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
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
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
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
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
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
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
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 3rd
edition
• Pediatrics in Review Vol.24 No.2 February 2003
Stereotypies are intermittent, involuntary, repetitive, purposeless, patterned movements that are usually rhythmic Transient tic disorder- Most common movement abnormality of childhhood, lasts for weeks to <yr, M>F. +ve family history, Chronic tic- motor tic lasting for more than year Gilles de la tourette synd- AD, Atleast one vocal tic, OCD and ADHD Motor tics can be classified further by speed and quality as clonic (abrupt and fast) or dystonic/tonic (slow and sustained). Simple motor tics include blinking, nose twitching, grimacing, neck jerking, shoulder elevation, sustained eye closure, gaze shifts, bruxism, and abdominal tensing. Simple vocal tics include sniffing, throat clearing, grunting, squeaking, humming, coughing, blowing, and sucking sounds. Complex tics appear more "purposeful" than simple tics and may include combinations of movements of multiple body parts. Examples are head shaking, trunk flexion, scratching, touching, finger tapping, hitting, jumping, kicking, and gestures (obscene gestures are termed copropraxia). Complex vocal tics can encompass spoken syllables words or phrases; shouting of obscenities or profanities (coprolalia); repetition of the words of others (echolalia); and repetition of the final syllable, word, or phrase of one’s own words (palillalia)
Hungtington’s disease- AD inheritance, expanded sequence of CAG repeats, Progressive chorea and presenile dementia
Tardive dyskinesia- facial movement, late onset,
Tardive dyskinesia- facial movement, late onset,
Rigidity- muscle stiffness through out the range of motion in both flexors and extensors due to dysfn in BG D/d- Spasticity- increased tone is velocity dependent due to UMN dysfn
Segmental- genetic form of torsion dystonia or idiopathic or acquired due to overuse Individuals who have dystonia often find that touching one part of the body relieves the dystonic spasms; this phenomenon is called a sensory trick or geste antagoniste . For example, rubbing the back of the hand may diminish writer’s cramp
Generalised primary dystonia- torsion dystonia or dystonia musculorum deformans- Genetic disorder, tiptoe walking, aggravated by stress
Opsoclonus- myoclonuc- conjugate eye movement, severe myoclonic jerking of head. Idiopathic, encephalitis, neuroblastoma
Opsoclonus- myoclonuc- conjugate eye movement, severe myoclonic jerking of head. Idiopathic, encephalitis, neuroblastoma
Thallium- used in home as pesticide Anticonvulsants like phenytoin Genetic disorder- Dominant recurrent ataxia, Episodic ataxia Type 1 and 2, Hartnup disease, MSUD, Pyruvate dehydrogenase disorder Postinfectious/ Immune- Varicella, Miller Fisher syndrome, Multiple sclerosis, Myoclonic encephalopathy/ Neuroblastoma Vascular disorder- Cerebellar hemorrhage, Kawasaki disease Ataxia telangiectasia- involves immunolgic, neurologic, endocronologic, hepatic and cutaneous abnormalities.
Juvenile idiopathic dystonia- sporadic or hereditary
Generalised primary dystonia- torsion dystonia or dystonia musculorum deformans- Genetic disorder, tiptoe walking, aggravated by stress
Rigidity- muscle stiffness through out the range of motion in both flexors and extensors due to dysfn in BG D/d- Spasticity- increased tone is velocity dependent due to UMN dysfn
Milkmaid grip- relaxing and tightening hand shake Choreic hand- Spooning of the extended hand by flexion at the wrist and extension of finger Darting tongue- Tongue cannot be protruded for longer than a few seconds Pronator sign- The arms and palms turn outward when held above the head