IntroductionIntroductionQuestions and AnswersQuestions and Answers

Dr Janice Bothwell

Consultant Paediatrician

Belfast HSCT

ObjectivesObjectives

Role of Paediatrician in management of children with hemiplegia

Multidisciplinary team Some questions parents have when told

their child has a hemiplegia Other questions?

What is the role of the doctor ?What is the role of the doctor ?

A Paediatrician is a doctor who specialises in the care of children.

Neurodevelopmental Paediatricians specialise in the care of children with developmental disorders and/or medical conditions such as hemiplegia which can affect the development of skills in a growing child

Neurologists specialise in children who present with neurological conditions such as hemiplegia

Assessment processAssessment process

Unidisciplinary Multidisciplinary

Medical AssessmentMedical Assessment

History of pregnancy, birth, family history, significant illnesses, any injuries sustained, medications taken, developmental progress, educational history

Physical examination

- examination of heart, lungs, abdomen, central and peripheral nervous system, hearing and vision

- height and weight

Developmental AssessmentDevelopmental Assessment

Gross Motor Skills Fine Motor Skills Speech and Language – ability to both

speak and understand language Personal and social skills Cognitive ability

Child development teamChild development team

Doctor

Occupational therapist

Speech & Language therapist

Physiotherapist

Child

Social Worker

Dentist

InvestigationsInvestigations

Neuroimaging Blood investigations EEG Hearing tests Vision tests

Diagnosis - HemiplegiaDiagnosis - Hemiplegia

Congential Acquired

What questions do parents ask What questions do parents ask when their child is diagnosed when their child is diagnosed

with hemiplegia?with hemiplegia?

What does hemiplegia What does hemiplegia mean?mean?

Paralysis of one side of the body resulting from disease or injury to the motor centres of the brain.

My child has hemiplegia does My child has hemiplegia does this mean he/she has cerebral this mean he/she has cerebral palsy?palsy? Cerebral palsy is an umbrella term covering a

group of non-progressive but often changing motor-impairment syndromes secondary to anomalies or lesions in the brain

These anomalies or lesions can be prenatal/perinatal/postnatal in origin

Hemiplegic CP is classified as a unilateral motor disability

What is a Congenital What is a Congenital Hemiplegia?Hemiplegia?

A congenital hemiplegia is one which occurs as a result of a prenatal/perinatal or early postnatal problem (<28 days after birth).

What went wrong?What went wrong? Three main processes can occur, each occur at

different developmental stages during pregnancy/birth- cerebral malformations originating early in the pregnancy- periventricular lesions mainly arising during 24 – 34 weeks (either during pregnancy or after a preterm birth)- cortical infarctions occurring just before or around the time of a term birth

What does a normal brain look What does a normal brain look like?like?

What are cerebral What are cerebral malformations?malformations?

All malformations originate from early fetal life Neuronal migration disorder

- agyria or lissencephaly at early stage

- schizencephaly and pachygyria at later stage

- heterotopias and polymicrogyria at later stages (up to 30 weeks gestation)

What do these conditions look What do these conditions look like on MRI?like on MRI?

lissencephaly schizencephaly polymicrogyria

What are periventricular lesions?What are periventricular lesions?

During the period between 24th and 34th week of gestation the brain is very vulnerable to bleeding

As a result periventricular leukomalacia or periventricular haemorrhagic infarctions can develop which may result in a congenital hemiplegia

What does this look like on a What does this look like on a scan?scan?

How can this cause How can this cause hemiplegia?hemiplegia?

A simplified diagram to show how the corticspinal tracts can be affected by PVL and hydrocephalus

What are cortical infarctions?What are cortical infarctions?

Cortical infarctions occur around term and are caused by ischaemia (lack of oxygen and/or blood flow to part/parts of brain tissue).

This is actually the least common type

What does this look like?What does this look like?

My child has a congenital My child has a congenital hemiplegia – why were there no hemiplegia – why were there no signs when he/she was born?signs when he/she was born? Clinical signs of a hemiplegia may not be

evident until the child is old enough to use the affected limb.

The child may show signs such as being slower to crawl, pull to stand, walk or may develop hand dominance at a very early age.

What else can you tell me What else can you tell me about congenital hemiplegia?about congenital hemiplegia?

This affects about 4 babies per 10,000 Its more common in males 1.4:1 R sided hemiplegia is more common

What is an acquired What is an acquired hemiplegia?hemiplegia?

An acquired hemiplegia is a weakness which develops in a child who has previously been well– Acute– Chronic progressive

What is an acute hemiplegia?What is an acute hemiplegia?

This is a sudden onset of one sided weakness which can be associated with seizures

What causes an acute What causes an acute hemiplegia?hemiplegia?

An acute hemiplegia can have a number of causes.

A brief outline of some of the causes will be given

What is an alternating What is an alternating hemiplegia?hemiplegia?

This is a hemiplegia which appears and any time from birth to 54 months.

The initial signs are mild delay in your child’s development

This may be followed by abnormal positioning of your child’s limbs or weakness of one side and can be associated with jerking movements of the eyes

How long does the hemiplegia How long does the hemiplegia last for?last for?

In alternating hemiplegia the duration varies from minutes to days and the seriousness of symptoms can vary.

The side affected with weakness can alternate and the arm is usually more affected than the leg.

Hemiplegia is also noted to disappear with sleep and reappear on wakening

Will a child recover from Will a child recover from alternating hemiplegia?alternating hemiplegia?

After each episode the child may recover to a degree, but after multiple episodes the child may have learning difficulties and possibly lose some skills

Medications can be used with various degrees of success

My child has been diagnosed My child has been diagnosed as having a stroke why?as having a stroke why?

There are many reasons why a child will have a stroke which results in a hemiplegia.

These reasons can include clotting problems, heart disease, trauma, infections, vascular problems, tumours

What investigations will What investigations will doctors do to identify why?doctors do to identify why?

Investigations include multiple blood investigations, urine sampling, heart scans, brain scans

What do strokes look like on a What do strokes look like on a scan?scan?

Can infection cause Can infection cause hemiplegia?hemiplegia?

An acute hemiplegia can be caused by brain infections such as meningitis or encephalitis

Infection which causes hemiplegia is also usually associated with seizures

What causes progressive What causes progressive hemiplegias?hemiplegias?

Progressive hemiplegias can be caused by– AV malformations– Brain abscesses– Tumours– Demyelinating disease– Sturge Weber syndrome

What is Sturge Weber What is Sturge Weber syndrome?syndrome?

What are the signs of Sturge What are the signs of Sturge Weber syndrome?Weber syndrome?

Port wine stain on face (cutaneous angioma)

Epilepsy in 80% Hemiplegia evident in 50% affecting limbs

on side opposite to facial signs

We have a diagnosis of We have a diagnosis of hemiplegia what other hemiplegia what other problems may be present?problems may be present?

Children with hemiplegia may also have– Learning disability (1 in 5 children)– Epilepsy present in 25 to 33% of children– Vision: significant problems rare but squints

and refraction errors are common– Hearing most often normal– Speech is well preserved

Who should be involved with Who should be involved with my child after diagnosis?my child after diagnosis?

Child

Paediatrician Orthopaedic surgeon

Physiotherapist

Occupational therapist

Educational Psychologist

Neurologist

Voluntary agencies

How do we treat hemiplegia?How do we treat hemiplegia?

Main treatment aims are– Child will walk and aims of treatment are to

maximise efficiency and look of walking– The arm can be very often ignored by the child

so early awareness of its use and encourage use of this limb is important. This should be combined with improvement of efficiency and look

– Monitor spine

Is early treatment important?Is early treatment important?

Yes to ensure– Greater symmetry of posture and movement– Develop an awareness of affected side– Prevention of fixed deformity– Provide support for families

Are there any surgical or Are there any surgical or medical treatment available?medical treatment available?

Yes surgical treatments are offered by the orthopaedic surgeons to correct deformities or to improve limb function

Medical treatment includes treatment for spasticity (tight muscles) this includes baclofen, diazepam

Botulinum toxin now used as injection form to help spastic limbs

What other therapeutic options What other therapeutic options are there?are there?

Numerous – need to look at evidence base for effectiveness

Any other questions ????Any other questions ????