Paediatric Neurology Flashcards
What is the underlying cause of cerebral palsy?
An insult to the developing brain before 3 years of age
Most insults leading to cerebral palsy are thought to occur at what time period?
Antenatally
What is the most common presentation of cerebral palsy, generally?
Delayed motor development
What is the most common subtype of cerebral palsy, making up 75-80% of cases? What characterises this type?
Spastic- a persistent increase in muscle tone
Which subtype of cerebral palsy affects the basal ganglia, and is characterised by involuntary movements and variable tone?
Dyskinetic
Which subtype of cerebral palsy affects the cerebellum, and is characterised by unsteadiness and poor spatial awareness?
Ataxic
What term is used to describe spastic cerebral palsy affecting a) both legs, b) a unilateral arm and leg, and c) all four limbs?
a) diplegic, b) hemiplegic, and c) quadriplegic
How should a child with cerebral palsy be fed if they are deemed to be at high risk of aspiration?
NG tube or gastrostomy
What medications can be used to help reduce spasticity associated with cerebral palsy?
Botox injections or oral baclofen
What are some musculoskeletal complications that may occur as a result of cerebral palsy, especially in those bound to a wheelchair?
Scoliosis and hip dislocation
What is the most likely cause of death in a child with cerebral palsy?
Aspiration pneumonia
Which anatomical structures are affected in muscular dystrophy?
Lower motor neurones and muscles
How do children with neuromuscular diseases usually present?
Floppy weakness and delayed motor milestones
How are muscular dystrophies inherited?
X linked recessive
The defect that occurs in muscular dystrophy impairs the formation of what protein?
Dystrophin
Gower’s sign is seen in what neurological condition affecting children?
Duchenne’s muscular dystrophy
What is the typical age of onset of a) Duchenne’s and b) Becker’s muscular dystrophy?
a) 2-3 years, b) teenage years
What is the most important blood test to perform on children with suspected muscular dystrophy?
Creatinine kinase
What investigation is used to make a definitive diagnosis of muscular dystrophy?
Genetic testing
Most patients with Duchenne’s muscular dystrophy die in their mid twenties from what complication?
Respiratory failure
Most patients with Becker’s muscular dystrophy die in their forties from what complication?
Cardiac failure (often cardiomyopathy)
What orthopaedic complication is likely to be seen in children with muscular dystrophy, particularly Duchenne’s?
Scoliosis
Congenital anomalies of the CNS are the result of insults to the developing foetus at any time up to when in development?
20 weeks
What are the two most common congenital anomalies of the CNS?
Neural tube defects and hydrocephalus