
	16: Anti-NMDA Receptor Encephalitis and Other Autoimmune and Paraneoplastic Movement Disorders
		Patient Vignette
		Introduction
		General Concepts
		General Classification
		Anti-NMDAR Encephalitis and Other Disorders Resulting in an Excess of Movements
			Anti-NMDAR Encephalitis
			Anti-NMDAR Encephalitis After Herpes Simplex Encephalitis
			Paraneoplastic Chorea and CRMP5 Antibodies
			Pseudoathetoid Movements in Paraneoplastic Sensory Neuronopathy
			Opsoclonus–Myoclonus–Ataxia Syndrome
			Sydenham’s Chorea
			Myoclonic-Like Movements in Patients with LGI1 Antibodies
			Tremor and Ataxia in Paraneoplastic Cerebellar Degeneration
		Disorders Resulting in a Paucity of Movement or Stiffness
			Anti-Ma2 Encephalitis and Hypokinesis
			Basal Ganglia Encephalitis
			Stiff-Person Syndrome
			Peripheral Nerve Hyperexcitability
		General Management Considerations
		References
	17: Psychosis and Parkinson’s Disease
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Phenomenology
		Epidemiology
		Factors That Influence Development of PD Psychosis
		Pathophysiology
		Evaluation
		Treatment
		Follow-Up of Patient Vignettes
		Conclusion
		References
	18: Perioperative Emergencies Associated with Deep Brain Stimulation
		Patient Vignettes
			Perioperative Emergencies
				Patient 1: Intraventricular Hemorrhage
				Patient 2: Intracerebral Hemorrhage
				Patient 3: Intraoperative Seizure
				Patient 4: Venous Infarct
				Patient 5: Neuroleptic Malignant Syndrome (Parkinsonism-Hyperpyrexia Syndrome) [1]
				Patient 6: Myocardial Infarction
			Postoperative Emergencies
				Patient 7: Superficial Wound Infection
				Patient 8: Deep Infection
				Patient 9: Intracerebral Infection
				Patient 10: Lead Migration
				Patient 11: Hardware Malfunction
		Introduction
		Perioperative Management
			Intracranial Hemorrhage
			Subdural Hematoma
			Air Embolus
			Dyskinetic Storm
			Intraoperative Seizure
			Neuroleptic Malignant Syndrome (Parkinsonism-Hyperpyrexia Syndrome)
			Behavioral and Cognitive Problems
			Myocardial Infarction
		Postoperative Management
			Suicide Attempt and Ideation
			Hardware Infection
			Lead Migration
			Hardware Malfunction
			Accidental On/Off and Symptom Rebound
			Aseptic Intraparenchymal Cyst
		Conclusion
		References
Part IV: Emergencies of Recognition: Pitfalls in Diagnosis
	19: Startle Disorders
		Patient Vignette
		Introduction
		Normal Startle Reflex
		Startle Syndromes
			Stimulus-Induced Disorders
			Neuropsychiatric Startle Disorders
			Hyperekplexia
				Etiology and Genetics
				Clinical Features
				Treatment/Management
				Future Studies
		Conclusion
		References
	20: Pseudodystonic Emergencies
		Patient Vignettes
			Patient 1
			Patient 2
		Concept of Pseudodystonia
			Pseudodystonic Emergencies
		Evaluation and Treatment of Pseudodystonic Emergencies
		References
	21: Functional Movement Disorders
		Patient Vignette
		Introduction
		A Note on Terminology
		Pathophysiology
		Diagnosis
			General Diagnostic Features of FMD
				Functional Tremor
				Functional Dystonia
				Functional Gait Disorder
				Functional Myoclonus/Jerks
				Functional Parkinsonism
				Functional Tics
				Functional Weakness
		Management of Functional Movement Disorders in the Emergency Setting
		Conclusion
		References
	22: Tardive and Neuroleptic-Induced Emergencies
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Neuroleptic-Induced Respiratory Phenomena
		Neuroleptic-Induced Gastrointestinal Phenomena
		Oculogyric Crisis
		Treatment
		Conclusion
		References
	23: Abductor Paresis in Shy-Drager Disease
		Patient Vignette
		Introduction
		Clinical Aspects of VCAI
			Symptoms and Diagnosis of VCAI
			Acoustic Analysis of Stridor
		Pathogenesis of VCAI
		Laryngeal Abnormalities Other than VCAI
			Supraglottic Collapse and LM
			Supraglottic Collapse and OSAS
		Treatments
			Treatment for VCAI
			Treatment for FE and FA
			CPAP Failure
			Combination with UAO and CRD
		Conclusion
		References
	24: Dopa-Responsive Dystonia and Related Disorders
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Clinical Observations
			Classic DRD
			Phenotypic Heterogeneity
			Adult-Onset Parkinsonism
			DRD Simulating Cerebral Palsy or Spastic Paraplegia
			Various Types of Focal Dystonia
			Other Involuntary Movements
			Non-motor Symptoms
		Molecular Genetics
			GTPCH1-Deficient DRD
			TH-Deficient DRD
			SR-Deficient DRD
		Genetically Related Disorders
			Severe GTPCH1 Deficiency (GTPCH1-Deficient HPA)
			Moderate GTPCH1 Deficiency (Dystonia with Motor Delay)
			Contiguous Gene Deletion Syndrome Relating to GTPCH1 Deficiency
			Other BH4-Related Enzyme Deficiencies Including Severe SR Deficiency
			Very Severe TH Deficiency (TH-Deficient Progressive Infantile Encephalopathy)
			Severe TH Deficiency (TH-Deficient Infantile Parkinsonism with Motor Delay)
			Atypical Forms of Severe TH Deficiency
				TH-Deficient Myoclonus-Dystonia
				TH Deficiency with Exacerbation by Viral Infections
				TH Deficiency with a Biphasic Clinical Course
		Laboratory Investigations
			CSF Analysis
			Activity Assay
			Phenylalanine Loading Test
		Neuroimaging
		Neuropathology
		Neurochemistry
		Diagnosis
		Treatment
		Conclusion
		References
	25: Wilson’s Disease
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		First WD Emergency: Timely Diagnosis
			Clinical Presentation
		Diagnosis of WD
		Second WD Emergency: Timely Treatment
		Third WD Emergency: Paradoxical Treatment-Induced Worsening
		Conclusion
		References
	26: Wilson Disease Presenting as Opsoclonus-Myoclonus Syndrome
		Patient Vignette
		Introduction
		Pathophysiology
		Signs and Symptoms
		Assessment
		Diagnosis
		Treatment
		Conclusion
		References
	27: X-Linked Adrenoleukodystrophy: Addisonian Crisis in a Patient with Spastic Paraparesis-Ataxia Syndrome
		Patient Vignette
		Introduction
		Pathophysiology
		Clinical Presentations
			Neuropsychiatric Manifestations
			Endocrine Dysfunction
			Dermatologic Signs
			Radiologic Findings
		Diagnosis
		Differential Diagnosis
		Treatment
		References
	28: Whipple’s Disease
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Tropheryma whipplei
		Epidemiology
		Pathophysiology and Immunopathology
		Clinical Presentations of Whipple’s Disease
		Neurological Manifestations of Whipple’s Disease
		Radiologic Findings
		Investigations and Diagnosis
		Treatment
		Conclusion
		References
Part V: Practical Risks in the Clinic: Pitfalls in Management
	29: Emergencies in Huntington’s Disease
		Patient Vignettes
			Patient 1
			Patient 2
			Patient 3
		Introduction
		Emergencies in HD
			Psychiatric Emergencies in HD
			Trauma
			Pneumonia and Aspiration Pneumonitis
		Conclusion
		References
	30: Genetics and Genetic Counseling-Related Issues
		Patient Vignettes
			Patient 1
			Patient 2
		Introduction
		Psychosocial Emergencies Following a Diagnostic Gene Test
		Requests for “Emergency” Diagnostic Testing
			Patient 3
			Patient 4
		Psychosocial Complications of Predictive Testing
		Prenatal Testing
		Family Crises Related to a Genetic Diagnosis
		Conclusion
		References
	31: Driving in Parkinson’s Disease
		Patient Vignettes
			Patient 1
			Patient 2
			Patient 3
		Introduction
		The Scope of the Problem
		The Framework to Start Driving
		Neural Substrates of Driving
		Driving Research in PD
			Off-Road Evaluation
				Demographics
				Assessments of Cognition, Vision, and Motor Function and Indices of Parkinsonism Sleep and Psychiatric Problems
					Effect of Sleep-Related Impairments
					Effect of PD Treatment
			Standardized Road Tests
				Cross-Sectional Studies
				Longitudinal Studies
				Effects of Multitasking on Driving in PD
			Driving Simulation
			Real-Life Driving Outcomes
				Driving History and Habits
		Driver Rehabilitation
		Policy Issues
		Conclusion
		References
	32: Suicide Risk in Parkinson’s Disease
		Patient Vignette
		Introduction
		Suicidal Ideation
		Completed Suicides
		Deep Brain Stimulation and Suicidal Outcomes
		Conclusion
		References
Index