INFORMATION FOR CANDIDATE:

A young father, James Miller, brings his 8 months old

boy, Sasha, for his vaccination which they missed at 6
months because his grandmother died and they had no
time to come to you earlier. James informs you that
Sasha has some problems with sitting up and that he
seems to be arching his back when held in his arms.
YOUR TASK IS TO:
Take a history
Perform an examination
Discuss your diagnosis and management with the
father

HOPC: The good candidate will greet and express some sympathy regarding
grandmothers death and then go on to history taking.
Pregnancy was a bit complicated by toxaemia with high blood pressure which was well
controlled with tablets. Sasha was born prematurely (4 weeks) and did not breath for 5
minutes. He required some resuscitation and was in the NICU for a few weeks. Mum was
able to breast feed. His development so far showed some gross motor delay (late lifting
head, rolling over and trying to sit up). Language and personal/social development o.k.
His legs seem to be quite stiff when held in fathers or mothers arms and they seem to
cross over each other (scissoring).
No other symptoms or notices signs of associated disorders:
Visual problems
Hearing deficits
Communication disorders
Epilepsy
Intellectual disability
learning difficulties
Perceptual problems
FHx.: unremarkable
SHx: first child of married couple, no problems.
EXAMINATION: child is at lower end of head circumference, growth and development
charts. Except for spasticity and increased deep tendon reflexes the physical examination
is normal.
INVESTIGATIONS: cranial MRI is done; it can detect abnormalities in most cases.
DIAGNOSIS: CEREBRAL PALSY
Cerebral palsy refers to nonprogressive syndromes characterized by spasticity, ataxia or
impaired voluntary movement or posture and resulting from prenatal developmental
malformations or perinatal / postnatal CNS damage. Syndromes manifest before age 5 yr.
Diagnosis is clinical.
CP syndromes occur in 0.1 to 0.2% of children and affect up to 15% of premature infants.
Etiology is multifactorial, and a specific cause is often hard to establish. Prematurity, in
utero disorders, neonatal encephalopathy, and kernicterus often contribute. Perinatal
factors (eg, perinatal asphyxia, stroke, CNS infections) probably cause 15 to 20% of
cases. Spastic diplegia after premature birth, spastic quadriparesis after perinatal asphyxia,
and athetoid and dystonic forms after perinatal asphyxia or kernicterus are examples of
types of CP. CNS trauma or a severe systemic disorder (eg, stroke, meningitis, sepsis,
dehydration) during early childhood may also cause a CP syndrome.
Symptoms and Signs:
Before a specific syndrome develops, symptoms include lagging motor development and
often persistent infantile reflex patterns, hyperreflexia, and altered muscle tone.
Categories: Syndromes are categorized mainly as one of the following, depending on
which parts of the CNS are malformed or damaged:
Spastic syndromes occur in > 70% of cases. Spasticity is a state of resistance to passive
range of motion; resistance increases with increasing speed of that motion. It is due to
upper motor neuron involvement and may mildly or severely affect motor function. These
syndromes may cause hemiplegia, quadriplegia, diplegia, or paraplegia. Usually, deep
tendon reflexes in affected limbs are increased, muscles are hypertonic, and voluntary

movements are weak and poorly coordinated. Joint contractures develop, and joints may
become misaligned. A scissors gait and toe walking are typical. In mild cases, impairment
may occur only during certain activities (eg, running). Corticobulbar impairment of oral,
lingual, and palatal movement, with consequent dysarthria or dysphagia, commonly
occurs with quadriplegia.
Athetoid or dyskinetic syndromes occur in about 20% of cases and result from basal
ganglia involvement. The syndromes are defined by slow, writhing, involuntary
movements of the proximal extremities and trunk (athetoid movements), often activated
by attempts at voluntary movement or by excitement. Abrupt, jerky, distal (choreic)
movements may also occur. Movements increase with emotional tension and disappear
during sleep. Dysarthria occurs and is often severe.
Ataxic syndromes occur in < 5% of cases and result from involvement of the cerebellum
or its pathways. Weakness, incoordination, and intention tremor cause unsteadiness, a
wide-based gait, and difficulty with rapid or fine movements.
Mixed syndromes are commonmost often, with spasticity and athetosis.
Associated findings: About 25% of patients, most often those with spasticity, have other
manifestations. Strabismus and other visual defects may occur. Children with athetosis
due to kernicterus commonly have nerve deafness and upward gaze paralysis. Many
children with spastic hemiplegia or paraplegia have normal intelligence; children with
spastic quadriplegia and mixed syndromes may have severe intellectual disability.
Prognosis
Most children survive to adulthood. Severe limitations in sucking and swallowing, which
may require feeding by gastrostomy tube, decrease likelihood of survival. The goal is for
children to develop maximal independence within the limits of their motor and associated
deficits; with appropriate management, many children, especially those with spastic
paraplegia or hemiplegia, can lead near-normal lives.
MANAGEMENT:
Treatment may include physical and occupational therapy, braces, drug therapy or
botulinum toxin injections, orthopedic surgery, intrathecal baclofen, or, in certain cases,
dorsal rhizotomy (severing spinal nerve roots).
Assistive devices
Physical therapy and occupational therapy for stretching, strengthening, and facilitating
good movement patterns are usually used first. Bracing, drug therapy, and surgery are
used to treat spasticity. Botulinum toxin may be injected into muscles to decrease their
uneven pull at joints and to prevent fixed contractures. Drugs such as baclofen,
benzodiazepines and sometimes may diminish spasticity. Intrathecal (via subcutaneous
pump and catheter) is the most effective treatment for severe spasticity. Orthopedic
surgery (eg, muscle-tendon release) may help reduce restricted joint motion or
misalignment. Selective dorsal rhizotomy may help a few children if spasticity affects
primarily the legs and if cognitive abilities are good.
When intellectual and physical limitations are not severe, children should attend
mainstream schools. However, some children require varying degrees of lifelong
supervision and assistance. Speech training or other forms of facilitated communication
may be required. Even severely affected children can benefit from training in activities of
daily living (eg, washing, dressing, feeding), which increases their independence and selfesteem and greatly reduces the burden for family members or other caregivers. Assistive
devices may increase mobility and communication, help maintain range of motion, and
help children and their caregivers with activities of daily living.

Parents of a child with chronic limitations need assistance and guidance in understanding
the child's status and potential and in dealing with their own feelings of guilt, anger,
denial, and sadness. Such children reach their maximal potential only with stable, sensible
parental care and the assistance of public and private agencies (eg, community health
agencies, vocational rehabilitation organizations, lay health organizations).
Management of commonly associated disabilities and health problems:
Hearing and vision assessment
Assessment of epilepsy
Cognitive assessment
Nutritional problems like obesity or underweight should be discussed with
dietitian!
Gastro-oesophageal reflux is common and would need attention
Constipation requires diet and/or laxatives
Aspiration and lung disease happen often
Psychological and social difficulties require attention