Approach to child with Involuntary movements

The approach outlined here covers the following four types of involuntary movement: chorea, athetosis,
dystonia and tremor. Hemiballismus, tics, myoclonus and seizure activity are mentioned briefly.

Types of involuntary movements

Involuntary movement Description
Chorea. This describes irregular rapid
movements involving any
muscle group, especially distal.
Athetosis This describes slow writhing
movements of proximal
extremities.
Dystonia. This comprises sustained
abnormal posturing, which may
be brought on rapidly in
‘dystonic spasms’.
etiology
Causes include CP, Sydenham
chorea, Wilson disease (WD),
systemic lupus erythematosus
(SLE), moyamoya disease and
degenerative conditions such as
AT, Huntington disease (HD),
Lesch–Nyhan syndrome (LNS)
and phenylketonuria (PKU).
Chorea is due to pathology
affecting the corpus striatum.
It can accompany chorea, as in
dyskinetic CP, WD, LNS and AT.
Athetosis is due to pathology
affecting the outer region of the
putamen.
Causes include drugs (tardive
dystonia), degenerative
disorders such as WD, HD and
post-hemiplegic.

Tremor. 1. Static tremor: present at rest, Causes include WD, HD and

There are three basic types:
Hemiballismus.
disappears with action.
2. Postural tremor is most
notable when the arms are
outstretched in front of the
body, but can occur through a
range of movement.
3. Intention tremor is marked at
end points of movement, but is
not present during the course of
movement.
This is unilateral random gross
rotatory movements of the
Parkinson diseases.
Causes include thyrotoxicosis,
phaeochromocytoma, familial
tremor, physiological tremor
and WD.
Causes include many disorders
affecting the cerebellar
hemispheres and pathways,
including WD. Note that
asterixis, or ‘flapping tremor’, is
not actually a tremor, and
should be differentiated from
this. Causes include liver failure
and hypercapnia.
it is due to pathology in the sub-
thalamic region on the side
 proximal portion of a limb.
Exceptionally rare in
paediatrics,
Tics. These are brief, separate,
defined movements, usually
involving the head and face,
that can be voluntarily
suppressed.
Myoclonus. This is sudden, disorganised,
irregular contraction of a
muscle or muscle group
(distinguished from
fasciculations, which cannot
cause movement of a complete
muscle group).
WD and CP can cause choreoathetosis, tremors, dystonia, or myoclonus, so the examination needs to
thoroughly evaluate these two conditions, irrespective of the type of movement disorder.
opposite to the affected side.
Causes include benign
childhood tics, and the Gilles de
la Tourette syndrome.
Causes include seizure disorders
(e.g. infantile spasms, benign
juvenile myoclonic epilepsy),
degenerative conditions
(neurocutaneous syndromes,
WD), structural brain anomalies
(Aicardi syndrome,
porencephaly), cerebrovascular
accidents, anoxic brain injury,
infections (SSPE) and metabolic
disorders (aminoacidopathies).

Examination
the outline given below is essentially similar, whether the problem is chorea, athetosis, dystonia, or
tremor. Remember that movement disorders can coexist

1. Make A Start by introducing yourself to the patient and parent.

2. Try to gain an impression of whether there is any intellectual impairment (e.g., CP, HD, PKU) or
hearing impairment (e.g., kernicterus) and note the speech (e.g., cerebellar dysarthria, palilalia
with Parkinson disease).
3. Stand back and inspect for evidence of stigmata of chronic liver disease (WD), telangiectasia
(AT), facial butterfly erythema (SLE), fair complexion with blond hair (PKU), mask facies
(Parkinson), prominent eyes (thyrotoxicosis), evidence of self-mutilation (LNS) or spastic
posturing (CP).
4. Make a point of looking at the parents (HD).
5. Describe the quality and distribution of the movements: whether they are unilateral or bilateral;
involve the face, arms, trunk, or legs; are fast or slow, regular or irregular, distal or proximal.
6. A series of maneuvers can then be performed to establish more clearly which sort of movement
is occurring.
 Have the child shake hands with you and then squeeze your finger. This is to detect a ‘milkmaid
grip,’ which occurs with chorea.
 Then ask the child to hold out his or her hands, first with palms up and then with palms down.
This may detect static tremors or chorea.
  Ask the child to hold the arms outstretched to either side of the body. Then have the child try to
put both index fingers to either side of the nose, as close as possible to the nose without
touching. This is a sensitive test for several involuntary movements, including intention tremors.
 Finally, have the child hold his or her wrists back in extension to exclude asterixis.
 Have the child hold both arms up above the head. Look for the development of pronation
(pronator sign) with chorea.
 If there is any suggestion of intention tremor, check for dysdiadochokinesis.
7. Check the upper limb tone (decreased with chorea, increased with CP), power, and reflexes.
8. A rapid functional assessment (e.g., write your name, drink from a cup) may be performed at
this stage to assess the degree of incapacity caused by the movement.

This stage may be clear whether the problem is (most probably) chorea or tremor. This will allow much
of the following to be omitted, as it will not be relevant.

1. If the type of movement is not yet clear, a full gait examination can be performed, looking for
CP, cerebellar disease, and WD or HD.
2. Take note of heel-toe walking (cerebellar disorders), squatting (thyrotoxicosis), and also have
the child walk, turn quickly, stop and recommence walking (Parkinson).
3. This can be followed by a neurological lower limb evaluation for tone, power, reflexes, and
cerebellar function.
4. The head may then be examined. First, measure the head circumference (decreased with CP).
5. Inspect the face for malar flush (SLE).
6. Look at the eyes for lid retraction or proptosis (thyrotoxicosis), telangiectasia (AT), Kayser-
Fleischer rings (WD), nystagmus (cerebellar disease, AT), and oculomotor dyspraxia (AT).
7. Test the extraocular movements, looking for nystagmus, and check for lid lag (thyrotoxicosis).
Check the ears for telangiectasia (AT);
8. test the hearing (kernicterus, CP), and if it is abnormal, perform Rinne’s and Weber’s tests.
9. Have the child poke out the tongue to detect a ‘Jack-in-the-box’ tongue, which may occur with
chorea.
10. Check the neck for goiter (thyrotoxicosis).
11. Next, the cardiovascular system can be examined for evidence of rheumatic heart disease
(Sydenham chorea).
 Check the pulse for abnormal waveform (e.g., aortic incompetence) and tachycardia
(thyrotoxicosis).
 Request or take the BP (for phaeochromocytoma as the cause of tremor).
 Palpate and auscultate the praecordium for valvular disease.
12. Examine the abdomen for prominent abdominal wall veins, hepatosplenomegaly, or ascites
(WD) and look for peripheral signs of chronic liver disease.
13. If the tremor is the problem, also look for abdominal wall needle marks (diabetic hypoglycemia
as the cause of tremor) and palpate for the adrenal glands (but check with the examiners that
there is no contraindication to deep palpation, as palpating a phaeochromocytoma can cause an
acute hypertensive crisis).
14. After the case, summarise your findings, present a differential diagnosis and discuss which
investigations would be in order