Term
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Definition
| An umbrella term for a group of disorders of development and posture. |
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Term
| What can cerebral palsy be attributed to? |
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Definition
| Non-progressive disturbance occurring in the foetal/infant brain. |
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Term
| What is the most common classification of cerebral palsy? |
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Definition
| Spastic cerbral palsy, 80-90%. |
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Term
| What pathophysiology is spastic cerebral palsy associated with? |
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Definition
| Damage to the cortex, therefore no inhibition of the reflexes causing hypereflexia. |
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Term
| Name the three different types of spastic cerebral palsy? |
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Definition
| Quadriplegic (10%)- all four limbs, diplegia (35%)-both legs more commonly than both arms and hemiplegia (40%)-one side of the body. |
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Term
| What percentage of cerebral palsy is extrapyramidal? |
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Definition
| 5-10%- associated with damage to the extrapyramidal system. |
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Term
| What are the specific causes of extrapyramidal cerebral palsy? |
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Definition
Acute neonatal hyperbilirubinaemia.
Acute neonatal hypoxic ischaemic injury |
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Term
| Describe dystonic cerebral palsy as a type of extrapyramidal cerebral palsy |
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Definition
| Dystonia - Abnormal sustained contraction of agonists & antagonists. This results in abnormal posture with strongest muscle group out competing. |
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Term
| Describe choreo-athetoid cerebral palsy as a type of extrapyramidal cerebral palsy |
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Definition
Atheoid- slow writhing movements
Chorea- Rapid sudden involuntary movements. |
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Term
| What percentage of cerebral palsy is ataxic? |
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Definition
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Term
| Describe ataxic cerebral palsy |
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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. |
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Term
| Describe hypotonic cerebral palsy |
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Definition
| Hypotonia, hyperreflexia and persistance of primary reflexes. |
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Term
| What is mixed cerebral palsy and why might this happen? |
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Definition
| Mixed picture of all types, due to damage to more than one part of the brain. |
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Term
| What co-morbidities can be present with cerebral palsy? |
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Definition
| Learning Difficulties (30%), Hearing Impairment (~25%), Visual Impairment (may be due to visual cortex damage or oculomotor process), Epilepsy |
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Term
| What are the pre-natal causes of cerebral palsy? |
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Definition
| Infection, Toxins, Drugs, Trauma, ↓Foetal movement |
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Term
| What are the perinatal causes of cerebral palsy? |
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Definition
| Asphyxia, Interventricular Haemorrhage |
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Term
| What ate the postnatal causes of cerebral palsy? |
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Definition
| Vascular Diseases (CVA), Infection, Trauma, Encephalopathy, Metabolic Disturbance e.g. ↑Bilirubin/Glycaemia |
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Term
| What are the prenatal suggestive factors of cerebral palsy? |
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Definition
| Infection, Toxins, Drugs, Trauma, ↓Foetal movement |
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Term
| What are the perinatal suggestive factors of cerebral palsy? |
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Definition
| Prematurity, Difficult birth, Abnormal tone, Post-birth pathology |
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Term
| What developmental signs might make you consider a diagnosis of cerebral palsy in infancy? |
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Definition
| Delayed motor development and missed milestones. |
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Term
| On examintaion, what would make you consider a diagnosis of cerebral palsy? |
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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) |
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Term
| What management options are available for cerebral palsy? |
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Definition
Therapy-physio, occupational and SALT.
Orthotics
Antispasmodics- Baclofen and Botulinum toxin.
Surgery- Orthapaedic and selective dorsal rhizotomy. |
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Term
| How does baclofen as an antispasmodic for cerebral palsy work? |
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Definition
| GABA antagonist (GABA inhibitory neurotransmitter), reduces spasticity with varying effect, PO or IV. |
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Term
| How does botulinum work as an antispasmodic in cerebral palsy? |
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Definition
| Blocks ACh release, reduced tone for 3-4 months post treatment. Administered via injection. |
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Term
| What is selective dorsal rhizotomy? |
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Definition
Cuts sensory fibres reducing muscle stimulus by reducing reflexes.
Eliminates spasticity but leaves the patient very weak. |
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Term
| What is the current prevalance of autistic spectrum disorders and how has this changed? Why? |
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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 |
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Term
| What are the associated risk factors of autistic spectrum disorders? |
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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%) |
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Term
| What are the classical clinical features of autistic triad? |
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Definition
| Impairment of social relationships, Impairment of Social Communication, and Impairment of Imagination. |
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Term
| How would impairment of social relationships present as a sign of autism? |
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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 |
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Term
| How would impairment of social communication present as a sign of autism? |
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Definition
Non-Verbal Communication
Verbal Communication - Use formal/pedantic spoken language, lacking in inflexion
Interpretation – Take things literally & have difficulty interpreting implied meanings |
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Term
| What characteristic signs of autism would you expect to see in childhood? |
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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 |
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Term
| How would impairment of imagination present as a sign of autism? |
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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 |
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Term
| How is the differential diagnosis of autism assessed? |
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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. |
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Term
| What semi-structured interview schedules and observation tools are used in the assessment of autism? |
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Definition
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Term
| What other variables should be considered in the diagnosis of autism? |
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Definition
Pregnancy and birth
Developmental milestones- in particular language. |
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Term
| In the presentation of autism, what is regression of milestones? |
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Definition
| Children have normal developmetn before regressing e.g stopping communication at 18-30 months or losing toilet training. |
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Term
| By what age does developmental delay usually become apparent in autism? |
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Definition
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Term
| What are the classic language signs of autism? |
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Definition
•Language Delay/Absence
•Echolalia - immediately or delayed
-‘Parroting’ of parents orders
-Repetition of chunks of dialogue from TV/film out of context |
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Term
| What are the classic social interaction signs of autism? |
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Definition
Absence of protodeclarative pointing
Failure to follow point of other
Lack of eye contact
Indifference to presence of parents or others |
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Term
| What are the classic environmental stimuli signs of autism? |
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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 |
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Term
| What are the classic play signs of autism? |
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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 |
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Term
| What are the classic movement signs of autism? |
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Definition
• Patterned, repetitive movements e.g.Hand Flapping - tends to occur when child excited
Tics & ‘choreoatheotic’ movements
Self injurious behaviours |
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Term
| When does aspergers and high functioning autism typically present? |
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Definition
| School age rather than pre school. |
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Term
| How does aspergers typically present in children? |
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Definition
IQ >70
Impairments in all 3 areas of Autistic triad
No delay in language |
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Term
| Hows does higher functioning autism classically present in children? |
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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 |
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Term
| What are the classic language and communication signs of both aspergers and higher functioning autism at school age? |
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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 |
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Term
| How might you describe the interests of a school age child with classic aspergers or a higher functioning autism presentation. |
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Definition
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Term
| What are the classic relationship signs of both aspergers and higher functioning autism at school age? |
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Definition
Social isolation
Difficulty in making friends (+/- lack of interest in doing so), this may lead to depression in teenage years. |
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Term
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Definition
| Diagnosis for those who present with subthreshold signs of autism or present with signs of autism after the age of 30 months. |
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Term
| What interventions are indicated in autistic spectrum disorders? |
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Definition
Family education & support
Language therapy
Behavioural therapy
Psychological treatment
Pharmacological treatment
Risperidone - Used for severe aggression- Atypical Antipsychotic
Diet |
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