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Week 222
Cerebral palsy and autism.
50
Medical
Undergraduate 2
02/05/2014

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Term
What is cerebral palsy?
Definition
An umbrella term for a group of disorders of development and posture.
Term
What can cerebral palsy be attributed to?
Definition
Non-progressive disturbance occurring in the foetal/infant brain.
Term
What is the most common classification of cerebral palsy?
Definition
Spastic cerbral palsy, 80-90%.
Term
What pathophysiology is spastic cerebral palsy associated with?
Definition
Damage to the cortex, therefore no inhibition of the reflexes causing hypereflexia.
Term
Name the three different types of spastic cerebral palsy?
Definition
Quadriplegic (10%)- all four limbs, diplegia (35%)-both legs more commonly than both arms and hemiplegia (40%)-one side of the body.
Term
What percentage of cerebral palsy is extrapyramidal?
Definition
5-10%- associated with damage to the extrapyramidal system.
Term
What are the specific causes of extrapyramidal cerebral palsy?
Definition
Acute neonatal hyperbilirubinaemia.
Acute neonatal hypoxic ischaemic injury
Term
Describe dystonic cerebral palsy as a type of extrapyramidal cerebral palsy
Definition
Dystonia - Abnormal sustained contraction of agonists & antagonists. This results in abnormal posture with strongest muscle group out competing.
Term
Describe choreo-athetoid cerebral palsy as a type of extrapyramidal cerebral palsy
Definition
Atheoid- slow writhing movements
Chorea- Rapid sudden involuntary movements.
Term
What percentage of cerebral palsy is ataxic?
Definition
5-10%
Term
Describe ataxic cerebral palsy
Definition
Damage to the cerebellum.
Ataxia- abnormality in the smooth approach to an object, wide amplitude corrections needed. This may therefore affect walking, speech and movements.
Term
Describe hypotonic cerebral palsy
Definition
Hypotonia, hyperreflexia and persistance of primary reflexes.
Term
What is mixed cerebral palsy and why might this happen?
Definition
Mixed picture of all types, due to damage to more than one part of the brain.
Term
What co-morbidities can be present with cerebral palsy?
Definition
Learning Difficulties (30%), Hearing Impairment (~25%), Visual Impairment (may be due to visual cortex damage or oculomotor process), Epilepsy
Term
What are the pre-natal causes of cerebral palsy?
Definition
Infection, Toxins, Drugs, Trauma, ↓Foetal movement
Term
What are the perinatal causes of cerebral palsy?
Definition
Asphyxia, Interventricular Haemorrhage
Term
What ate the postnatal causes of cerebral palsy?
Definition
Vascular Diseases (CVA), Infection, Trauma, Encephalopathy, Metabolic Disturbance e.g. ↑Bilirubin/Glycaemia
Term
What are the prenatal suggestive factors of cerebral palsy?
Definition
Infection, Toxins, Drugs, Trauma, ↓Foetal movement
Term
What are the perinatal suggestive factors of cerebral palsy?
Definition
Prematurity, Difficult birth, Abnormal tone, Post-birth pathology
Term
What developmental signs might make you consider a diagnosis of cerebral palsy in infancy?
Definition
Delayed motor development and missed milestones.
Term
On examintaion, what would make you consider a diagnosis of cerebral palsy?
Definition
Abnormal Tone
Athetosis/Chorea/Ataxia
Reflex Abnormalities:
Hyperreflexia
Persistence Of Primitive Reflexes e.g.:
•Morro Reflex (Startling on dropping backwards)
•Asymmetric Tonic Neck Reflexes (Turning head to left (actively or passively) causes extension of left limbs & flexion of right limbs +v-v)
Term
What management options are available for cerebral palsy?
Definition
Therapy-physio, occupational and SALT.
Orthotics
Antispasmodics- Baclofen and Botulinum toxin.
Surgery- Orthapaedic and selective dorsal rhizotomy.
Term
How does baclofen as an antispasmodic for cerebral palsy work?
Definition
GABA antagonist (GABA inhibitory neurotransmitter), reduces spasticity with varying effect, PO or IV.
Term
How does botulinum work as an antispasmodic in cerebral palsy?
Definition
Blocks ACh release, reduced tone for 3-4 months post treatment. Administered via injection.
Term
What is selective dorsal rhizotomy?
Definition
Cuts sensory fibres reducing muscle stimulus by reducing reflexes.
Eliminates spasticity but leaves the patient very weak.
Term
What is the current prevalance of autistic spectrum disorders and how has this changed? Why?
Definition
100 per 100,000 persons have an autistic spectrum disorder, 4:1 male:female ratio.
~4 per 100,000 classified as autistic in 1960s
Due to increased prevalence of autism, or increased diagnosis
Term
What are the associated risk factors of autistic spectrum disorders?
Definition
Genetic factors- 5-6% chance with older siblings diagnosed.
Elderly parenthood- both maternal and paternal.
Associated syndromes- <10% of cases
foetal alcohol syndrome, phenylketonuria, angelman syndrome.
Co-Ocurring disorders- ADHD (20-30%)
Term
What are the classical clinical features of autistic triad?
Definition
Impairment of social relationships, Impairment of Social Communication, and Impairment of Imagination.
Term
How would impairment of social relationships present as a sign of autism?
Definition
Social Isolation - Often content to be alone, Find making friends difficult
Lack Understanding Of Social Behavior
Find social cues difficult to read; lack of empathy
Inappropriate behavior – May cause offence & appear insensitive/egocentric
Term
How would impairment of social communication present as a sign of autism?
Definition
Non-Verbal Communication
Verbal Communication - Use formal/pedantic spoken language, lacking in inflexion
Interpretation – Take things literally & have difficulty interpreting implied meanings
Term
What characteristic signs of autism would you expect to see in childhood?
Definition
Echolalia: Child may repeat/mimic chunks of language out of context
Absence of Protodeclarative Pointing: Child doesn’t point to declare interest
Using Parents/Carers As Tools - Child may grab person’s hand and place it on an object to get person to do something
Term
How would impairment of imagination present as a sign of autism?
Definition
Preference for repetitive activities – e.g. ordering/lining up/stacking toys,
Attention drawn to trivial aspects of environment with lack of appreciation of whole
Devotion to routine – e.g. bedtime rituals, following exact routes
Devotion to specific topics – e.g. repetition of facts on certain topics
Term
How is the differential diagnosis of autism assessed?
Definition
The use of multi-disciplinary teams (e.g. Doctors, Psychologists, SALTs, OTs) and multiple sources (e.g. home, school, parents) along-side a semi-structured interview and observation tools.
Term
What semi-structured interview schedules and observation tools are used in the assessment of autism?
Definition
DISCO - 11
ADI
ADOS
Term
What other variables should be considered in the diagnosis of autism?
Definition
Pregnancy and birth
Developmental milestones- in particular language.
Term
In the presentation of autism, what is regression of milestones?
Definition
Children have normal developmetn before regressing e.g stopping communication at 18-30 months or losing toilet training.
Term
By what age does developmental delay usually become apparent in autism?
Definition
Before 30 months.
Term
What are the classic language signs of autism?
Definition
•Language Delay/Absence
•Echolalia - immediately or delayed
-‘Parroting’ of parents orders
-Repetition of chunks of dialogue from TV/film out of context
Term
What are the classic social interaction signs of autism?
Definition
Absence of protodeclarative pointing
Failure to follow point of other
Lack of eye contact
Indifference to presence of parents or others
Term
What are the classic environmental stimuli signs of autism?
Definition
Interest in moving geometric patterns
Inappropriate reactions to loud sounds e.g. incessant screaming, suggestion of pain
Abnormal responses to physical pain: no crying/running to parent
Term
What are the classic play signs of autism?
Definition
Absence of symbolic play (tea parties, playing with toy telephone)
Repetitive Play e.g. repeatedly tapping objects
Ordering objects or dropping from a particular height
Term
What are the classic movement signs of autism?
Definition
• Patterned, repetitive movements e.g.Hand Flapping - tends to occur when child excited
Tics & ‘choreoatheotic’ movements
Self injurious behaviours
Term
When does aspergers and high functioning autism typically present?
Definition
School age rather than pre school.
Term
How does aspergers typically present in children?
Definition
IQ >70
Impairments in all 3 areas of Autistic triad
No delay in language
Term
Hows does higher functioning autism classically present in children?
Definition
IQ >70
Impairments in all 3 areas of autistic triad
Delay in language acquirement +/- Abnormality in language functioning, delay may only be picked up in retrospect
Term
What are the classic language and communication signs of both aspergers and higher functioning autism at school age?
Definition
Limited use of gestures
Awkward body language
Absence of facial expression
Pedantic speech
Poor use of inflexion
Socially inappropriate & offensive communication
Literal interpretation of orders
Term
How might you describe the interests of a school age child with classic aspergers or a higher functioning autism presentation.
Definition
Peculiar and narrow.
Term
What are the classic relationship signs of both aspergers and higher functioning autism at school age?
Definition
Social isolation
Difficulty in making friends (+/- lack of interest in doing so), this may lead to depression in teenage years.
Term
Describe atypical autism
Definition
Diagnosis for those who present with subthreshold signs of autism or present with signs of autism after the age of 30 months.
Term
What interventions are indicated in autistic spectrum disorders?
Definition
Family education & support
Language therapy
Behavioural therapy
Psychological treatment
Pharmacological treatment
Risperidone - Used for severe aggression- Atypical Antipsychotic
Diet
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