CEREBRAL PALSY AND MENTAL RETARDATION AUTISM Flashcards

1
Q

Cerebral Palsy
• A disorder of ________ and ________ resulting from a (temporary or permanent?) (progressive or non-progressive?) defect or lesion of the
(Mature or immature?) brain.

• The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behaviour, and/or by a seizure disorder’.

A

movement ; posture

permanent ; non-progressive

immature

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2
Q

Aetiology of CP

Pre-natal, Perinatal, Postnatal –
• Commonest Causes
Nigeria and Developing Countries
– Severe ______________
– ______________
– Infections (Intrauterine and Perinatal)
– Metabolic (______________)

Developed Countries
– Extreme ______________
– Inborn Errors of Metabolism

A

Severe Perinatal Asphyxia

Kernicterus

(Hypoglycaemia

prematurity

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3
Q

Classification (CP)
• _________
• _________
• The _______________ Classification System (GMFCS)

A

Clinical

Anatomical

The Gross Motor Function Classification System (GMFCS)

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4
Q

Classification (CP)
• Clinical (_________ , __________ , extra-pyramidal [__________, __________ , __________] and __________).

• Anatomical (__________plegia, __________plegia, _____plegia and
_________plegia)

A

spastic, flaccid, extra-pyramidal [dystonic, ataxic, choreo-athetoid] and mixed).

monoplegia, hemiplegia, triplegia and
quadriplegia)

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5
Q

The Gross Motor Function Classification System (GMFCS) is a recently developed system which classifies children with CP by their age specific motor activity.
– It is based on the assessment of severity of CP in children 0-____
years of age based on their ______________ rather than their
___________ .
– The GMFCS describes the functional characteristics in five levels, from I to V, level I being the mildest.

A

12

functional abilities

limitations

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6
Q

Aetiology (MR) 1
Before or At Conception

• Inherited disorders (such as ___________, ________ disease, neurofibromatosis, hypo__________, and _________ syndrome)
• Chromosome abnormalities (such as _______________)

A

phenylketonuria

Tay-Sachs disease

hypothyroidism

Fragile X syndrome)

Down syndrome

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7
Q

Aetiology (MR) 1

During Pregnancy
• Severe maternal ____________
• Infections with HIV, cytomegalovirus, herpes simplex, toxoplasmosis, rubella virus
• Toxins (such as _______ and _______ )
• Drugs (such as phenytoin, valproate, isotretinoin, and cancer chemotherapy)
• Abnormal brain development (such as poro-encephalic cyst, _______________, and ___________)
• Pre-eclampsia and ________ births

A

malnutrition

alcohol ; lead

Grey matter heterotopia ; encephalocoele

multiple births

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8
Q

Aetiology (MR) 2
During Birth
• Insufficient ________ (_______)
• Extreme __________

A

oxygen (hypoxia)

prematurity

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9
Q

Aetiology (MR) 2

After Birth
• Brain _________ (such as meningitis and encephalitis)
• Severe _________
• _________ of the child
• Severe emotional neglect or abuse
• Toxins (such as lead and _________)
• Brain tumors and their treatments

A

infections ; head injury

Malnutrition ; mercury

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10
Q

A specific cause can only be identified in only about _______ third of
people with mild MR/ID and in _______ thirds of people with moderate to
profound MR/ID.

A

one

two

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11
Q

Classification (MR)
• In general an IQ of ______ or below indicates mental retardation.

A

70

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12
Q

The Developing Brain
• Critical Periods of Brain growth
– ________ – neural tube
– _______ – All the lobes and major divisions complete
– _______ post-natal – 2/3 adult size
– _________ age – 75% adult size
– _________ – 90% adult size

A

1 month
4th month
1 year post-natal
2 years age
5 years

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13
Q

Potential for Neurogenesis (peaks in _________) and Synaptogenesis (peaks by ________) continues throughout life.

A

utero

5 years

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14
Q

Normal Development

Maturation of the nervous system
underlies development and it progresses _____________ (head downward). It is also a smooth continuous process.

A

cephalo-caudally

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15
Q

Motor
• Primitive reflexes (disappear by 3-4 months)
• Neck control _______ months (earlier in African children)
• Sitting _______ months
• Rolls _______ months
• Crawls _______ months
• Stands with support _______ months
• Walks _______ months
• Climbs up and down stairs _______ months

A

• Sitting 5-6 months
• Rolls 7 months
• Crawls 7-8 months
• Stands with support 10 months
• Walks 12 months
• Climbs up and down stairs 20 months

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16
Q

Social
• Smiles _______ (reflex smiles from birth)
• Follows person with the eyes ____ months
• Turns head to person talking _____ months
• Shows toy ____ months
• Waves bye-bye ______ months
• Knows 2 body parts ______

A

Social
• Smiles 6 weeks (reflex smiles from birth)
• Follows person with the eyes 3 months
• Turns head to person talking 6 months
• Shows toy 8 months
• Waves bye-bye 10 months
• Knows 2 body parts 2 years

17
Q

Language
• Babbles ______ months
• Says mama/dada ______ months
• Understands “No” ______ months
• Babbled conversation ______ months
• Understands 6-20 words ______ months
• 2 word sentences ______ months

A

Language
• Babbles 6 months
• Says mama/dada 9-12 months
• Understands “No” 12 months
• Babbled conversation 18 months
• Understands 6-20 words 20 months
• 2 word sentences 24 months

18
Q

Intelligence is the ability to _________ and _______ to the _____________ of everyday life. It involves verbal ability and problem solving skills.

A

learn ; adapt

experience

19
Q

IQ = _____/_____ x 100

• Score of _____ is considered average.

A

IQ = MA/CA x 100

100

20
Q

Common intelligence tests used:
• ____________ Test
• ____________ Scales
• ____________ Developmental Scales
• ____________ Scales
• Griffiths Scales
• Raven’s Progressive Matrices

A

Stanford-Binet Test
• Weschler Scales
• Denver Developmental Scales
• Bayley Scales

21
Q

Quantification of milestone achievement can yield three types of abnormalities: _______,___________, and __________.

A

delay

dissociation

deviance.

22
Q

Delay means a significant __________ rate of development. It can be global or affect one stream of development. It is the commonest reason for referral.

A

sub-average

23
Q

• Dissociation is a state that exists when ????????.

A

one phase of development is out synchrony with the others

24
Q

Deviance refers to _________________
e.g. a child who walks without crawling.

A

non-sequential development

25
Approach to Management 2 • Investigations. No single investigation is appropriate. In places where facilities are available the following are advisable: – ___________ studies – _____________ of the brain (CT or MRI) – _____________ studies – Others include enzyme assays, tissue biopsies, toxicological screening, TORCHES screening.
– Chromosomal studies – Radiological imaging Metabolic studies
26
Most children with MR/ID do not develop symptoms that are noticeable until the ____________ period.
preschool
27
Autism (Boys or Girls?) are 3-4 times more affected than (boys or girls?).
Boys are 3-4 times more affected than girls.
28
Causes of AUTISM • Unknown. However it has been associated with: – Intrauterine infections (TORCHES) – ______- or _________ – Metabolic diseases (______ deficiency) – ______ encephalopathy – Intracranial infections – Brain tumours – Genetic problems (_______ syndrome) • Neuro-radiological and neuro-pathological studies show abnormalities in the limbic system and the cerebellum.
Micro- or Hydrocephalus Vitamin D ; Lead Fragile X syndrome
29
Clinical features of autism • Age of onset: 50% occur by the ______ year. • Language impairment: usually non-existent. If present is characterized by _________ and unintelligible jargon. • Affect and sociability: some are withdrawn while others are emotionally labile. • Play: lacks imagination and is characterized by purposeless, repetitive manipulation of toys (usually favourite ones). They may have stereotypic body movements (hand flapping). • Activity: insistence on following routines. • Seizures: 15-35% may be associated with seizures • Cognitive function: 75% are below average by standard IQ tests. Some may have exceptional talents (high- functioning).
2nd echolalia
30
What else might be associated with Autism? • _______ • _________ • Mental disability • __________ skills
ADHD • Seizures • Mental disability • Exceptional skills
31
Autism Spectrum Disorders • Autism (all criteria met) •_______________[PDDNOS] •_________ Syndrome •________ Syndrome •_______________________ [CDD]
Pervasive Developmental Disorder-not otherwise specified Asperger’s Rett’s Childhood Disintegrative Disorder
32
Asperger’s Syndrome (may have (mild or severe?) issues with social interaction and imaginative play communication is usually _________ and ___________ is normal or above average)
Mild unaffected; intelligence
33
Asperger’s Syndrome (may have mild issues with social interaction and imaginative play but communication is usually unaffected and intelligence is normal or above average) • Rett’s Syndrome (severe neurological form affects only girls with regression, small head size, hand regard) • Childhood Disintegrative Disorder [CDD] (severe developmental disorder)
34
Rett’s Syndrome (severe neurological form affects only (boys or girls?) with regression, _______ head size, hand regard)
Girls small
35
• Childhood Disintegrative Disorder [CDD] is a (mild or severe?) developmental disorder
Severe
36
Cure for autism??
There is NO cure for autism