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Cerebral palsy
Definition:- is a diagnostic term used to describe a group of permanent disorders of
movement and posture causing activity limitation, that are attributed to nonprogressive
disturbances in the in the developing fetal or infant brain. some of the neurologic features of
CP, such as movement disorders and orthopedic complications including scoliosis and hip
dislocation, can change or progress over time
It is often associated with epilepsy
,
abnormalities of speech , vision , intellect, hearing and dental defect. Many children and
adults with CP have no any sign of cognitive dysfunction. the incidence is 3.6/1000 with a
male/female ratio of 1.4/1. The prevalence of CP has increased somewhat 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:1) Pre natal :- 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 biluribinemia , hypo glycemia , hyper
natremia , subdural hematoma and toxins ).
Classification :1) Physiological: identifies the major motor abnormality (spastic , rigid , athetoid , a taxic , a
tonic , tremor , mixed , unclassified ).
2) Topographical : indicates the involved extremities (monoplegia , paraplegia , triplegia ,
quadriplegia , hemiplegia (arms > leg ) , double hemiplegie , diplegia ( legs > arms).
3) Functional
a) Class I →
no limitation of activity.
B) Class II →
slight – moderate limitation .
c) Class III → moderate – severe limitation.
d) Class IV →
no useful physical activity.
C.F :- it depend on the mode of presentation and the associated defect .
Spastic hemiplegia (25%) :- Decreased spontaneous movement on the effected side and
show hand preference at a very early age, delayed walking until ( 18 – 24 )m ,
circumductional gait, dystonia on running, growth arrest of affected side. Equina varus,
walking on tip to, ankle clonus, increase DTR, babinski
sign. About one third of patients have a seizure disorder that usually develops in first year or
two, 25% have cognitive abnormalities including MR. CT scan or MRI study may show
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an atrophic cerebral hemisphere with a dilated lateral ventricle contra lateral to the side of
affected extremities
Spastic diplegia (35%) :- Commando crawl, difficult application of diaper because of
excessive adduction of the hips, scissoring posture of lower extremities, it is the commonest
type and it is always congenital. Seizure in minority of cases, the prognosis for normal
intellectual development is excellent.
Spastic quadriplegia (20%) :- Most severe type, high association with seizure and MR,
bulbar palsy leading to swallowing difficulties and aspiration pneumonia. Children with
spastic quadriparesis often have evidence of athetosis and may be classified as having mixed
CP.
Athetoid (choreoathetoid, extrapyramidal, or dyskinetic ) C.P ( 15%) :- most likely to
be associated with birth asphyxia, and can also caused by kernicterus, characterized by
hypotonia with poor head control and marked head lag and develop increased tone with
rigidity and dystonia over several years, upper extremities are generally more affected than
the lower extremities, feeding difficulties and drooling may be prominent, athetoid
movement may not become evident until one year of age, speech typically affected, UMN
signs aren’t present. Seizure is uncommon and intellect is preserved in most patients.
D.D:- degenerative diseases, metabolic disorders, spinal cord tumor, or muscular dystrophy.
Diagnosis :- History, physical exam, MRI scan of the brain and spinal cord, tests for hearing
and visual functions, genetic evaluation should be considered in patients with congenital
malformations or evidence of metabolic disorders .
Complication :- Acquired dislocation of the hip, scoliosis, epilepsy, speech defect due to
high tone deafness or associated with MR, eye problems ( squint, nystagmus, blindness ),
dental problems ( tooth grinding, malocclusion, gingivitis ), hearing loss and MR.
Treatment :- In the ( C.P. clinic ) in which a team of physicians from different specialty as
well as occupational and physiotherapist, speech pathologist, social worker, educational, and
developmental psychologist.
The aim of the treatment is to make use of patient abilities as effectively as possible by :
1) Physiotherapy.
2) Occupational therapy.
3) Education of the parents of how to handle their child in daily activities.
4) Use of adaptive equipment ( motorized wheel chair , special feeding device ).
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.
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7) Treatment of sever spasticty by: oral diazepam , oral baclofen ,dantrolene, intrathecal
baclofen , and botulinum toxin injected into specific muscle groups. Botulism toxin
injected into salivary glands may also help reduce the severity of drooling, which is
seen in 10-30% of patients with CP
8) Treatment of dystonia by: small doses of levodopa . Artane (trihexyphenidyl) is
sometimes useful and can increase use of the upper extremities and vocalizations.
Carbamazapine may also be useful.
9) Reserpine
or tetrabenzine can be useful for hyperkinetic movement disorders
including athetosis or chorea.
10)Hearing aids.
11) Special education .
12) Treatment of seizures by anticonvulsant.
13 ) Enhancement of communication skills.
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 with magnesium
Prognosis:- depend on :
1) Severity of the condition.
2) Associated intellectual defect.
3) Adequate education and available facilities.
4) Centers that treats these conditions.
5) Type of C.P.
Ataxias
Ataxia is the inability to make smooth, accurate , and 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: 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.
Causes of floppy baby syndrome
1) Brain→ hypotonic C.P., Brain stem stroke, Brain stem encephalitis.
2) Spinal cord→ werdnig – hoffman disease, (hypotonia, a reflexia, tongue fasciculation
and repeated chest infection).
3) Peripheral nerve: Guillian – Barre disease, lead poisoning.
4) Neuromuscular junction : myasthenia gravis.
5) Muscles→ myopathy.
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