Movement Disorders in Children

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Movement Disorders in Children
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Overview

• Childhood movement disorders occur secondary to a
  wide range of genetic and acquired disorders
  affecting brain development. 
•  Classification by type of abnormal movement
• Bradykinetic disorders
• Hyperkinetic disorders
• Classification by Etiology
• Primary
• Secondary
• Fixed Structural lesion
• Degenerative
• Metabolic
• Drug Induced
• Infectious
• Important point
• Any disorder that affects the basal ganglia can
  cause a wide array of different movement
  disorders
• Static brain injury may nonetheless cause a
  changing movement disorder, as development and
  brain plasticity alter the brains response to
  injury.

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Basal Ganglia

• Group of deep nuclei
• Caudate nucleus
• Putamen
• Globus pallidus
• Substantia nigra
• dopamine-rich pars compacta
• pars reticularis
• Inputs Corpus striatum (caudate nucleus and
  putamen) receives input from the cerebral cortex
  and the thalamus
• Outputs projects by way of the thalamus to the
  cerebral cortex and then to the pyramidal system

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Bradykinetic vs. Hyperkinetic

• Bradykinetic disorders
• Very rare in children
• Parkinson disease is the most common bradykinetic
  disorder
• Hyperkinetic disorders
• Tic Disorders
• Dystonia
• Sterotypies
• Chorea
• Athetoses
• Ballismus
• Tremor
• Myoclonus
• Dyskinesia

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Tic Disorders

• Tics are repeated, intermittent movements that
  are almost always briefly suppressible and are
  usually associated with awareness of an urge to
  perform the movement
• Tourette syndrome Multiple motor and vocal tics
• Etiology
• Primary
• the vast majority
• Secondary
• Huntingtons, encephalitis, medication induced,
  carbon monoxide poisoning, neuroacantocytosis

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Tics

• Diagnosis
• Typical movements
• Dont occur in sleep
• Patient usually unaware of it occurring
• Patient can usually suppress for a short time
• But when they do, it is accompanied by a
  discomfort and a strong urge to do the tic (a
  compulsion)
• Wax and wane over time
• Worsen with stress
• Associated with ADHD and OCD
• Make sure to ask both of patient and family
  history
• PANDAS
• Treatment
• Reassurance
• Tics tend to wax and wane most children outgrow
  them
• Medications (when necessary)
• Stimulants bring out tics if the have ADHD, they
  cant use stimulants
• Tenex
• Risperidone

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Dystonia

• Involuntary sustained or intermittent muscle
  contractions that cause twisting and repetitive
  movements, abnormal postures, or both.

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Dystonia
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Classification of Dystonia

• By location
• Generalized dystonia affects most or all of the
  body.
• Focal dystonia is localized to a specific part of
  the body.
• Blepharospasm, Cervical Dystonia, Task Specific
  Dystonia (eg Writers cramp)
• Multifocal dystonia involves two or more
  unrelated body parts.
• Segmental dystonia affects two or more adjacent
  parts of the body.
• Hemidystonia involves the arm and leg on the same
  side of the body.
• By etiology
• Primary by definition, no other neurologic
  impairment
• Secondary Cerebral Palsy the most common cause
  in children
• Psychogenic

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Primary Dystonias

• Genetic Dystonias
• DYT1 dystonia 
• dominantly inherited generalized dystonia
• typically begins in childhood, affects the limbs
  first, and progresses
• A great deal of phenotypic variability
• Dopa-responsive dystonia (Segawas disease)
• onset during childhood and have progressive
  difficulty with walking.  
• Symptoms characteristically fluctuate and are
  worse late in the day and after exercise. 
• Some forms are due to mutations in the DYT5 gene
  for GTP cyclohydrolase 1.   
• Patients with this disorder have dramatic
  improvements in symptoms after treatment with
  levodopa
• Many other genes that cause dystonic syndromes
  have been found

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Secondary Dystonias

• Fixed Injury/Structural
• Kernicterus,, head trauma, encephalitis tumors,
  stroke, congenital malformations
• Degenerative Fahr's disease ,
  pantothenate-kinase associate dneurodegenerative
  disease (Hallervorden-Spatz disease),
  Huntington's disease, spinocerebellar ataxias
  neuronal ceroid lipofuscinosis Rett syndrome
  Tay-Sachs disease Sandhoff's disease
  Niemann-Pick type C metachromatic
  leukodystrophy Leigh's disease
  neuroacanthocytosisPelizaeus-Merzbacher
  disease ataxia-telangiectasia
• Chemical/Metabolic Glutaric aciduria
  mitochondrial disorders Wilson's disease
  homocystinuria Lesch-Nyhan disease
  methylmalonic aciduria tyrosinemia, vitamin E
  deficiency
• Drug- or Toxin-induced
• Neuroleptic and anti-emetic medications (e.g.,
  haloperidol, thorazine, olanzapine, risperidone,
  quetiapine, compazine, prochlorperazine,
  metoclopramide, etc.) calcium channel blockers
  stimulants , anticonvulsants (e.g.,
  carbamazepine, phenytoin, ) thallium manganese
  carbon monoxide ethylene glycol cyanide
  methanol wasp sting
• Paroxysmal
• Paroxysmal kinesogenic choreoathetosis familial
  periodic paralysis complex migraine alternating
  hemiplegia paroxysmal torticollis of infancy
• Psychogenic
• Disorders That Mimic Dystonia
• Tonic seizures, syringomyelia Arnold-Chiari
  malformation type II posterior fossa mass
  cervical spine malformation, Sandifer's syndrome
  spasmus nutans tics self-stimulation
  spasticity myotonia hyperexplexia disorders

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Work up of Dystonia

• Take careful history of medication, drug and
  supplement use
• Consider Genetic testing (especially DYT1)
• Consider empiric trial of levodopa
• Consider metabolic testing amino acids, organic
  acis, Wilsons testing, lysosomal storage diseases
• Consider MRI

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Treatment of Dystonia

• Botulinum toxin
• Particularly for focal dystonias
• Medications
• Anticholinergic agents trihexyphenidyl and
  benztropine.
• GABAergic agents  benzodiazepines, baclofen
• Dopaminergic agents tetrabenazine
• Levodopa for Dopa-responsive dystonia (DRD)
• Deep brain stimulation (DBS)
• Physical and other therapies

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Sterotypies

• Repetitive, simple movements that can be
  voluntarily suppressed.
• Examples include repetitive chewing, rocking,
  twirling, or touching movements
• Most common in children with autism or mental
  retardation can occur in otherwise normal
  children.

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Rett Syndrome
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Chorea, athetosis and ballismus

• Chorea
• an irregular, rapid, uncontrolled, involuntary,
  excessive movement that seems to flow randomly
  from one part of the body to another.
• The affected child often appears fidgety or
  restless and cant sit still
• Athetosis
• A slower writhing and twisting movement.
• Ballism (ballismus)
• chorea that affects proximal joints such as
  shoulder or hip, leading to large amplitude
  flailing movements of the limbs

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Etiology of Chorea

• Fixed injury/Structural
• Cerebral Palsy (Kernicterus), Tumor, Trauma,
  Stroke
• Degenerative
• Huntingtons, Neuroacanthocytosis,
  Ataxia-telangiectasia Fahr's disease
  pantothenate kinase 2 deficiency
  ("Hallervorden-Spatz disease") Rett syndrome
  Niemann-Pick disease type C , Pelizaeus-Merzbacher
  disease GM1 gangliosidosis, metachromatic
  leukodystrophy Wilson's disease ,etc
• Drug Induced/Toxins
• Neuroleptics, antiparkinson drugs, tricyclics ,
  amphetamines, anticonvulsants, anticholinergics
• Metabolic
• Hyperthyroid, hypoparathyroid, pregnancy, hyper
  and hypo natremia, hypomagnesemia, hypoclaciemia,
  nutrional deficiencies (beriberi, pellagra, B12
  deficiency)
• Acyl-CoA dehydrogenase deficiency Lesch-Nyhan
  disease methylmalonic aciduria vitamin E
  deficiency propionic acidemia
• Infectious
• Sydenhams chorea
• Immunological
• SLE, HSP
• Migraine
• Psychogenic

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Kernicterus
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Sydenham chorea

• Sydenham chorea is a movement disorder
  characterized by chorea, emotional lability, and
  hypotonia. It is one of the major clinical
  manifestations of acute rheumatic fever (ARF).
• Symptoms of SC usually begin one to eight months
  after the onset of ARF. The symptoms typically
  improve gradually, with a mean duration of 12 to
  15 weeks (
• At least 30 percent of individuals have clinical
  carditis in association with SC.
• Diagnosis
• The diagnosis of SC is made clinically, based on
  characteristic neurological findings and a
  careful cardiac examination. If carditis is
  present, this confirms the diagnosis.
• The antistreptolysin O (ASLO) titer is of limited
  use in patients with SC, because titers generally
  peak before the onset of SC symptoms and children
  without rheumatic fever or SC often have low
  positive titers of ASLO.
• The antideoxyribonuclease (anti-DNAse) B titer is
  more useful for supporting the diagnosis of SC
  because it tends to remain elevated longer.
• If not clinicually definite, other causes of
  chorea should be excluded, including systemic
  lupus erythematosus, Huntingtons disease, and
  Wilsons disease.
• Treatment
• Most patients with SC recover fully without
  treatment, with symptoms lasting from a few weeks
  to one year or more.
• For those with significant impairment of motor
  function and the possibility of self injury
  consider corticosteroids (prednisone 1 mg/kg
  daily for two weeks and then tapered over two to
  three weeks)
• Valproic acid if needed to treat chorea
• Up to 30 percent of individuals with SC
  experience a recurrence, usually within a few
  years of the initial episode. The risk is
  probably reduced, by chronic treatment with
  prophylactic antibiotics.

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Sydenham Chorea
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Athetosis

• http//www.youtube.com/watch?vgNKKZAfMr8M

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Work up

• Take careful history of medication, drug and
  supplement use
• If acute onset throat culture and streptococcal
  blood antigen test (ASLO, anti-DNAse),
  electrolytes, magnesium, calcium, thyroid
  function, CBC
• Consider amino and organic acid studies, ammonia,
  antinuclear antigen (ANA), antiphospholipid
  antibodies (APLA), work up for Wilsons disease
  (start with ceruloplasmin), evaluation of CBC for
  acanthocytes.
• Consider MRI

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Treatment of Chorea

• May be difficult to treat.
• Taper or discontinue any medications that can
  cause or worsen chorea
• In adults, the mainstay of treatment in adults is
  neuroleptics, including haloperidol and pimozide.
  
• In children the incidence of side effects in
  children is high.
• Therefore, treatment is usually
• Benzodiazepine, particularly clonazepam,
  diazepam, or clobazam
• Valproate, especially in Sydenham's chorea.
• Sydenham's chorea
• There is considerable debate about whether
  children with Sydenham's chorea due to
  streptococcal infection should be given long-term
  antibiotics. There is not yet scientific evidence
  to support this, although short-term treatment is
  certainly needed in order to prevent
  complications such as rheumatic fever.

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Tremor

• A rhythmic back-and-forth or oscillating
  involuntary movement about a joint.

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Classification of Tremor

• Classification by type of tremor
• Rest Tremor
• Parkinsons, Wilson Disease, Severe essential
  tremor
• Action Tremor
• Postural
• Kinetic
• Intention Cerebellar Tremor
• Task Specific
• Isomeric
• Classification by Etiology
• Physiologic tremor
• Essential tremor
• Associated w/ Peripheral Neuropathy Charcot
  MarieTooth
• Psychogenic

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Etiology of Tremor

• Primary Tremors
• Enhanced physiologic tremor
• Essential Tremor
• Static (fixed) injuryStroke (particularly in
  the midbrain or cerebellum) multiple sclerosis
• Degenerative
• Juvenile parkinsonism Wilson's disease
  Huntington's disease Tay-Sachs disease
• Chemical/metabolic
• Hyperthyroidism hyper-adrenaline state
  (including anxiety or pheochromocytoma)
  hypomagnesemia hypocalcemia hypoglycemia
  hepatic encephalopathy
• Drug-induced
• Valproate lithium thyroid hormone albuterol,
  tricyclic antidepressants stimulants,
  neuroleptics cyclosporine mercury thallium
  nicotine lead manganese arsenic cyanide
  ethanol
• Psychogenic tremor
• Other causes of tremor
• Peripheral neuropathy, cerebellar disease or
  malformation,spasmus nutans

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Essential Tremor

• Tremor should be the only neurologic
  manifestation
• Usually benign, but may progress to a disabling
  movement disorder.
• Hereditary ET can begin in infancy
• hereditary chin tremor and shuddering attacks.

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Work up of Tremor

• Any medications that may worsen tremor should be
  avoided, if possible.
• Check electrolytes, including glucose, calcium
  and magnesium, thyroid function, copper in the
  urine (for Wilson's disease), and possibly the
  amount of adrenaline metabolites (for
  pheochromocytoma).
• Consider MRI if the tremor had sudden onset,
• Consider EEG if there is suspicion for seizures.
• If parkinsonian features are present, consider a
  trial of L-DOPA
• If there is a family history of tremor, it may be
  helpful to of alcohol ( in the affected family
  member). This suggests essential tremor.

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Treatment of Tremor

• Often, mild tremor does not require treatment.
• Medications
• Propranolol
• Primidone
• benzodiazepines (i.e., clonazepam, diazepam,
  lorazepam).

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Myoclonus

• Sudden, brief, jerky, shock-like involuntary
  movements.
• May be triggered by attempts at voluntary
  movement, sensory stimulation or startle
• Myoclonus is not suppressible and is often
  activated by volitional movement.
• Negative myoclonus is a sudden involuntary
  relaxation of a muscle, rather than a
  contraction.
• Myoclonus is often associated with epilepsy. 

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Classification and Etiology of Myoclonus

• Physiological e.g., sleep myoclonus, benign
  myoclonus of infancy
• Essential Myoclonus familial essential
  myoclonus, essential myoclonus-dystonia,
  stimulus-sensitive myoclonus
• Epileptic e.g., juvenile myoclonic epilepsy,
  progressive myoclonic epilepsies, epilepsia
  partialis continua, Rasmussen's encephalitis,
  early infantile myoclonic encephalopathy,
  infantile spasms, Lennox-Gastaut syndrome, benign
  familiar myoclonic epilepsy, Angelman syndrome
• Symptomatic
• Fixed injury e.g., carbon-monoxide poisoning,
  hypoxic injury or near-drowning, heatstroke,
  trauma, stroke, electrocution
• Storage/Degenerative diseases e.g., sialidoses,
  lipidosis, storage diseases, Wilson's disease,
  Rett syndrome, mitochondrial disorders,
  spinocerebellar ataxias
• Infections/Para-infectious e.g., Creutzfeldt-Jaco
  b disease, steptococcus, viral encephalitis
• Endocrine e.g., hyperthyroidism, hyponatremia,
  hypoglycemia
• Structural e.g., tumors that irritate brain in
  direct manner or release chemicals into the blood
• Drug-induced/Toxins e.g., anti-seizure
  medications, antidepressants, stimulants,
  liver-toxic medications, respiratory depressants,
  corticosteroids, acyclovir, L-dopa
• Associated with systemic illness e.g., dialysis,
  renal failure, liver failure, pulmonary disease,
  carbon dioxide intoxication

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