Podcast
Questions and Answers
What is the most common movement disorder in children?
What is a characteristic feature of tics?
Which condition is associated with severe movement disorders and must be ruled out in pediatric cases?
Sydenham’s chorea is most commonly linked to which of the following?
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Dystonia in children is often characterized by which of the following factors?
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Why do most pediatric patients with tremor often not seek medical attention?
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Stereotypies in children are clinically relevant because they are usually associated with which conditions?
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What is a notable feature of parkinsonism in children?
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What characterizes dystonia as a movement disorder?
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How is tremor defined in the context of movement disorders?
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What distinguishes Tourette's syndrome from other tic disorders?
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In what age group do tics typically present the most common?
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Which of the following is a common association with Tourette’s syndrome?
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What is unique about the feature of tics compared to other movement disorders?
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What genetic condition is the most common cause of generalized dystonia in patients under 26?
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What is the typical onset age for symptoms of DYT1?
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What defines dopa-responsive dystonia (DYT5) apart from other dystonias?
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Which neurological signs are sometimes observed in DYT5 patients?
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What percentage of children with tics may demonstrate symptoms requiring special education services?
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What is coprolalia in the context of tics?
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What is the prognosis for individuals with Tourette's syndrome regarding long-term outcomes?
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What term is used when tics are secondary to identifiable causes?
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Which of the following features is NOT suggestive of a psychogenic movement disorder?
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What type of stereotypies are most commonly observed in children with classical autism?
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Which statement is true regarding Rett syndrome?
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What is a common characteristic of Parkinsonian syndrome in children?
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Which condition is the most common cause of chorea in children worldwide?
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Stereotypies in children can be associated with which of the following conditions?
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In children, what is typically true about the prognosis of tics?
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Which condition should always be ruled out in children with a movement disorder due to its effective treatment options?
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Which characteristic is NOT typically associated with movement disorders in children?
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What are the common features of stereotypies observed in both autism and Rett syndrome?
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What is the unique distinguishing feature of DYT5?
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Which condition describes the most common form of secondary dystonia in children?
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What factor is the major cause of disability in patients with Sydenham's chorea?
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What is a common developmental feature associated with Sydenham’s chorea?
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Which neurological condition is distinguished by the absence of other features associated with rheumatic fever?
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In children, what is the most common cause of physiological tremor enhanced by drugs?
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Which of the following conditions is characterized by a rigid-akinetic syndrome rather than chorea?
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How is the diagnosis of Sydenham's chorea primarily confirmed?
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Which statement correctly describes the movement severity in children with cerebral palsy?
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What characterizes essential tremor in children?
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What is the genetic basis of benign hereditary chorea?
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Which condition may develop recurrence of chorea later in life when pregnant?
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What is a common feature of children diagnosed with essential tremor?
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Which condition is generally associated with psychogenic tremors in children?
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Study Notes
Movement Disorders in Childhood
- Movement disorders are common in childhood, with unique features compared to adults.
- Tics are the most frequent, often mild and with a good prognosis.
- Dystonia is less common but often genetic, causing significant disability.
Tics
- Affect nearly one-fifth of elementary school students.
- More common in boys, onset around age 8.
- Tourette’s syndrome (TS) is the most common type.
- Characterized by:
- Multiple motor tics.
- At least one phonic tic.
- Duration of at least one year.
- Onset before age 21.
- Can be suppressed, but this leads to discomfort.
- Most cases involve simple movements in the head and face.
- Severe cases can involve complex, widespread movement patterns.
- Coprolalia, the emission of obscenities, occurs in less than 25% of TS patients.
- Strong association with behavioral problems such as obsessive-compulsive disorder and ADHD.
- Long-term prognosis is favorable, with spontaneous improvement in up to 70% of cases.
Dystonia
- Primary dystonias, without a structural brain lesion, are usually idiopathic or genetic.
- Oppenheim’s dystonia (DYT1) is the most common cause of generalized dystonia in children under 26 years old.
- Autosomal dominant condition with variable penetrance and expression.
- Onset typically in the first decade of life with unilateral foot dystonia.
- Gradually spreads to other limbs, trunk, and even the neck and face.
- More common in Ashkenazi Jews.
- Many cases have mild presentation with focal or segmental dystonia.
- Dopa-responsive dystonia (DYT5, Segawa’s disease) is another autosomal dominant condition. .
- Greater expression in females.
- Onset typically in the first decade of life with action dystonia of the foot.
- Often presents with fluctuations in symptom severity throughout the day and after exercising.
- Some patients present with parkinsonian findings.
- DYT5 is uniquely responsive to low-dose L-dopa, which leads to complete disappearance of dystonia.
- It is crucial to treat all childhood-onset dystonia patients with L-dopa for at least 60 days to differentiate DYT5 from other dystonia forms.
- Cerebral palsy (CP) is the most common cause of secondary dystonia in children.
- Characterized by various movement disorders and neurological findings, including dystonia, chorea, tremor, myoclonus, ataxia, and spasticity.
- Dystonia is typically more severe in the upper body.
- CP patients commonly have severe dysarthria.
Chorea
- Sydenham’s chorea (SC) is the most common cause of acute chorea in children worldwide.
- A major manifestation of rheumatic fever.
- Presumably caused by antibodies induced by b-hemolytic streptococci that cross-react with brain antigens.
- Average age at onset is 9 years.
- More common in girls.
- In addition to chorea, patients may experience other motor and non-motor features such as decreased muscle tone, vocalizations, emotional lability, obsessions, compulsions, attention deficit and hyperactivity, and carditis.
- Cardiac complications are the most significant source of disability.
- Can have a protracted course lasting 2 or more years (persistent SC).
- Patients may experience recurrences later in life, such as during pregnancy (chorea gravidarum) or when taking oral contraceptives.
- SC diagnosis is made based on clinical findings as there are no specific biological markers.
- Systemic lupus erythematosus (SLE) is a much rarer cause of chorea in children.
- Diagnostic confusion with SC is common.
- Patients with SLE typically have other non-neurological manifestations such as arthritis, pericarditis, serositis, and skin abnormalities.
- The neurological picture is more complex and may include psychosis, seizures, or other movement disorders.
- Patients with SLE will eventually develop other features that meet the diagnostic criteria for this condition.
- Benign hereditary chorea (BHC) is likely the main cause of isolated genetic chorea in children.
- Autosomal dominant inheritance pattern.
- Mutation in the TITF1/NKX2-1 gene, which codes for a transcription factor essential for organogenesis in the lung, thyroid, and basal ganglia.
- Characterized by a variable combination of chorea, mental retardation, congenital hypothyroidism, and chronic lung disease.
- Also known as brain–thyroid–lung syndrome.
Tremor
- Physiological tremor, often enhanced by drugs, is the most common cause of tremor in childhood.
- Commonly caused by medications for neuropsychiatric conditions (stimulants, neuroleptics, antiepileptic drugs, antidepressants) or non-neurological conditions (asthma, cardiac arrhythmia).
- Presentation is a high-frequency postural and kinetic tremor of the upper limbs, rarely disabling.
- Essential tremor (ET) is often regarded as the most common movement disorder among adults.
- Up to 30% of adults had tremor during childhood.
- Mean age of onset is 6 years.
- Diagnosis is based on clinical grounds since there is no biological marker for ET.
- Psychogenic tremor is the most common cause of tremor in children seen in emergency units.
- Characterized by acute onset, paroxysmal nature, irregular frequency and amplitude, entraining, disappearance with distractability, co-existent non-neurologic signs, and co-existent psychiatric disorders.
Stereotypy
- The most common cause of stereotypies in children is Autism Spectrum Disorder (ASD).
- Stereotypies in ASD can be motor or vocal.
- Common examples include hand-flapping, finger wiggling, trunk rocking, pacing, skipping, and shouting simple sounds.
- Less common stereotypies include shaking objects, jumping, running, and shouting complete sentences.
- Stereotypies in ASD are performed repeatedly, are voluntarily suppressible, and tend to disappear with distraction.
- Rett syndrome (RS) is another neurodevelopmental disorder with stereotypies, particularly hand clapping.
- Typically affects girls.
- Associated with MECP2 mutations.
- Has very poor prognosis.
- Characterized by developmental arrest and regression, poor sociability, lack of communication, irritability, anxiety, dystonia, respiratory pauses, loss of hand skills, deceleration of head growth, progressive motor deterioration and stereotypies.
Parkinsonism
- Parkinsonian syndrome (PS) is uncommon in children.
- Most cases are caused by infectious or autoimmune encephalitis.
- Drug-induced PS is also common.
- Typically characterized by a rigid-akinetic syndrome without tremor.
Wilson’s Disease (WD)
- A rare autosomal recessive disorder.
- Characterized by copper accumulation in the liver, cornea, brain, and other organs.
- Must be ruled out in every child with a movement disorder.
- Can lead to death if left untreated.
- There are effective treatments if diagnosed early.
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Description
Explore the unique features of movement disorders in children, focusing particularly on tics and their impact. Understand the characteristics of Tourette’s syndrome and its prevalence amongst elementary students. Gain insight into the prognosis and associated behavioral issues.
