Involuntary Movements

Purposeless movements that occur at restand usually disappear during sleep. >1 type can occur in thesame individual.

Principal Causes of Involuntary Movements

1. Tics
   1. Transienttic disorder
   2. Tourette syndrome
   3. Drugs
2. Chorea
   1. Sydenham chorea
   2. Benign familial chorea
   3. Huntington disease
   4. Drugs
3. Athetosis
4. Dystonia
   1. Dopa-responsive dystonia
   2. Idiopathic torsion dystonia (dystoniamusculorum deformans)
   3. Hypoxic-ischemic encephalopathy
   4. Wilson disease
   5. Hallervorden-Spatz disease
   6. Drugs
5. Myoclonus
   1. Benign neonatal sleep myoclonus
   2. Essential myoclonus
   3. Myoclonic encephalopathy
   4. Other
6. Tremor
   1. Physiologic tremor
   2. Pathologic tremor
7. Spasmus nutans
8. Ballismus
9. Habit spasms

Clinical Features and Diagnosis

Tics

Brief, sudden,repetitive movements that may be motor, vocal, or both.

Motor tics usually involve face, neck,or shoulder and last 1–2 secs. Consist of eye blinking, grimacing,head jerking, and shoulder shrugging.

Vocal tics include throat clearing,sniffing, coughing, grunting, and humming.

Social impairing tics [e.g.,coprolalia (foul language) and copropraxia (foul gestures)] are lesscommon.

Transient Tic Disorder

Usuallycharacterized by ≥1 simple tics, typically involving head orupper body.

Duration of symptoms is <12mos.

No markers identify which childrenwill progress to have Tourette syndrome.

Tourette Syndrome

Autosomal-dominantdisorder characterized by motor and/or vocal tics of ≥1 yr'sduration.

Gene locus has been mapped to chromosome11q23.

Attention deficit hyperactivity disorder,obsessive compulsive behaviors, and other psychiatric disordersare commonly associated with Tourette syndrome.

Drugs

Onset and severity of tics may be associatedwith stimulant use, although recent epidemiologic evidence appearsto make this association less clear. Other implicated drugs includeanticonvulsants, neuroleptics, and levodopa.

Chorea

Consists of repetitive, aimless, irregular,jerky movements of face, trunk, and extremities. Can be more apparentduring sustained muscle contraction or action movements.

Sydenham Chorea

Manifestationof acute rheumatic fever that can occur alone or with other manifestationsof rheumatic fever.

May occur months after primary infectionwith group A Streptococcus.

Besides chorea, characteristic featuresinclude emotional lability, slurred speech, difficulty in holdingobjects, and worsening handwriting. Anxiety, inattentiveness, anddeterioration in learning may occur before chorea becomes apparent.

Antineuronal antibodies as well asantibodies to streptolysin O and DNAse B may be found in many childrenwith Sydenham chorea.

Benign Familial Chorea

Onset isin infancy or early childhood.

Both autosomal-dominant and -recessiveforms occur.

Age of onset, family history, and lackof neurologic deterioration are diagnostic.

Less pronounced in adolescence.

Huntington Disease

Autosomal-dominantdisease that can appear as early as 5 yrs of age, but usual onset isin middle of adult life.

Gene locus has been mapped to chromosome4p16.3.

Characteristic features include chorea,rigidity, and dementia. Speech and behavioral changes may precedeintellectual deterioration.

CT or MRI often shows atrophy of caudatenucleus and putamen.

Clinical and radiologic findings alongwith family history are diagnostic. Molecular genetic analysis isconfirmatory.

Drugs

Phenytoin, methylphenidate, dextroamphetamine,ethosuximide, and oral contraceptives can cause chorea-like movements.

Athetosis

Consistsof slow, writhing, purposeless, often continuous movements of head,neck, and extremities. Excitement or agitation increases their amplitude.

Hypoxic-ischemic encephalopathy andcerebral palsy are common causes of these movements, which may mergewith chorea-like movements to produce choreoathetosis.

Familial paroxysmal choreoathetosisis autosomal-dominant disorder that may occur in children who areotherwise normal.

Dystonia

Slow, writhing,irregular, twisting movements of limbs, trunk, or face that produce abnormalpostures. Movements may begin with initiation of reaching or walking.

Increases with anxiety and stress andtend to disappear during sleep.

Dopa-Responsive Dystonia

May be transmittedas autosomal-dominant or -recessive trait. Dominant form is causedby mutations in GTP cyclohydrolase 1 gene on chromosome 14q22.1-q22.2,while recessive form is caused by mutations in tyrosine hydroxylasegene on chromosome 11p15.5.

Onset is usually in childhood withgait disturbance produced by dystonic leg movements, which may worsenas day progresses. Posturing movements of arms also may occur. Eventually,parkinsonian features (e.g., rigidity and bradykinesia) develop.

Diagnosis generally confirmed by clinicalresponse to small doses of levodopa. Molecular genetic analysisis definitive.

Idiopathic Torsion Dystonia (Dystonia Musculorum Deformans)

There isevidence that mutations in DYP 1 gene on chromosome 9q34 can causethis autosomal-dominant disorder.

Onset of this slowly progressive diseaseis usually 5–15 yrs of age.

Posturing of trunk and neck interfereswith sitting, standing, and walking. Involvement of face and tonguemay impair speech and swallowing. Intellect is preserved and seizuresdo not occur.

Molecular genetic analysis is confirmatory.

Hypoxic-Ischemic Encephalopathy

1 of theprincipal causes of chronic dystonia in pediatric population.

Some common causes of hypoxic-ischemicencephalopathy are perinatal asphyxia, head trauma, near drowning,and carbon monoxide poisoning.

Wilson Disease

Autosomal-recessivedisorder of copper transport that is characterized by liver involvementand degenerative changes in brain, primarily basal ganglia.

Neurologic findings (e.g., dystoniaand speech disturbance) usually occur after 10 yrs of age, whereashepatic disease usually occurs before this age.

See Chap.36, Jaundice.

Hallervorden-Spatz Disease

Mutationsin pantothenate kinase gene located on chromosome 20p12.3-p13 have beenfound to cause this autosomal-recessive disorder.

Onset is usually 2–10 yrsof age with generalized dystonia followed by spasticity, speech difficulty,retinal degeneration, and intellectual deterioration.

MRI shows lesions involving the globuspallidus. Molecular genetic analysis is confirmatory.

Drugs

Acute dystonia may be due to phenothiazines,haloperidol, and metoclopramide.

Myoclonus

Fast, repetitivecontractions of muscle or muscle group in any part of body.

Muscular jerks may be epileptic ornonepileptic. Epileptic form is discussed in Chap. 59, Seizures.

Occurrence of myoclonus in restrictedgroup of muscles (arm or leg) is called myoclonus simplex, whereasgeneralized contraction of muscle groups is termed polymyoclonus.

Benign Neonatal Sleep Myoclonus

Consistsof rhythmic jerks of extremities, which may last a few seconds orcontinue for a few hours, and cease on awakening. They usually disappearin a few months.

Child's development and electroencephalogramare normal.

Essential Myoclonus

Autosomal-dominantdisorder of unknown cause that consists of brief, shocklike musclecontractions with onset usually after several years of age.

Proximal limb muscles are primarilyaffected.

Course is benign, with no seizuresor other neurologic impairment.

Myoclonic Encephalopathy

Myoclonic jerks of head and trunk; bizarre,chaotic, conjugate eye movements; and truncal ataxia characterizethis disorder, which can be associated with neuroblastoma or postviralencephalitis (see Chap. 4, Ataxia).

Other

Polymyoclonus may be associated with severaldisorders, including phenylketonuria, maple syrup urine disease,Tay-Sachs disease, Hallervorden-Spatz disease, tuberous sclerosis,hypoxic-ischemic encephalopathy, lead intoxication, and encephalitis.

Tremor

Regular, rhythmic, alternating contractionsof muscle group and its antagonists that may occur at rest or withmovement. Hands and fingers are usually affected.

Physiologic Tremor

All humanshave tremor of hands that may be detected by special electrophysiologic techniques.

Enhanced physiologic tremor is actionthat is visible to naked eye. May be caused by fear, anxiety, fatigue,hyperthyroidism, caffeine, cigarette smoking, and drugs (beta adrenergicbronchodilators, lithium, valproate, selective serotonin reuptakeinhibitors).

Pathologic Tremor

Includesresting, action, and intention tremors.

Resting tremor occurs when extremityis relaxed, while action tremor is seen with sustained extensionof the arm, or while performing motor tasks (e.g., writing). Bothforms of tremor may occur with Wilson disease and Parkinson disease,though the latter is rare in pediatric population.

Most common form of action tremor isessential tremor, which involves hands but can also affect head,trunk, and legs. This autosomal-dominant disorder may begin in childhoodand slowly progress.

Intention tremor occurs when exactor precise movement is attempted (e.g., finger-to-nose maneuver).Often accompanied by other signs of cerebellar disease (e.g., ataxia,nystagmus, and slurred speech).

Spasmus Nutans

Disorderof unknown cause that usually occurs after 6 mos of age and resolvesby 3–4 yrs of age.

Consists of triad of nystagmus, headnodding, and torticollis, but all do not have to occur together.

Usually disappears within a few monthsor 1 or 2 yrs.

Ballismus

Flailingmovements of arms and legs.

No meaningful distinction between choreaand ballismus exists except one of degree.

In childhood, ballismus is most commonlyseen as extreme manifestation of chorea, usually Sydenham chorea.

Habit Spasms

Occur mostoften in older children and adolescents and consist of sniffing,throat clearing, grimacing, head jerking, and eye blinking.

Tension or stress may precipitate theiroccurrence.

With passage of time, individual becomesunaware of them and cannot control them.

Diagnostic Approach

Movementdisorders generally must be identified by clinical features, incontext of history, family history, and careful physical exam. Oncespecific type of movement is recognized, specific cause should beinvestigated.

In absence of any associated neurologicdeficits, neuroimaging and electroencephalography are not routinelyuseful for childhood-onset movement disorders.

References

1. Aicardi J. Diseases of the nervous system,2nd ed. London: Mac Keith Press, 1998.
2. Behrman RE, et al., eds. Nelson textbook of pediatrics,16th ed. Philadelphia: WB Saunders, 2000.
3. Fenichel GM. Clinical pediatric neurology: a signsand symptoms approach, 4th ed. Philadelphia: WB Saunders, 2001.
4. Fleisher GR, Ludwig S, eds. Textbook of pediatric emergencymedicine, 4th ed. Philadelphia: Lippincott Williams & Wilkins,2000.
5. Golden GS. Tics, twitches and habit spasms. Curr ProblPediatr 1978;8:29–41.
6. Lang AE, Lozano AM. Parkinson's disease. NEngl J Med 1998;339:1044–1053, 1130–1143.
7. Leckman JF, Cohen DJ. Tourette's syndrome—tics,obsessions, compulsions: developmental psychopathology and clinicalcare. New York: John Wiley & Sons, 1999.
8. Louis ED. Essential tremor. N Engl J Med 2001;345:887–891.
9. Online Mendelian Inheritance in Man (OMIM). McKusick-NathansInstitute for Genetic Medicine, Johns Hopkins University (Baltimore,MD) and National Center for Biotechnology Information, NationalLibrary of Medicine (Bethesda, MD), 2000. World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim.
10. Rudolph AM, ed. Rudolph's pediatrics, 20thed. Stamford, CT: Appleton & Lange, 1996.
11. Swaiman KF. Movement disorders and disorders of thebasal ganglia. In: Swaiman KF, Ashwal S, eds. Pediatric neurology:principles & practice, 3rd ed. St. Louis: CV Mosby, 1999:801–831.

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Book Source Details

• Book Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics
• Author(s): Paul S. Bellet
• Year of Publication: 2006
• Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2006 Lippincott Williams & Wilkins.

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