Pediatric

Cerebral palsy neurology

Non progressive disturbances in the developing fetal or infant brain. lecture
By Dr.
 Key Aspects Russul
 Cerebral palsy is a disorder of motor function with or without Feihan
abnormal posture.
 The clinical manifestations of the disorder may change over time,
but the causative lesion is static.
The incidence is 3.6/1000 with a male/female ratio of1.4/1.
The prevalence of CP has increased due to the enhanced survival of very
premature infants weighing <1,000 g, who go on to develop CP at a rate of
approximately 15/100.
Etiology: -
Most common cause of cp is prematurity and low birth weight not hypoxia
1) Prenatal: - Congenital infections like (CMV, toxoplasmosis and rubella),
cerebral agenesis, intra uterine bleeding, radiation, toxins, anoxia, genetic and
metabolic disorders).
2) Perinatal :-( cerebral anoxia, intra ventricular hemorrhage, birth trauma).
3) Postnatal :-( meningitis, encephalitis, hyper bilirubinemia, hypo glycaemia,
hyper natremia, subdural hematoma and toxins).

 Types according to physiological classification

 Spastic: 70%
-hemiplegia
-diplegia
-quadriplegia
 Dyskinetic
-chorathetiod
-dystonic
 Hypotonic
 Mixed
 FUNCTIONAL classification :
• Class I → no limitation of activity.
• Class II → slight – moderate limitation.
• Class III → moderate – severe limitation.
• Class IV → no useful physical activity.

whereas the topographic taxonomy indicates the involved extremities.

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Spastic hemiplegia (25%): -
Cause: Stroke: in utero or neonatal due to Thrombophilic disorders,
Infection, Genetic/developmental, Periventricular hemorrhagic infarction
C.F.:
-Decreased spontaneous movement on the effected side and show hand
preference at a very early age.
- Circumduction gait, dystonia on running, growth arrest of affected side.
Equina varus, walking on tip to, ankle clonus, increases DTR.
-About one third of patients have a seizure disorder that usually develops in
first year or 2nd.
-25% have cognitive abnormalities.
Dx. A CT scan or MRI study may show an atrophic cerebral hemisphere with a
dilated lateral ventricle contra lateral to the side of affected extremities.

Spastic diplegia (35%): - bilateral spasticity of the legs

Cause: is strongly associated with damage to the immature white matter
(prematurity).
C.F.
-In sever spasticity difficult application of diaper because of excessive
adduction of the hips. - Scissoring posture of lower extremities during vertical
suspension.
-Commando crawls.
-Seizure in minority of cases, the prognosis for normal intellectual
development is good.
The most common neuropathological finding in children with spastic Diplegia
is PVL (periventricular leukomalacia), Periventricular cysts or scars in white
matter, enlargement of ventricles, which is visualized on MRI in more than
70% of cases.

Spastic quadriplegia (20%): -cause hypoxic ischemic encephalopathy

Is the most severe form of CP because of marked motor impairment of all
extremities and the high association with intellectual disability and \seizures.
Swallowing difficulties are common as a result of supranuclear bulbar palsies.
The most common lesions seen on pathologic examination or on MRI scanning
are severe PVL and Multicystic cortical encephalomalacia.

Athetoid (choreoathetoid, extrapyramidal, or dyskinetic) (15%): -

Affected infants are characteristically hypotonic with poor head control and
marked head lag and develop variably increased tone with rigidity and dystonia
over several years.

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Unlike spastic diplegia, the upper extremities are generally more affected than
the lower extremities in extrapyramidal CP.
Feeding may be difficult, and tongue thrust and drooling may be prominent.
Speech is typically affected because the oropharyngeal muscles are involved.
Speech may be absent or sentences are slurred.
Generally, upper motor neuron signs are not present, seizures are uncommon,
and intellect is preserved in many patients.
dyskinetic CP is the type most likely to be associated with birth asphyxia.
Athetoid CP can also be caused by kernicterus secondary to high levels of
bilirubin, and in this case the MRI scan shows lesions in the globuspallidus
bilaterally.
Extrapyramidal CP can also be associated with lesions in the basal ganglia and
thalamus caused by metabolic genetic disorders such as mitochondrial
disorders and glutaric aciduria.

Diagnosis of CP:
-History (also review pregnancy and delivery records).
-Physical exam (including thorough neurologic exam).
-EEG, CT scan, MRI.
-Tests for hearing and visual functions.
-Genetic evaluation in patients with congenital malformations or evidence of
metabolic disorder.
Management
Multidisciplinary approach
1) Pediatrician: co-ordinating role
2) Physiotherapist.is involve with the child from an early stage, help in
preventing severe contractures and deformity.
3) Occupational therapist. Is involved in adapting the environment to aid
the child functioning. Use of adaptive equipment (motorized wheel
chair, special feeding device).
4) Communication therapist: is involved early, providing help with feeding
problem and later with speech
5) Surgery: for CDH. (Adductor tenotomy and psoas transfer and release),
rhizotomy procedure (division roots of spinal nerve) which used in
severe spastic diplegia, tenotomy for Achilles’ tendon.
6) Care for lower urinary tract dysfunctions.
7) Drugs
a. Treatment of severe spasticity by: oral diazepam, oral baclofen,
dantrolene, intrathecal baclofen, and botulinum toxin injected into specific
muscle groups.

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 b. Botulinum toxin could be injected into salivary glands to reduce the
severity of drooling that is seen in 10-30% of patients with CP.
c. reserpine or tetrabenazine can be useful for hyperkinetic movement
disorders including athetosis or chorea.
d. treatment of dystonia by small doses of levodopa. Artane and
Carbamazepine is sometimes useful
e. Treatment of seizures by anticonvulsant.
8) Hearing aids.
9) Special education.

Prevention: -
1) Good pre natal care.
2) Prevention of kernicterus.
3) Care for LBW infants.
4) Treatment of apneic episodes.
5) Prenatal treatment of the mothers in premature labour with magnesium
sulfate.
6) several large trials have shown that cooling term infants with hypoxic-
ischemic encephalopathy to 33.3°C for 3 days, starting within 6 hr of
birth, reduces the risk of the dyskinetic or spastic quadriplegia form of
CP.

Prognosis: - depend on:

1) Severity of the condition.
2) Associated intellectual defect.
3) Adequate education and available facilities.
4) Centers that treat these conditions.
5) Type of C.P.

Ataxias
Ataxia is the inability to make coordinated movements, usually due to a
disorder of the cerebellum and/ or sensory pathways in the posterior columns
of the spinal cord. Ataxias may be generalized or primarily affect gait or the
hands and arms. They may be acute or chronic.
Causes of acute or recurrent ataxia
1- Brain tumors.
2- Drugs: piperazine, phenytoin, alcohol.
3- Encephalitis (brain stem).
4- Migraine: Basilar, and Benign paroxysmal vertigo.
5- Post infectious/ immune:

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 a- Acute cerebellar ataxia (ACA): occurs primarily in children between
1-3 years of age and is a diagnosis by exclusion. It often follows a
viral infection like (varicella, coxsackie virus, or echo virus) infection
by 2-3 weeks.
b- Cerebellar abscess.
c- Acute labyrinthitis.
6- Trauma.
7- Vascular disorders: Cerebellar hemorrhage, and Kawasaki disease.
8- Pseudo ataxia (Epileptic).
9- Genetic disorders: (Maple syrup disease, and Pyruvate dehydrogenase
deficiency).
Causes of Chronic or Progressive Ataxia
1- Brain tumors: Cerebellar tumors, Medulloblastoma, Ependymoma,
Supra tentorial tumors, Neuroblasoma.
2- Congenital malformation: Dandy-Walker malformation, Chiare
malformation.
3- Hereditary ataxia: Abetalipoproteinemia, Ataxia- telangectasia,
Friedreich ataxia.