This document outlines the approach to examining a child presenting with involuntary movements. It describes the key features and potential causes of different movement disorders including chorea, athetosis, dystonia, tremor, hemiballismus, tics and myoclonus. The examination involves detailed observation of movements, neurological assessment, and inspection for signs of underlying conditions such as Wilson's disease, cerebral palsy or thyroid disorders. Relevant investigations are then determined based on the differential diagnosis.

1. APPROACH TO
CHILD WITH
INVOLUNTARY
MOVEMENTS

2. 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.

3. Chorea This describes irregular rapid
movements involving any
muscle group, especially
distal.
Chorea is due to pathology
affecting the corpus
striatum.
Causes include
• CP
• Sydenham chorea,
• Wilson disease (WD),
• systemic lupus
erythematosus
• Moya Moya disease
• Thyrotoxicosis/hypothyroidis
m
• AT (ataxia telangiectasia)
• Huntington disease (HD),
• Lesch–Nyhan syndrome
(LNS)
• phenylketonuria (PKU).

4. Athetosis This describes slow
writhing movements
of proximal
extremities.
Athetosis is due to
pathology affecting
the outer region of the
putamen.
It can accompany chorea,
as in
• dyskinetic CP,
• WD (Wilsons's disease)
• LNS (lesch-nyhan
syndrome)
• AT. (Ataxia
telengectasia)

5. Dystonia. This comprises sustained
abnormal posturing, which
may be brought on rapidly in
‘dystonic spasms’.
Causes include drugs
(tardive dystonia),
degenerative disorders
such as WD(Wilsons's
disease), HD and post-
hemiplegic.

6. Tremor
There are three basic types:
1.Static tremor:
present at rest, disappears with action.
Causes include WD, HD and Parkinson
diseases.
2. Postural tremor
It is most notable when the arms are
outstretched in front of the body but can
occur through a range of movement.
Causes include thyrotoxicosis,
phaeochromocytoma, familial tremor,
physiological tremor and WD.
3. Intention tremor
It is marked at end points of movement
but is not present during movement.
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.

7. Hemiballismus. This is unilateral
random gross
rotatory
movements of
the proximal
portion of a limb.
Exceptionally
rare in
paediatrics,
it is due to
pathology in the
sub-thalamic
region on the side
opposite to the
affected side.

8. Tics. These are brief,
separate, defined
movements,
usually involving
the head and face,
that can be
voluntarily
suppressed.
Causes include
benign childhood
tics, and the
Gilles de la
Tourette
syndrome.

9. 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).
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).

10. WD (Wilson's disease)and CP (cerebral palsy) can cause
choreoathetosis, tremors, dystonia, or myoclonus, so the
examination needs to thoroughly evaluate these two
conditions, irrespective of the type of movement disorder.

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

12. 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).

13. 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

14. •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.

15. 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.

16. 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.

17. 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).

18. Inspect
Inspect the face
for malar flush
(SLE).
Look
Look at the eyes
for lid retraction
or proptosis
(thyrotoxicosis),
telangiectasia
(AT), Kayser-
Fleischer rings
(WD), nystagmus
(cerebellar
disease, AT), and
oculomotor
dyspraxia (AT).
Test
Test the
extraocular
movements,
looking for
nystagmus, and
check for lid lag
(thyrotoxicosis).
Check the ears
for telangiectasia
(AT);
Test
test the hearing
(kernicterus, CP),
and if it is
abnormal,
perform Rinne’s
and Weber’s
tests.
Have
Have the child
poke out the
tongue to detect
a ‘Jack-in-the-
box’ tongue,
which may occur
with chorea.

19. 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.

20. 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