‫كلية طب نينوى‬

6/ 6 /2021 ‫علي عادل الحيالي‬.‫د‬

‫أطفال‬ CNS )2( ‫محاضرة‬

Cerebral Palsy
Definition:
Is a diagnostic term used to describe a group of motor syndromes resulting
from nonprogressive disturbances in the developing fetal or infant brain.

 It indicate that the condition is non progressive, excluding conditions such

as cerebral tumours, the degenerative brain diseases.
 All children with C .P have suffered some form of brain damage and this
has involved the motor pathways.
 This does not mean that C.P is a static condition ,the clinical pattern
changes as brain maturation continues through out childhood resulting in
dynamic clinical pattern despite a static pathology.
 The term C.P differentiates such children from those with spinal palsy as
seen in the spina bifida complex, traumatic paraplegia or familial paraplegia
,etc.
 The child with damage to motor centers would be expected to have
damage to the other parts of brain (so epilepsy and abnormalities of
speech, vision, hearing, behavior, cognition, and intellect may be
associated with C.P).

Epidemiology:

 the incidence is 3.6/1000 live births.

 CP is the most common and costly form of chronic motor disability that
begins in childhood.

Etiology:

1) Prenatal causes : Congenital infections like (CMV , toxoplasmosis and

rubella ), chorioamnionitis, cerebral agenesis , radiation , toxins , anoxia
, genetic and metabolic disorders ).
2) Perinatal causes: hypoxic ischemic encephalopathy (HIE), intra
ventricular hemorrhage, prematurity , meconium aspiration, placental
abruption

1
 3) Postnatal causes : meningitis , encephalitis , hyper biluribinemia ,
hypoglycemia , subdural hematoma, intraparenchymal hemorrhage ,
toxins, cerebral infarction, prolonged ventilation

Classification of CP:

1) Physiologic classification: identifies the major motor abnormality.

2) Topographic classification: indicates the involved extremities.
3) Functional classification :
4) Etiologic classification:

Physiologic classification:

70-80% of c.p cases, It results from injury to the upper

1 Spastic C.P
motor neurons of the pyramidal tract
2 Choreoathetotic C.P Due to hyperbilirubinemia (it is rare now)
3 Mixed type 10%-15%of all cases
4 Ataxic C.P <5% of cases, results from cerebellar injury
5 Atonic C.P Marked hyoptonia ,floppy infant

Functional classification:

- Class I—no limitation of activity

- Class II—slight to moderate limitation
- Class III—moderate to great limitation
- Class IV—no useful physical activity

Topographic classification: indicates the involved extremities( usually for

spastic C.P)

2
 1 Spastic quadriplegia the 4 extremities about equally affected
2 Spastic diplegia legs involved more than arms
one side of the body involved (The arm is often more
3 Spastic hemiplegia
involved than the leg)
4 Monoplegia One limb affected (usually an arm), less common
5 Triplegia 3 limbs affected (usually both arms &1 leg), less common

Clinical presentation

Many children who develop cerebral palsy will have been identified as being at
risk in the neonatal period.

Early features of cerebral palsy are:

1. Delayed motor milestones.

2. Abnormal limb tone and limb and/or trunk posture in infancy.
3. May be accompanied by slowing of head growth
4. Feeding difficulties, with oromotor incoordination, slow feeding, gagging
and vomiting.
5. Abnormal crawling or gait once walking is achieved.
6. Asymmetric hand function before 12 months of age and hand preference at
a very early age.
7. Persistence of primitive reflexes.

Spastic quadriplegia (20%):

1. The most severe form of CP because of marked motor impairment of all

extremities and the high association with mental retardation and seizures.
2. Swallowing difficulties are common owing to supranuclear bulbar palsies,
and they often lead to aspiration pneumonia.
3. Neurologic examination shows increased tone and spasticity in all
extremities, decreased spontaneous movements, brisk reflexes, and plantar
extensor responses(UMNL signs).

3
4. Flexion contractures of the knees and elbows are often present by late
childhood.
5. Associated developmental disabilities, including speech and visual
abnormalities, are particularly prevalent in this group of children.
6. Children with spastic quadriparesis often have evidence of athetosis and
may be classified as having mixed CP.

Spastic diplegia(35%): Bilateral Spasticity of the legs(legs >arms)

1. The first indication of spastic diplegia is often noted when an affected infant
begins to crawl. The child uses the arms in a normal reciprocal fashion but
tends to drag the legs behind more as a rudder (commando crawl) rather
than using the normal four-limbed crawling movement.
2. If the Spasticity is severe, application of a diaper is difficult owing to
excessive adduction of the hips.
3. Walking is significantly delayed; and the child walks on tiptoes.
4. Neurologic examination shows spasticity in the legs with brisk reflexes,
ankle clonus, and a bilateral Babinski sign(UMNL signs) .When the child is
suspended by the axillae, a scissoring posture of the lower extremities is
maintained..
5. Severe spastic diplegia is characterized by disuse atrophy and impaired
growth of the lower extremities and by disproportionate growth with normal
development of the upper torso.
6. The prognosis for normal intellectual development is excellent for these
patients, and the likelihood of seizures is minimal

spastic hemiplegia(25%):
1. Decreased spontaneous movements on the affected side and show hand
preference at a very early age.
2. The arm is often more involved than the leg and difficulty in hand
manipulation is obvious by 1yr of age.
3. Walking is usually delayed until 18–24 mo, and a circumductive gait is
apparent.
4. Examination of the extremities may show:
- growth arrest, particularly in the hand and thumbnail
- U.M.N.L signs of affected extremities Spasticity is apparent in the
affected extremities, particularly the ankle, Ankle clonus and a Babinski

4
 sign may be present; the deep tendon reflexes are increased; and
weakness of the hand and foot dorsiflexors is evident
- walks on tiptoes because of the increased tone.
- the affected upper extremity assumes a dystonic posture when the child
runs.
5. 1/3 of patients with spastic hemiplegia have a seizure disorder that usually
develops during the first or second year .
6. Approximately 25% have cognitive abnormalities including mental
retardation.
7. A CT scan or MRI may show an atrophic cerebral hemisphere.

Athetoid CP(choreoathetoid or extrapyramidal CP):

1. Is relatively rare, especially since the advent of aggressive management of

hyperbilirubinemia and the prevention of kernicterus.
2. These infants are characteristically hypotonic and have poor head control
and marked head lag.
3. Feeding may be difficult, and tongue thrust and drooling may be prominent.
4. The athetoid movements may not become evident until 1 yr of age.
5. Speech is typically affected due to involvement of the oropharyngeal
muscles. Sentences are slurred .
6. upper motor neuron signs are not present and seizures are uncommon.
7. intellect is preserved in most patients.

Differential diagnosis of C.P:

 Degenerative brain diseases

 Metabolic disorders
 Spinal cord diseases & tumor.
 Muscular dystrophy

Diagnosis:
History, physical examination , CT scan, MRI of the brain and spinal
cord, EEG,tests for hearing and visual functions, genetic evaluation

5
should be considered in patients with congenital malformations or
evidence of metabolic disorders .
Treatment:
 The objectives of treating these children are to attain the highest possible
level of independent functioning within their family and community , to
prevent further complications and treat co morbid conditions.
 A team of physicians from various specialties as well as the occupational
and physical therapists, speech pathologist, social worker, educator, and
developmental are involved.
1) Physiotherapy: series of exercises designed to prevent the development
of contractures, especially a tight Achilles tendon.

2) Adaptive equipment:
Such as walkers, standing frames and motorized wheelchairs.
3) Treatment of spasticity:
a) Pharmacological : by drugs which will decrease tone and spasticity as
dantrolene sodium, benzodiazepines, baclofen , Botulinum toxin and
Intrathecal baclofen.
b) Surgical : by doing Selective dorsal rhizotomy , in which the roots of the
spinal nerves are divided.
4) Controlling Seizures with anticonvulsants.
5) Treatment of associated disabilities :
- If there is evidence of hip dislocation, consideration should be given
to performing surgical soft tissue procedures that reduce muscle
spasm around the hip girdle, including an adductor tenotomy or psoas
transfer and release
- consult with ophthalmologist for Strabismus, nystagmus, and optic
atrophy.
- Psychotherapy may be of value in some children especially those
with learning and attention deficit disorders and mental retardation .
- relieve feeding difficulties caused by dysphagia and
gastroesophageal reflux.

6
 Acute post-infectious polyneuropathy (Guillain-Barré syndrome)

Guillain-Barré syndrome is a postinfectious polyneuropathy involving mainly

motor but sometimes also sensory and autonomic nerves.

The paralysis usually follows a nonspecific viral infection by about 10 days.

rarely it may follow vaccination especially influenza.

The original infection may have caused only gastrointestinal ( especially

Campylobacter jejuni, but also Helicobacter pylori) or respiratory tract
(especially Mycoplasma pneumoniae) symptoms.

C.Jujeni accounts for 1/3 of GBS because of mimicry between gangliosides

and lipopolysaccharides of the bacteria.

Among the first symptoms are pain, numbness, paraesthesia or weakness in

the limbs.

Weakness begins usually in the lower extremities and progressively involves

the trunk, the upper limbs, and finally the bulbar muscles, a pattern known as
Landry ascending paralysis. Proximal and distal muscles are involved
relatively symmetrically, but asymmetry is found in 9% of patients.

Maximal weakness is reached within 2-4 weeks. Patient then have a plateau
phase of variable duration ranging from days to several weeks followed by
recovery phase of variable duration.

Bulbar involvement occurs in about half of cases leading to difficulty in

swallowing and aspiration .

Respiratory insufficiency may result. Dysphagia and facial weakness are often
impending signs of respiratory failure.

Urinary incontinence or retention of urine is a complication in about 20% of

cases but is usually transient.

Tendon reflexes are lost, usually early in the course, but are sometimes
preserved until later.

7
Autonomic dysfunction may present as:

- Cardiac dysrhythmias
- Hypotension or hypertension
- Paralytic ileus
- Bladder dysfunction
- Abnormal sweating

Differential diagnosis :
- Poliomyelitis(purely motor disorder with meningitis, asymmetric)
- Spinal cord Compression (asymmetric)
- Transverse myelitis(abrupt bilateral leg weakness with sensory level)
- Botulism( descending paralysis)
- Myasthenia gravis
- Acute disseminated encephalomyelitis (ADEM)
- Periodic paralyses and hypokalemia
- Traumatic Neuritis (onset of acute flaccid paralysis in lower limb occurs 1
hour to 5 days after injection in gluteal region.)

Laboratory findings and Diagnosis:

 The CSF protein is characteristically markedly raised, but this may not be
seen until the second week of illness.
 The CSF white cell count is not raised(Fewer than 10 white blood
cells/mm3 are found). (Cytoalbumin dissociation of CSF)
 Nerve conduction velocities are reduced.
 MRI of the brain and spinal cord may be indicated in some atypical cases.

TREATMENT:

 Patients in early stages of this acute disease should be admitted to the

hospital for observation because the ascending paralysis may rapidly
involve respiratory muscles during the next 24 hr.
 Patients with slowly progressive disease may simply be observed for
spontaneous remission with supportive therapy.
 Patients with rapidly progressive ascending paralysis should be admitted to
the ICU and treated with intravenous immunoglobulin (IVIG), (2 g/kg over 2
or 5 days).

8
 Children should admitted to the pediatric intensive care unit(PICU) if they
have one or more of the following :
1) Flaccid tetraparesis.
2) Severe rapidly progressive course.
3) Reduced vital capacity at or below 20 ml/kg.
4) Bulbar palsy with symptoms.
5) Autonomic cardiovascular instability that is persistent hypertension or
labile blood pressure or arrhythmias.
 Plasmapheresis, and/or immunosuppressive drugs are alternatives, if IVIG
is ineffective.
 Steroids are not effective.
 Supportive care, such as respiratory support and monitoring, prevention
of bedsores in children with flaccid tetraplegia, and treatment of secondary
bacterial infections is important.

PROGNOSIS

 The clinical course is usually benign, and spontaneous recovery begins

within 2–3 wk.
 Most patients regain full muscular strength, although some are left with
residual weakness.
 The tendon reflexes are usually the last function to recover.
Improvement usually follows a gradient inverse to the direction of
involvement, with recovery of bulbar function first and lower extremity
weakness resolving last.
 Bulbar and respiratory muscle involvement may lead to death if the
syndrome is not recognized and treated.
 Most who had slow recovery over the first 6 mo could eventually walk,
although some required years to recover.