2. Introduction
• A group of heterogenous, nonprogressive, clinical syndromes,
characterized by motor and postural dysfunction
• Range in severity, but all of them are due to abnormalities of the
developing brain
• Nonprogressive, but the appearance of neuropathologic lesions and
their clinical expression may change over time as the brain matures
3. Risk Factors
• Prematurity, along with its complications
• Hypoxic-ischemic encephalopathy
• Antenatal infection
• Multiple gestation
• IUGR in males
• Endocrine/metabolic causes
4. Clinical Features
• Abnormalities of motor activity and posture
• Voluntary movement:
• complex, coordinated, varied uncoordinated, stereotypical, limited
• Differ according to the pattern of neurologic involvement,
neuropathology and etiology
5. Classification
Spastic syndromes
• Features of upper motor neuron lesions (both positive and negative).
• A positive sign: an abnormality that leads to involuntary increase in
muscle activity
• A negative sign: insufficient muscle activity or insufficient control that
interferes with function
6. Classification
• Depends on the distribution of abnormal upper motor neuron signs
• Spastic diplegia: more prominent in the lower limbs
• Spastic hemiplegia: one side is involved
• Spastic quadriplegia: all limbs are affected, but the upper limbs may
be more involved than the lower limbs
• Highly associated with prematurity and neonatal infection
7. Classification
Dyskinetic syndromes
• Usually results from severe, acute perinatal asphyxia
• Kernicterus is another important cause
• Selective neuronal necrosis in the hippocampus, thalamus, basal
ganglia, reticular formation and Purkinje cells of the cerebellum
• Presentation: encephalopathy, lethargy, decreased spontaneous
movement, hypotonia, and suppressed primitive reflexes
8. Classification
• Divided according to the predominant type of abnormal movement
• Athetoid type: movements are athetoid, choreiform, or a combination
of both
• Dystonic type: dystonia, with repetitive, patterned, twisting and
sustained movements of the trunk and limbs, can be slow or rapid
• Compared to spastic syndromes, muscle tone in dyskinetic syndromes
is typically normal or hypotonic.
9. Classification
Ataxic Syndromes
• Variable presentations and etiologies
• Delayed motor milestones and language skills
• Ataxia usually improves with time
• Speech is slow, jerky and explosive
• A diagnosis of exclusion
10.
11. Diagnosis
• A progressive disorder should be excluded through thorough history
and physical examination
• Brain MRI: indicated to determine the location and extent of
structural lesions or associated congenital malformations
• Spinal MRI: indicated if there is any question about spinal cord
pathology
12. Treatment
• Preventive:
• MgSO4 IV administration to mothers in premature labor with birth imminent
before 32 weeks, although benefit is controversial
• Cooling term infants with hypoxic-ischemic encephalopathy to 33.3 C for 3
days, starting 6 hours of birth
• Curative:
• There is no cure for CP
• A multidisciplinary team is important to reduce the abnormalities of
movement and tone and to optimize normal psychomotor development
• Family educations Is important
13. Treatment
• Physical therapy, with assistant tools is often required
• Sometimes, neurosurgery and orthopedic surgery can help reduce
morbidity
• Intrathecal baclofen, delivered via an implanted pump showed
success in many children with severe spasticity
• Botulinum toxin injected into specific muscle groups for the
management of spasticity
• Deep brain stimulation for selected refractory patients
• Hyperbaric oxygen showed no improvement
14. Associated Disorders
• Developmental: intellectual disability (50%), epilepsy (25%),
behavioral disorders (25%), sleep disorders (20%)
• Functional: unable to walk (30%), unable to talk (25%), blind
(10%), deaf (4%)
• Physical: pain (75%), hip displacement (30%), bladder control
problems (25%), drooling (20%), requiring tube feeding (7%)
15. Neurologic Deterioration
• CP is static
• However, neurologic signs may change or worsen with increasing age
• Cervical spondylotic myelopathy resulting from exaggerated neck
flexion or extension
• Some individuals with evidence of static brain injury beginning at
birth developed movement disorders in adulthood, mechanism is
uncertain
16. Questions
• What is the most common presentation of CP?
1. Spastic diplegia
2. Spastic quadriplegia
3. Hemiplegia
4. Dyskinetic CP
5. Ataxic CP
17. Questions
• What is the most common presentation of CP?
1. Spastic diplegia
2. Spastic quadriplegia
3. Hemiplegia
4. Dyskinetic CP
5. Ataxic CP
18. Questions
• Commando crawl is a characteristic presentation of which of the
following types of CP?
1. Spastic diplegia
2. Spastic hemiplegia
3. Spastic quadriplegia
4. Ataxic CP
5. Dyskinetic CP
19. Questions
• Commando crawl is a characteristic presentation of which of the
following types of CP?
1. Spastic diplegia
2. Spastic hemiplegia
3. Spastic quadriplegia
4. Ataxic CP
5. Dyskinetic CP
20. Questions
• Which of the following results from an insult to extrapyramidal
structures of the brain?
1. Spastic diplegia
2. Spastic quadriplegia
3. Spastic hemiplegia
4. CP with scissoring posture
5. Dyskinetic CP
21. Questions
• Which of the following results from an insult to extrapyramidal
structures of the brain?
1. Spastic diplegia
2. Spastic quadriplegia
3. Spastic hemiplegia
4. CP with scissoring posture
5. Dyskinetic CP
22. Questions
• Which of the following is considered a preventive measure against
CP?
1. IV MgSO4 administration
2. Intrathecal baclofen pump
3. Physiotherapy
4. Orthosis
5. Botulinum toxin administration
23. Questions
• Which of the following is considered a preventive measure against
CP?
1. IV MgSO4 administration
2. Intrathecal baclofen pump
3. Physiotherapy
4. Orthosis
5. Botulinum toxin administration
24. Questions
• Which of the following non-motor disorder is most frequently seen in
patients with CP
1. Intellectual disability
2. Sleep disorders
3. Blindness
4. Deafness
5. Abnormal behavior
25. Questions
• Which of the following non-motor disorder is most frequently seen in
patients with CP
1. Intellectual disability
2. Sleep disorders
3. Blindness
4. Deafness
5. Abnormal behavior
Notes de l'éditeur
Notably intracerebral hemorrhage and periventricular leukomalacia
Spasticity: increased muscle tone, especially with the speed of passive muscle stretching and is greatest in antigravity muscle
Positive signs:
Spastic hypertonia
Hyperreflexia caused by hyperexcitability of the stretch reflex
Extensor plantar responses
Clonus
Negative signs:
Slow effortful voluntary movements
Impaired fine-motor function
Difficulty in isolating individual movements
Fatigability
Spastic diplegia: often noted when the affected infant begins to crawl, tends to drag the legs behind more as a rudder (commando crawl). In severe cases, applying a diaper is difficult because of the excessive adduction of the hips. When suspending the child by the axillae, a scissoring posture of the lower extremities is maintained.
Patients with spastic hemiplegia show hand preference at a very early age, the upper limbs are usually more involved than the leg and difficulty in hand manipulation is obvious by 1 year of age. Walkin is delayed and circumductive gait is apparent
Patients present with poor head control and marked head lag, develop rigidity and dystonia over several years.
Upper motor neuron symptoms are not present, but speech and feeding are affected as the lesions here are extrapyramidal
Athetosis — Athetosis consists of slow, smooth, writhing movements that involve distal muscles. There is dyssynergia (antagonistic action) of opposing muscle groups, such as flexion and extension or pronation and supination. Emotion, change in posture, or intended movement may accentuate or induce the abnormal movements. Athetosis is most apparent during reaching, as the fingers extend and abduct. Primitive reflexes often are retained. Oropharyngeal difficulties may result from facial grimacing.
Chorea — Chorea consists of rapid, irregular, unpredictable contractions of individual muscles or small muscle groups that involve the face, bulbar muscles, proximal extremities, and fingers and toes. Stress, excitement, or fever may exacerbate the chorea. In some cases, fever may result in ballismus, a form of severe, coarse chorea