Neurology and development: Flashcards
List some common neurological disorders in children:
Brain - Cerebral palsy, epilepsy, stroke, migraine
Spine - Spina bifida, scoliosis
Nerves - Peripheral neuropathy syndrome
Muscles - Muscular dystrophies, myotonic dystrophies
When does a child typically start to show a hand preference?
Not until roughly 2y/o
What is loss of skills known as?
Developmental regression
What are the four main parts of a neurological examination?
- Gait - watch walking, tip-toe, heel-walking, running. Sit to stand (Gowers sign)
- Cerebellar - Gait, finger-nose, eye movements
- Cranial nerves - CN 1-12, facial movements, fundoscopy
- Peripheral - Tone, power, reflexes, sensation
What is Gowers sign?
When a child uses their arms completely to stand up with. Often a sign in muscular dystrophy
What nerve is responsible for Bells palsy
CN VII (facial paralysis)
Give 5 contraindications for a lumbar puncture:
- GCS <13
- ^ ICP
- Bleeding tendency
- Septicaemic shock
- Focal neurology
What is the commonest seizure in childhood?
Febrile convulsions
What age do febrile convulsions typically present?
3 months - 6 y/o
Describe the seizures in febrile convulsions:
- Seizures associated with fever
- Typically generalised
- Short duration
What is the most common cause of febrile convulsions?
Otitis media and tonsillitis. Febrile convulsions are typically caused by UTI, URTI or other infections
In febrile convulsions, is there CNS involvement?
No there is not usually any CNS involvement. Meningitis/meningococcal disease should however be excluded.
Outline the management of febrile seizures:
1) ABC (+DEFG -don’t ever forget glucose)
2) Symptomatic relief of high fever with anti-pyretics
3) Find a focus for the fever - Abx
4) Educate parents about prognosis/outcomes
When is a febrile seizure defined as ‘febrile status epilepticus’?
After 30mins. can lead to brain damage
What is the prognosis of Febrile seizures?
- Risk of recurrent febrile seizures 1/3
- Risk of epilepsy ^ 6 fold
List two other conditions which cause seizures which aren’t epilepsy?
- Breath holding attacks
- reflex anoxic seizures
What differentiates typical and atypical febrile seizures?
Typical: - Generalised tonic clonic - <15mins - DO NOT reoccur in 24hrs Atypical: - Focal seizures - >15mins duration - Recurrence in 24hrs - Incomplete recovery in 24hrs
What are breath holding attacks?
(common in 6month to 2 years) Pain or anger followed by brief crying, child takes a deep breath and stop breathing, turns BLUE and limbs extend. Then limp with LOC. Sometimes a few convulsive jerks; no post-ictal phase
What is a reflex anoxic seizure?
Equivalent to faint in older children. (child turns pale and falls to the floor)
triggers: minor injury, cold food, fright, fever
What causes the reflex anoxic seizures?
Vagal activation causes bradycardia
Define what an epileptic seizure is:
A transient clinical event that is the result of abnormal or excessive electrical activation of synchronised populations of neurones.
When is epilepsy diagnosed in children?
2 or more seizures
What is a generalised seizure?
Seizure whose initial presentation indicates more than minimal involvement of both cerebral hemispheres ( with impaired consciousness)
Give examples of generalised seizures:
- Tonic-clonic seizures
- Absence seizures
- Myoclonic seizures
- Atonic seizures (wear helmet)
What is a focal seizure?
A seizure whose initial presentation indicates activation of only part of one cerebral hemisphere (with or without loss of consciousness).
Give examples of focal seizures:
- Temporal lobe epilepsy
- Frontal seizures
- parietal lobe seizures
What is used to treat seizures in neonates? When is use of it contraindicated?
- Phenobarbitone
- Contraindicated in Absence seizures
What is absence epilepsy of childhood? How can it be precipitated? How long do they last?
- Transient loss of consciousness (up to 20 a day)
- Can be precipitated by hyperventilation
- Duration <30s
What EEG features are present in those with absent seizures?
- 3Hz spike
- Wave abnormality
What is the treatment for those with absent seizures?
Sodium valproate
What is the prognosis of absent epilepsy?
Most children ‘out grow’ it
If a child has infantile spasms, what differential might to enquire about?
Wests syndrome - onset of infantile spasm in 1st year of life
What else is Wests syndrome linked to?
Tuberosclerosis (examine skin)
What is the triad of Wests syndrome?
1) Infantile spasms (flexor spasms)
2) Developemental delay
3) Hyperarrythmia on EEG (chaotic pattern)
What is the Rx for those with infantile spasms?
- Treated with steroids or vigabatrin (anti-epileptic)
What is the prognosis for those with infantile seizures/Wests syndrome?
- Poor with long tem developmental and behavioural difficulties
- Most will go on to have other seizure types
What are the domains of a developmental assessment?
- Gross motor
- Fine motor
- Language
- Social/behavioural
Outline the key developmental milestones:
3 months - Smiles socially (6-8 weeks) (Social)
6 months - Transfers objects between hands (fine motor)
1 year - Pincer grasp (fine motor)
1.5 years - Walking (gross motor), hand preference
2 years - 2 word sentences (Language)
3 years - rides tricycle, jumps in puddles (gross motor)
What does hemiplegia cause?
On affected side:
- Weakness
- Increased tone and reflexes
What can cause cerebral palsy surrounding the birth?
- Prematurity
- Birth asphyxia
List 5 differentials for hypotonia:
- Downs
- Birth trauma
- Metabolic disorders
- Sepsis
- Hypoglycaemia
- Myotonic dystrophy
List 4 features present in a cerebral palsy presentation:
- Delayed motor development
- Evolving CNS signs
- Learning difficulties
- Epilepsy
Define cerebral palsy:
Non-progressive brain lesions that manifest as motor or postural abnormalities, the lesion can occur at any point from conception & 3 years of age
List 3 causes of CP:
Trauma
Infectious
Hypoxic-ischaemic encephalopathy
(or congenital malformations)
List the domains used to classify cerebral palsy:
1) Predominant type of tone or movement abnormality (e.g. spastic or dyskinetic)
2) Distribution pattern of affected limbs (e.g. hemiplegia, diplegia, duadriplegia)
3) Associated functional limitations (e.g. gross motor functional classification system (GMFCS 1-5) and manual ability classification system (MACS 1-5))
List 3 complications of cerebral palsy:
- Muscle spasms and contractures (muscles shorter than they should be)
- Feeding difficulties and nutritional problems
- Scoliosis
- Epilepsy
Outline the management of CP:
Multidisciplinary:
- Paeditrician - developmental assessment, epilepsy, muscle relaxants for spasticity
- Physiotherapy/occupational health (helps prevent spasticity and muscle contractures)
- Speech and language therapy - language difficulties
- Education - special needs teaching
Give a muscle relaxant which can be used for spasticity:
Baclofen
Give 2 signs of cerebral palsy:
- Scissoring gait
- Microcephaly
Give 4 investigations which can be performed in those with suspected CP and their results:
- USS: preterm babies ^ risk
- MRI: White matter lesions
- CT: structural abnormalities/haemorrhages
- EEG: Epilepsy
What is dyskinesia?
Involuntary convoluted writhering movements of fingers, arms, legs and neck. Athetosis (writhing movements).
What hereditary pattern is seen in Duchenne Muscular dystrophy?
X- linked recessive: therefore men affected.
Xp21 site on short arm of X-chromosome - codes for dystrophin which maintains the integrity of muscle cell wall.
Absence of dystrophin leads to progressive muscle cell damage.
When does Duchenne Muscular Dystrophy typically present?
4-5y/o
How do those with Duchennes Muscular Dystrophy typically present?
- Gross motor delay with ‘clumsiness’, waddling gait and proximal myopathy
- Reduced or absent reflexes though ankle jerk often preserved
- Gowers sign
- Calf pseudohypertrophy - muscle replaced with fat and fibrous tissue
What investigations are performed in someone with suspected Duchenne Muscular Dystrophy and what are their typical results?
- Raised Creatinine Kinase levels (CK)
- Genetic studies (Xp21 gene)
- EMG (myopathy pattern)
- Muscle biopsy (absent dystrophin on immunohistochemistry)
What is Duchennes prognosis like and what are any complications?
- Need for walking frame 8-10 and wheelchair aged 10-14
- Associated cardiomyopathy
- Respiratory problems
- Scoliosis
- Cognitive impairment
What is a common cause of death in those with Duchenne Muscular dystrophy?
Respiratory/heart failure in early 20’s.
