An approach to a child with abnormal movement

1. An Approach to a Child with
Abnormal Movement
Sunil Agrawal
1
st
year MD Pediatrics
IOM

2. Contents
•Introduction
•Pathophysiology
•Classification
•History
•Examination
•Investigations
•Management

3. Introduction
- Dysfunction in the implementation of appropriate
targeting and velocity of intended movements,
- dysfunction of posture,
- the presence of abnormal involuntary movements,
-the performance of normal-appearing movements
at inappropriate or unintended times.

4. Introduction- Contd…
•Can be the primary or secondary manifestation of
numerous neurologic disorders
•Classification can be difficult
•Can resemble , sometimes difficult to distnguish
from each other

5. Pathophysiology
•The components typically implicated in disorders of
movement are
–the basal ganglia (caudate, putamen, globus pallidus,
subthalamic nucleus, substantia nigra) and
–frontal cortex.
•The accomplishment of smooth, coordinated
movement requires a multifaceted network of brain
regions, including basal ganglia and frontal cortex,
but also thalamus, cerebellum, spinal cord,
peripheral nerve, and muscle.

9. Movement Disorder according to lesionMovement Disorder according to lesion
. Lesion in globus pallidus – athetosis. Lesion in globus pallidus – athetosis
. Lesion in the subthalamic nucleus –hemiballismus . Lesion in the subthalamic nucleus –hemiballismus

. Multiple small lesion in putamen – s.chorea. Multiple small lesion in putamen – s.chorea
. Lesion in caudate nucleus- huntington chorea. Lesion in caudate nucleus- huntington chorea
. Lesion in substantia nigra –parkinson’s disease. Lesion in substantia nigra –parkinson’s disease

10. MOVEMENT DISORDERS
PYRAMIDAL
SYMPTOMS
BASAL GANGLIA
DISORDERS
CERBELLAR
DISORDERS
SPASTICITY
ATAXIA
HYPOKINESIA
S
HYPERKINESIAS MOTOR-SENSORY
BEHAVIOUR
AKINESI
A
RIGIDITY
TREMOR
DYSTONI
A
MYOCLONU
S
CHOREA/
ATHETOSI
S
TICS/
STERIOTYPIE
S
COMPULSION
MANNERISM

11. Fernandez alvarez, 2005
684 patient< 18 years
•Tics - 43%
•Dystonia- 23%
•Tremor- 16%
•Myoclonus 6%
•Mixea- 4%
•Chorea- 3%
•Hypokinetic 3%

12. Biochemistry - neurotransmitters
Dopamine Acetylcholine
Serotonin Receptor Histamine
GABA Glutamate
Substance P
Hypokinetic - dopamine decreased - treat with dopamine
replacement or anticholinergic drugs
Hyperkinetic - dopamine increased and acetylcholine decreased
- treat with a dopamine antagonist or cholinergic drug

13. Definitions

14. Tics
•Spasmodic, involuntary, repetitive, stereotyped
movements that are nonrhythmic, often exacerbated
by stress
•May affect any group of muscle
•Classification-
–Transient tics of childhood ( < 1 year)
25 to 30% of children – Most common movement
abnormality of childhood
–Chronic tics (> 1 year)
–Tourette syndrome

15. Chorea
•“Dance” in Greek
•Irregular, rapid, uncontrolled, involuntary
movements
•Worsen on rest, but remain or improve with
voluntary movement
•Incorporated into semipurposeful acts to
modify the movement
•Tone - normal

16. Chorea-Contd..
•Causes-
•Parainfectious and autoimmune disorders-
–Syndenham’s chorea
–SLE
•Structural basal ganglia lesions-
–Vascular chorea in stroke
–Mass lesions

17. Chorea- causes Contd…
•Genetic-
–Huntington’s disease
–Ataxia telangiectasia
•Infectious chorea-
–HIV encephalopathy
–Cysticercosis
–Toxoplasmosis
–Diphtheria
–Scarlet fever
–Viral encephalitis( Mumps, measles, varicella)

18. Chorea- causes Contd…
•Metabolic or toxic encephalopathies-
–Hypo/ hypernatremia
–Hypocalcemia
–Hyperthyroidism
–Hypoparathyroidism
–Hepatic/ Renal failure
–Carbon monoxide, Manganese, mercury, OP
poisoning

19. Chorea- causes Contd…
•Drug induced chorea-
–Dopamine receptor blocking agents-
•Phenothiazines
–Antiparkinsonian drugs-
•L-dopa
•Dopamine agonists
•Anticholinergics
–Antiepileptic drugs-
•Phenytoin
•Carbamazepine

20. Chorea- causes Contd…
•Drug induced chorea-
–Dopamine receptor blocking agents-
•Phenothiazines
–Antiparkinsonian drugs-
•L-dopa
•Dopamine agonists
•Anticholinergics
–Antiepileptic drugs-
•Phenytoin
•Carbamazepine

21. Athetosis
•Distal writhing movements of extremities
•Choreoathetosis
•Also has rigidity
•Causes-
–Extrapyramidal CP- asphyxia, kernicterus or genetic
metabolic disorder like glutaric aciduria
–CP due to prematurity
–Post- infectious
–Cirulatory arrest for complex cardiac surgery
–Drugs like phenothiazines

22. Tremor
•Rhythmic oscillations of a part of the body around the central point
•Rest -
•Intention -
Causes-
. Physiological
•Essential tremor
•Drugs-
–Valproic acid
–Neuroleptics
–Caffeine
•Trauma- head injury
•Metabolic disorder
- hypoglycemia, thyrotoxicosis, neuroblastoma,
pheochromocytoma, Wilson disease

23. Dystonia
•Syndrome of sustained muscle contractions,
frequently causing twisting and repetitive
movements or abnormal postures
hallmark - simultaneous contraction of agonist and
antagonist muscle
•Focal
•Segmental
•Multifocal
•Hemi dystonia
•Generalized

24. Dystonia- Contd..
•Causes-
–Perinatal asphyxia
–Kernicterus
–Generalised primary dystonia
–Drugs
–Wilson disease- Dystonia most common
neurologic manifestation
.Segmental- genetic, idiopathic or overuse

25. Ballismus
•Form of chorea
•Movements more coarse and ballistic
•Hyper chorea
•Extremity flailing
•Causes
–Sydenham’s chorea
–Stroke
–Cerebral tumours and
–Trauma

26. Myoclonus
•Very brief, abrupt, involuntary, non-
suppressible, jerky contraction involving a
single muscle or muscle group- "shock like"
•Presence in normal (associated with sleep,
exercise, anxiety) and numerous pathologic
situations, both epileptic and nonepileptic
•Focal , segmental or generalized

27. Myoclonus
•Causes-
–Physiologic
–Benign nocturnal myoclonus
–Benign myoclonus of infancy
–Essential myoclonus
–Epileptic myoclonus( Juvenile myoclonic epilepsy)
–Opsoclonus- myoclonus
–Post CNS injury
–Basal ganglia disorders
–Drug induced

28. Myoclonus
•Causes-
–Physiologic
–Benign nocturnal myoclonus
–Benign myoclonus of infancy
–Essential myoclonus
–Epileptic myoclonus( Juvenile myoclonic epilepsy)
–Opsoclonus- myoclonus
–Post CNS injury
–Basal ganglia disorders
–Drug induced

29. Ataxia
•Inability to make smooth, accurate and coordinated
movements
•Due to disorder of cerebellum,sensory pathway in posterior
column of spinal cord
-Generalised or
- primarily affect gait or hands and arms
. acute or chronic
Causes:
•Acute or Recurrent-
–Brain tumor
–Drugs like alcohol, thallium, anticonvulsants
–Postinfectious/ immune
–Trauma
–Vascular disorder

30. Ataxia- Contd..
•Chronic or Progressive Ataxia-
–Brain tumors
–Congenital malformations-
•Cerebellar aplasias
•Dandy- Walker malformation
•Chiari malformation
–Hereditary ataxias

31. Hypokinesia
•Parkinsonism : bradykinesia, rigidity, tremor
or abnormal posture
•Is rare in childhood
•Causes-
–Post head trauma
–Post encephalitis
–Genetic disorders- Juvenile Huntington chorea,
Wilson disease, ataxia telangiectasia

32. Approach

33. Key questions
•Is the pattern of movements normal or abnormal?
•Is the number of movements excessive or diminished?
•Is the movement paroxysmal (sudden onset and offset),
continual (repeated again and again), or continuous (without
stop)?
•Has the movement disorder changed over time?
•Do environmental stimuli or emotional states modulate the
movement disorder?

34. Key questions- Contd..
•Can the movements be suppressed voluntarily?
•Are there findings on the examination suggestive of
focal neurologic deficit or systemic disease?
•Is there a family history of a similar or related
condition?
•Does the movement disorder abate with sleep?

35. History
•Age at onset-
•full term neonate : jitteriness
Infant : myoclonus, athetosis, transient dystonia
Older child : chorea
•Sex-
female: Sydenham’s chorea, thryrotoxicosis
male : tics, tremors

36. History- Contd..
Onset/duration
Acute : Infection, trauma
Slowly progressive : Wilson’s disease,
Tourette syndrome, Parkinson’s disease,
Hungtington’s chorea

37. History- Contd..
•Type of movement-
•rapid jerky: chorea
•slow movement : athetosis
•sustained: dystonia
• Involvement of body parts:
• distal limb : athetosis
• all body parts : chorea
• hand : writer’cramp ,focal dystonia
•Presence of movements in sleep :
•seizure disorder
•nocturnal myoclonus

38. History- Contd..
•H/o waxing and waning: Tics
•Aggravated with stress: tremor, tics, Tourette synd
Generalised primary dystonia, Nocturnal
myoclonus, Syndenham’s chorea
•Relieving factors-
•Behavioral abnormalities: chorea, Parkinsons disease


•Diurnal variation- with sleep : nocturnal myoclonus

39. History- Contd..
•H/o fever : infective origin
•poisoning
•Associated with signs of hepatic failure-
Wilson disease

40. History- Contd..
•Sydenham chorea
- Associated with hypotonia, emotionalability
-Other features of rheumatic fever
• Joint pain , rashes : SLE
•Associated with presenile dementia-
Huntington disease

41. History- Contd..
•H/o heat intolerance, increased appetite with
weight loss, increased stool frequency,
palpitation- thyrotoxicosis
•Features of increased ICP- Brain tumors

42. History- Contd..
•H/o intake of drugs
•Perinatal history-
• Dystonia- Asphyxia, Jaundice
• Athetosis- Asphyxia, jaundice and prematurity
•Cardiac surgery- Choreoathetosis

43. History- Contd..
•Developmental history:
-delayed milestones
• Immunization history :
polio, diptheria , pertusis
• Family history- Huntington disease(AD)
Wilson’s disease
Essential tremor
•Consanguinuty

44. On Examination
•General look
unconscious – CNS infection
•Vitals :–
•Raised temp - Infective
•Pulse, BP, Pattern of respiration : ICSOL, CNS
infection, thyrotoxicosis
•Ant fontanel

45. •Icterus : Wilsons disease
•Eye :
•opsoclonusmyoclonus syndrome
•blepharospasm : tics
•K-F ring
•Mask like face (Parkinsonism)
• Rash: Meningococal, Oculocutaneous
Telangiectasias

46. •Foreshortened occiput- Chiari malformation
•Prominent occiput- Dandy Walker
malformation

47. •Syndenham chorea- Milkmaid’s grip
Choreic hand
Darting tongue
Pronator sign

48. CNS Examination
GCS
Speech- vocal tics, dysarthria- chorea
CRANIAL NERVE
3
rd
and 6
th
nerve palsy – raised ICT
Motor exam-
 dystonia, hypotonia
 rigidity, bradykinesia:Parkinsons disease
 exaggerated reflex : thyrotoxicosis
 ataxic gait : cerebellar lesion, ataxia telangiectasis
Tip toe walking-generalised primary dystonia

49. CNS examination. Contd…
•Presence of primitive reflexes- cerebral palsy
•Signs of meningeal irritation?
• any cerebellar signs?

50. Other Systems
•Musculoskeletal examinaion
-side of the body- hemichorea,
hemiballismus
- which joint/limb- ballismus, dystonia
- joint tenderness

• C.V.S: any murmurs
•Abdominal:
hepatosplenomegaly , ascites
•Thyroid

51. Investigations
* CBC leucocytosis : infective
raised ESR : SLE

* Biochemical: RFT, LFT,RBS ,Electrolytes

* Throat culture

•Imaging: CT SCAN , MRI
• USG, ECHO

52. Investigations
4)Electrophysiological studies
•EMG- dystonia
•EEG
•ECG
•5)Special tests
• Serological assay- ASO, antiDnase, ANA
antiphospholipid Ab
• Serum Cu/ceruloplasmin/24 hr urinary copper/ liver biopsy
• Test for metabolic disorder
• Toxins
• Selective absence of IgA- Ataxia telangiectasia
*Other testing for rare disease- based in symptoms and clinical
suspicion

53. Principle of Management
•Symptomatic treatment
•Treatment of the cause
•Counselling

54. Drug Treatment
•Dystonia :
–Diphenhydramine iv may reverse drug related
dystonia
–Trihexyphenidyl, carbamazepine levodopa,
bromocriptine, diazepam
– Botilinum toxin injection
– Deep brain stimulation for generalized dystonia
–A trial of L-DOPA is indicated in all cases of chronic
dystonia.

55. Drug Treatment
•Tics:
Haloperidol, clonidine
•Chorea:
Diazepam, valproic acid, phenothiazine,
haloperidol
•Tremor:
B blockers, anticholinergics

56. References
•Nelson Text book of pediatrics
•Ghai ,Essential Pediatrics
•Movement Disorders in Children -- Schlaggar and
Mink 24 (2) 39 -- Pediatrics in Review
•Clinical pediatric neurology, Gerald.M.Fenichel 3
rd

edition
•Pediatrics in Review Vol.24 No.2 February 2003

57. Thank you