1 Comment

109. Movement Disorders

109.1 Basal Ganglia Anatomy, Biochemistry, and Physiology

109.2 Cerebellar Anatomy, Biochemistry, and Physiology

109.3 Classification of Movement Disorders

109.4 Diagnostic Evaluation of Children with Movement Disorders

    • History
    • Examination
      • The Physical and Neurologic Examination
        • The General Physical Examination
        • The Neurologic Examination
          • Mental Status and General Cognitive and Emotional Cerebral Function
          • Expressive and Receptive Language
      • Cranial Nerves
      • Motor Examination
      • Sensory Examination
      • Cerebellar Examination
      • Tremor Examination
    • Diagnosis
      • Patterns Based on Phenomenology and Time Course
      • Proximate Causes as Possible Etiologies for Acute- and Subacute-Onset, Acquired Movement Disorders
      • Stepwise, Organized Approach to More Difficult Chronic Diagnoses
        • Understanding the Phenomenology
        • Identifying the Anatomic Substrate
        • Incorporating the Time Course into the Diagnostic Process
        • Role of the Family History of Neurologic and Psychiatric Conditions
        • Nonneurologic, Key Features of History or Physical Examination
        • Utilizing electronic resources

109.5 Developmental Movement Disorders in Children

  • Benign Neonatal Sleep Myoclonus
  • Benign Myoclonus of Early Infancy (Benign Infantile Spasms)
  • Shuddering
  • Jitteriness
  • Paroxysmal Tonic Upgaze of Infancy
  • Spasmus Nutans
  • Head Nodding
  • Benign Paroxysmal Torticollis
  • Benign Idiopathic Dystonia of Infancy
  • Sandifer Syndrome
  • Posturing during Masturbation

109.6 Tics and Tourette Syndrome

  • Tic Phenomenology
  • Tic Disorders
    • Transient Tic Disorder
    • Provisional Tic Disorder (Tic Disorder-Diagnosis Deferred)
    • Chronic Motor or Phonic Tic Disorder (CMVTD)
    • Tourette Syndrome and Tourette’s Disorder
    • Tic Disorder; Not Otherwise Specified
    • Tourettism, Tourette-like, or Secondary Tic Disorder
  • Pathophysiology of Tic Disorders
    • Neuroanatomic Localization
      • Striatum
      • Cortical
    • Neurotransmitter Abnormalities
      • Dopamine
      • Serotonin
      • Glutamate
  • Etiology
    • Genetic Basis
    • Autoimmune Disorder
  • Associated Behaviors and Psychopathologies in Tic Disorders
    • Attention-Deficit Hyperactivity Disorder
    • Obsessive-Compulsive Disorder
    • Anxiety and Depression
    • Episodic Outbursts (Rage) and Self-Injurious Behavior
    • Other Psychopathologies
      • Academic Difficulties
        • Sleep Disorders
  • Treatment
    • General Principles
    • Tic Suppression
      • Nonpharmacologic Treatments
      • Pharmacotherapy
      • Tier One Medications
      • Tier Two Medications
        • Other Medications and Botulinum Toxin
      • Surgical Approaches

109.7 Motor Stereotypies

  • Definition
  • Differentiating Stereotypies from Other Disorders
  • Pathophysiology of Motor stereotypies
  • Classification of Motor Stereotypies
    • Primary
      • Common Stereotypies
      • Head-Nodding Stereotypies
      • Complex Hand and Arm Movement Stereotypies
    • Secondary
      • Associated with Autism and Mental Retardation
      • Rett Syndrome
      • Associated with Sensory Deprivation
      • Associated with Inborn Errors of Metabolism/Genetic
      • Drug-Induced
      • Associated with Psychiatric Disorders
  • Treatment

109.8 Paroxysmal Dyskinesias

  • Paroxysmal Kinesigenic Dyskinesia
  • Paroxysmal Nonkinesigenic Dyskinesia
  • Paroxysmal Exertion-induced Dyskinesia
  • Paroxysmal Hypnogenic Dyskinesia

109.9 Chorea, Athetosis, and Ballism

  • Definitions of Chorea, Athetosis, and Ballism
  • Clinical Characteristics-Phenomenology of Chorea, Athetosis, and Ballism in Children
    • Chorea
      • History
      • Examination
    • Ballism
    • Athetosis
  • Neuroanatomy of Chorea
  • Neurophysiology of Chorea
  • Localization and Pathophysiology
  • Physiologic Chorea
    • Chorea in Infancy
    • Chorea Minor
  • Primary Choreas
    • Benign Hereditary Chorea
    • Choreoathetosis, Hypothyroidism, Neonatal Respiratory Distress-Allelic to Benign Hereditary Chorea
  • Secondary (Acquired) Choreas
    • Choreas Occurring in Immunologic/Autoimmune Disease
    • Sydenham’s Chorea
    • Chorea in Primary Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus
    • Vascular/Hypoxic Ischemic
      • “Postpump Chorea”-Chorea after Neonatal and Infant Cardiac Surgery
    • Dyskinetic/Choreoathetoid Cerebral Palsy
    • Kernicterus/Chronic Bilirubin Encephalopathy
    • Hyperglycemia/Diabetes-Associated Subacute Hemichorea or Hemiballism
  • Multisystem and Genetic Diseases Where Chorea is Prominent
    • Huntington’s Disease in Late Teens
    • Infantile Bilateral Striatal Necrosis
  • Treatment of Chorea

109.10 Dystonia

  • Introduction and Definition
  • Clinical Characteristics of Dystonia
  • Localization and Pathophysiology
  • Etiologies
    • Early-Onset Primary (Generalized) Torsion Dystonia (DYT1)
    • Other Early-Onset Primary Dystonias (DYT2; DYT4)
    • Dopa-Responsive Dystonia (DYT5)
    • Adolescent-Onset Mixed-Type
    • Dystonia (DYT6)
    • Myoclonus Dystonia Syndrome (DYT11)
    • Rapid-Onset Dystonia-Parkinsonism (DYT12)
    • Early-Onset Multifocal/Segmental Dystonia (DYT13)
    • Early-Onset Dystonia-Parkinsonism (DYT16)
    • Dystonic Storm (Status Dystonicus)

109.11 Myoclonus

  • Definition of Myoclonus
  • Phenomenology of Myoclonus in children
  • Neurophysiology of Myoclonus
  • Localization and Pathophysiology
  • Physiologic Myoclonus-Myoclonus in Certain Settings, in Otherwise Healthy Individuals
  • Physiologic and Benign Forms of Myoclonus
  • Hemifacial spasm
  • Primary Myoclonic Disorders
    • Essential Myoclonus
    • Myoclonus-Dystonia (DYT11)
    • Benign Myoclonus of Early Infancy
  • Secondary Myoclonus
    • Opsoclonus Myoclonus (Ataxia) Syndrome
    • Postanoxic Myoclonus
    • Epilepsia Partialis Continua, Rasmussen’s Encephalitis, and Myoclonia Continua
  • Epileptic Myoclonus without Encephalopathy
    • Juvenile Myoclonic Epilepsy
    • Benign Familial Myoclonic Epilepsy
    • Myoclonia with Childhood Absence Epilepsies
  • Progressive Myoclonic Epilepsies
    • Mitochondrial Myopathies-Myoclonus Epilepsy and Ragged Red Fibers (MERRF)
    • Unverricht-Lundborg Disease
    • Lafora Disease
    • Neuronal Ceroid Lipofuscinoses
    • Sialidoses
    • Angelman Syndrome
  • Startle Syndromes
    • Hereditary Hyperekplexias
    • Symptomatic Startle Disorders
    • Startle Epilepsies
    • Neuropsychiatric Startle Syndromes
  • Clinical Differentiation of Myoclonus from Other Movement Disorders

109.12 Tremor

  • Definition of Tremor
  • Clinical Characteristics-Phenomenology of Tremor in Children
  • Clinical Differentiation from Other Movement Disorders, and Some Special Types of Tremor
  • Localization and Pathophysiology
  • Primary Tremors
    • Enhanced Physiologic Tremor
    • Intermittent Tremor in the Young Child
    • Intermittent Tremor in the Adolescent
    • Essential Tremor
  • Symptomatic Tremors
  • Approach to Diagnosis and Management

109.13 Ataxia

  • Definition of Ataxia
  • Clinical Characteristics-Phenomenology of Ataxia in Children
  • Localization and Pathophysiology
  • Diseases and Disorders
    • Nonprogressive Ataxia I: The Uncoordinated Child
    • Nonprogressive Ataxia II: Ataxia Associated with Congenital Cerebellar Malformations
    • Acute Ataxias
    • Subacute Ataxias
    • Acute Recurrent Ataxias
    • Metabolic Ataxias-Acute Intermittent
      • Episodic Ataxia Type 1 (EA1)
      • Episodic Ataxia Type 2 (EA2)
    • Chronic Progressive and Degenerative Ataxias
      • Autosomal Dominant Spinocerebellar Ataxias
        • Spinocerebellar Atrophy Type 1 (SCA1)
        • Spinocerebellar Atrophy Type 2 (SCA2)
        • Spinocerebellar Atrophy Type 3 (SCA3); also known as Machado-Joseph Disease
        • Spinocerebellar Atrophy Type 7 (SCA7)
        • Spinocerebellar Atrophy Type 13 (SCA13)
        • Autosomal Recessive Ataxias
          • Friedreich Ataxia
          • Ataxia Telangiectasia
          • Ataxia with Oculomotor Apraxias
          • Ataxia with Isolated Vitamin E Deficiency
          • Infantile-Onset Spinocerebellar Ataxia
          • Mitochondrial Recessive Ataxia Syndrome
          • Childhood Ataxia with Central Hypomyelination/Vanishing White Matter Disease
        • Metabolic Ataxias-Chronic Progressive
          • Refsum Disease (Heredopathia Atactica Polyneuritiformis)
          • Cerebrotendinous Xanthomatosis

109.14 Parkinsonism

  • Clinical Features of Parkinsonism
  • Pathophysiogy of Parkinsonism
    • Tremor
    • Bradykinesia
    • Akinesia
    • Rigidity
    • Postural Instability
  • Etiology of Parkinsonism in Children
    • Juvenile Parkinson’s Disease
  • Secondary Parkinsonism
    • Structural Lesions
    • Hereditary/Degenerative Diseases
      • Huntington’s Disease
      • Rett Syndrome
      • Neuronal Intranuclear Inclusion Disease
      • Pallido-Pyramidal Syndrome
      • Kufor-Rakeb Syndrome
    • Infectious and Postinfectious Diseases
    • Metabolic Diseases
      • Fahr Syndrome
    • Drug-induced Parkinsonism

109.15 Inherited Metabolic Disorders Associated with Extrapyramidal Symptoms

  • Pediatric Neurotransmitter Diseases
  • Tetrahydrobiopterin (BH4) Metabolism
  • BH4 Defects with Hyperphenylalaninemia
    • Autosomal recessively inherited forms of GTPCH-1 deficiency (autosomal recessive, 14q22.1-22.2)
    • 6-PTS deficiency (autosomal recessive,locus 11q22.3-23.3)
    • DHPR deficiency (autosomal recessive, locus 4p15.31)
  • BH4 Defects without Hyperphenylalaninemia
    • Dopa-responsive dystonia (DRD)
    • DHPR deficiency without hyperphenylalaninemia
    • Sepiapterin reductase (SPR) deficiency (autosomal recessive, locus 2p14-p12)
  • Primary Defects of Monoamine Biosynthesis
    • Tyrosine Hydroxylase Deficiency (Autosomal Recessive, Locus 11p15.5)
    • Aromatic l-Amino Acid Decarboxylase (ALAAD or AADC) Deficiency (Autosomal Recessive, Locus 7p12.1-12.3)
  • Succinic Semialdehyde Dehydrogenase Deficiency (4-Hydroxybutyric Acidurias, Autosomal Recessive, Locus 6p32)
  • Mineral Accumulation
    • Wilson’s Disease (Hepatolenticular Degeneration) (Autosomal Recessive, Locus 13q14.3)
    • Aceruloplasminemia (Autosomal Recessive Disorder, Localized to Chromosome 3)
    • Neurodegeneration with Brain Iron Accumulation (NBIA)
    • Pantothenate Kinase–Associated Neurodegeneration (PKAN)
      • HARP syndrome
    • Neuroferritinopathy
    • Infantile Neuroaxonal Dystrophy
  • Lysosomal Disorders
    • Neuronal Storage Diseases
      • GM 1 gangliosidoses
      • GM2 gangliosidoses
    • Niemann-Pick Diseases
    • Gaucher Disease
    • Neuronal Ceroid Lipofuscinoses
      • Infantile NCL (INCL, Haltia-Santavuori Disease)
      • Late-Infantile (LINCL, Jansky-Bielschowsky Disease)
      • Juvenile (JNCL, Batten Spielmeyer-Vogt-Sjögren disease)
    • Cerebral folate deficiency
  • White Matter (Dysmyelinating) Disorders
  • Amino Acid Disorders
    • Homocystinuria
    • Hartnup Disease
    • Maple Syrup Urine Disease
    • Phenylketonuria
  • Organic Acid Disorders
    • Disorders of Lysine Catabolism
      • Glutaric Aciduria Type 1 (Autosomal Recessive)
    • Branched-Chain Organic Acidurias
      • Methylmalonic Acidemia (Autosomal Recessive)
      • Propionic Acidemia (Autosomal Recessive)
      • 3-Methylglutaconic Aciduria
      • Methylhydroxybutyryl-CoA Dehydrogenase
      • Methylacetoacetyl-CoA or Acetoacetyl-CoA Thiolase (β-Ketothiolase) Deficiency
    • Other Organic Acidurias
      • l-2-Hydroxyglutaric Aciduria
      • D-2-Hydroxyglutaric Acidemia
  • Glycolysis, Pyruvate Metabolism, and Tricarboxylic Acid Cycle Disorders
    • Triosephosphate Isomerase Deficiency
    • Pyruvate Carboxylase Deficiency
    • Pyruvate Dehydrogenase Complex Deficiency
    • 2-Ketoglutarate Dehydrogenase Deficiency
    • Fumarase (Fumarate Hydratase) Deficiency
  • Mitochondrial Disorders
    • Leigh Syndrome
    • Other Mitochondrial Syndromes
    • Leber Hereditary Optic Neuropathy Plus Dystonia
    • Mohr-Tranebjaerg Syndrome
  • Disorders of Purine Metabolism
    • Lesch-Nyhan Disease
    • Adenylosuccinate Lyase Deficiency
    • Phosphoribosylpyrophosphate Synthase Superactivity
    • Phosphoribosylpyrophosphate Synthase Deficiency
  • Disorders of Creatine Metabolism
    • Guanidinoacetate Methyltransferase Deficiency (Creatine Deficiency Syndrome)
    • Other Creatine Deficiency Syndromes
  • Cofactor Disorders
    • Molybdenum Cofactor (Sulfite Oxidase) Deficiency
  • Leukoencephalopathies
    • Pelizaeus-Merzbacher Disease
    • Aicardi-Goutières Syndrome
    • Canavan, van Bogaert, Bertrand Disease
  • Glucose Transport Defect (GLUT1 Deficiency)
  • Neuroacanthocytosis
    • McLeod Syndrome
    • Bassen-Kornzweig Syndrome
  • Congenital Disorders of Glycosylation (Formerly Named Carbohydrate-Deficient Glycoprotein Syndrome)

109.16 Movements That Occur in Sleep

  • Overview of Sleep Physiology
  • Classification of Movements in Sleep
  • Sleep-related myoclonic disorders
    • Hypnic Jerks (Sleep Starts; Hypnagogic Jerks)
    • Benign Neonatal Sleep Myoclonus
    • Propriospinal Myoclonus
    • Excessive Fragmentary Myoclonus in NREM Sleep
    • Idiopathic Myoclonus in the Oromandibular Region during Sleep (Nocturnal Faciomandibular Myoclonus)
    • Narcolepsy
  • Restless Legs Syndrome
  • Periodic Limb Movements of Sleep
  • Paroxysmal Hypnogenic Dyskinesia
  • (Nocturnal Paroxysmal Dystonia)
  • REM Sleep Disorders (Second Half of Night)
    • Nightmares (Dream Anxiety Attacks)
    • REM Sleep Behavioral Disorder (RBD)
  • Movements associated with Parasomnias
    • Disorders of Arousal
      • Sleepwalking (Somnambulism)
      • Sleep Terrors (Night Terrors, Pavor Nocturnus)
      • Confusional Arousals
    • Sleep-Wake Transition Disorders
    • Rhythmic Movement Disorders
  • Hyperkinetic Movement Disorders That Are Present during the Daytime and Persist during Sleep
  • Seizures In and Around the Time of sleep
  • Sleep Bruxism (Nocturnal Tooth Grinding)

109.17 Psychogenic Movement Disorders

  • Clinical Features of Psychogenic Movement Disorders
  • Pathophysiology
  • Diagnostic Criteria for Psychogenic Movement Disorders
    • Documented PMD
    • Clinically Established PMD
    • Probable PMD
    • Possible PMD
  • Aids in Diagnosing Psychogenic Movement Disorders
  • Specific Movement Disorder Types in Psychogenic Movement Disorders
    • Tremor
    • Dystonia
    • Myoclonus
  • Treatment and Outcome

109.18 Drug-Induced Movement Disorders in Children

  • Phenomenology of Drug-Induced Movement Disorders in Children
  • DIMDs Associated with Dopamine Receptor Blockade: Typical Antipsychotics, Atypical Antipsychotics
    • Neuroleptic Malignant Syndrome
  • Drug-Induced Movement Disorders
    • DIMDs Associated with ADHD Treatment
    • DIMDs Associated with Other Medications
      • Serotonin Reuptake Inhibitors
      • Antiseizure Medications
  • DIMDs Associated with Chemotherapeutic Medications
Say something here...
Log in with ( Sign Up ? )
or post as a guest
Loading comment... The comment will be refreshed after 00:00.
  • This commment is unpublished.
    Derek Foden · 20 days ago
    My Parkinson disease symptoms started at the age of 47. The fingers on my left hand were stiff and were difficult to move. People noticed that my walk was not normal. I was often asked if I was hurt. I noticed nothing different about my walk. It was difficult getting up from a chair and getting out of a car. I was diagnosed a year later ,it was the onset of tremors starting in my right hand that caused my other symptoms to be recognized as Parkinson's.. I am now 59. With the new herbal medicine for Parkinson I purchased from  Multivitamincare . org  was my only way to get rid of my PD,the herbal formula effectively reversed my condition and alleviated all my symptoms, people are suffering from this Parkinson disease due to lack of information.