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Dyskinesias in Children/Adolescents. CPT Timothy L. Switaj, MC, FS, USA Neurology (Child) Intern. Objectives. To demonstrate the basic abnormal movements in children/adolescents To begin to think about a differential for each type of movement

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Dyskinesias in Children/Adolescents

CPT Timothy L. Switaj, MC, FS, USA

Neurology (Child) Intern


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Objectives

  • To demonstrate the basic abnormal movements in children/adolescents

  • To begin to think about a differential for each type of movement

  • Brief discussion of the most likely etiologies of the abnormal movements


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Basic Movement Types

  • * Chorea/Athetosis

    • Ballismus

  • Dystonia

  • Hemifacial Spasm

  • Mirror Movements

  • * Myoclonus

  • Stereotypies

  • * Tics

  • * Tremor

  • Fasciculations

  • Myokymia

  • Seizures


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Things to ask yourself when seeing patient

  • What does the movement look like?

    • Is it rhythmical, jerky or “dancelike”?

  • Can it be suppressed?

  • What medications is the patient taking?

  • Any Family History of similar movements?


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General Characteristics (1)

  • Chorea/Athetosis – usually seen together

    • Chorea – rapid movement affecting body part that is incorporated into voluntary movement to hide it, NO FIXED FORM

      • Constant movement (restlessness)

      • Movements flow from side to side and limb to limb

    • Athetosis – slow, writhing movement of the limbs

      • Can occur alone but usually associated with chorea – athetosis without chorea is due to perinatal brain injury (most likely perinatal asphyxia)

    • Ballismus – high-amplitude, violent flinging of a limb (an extreme form of chorea)

    • Tardive Dyskinesia – uncommon in children


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General Characteristics (2)

  • Dystonia – sustained muscle contractions

    • Can be focal, segmental, hemi or generalized

  • Hemifacial spasm – involuntary, irregular contraction of muscles innervated by one facial nerve

    • Very rare in children

  • Mirror movements – involuntary movements of one side of body that are mirror reversals of intended movements on the other side

    • Normal during infancy and disappear before age 10 – persistence can be familial trait

    • Obligatory movements are abnormal at any age


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General Characteristics (3)

  • Myoclonus – involuntary movements characterized by rapid muscle jerks

    • Can be rhythmic, nonrhythmic; focal, multifocal or generalized; spontaneous, action or reflex

  • Stereotypies – repeated, purposeless movements

    • Can be simple or complex


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General Characteristics (4)

  • Tics – “habit spasms”; complex, stereotyped movements or utterances that are sudden, brief and purposeless

    • As opposed to chorea, are stereotyped

    • Can be suppressed for short periods, with some discomfort and are never part of a voluntary movement


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General Characteristics (5)

  • Tremor – involuntary oscillating movement with a fixed frequency

    • Product of frequency and amplitude are constant

      • Frequency decreases with age, amplitude increases

    • Shuddering, ataxia and dysmetria are not tremor because they lack rhythm


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General Characteristics (6)

  • Fasciculations

    • Rippling movements of a small group of muscles, benign with low amplitude common in young

  • Myokymia

    • Slow, worm-like, undulating movements usually in the face but also in the large limb muscles

  • Seizures


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Chorea - Differential

  • Neurodegenerative diseases (Huntington’s)

  • Lesions of the basal ganglia

  • Drugs (Dopamine agonists, stimulants, opiates, antiepileptics, estrogens)

  • Metabolic conditions (Wilson’s, hyperthyroid, hyperglycemia, hypoglycemia, electrolyte disorders)

  • Systemic disorders (Syndenham’s, lupus, chorea gravidarum)

  • Essential chorea syndromes

  • Paroxysmal chorea

  • Cardiopulmonary bypass (1 to 10%)


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Huntington’s - Genetics

Autosomal dominant, full penetrance, 50% chance to pass to

Offspring, CAG repeat of greater than 39 is diagnostic


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Huntington’s - Features

  • Age of onset typically 35-45, but childhood to >80 has occurred

  • Chronic, progressive, generalized chorea

  • Failure of indirect pathway

  • Can have other movement disorders present (parkinsonism, dystonia and tic)

  • Dementia late in disease


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Syndenham’s - Features

  • A.K.A. Rheumatic Chorea

  • 10 to 30% of cases of rheumatic fever

  • Symptoms appear 1 to 6 months after infection and last 5 to 15 weeks

  • Recurs in 20% of patients

  • Can cause mental status changes

  • Most cases in ages 5 to 15

  • Migratory chorea of limbs and face

  • Cardinal features of chorea, hypotonia, dysarthria and emotional lability

  • Treatment with steroids and treatment for infection


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Chorea Gravidarum

  • Due to antiphospholipid antibody syndrome, with or without SLE

  • Usually during 2nd to 5th month, sometimes postpartum

  • Cognitive changes may be present

  • Symptoms resolve spontaneously in weeks to months


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Chorea – workup/treatment

  • Neuroimaging, glucose, electrolytes, thyroid studies, CBC with smear, copper studies, genetic studies

  • Treat underlying cause

  • Can use clonazepam as first line

  • Neuroleptics are second line

  • Follow-up important because chorea tends to evolve


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Myoclonus - Evaluation

  • Distribution

    • Generalized, focal, multifocal, segmental

  • Temporal profile

    • Continuous, intermittent

  • Activation

    • Rest, voluntary, stimulus


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Myoclonus - classification

  • First – determine major category

  • Second – match clinical and lab/radiology findings with diagnosis within major category


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Myoclonus - Categories

  • Physiological

  • Essential

  • Epileptic

  • Symptomatic


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Myoclonus - Physiologic

  • Neurologically normal persons

  • Sleep jerking – most common

  • Also be anxiety or exercise related

  • Diagnosis based on history alone

  • NO TREATMENT NEEDED


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Myoclonus - Essential

  • Clinically significant jerking occuring at any time

  • Usually most prominent or only finding

  • Differs from physiologic because of social or physical disability

  • Condition progresses slowly or not at all

  • Hereditary (Autosomal dominant) or sporadic

  • Face, trunk and proximal muscles

  • Clinical features and family history make diagnosis

  • Clonazepam drug of choice is treatment needed


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Myoclonus - Epileptic

  • In persons with chronic seizure disorder and epileptiform activity on EEG

  • Juvenile Myoclonic Epilepsy

    • Myoclonic seizures with generalized tonic-clonic or absence

    • Onset in adolescence with peak between 12 and 18

    • Abnormality on chromosome 6


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Myoclonus - symptomatic

  • Neurodegenerative syndromes

  • Infection/Postinfection

  • Drugs, toxins, metabolic disorders

  • Hypoxia

  • Focal or segmental

  • Paraneoplastic

  • Post-CNS injury


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Myoclonus – workup/treatment

  • Electrolytes, glucose, renal and hepatic function testing, drug and toxin screening, brain imaging, EEG

  • Genetic studies, tissue biopsy and CSF studies as clinically indicated

  • Treatment is clonazepam

  • Valproic acid for JME


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Tics

  • Can be suppressed for short periods of time

  • Simple or complex

  • Transient tics occur in 20% of children under 10 years of age

  • Gilles de la Tourette syndrome


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Tourette’s

  • 10 cases per 10,000 population

  • Onset between 2 and 15 (mean 6.5)

  • Vocal tics begin 1 to 2 years after motor tics

  • 75% are tic free by 18 years old

  • Increase in severity with stress, caffeine, stimulants, fatigue, heat, steroids

  • Decrease with THC, alcohol, nicotine and decrease in mental activity

  • Disability usually social but may be physical injury

  • 50% also with ADHD, 30 to 50% with OCD

  • Multiple other behavioral problems


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Tourette’s

  • Diagnosis:

    • Multiple motor and one or more vocal tics

    • Onset before age 18

    • Tics occur many times a day, nearly every day

    • Variation in location, frequency and complexity over time

    • Not related to toxins or CNS disease

    • Symptoms cause impairment


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Tourette’s

  • Genetic factors in 75% with bilineal transmission in 25%

  • Radiologic/Laboratory workup not needed

  • Treatment

    • Clonidine, benzodiazepines, haldol, risperdal, clozapine, reserpine

  • Surgery for drug-resistant tics, but not shown effective in Tourette’s


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Tremor

  • All people have a physiologic tremor inherent in movement that cannot be normally noticed unless measured

  • Fine or coarse

  • Resting, postural, action


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Connor GS et al. Esential Tremor: A Practical Guide to Evaluation,

Diagnosis, and Treatment. Clinician, 19(2): 2001.


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Tremor - differential Evaluation,

  • Drug induced (Anticonvulsants, antidepressants, caffeine, steroids)

  • Hyperthyroidism

  • Juvenile Parkison’s disease (Not common)

  • Paroxysmal Dystonic Head Tremor

  • Essential tremor


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Essential tremor Evaluation,

  • 1 in 20 arise in childhood

  • 70% of pediatric cases in males

  • Not associated with other neurologic disturbances

  • Genetics – ETM1 on 3q13, ETM2 on 2p25, complete penetrance, autosomal dominant

  • Most common movement disorder

  • Prevalence of 0.1 to 22% worldwide

  • 20 times more common than Parkinson’s




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Essential tremor - Features Evaluation,

  • 4 to 8 hertz

  • Usually in limbs, occassionally head and face

  • Appears first in hands because it is enhanced by greater precision movements

  • Can be postural (early) and action (later)

  • Generally life-long

  • Can impact writing and other functions

  • Worsening due to enhanced physiologic tremor

  • Enhanced by anxiety, attempts to suppress, fatigue

  • Tremor can become severe with significant disability


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Connor GS et al. Esential Tremor: A Practical Guide to Evaluation,

Diagnosis, and Treatment. Clinician, 19(2): 2001.


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Essential Tremor – workup/treatment Evaluation,

  • Neuroimaging normal, pathology not indicated, genetic research possible if familial

  • Treatment usually not needed

  • If needed use beta-blockers first

    • Then anticonvulsants, benzos, calcium channel blockers, botox

  • If severe tremor, drug-resistant, deep brain stimulation


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DBS - Thalamus Evaluation,


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DBS - Localizing Evaluation,


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DBS - Leads Evaluation,


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References Evaluation,

  • Postgraduate Medicine, 108(5), Oct 2000.

  • Pranzatelli MR. Movement Disorders in Childhood. Ped Rev, 17(11): 1996.

  • Gerald M. Fenichel. Clinical Pediatric Neurology, 4th Edition. 2001.

  • All videos courtesy of Dr. Difazio