NEUROLOGY PAEDS Flashcards

Question

Breath holding spells: Types

Answer 1

Cyanotic breath holding spells | Pallid breath holding spells (aka anoxic seizures)

Answer 2

Involuntary episodes during which a child holds their breath, usually triggered by something upsetting or scaring them Typically occur between 6 and 18 months

Answer 3

Occur when a child is really upset, worked up and crying They become Cyanotic and lose consciousness Within 1 min they regain consciousness and start breathing They can be a bit tired and lethargic after an episode

Answer 4

Occur when a child is startled Vagus nerve sends strong signals to the heart that causes it to stop beating Child will suddenly go pale, lose consciousness and may start to have seizure like muscle twitching Within 30 seconds the heart restarts and the child becomes conscious again

Answer 5

Exclude other pathology Reassure parents There has been a link to iron deficiency anaemia - treating this can help

Answer 6

``` Tension headaches Migraines ENT infections Analgesic headache Problems with vision RICP Brain tumours Meningitis Encephalitis CO poisoning ```

Answer 7

``` Rest, fluids, low stimulus environment Paracetamol Ibuprofen Sumatriptan Antiemetics (e.g. domperidone) ```

Answer 8

Propranolol (avoid in asthma) Pizotifen (often causes drowsiness) Topiramate (teratogenic)

Answer 9

More common in children than adults Central abdominal pain lasting > 1 hour May be associated: N&V, Anorexia, headaches, pallor May predispose to head migraines

Answer 10

Antenatal - maternal infections, trauma during pregnancy Perinatal - Birth asphyxia, pre-term birth Post-natal - Meningitis, severe neonatal jaundice, head injury

Answer 11

SPASTIC - hypertonia and reduced function from damage to UMN DYSKINETIC - problems with controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. Result of damage to the basal ganglia ATAXIC - problems with coordinated movement resulting from damage to the cerebellum MIXED - mix of spastic, dyskinetic and/or ataxic features

Answer 12

Monoplegia - one limb affected Hemiplegia - one side of body affected Diplegia - four limbs are affected, but mostly the legs Quadriplegia - four limbs affected more severely, often with seizures, speech disturbance and other impairments

Answer 13

Hemiplegic / diplegic gait: indicated UMN lesion Broad based gait /ataxic gait: indicates cerebellar lesion High stepping gait: indicates foot drop or LMN lesion Waddling gait: Indicates pelvic muscle weakness due to myopathy Antalgic gait: indicates localised pain

Answer 14

``` Learning disability Epilepsy Kyphoscoliosis Muscle contractures Hearing and visual impairments Gastroenteritis-oesophageal reflux ```

Answer 15

MDT APPROACH: Physio, OT, SALT, Dieticians, orthopaedic surgeon, Paediatricans, social workers, support groups Surgery may be able to release contractures or lengthen tendons Muscle relaxants (e.g baaclofen) Anti-epileptics Glycopyrronium bromide (for excessive drooling)

Answer 16

Squint in otherwise healthy children are usually idiopathic Other causes: - hydrocephalus - cerebral palsy - space occupying lesions e.g. retinoblastoma - trauma

Answer 17

Shine pen torch at the patient from 1m away When they look at it observe the reflection of the light source on their cornea Reflection should be central and symmetrical Deviation from the centre —> squint

Answer 18

Cover one eye and ask the patient to focus on an object in front of them Move the cover across to the other eye and watch the movement of the previously covered eye If this eye moves inwards (means it had drifted outwards when covered) = EXTROPIA If it moves outwards (means it had drifted inwards when covered) = ESOTROPIA

Answer 19

Visual fields develop until the age of 8 years - so treatment needs to start before then Delayed treatment increases risk of the squint becoming permanent OCCLUSIVE PATCH. - can be used to cover the good eye and force the weaker eye to develop. ATROPINE DROPS - in the good eye - making it blurry Management coordinated by ophthalmologist Treat any underlying pathology

Answer 20

CSF build up within brain and spinal cord Either due to - over production of CSF - problem with draining or absorbing CSF

Answer 21

4 ventricles - 2 x lateral ventricles - third ventricle - fourth ventricle CSF is created in the 4 x choroid plexuses (1 in each ventricle) and by the walls of the ventricles CSF is absorbed into the venous system by the arachnoid granulations

Answer 22

CONGENITAL: - aqueductal stenosis —> insufficient drainage of CSF (the cerebral aqueduct that connects the third and fourth ventricles is stenosed - blocking normal flow of CSF out the 3rd ventricle —> build up in the lateral and third ventricles) - most common - Arachnoid cysts can block the flow of CSF if they area large enough - Arnold-chiari malformation (cerebellum herniates downwards through the foremen magnum) - chromosomal abnormalities and congenital malformations

Answer 23

Cranial bones don’t fuse at sutures until about 2 yrs Babies with hydrocephalus before this age present with enlarging head and rapidly increasing head circumference (occipito-frontal circumference) Other signs - budging anterior fontanelle - poor feeding and vomiting - poor tone - sleepiness

Answer 24

VP shunt can be placed to drain CSF from the ventricles to another body cavity (usually perionteal cavity) Valve helps to regulate the amount of CSF that drains from the ventricles

Answer 25

Infection Blockage XS drainage Intraventricular haemorrhage (during shunt related surgery) Outgrowing them (generally need replacing every 2 years as child grows)

Answer 26

When sutures close prematurely —> abnormal head shape and restriction to growth of brain

Answer 27

``` Will lead to raised intracranial pressure Developmental delay Cognitive impairment Vomiting Irritability Visual impairment Neurological symptoms and seizures ```

Answer 28

ABNORMAL HEAD SHAPE - Head shape depends on the affected cranial suture (Saggital synostosis / coronal synostosis / metopic synostosis / lambdoid synostosis ) Anterior frontanelle closure before 1 year of age Small head in proportion to body

Answer 29

1st line: skull X-ray 2nd line: CT head with bone views can be used to confirm diagnosis or exclude it if any double on X-ray Management: mild cases monitored and followed up over time. More severe cases require surgical reconstruction Prognosis usually good with proper management

Answer 30

Common conditions which cause abnormal head shapes in otherwise healthy babies Plagio- = translates to oblique/slanted. - Plagiocephaly refers to flattering of one area of baby’s head Brachy- = translates to short - Brachycephaly refers to flattering at the back of the head, resulting in a short head from front to back

Answer 31

Exclude: Craniosynostosis (palpate sutures) Check for congenital muscular torticollis (shortening of SCM muscle on one side) - may be the reason child always lies on one side of head Usually resolves as child grows Measures to avoid child lying on flatterened area - position on rounded side for sleep - supervised tummy time - using rolled towels or other props - minimising time in push chairs and car seats PHAGIOCEPHALY HELMETS - although have limitations and can cause skin problems and psychosocial problems. Not provided on NHS

Answer 32

MS is an umbrella term for genetic conditions that cause a gradual weakening and wasting of muscles

Answer 33

Duchennes muscular dystrophy (the main one) Others: beckers MD, myotonic MD, Facioscapulohumeral MD, oculopharyngeal MD, Limb-girdle MD, emery-Dreifuss MD

Answer 34

Children with proximal muscle weakness use a specific technique to stand up from a lying position = Gower’s sign They get onto their hands and knees then push their hips up and backwards like the “downward dog” pose They shift weight backwards and transfer their hands to their knees Whilst keeping legs quite straight they walk hands up their legs (They do this because the muscles around the penis are not strong enough to get their body erect without help of their arms)

Answer 35

No curative treatment MDT - OT, physio, medical appliances (e.g. wheelchairs) Surgical and medical management of complications e.g. spinal scoliosis and HF STEROIDS have been shown to slow progression of muscle weakness by as much as 2 years CREATINE supplementation can give slight improvement

Answer 36

X linked recessive Defective gene codes for dystrophin (a protein that holds muscles together at the cellular level) Females usually not affected as they have a spare copy of the gene

Answer 37

Rare autosomal recessive condition causing progressive loss of motor neurones, causing progressive muscular weakness Affects LMN in the spinal cord —> LMN signs

Answer 38

Fasiculations, reduced muscle bulk, reduced tone, reduced power, reduced/absent reflexes

Answer 39

SMA type 1: onset in first few months of life, usually progressing to death within 2 years SMA type 2: onset within first 18 months. Most never walk but survive into adulthood SMA type 3: onset AFTER the first year of life. Most walk without support, but subsequently lose that ability. Respiratory muscles less affected and life expectancy is close to normal. SMA type 4: onset in 20s. Most retain ability to walk short distances but require a wheelchair for mobility. Everyday tasks cause severe fatigue. Respiratory muscles and life expectancy not affected.

Answer 40

No cure Supportive and MDT approach Physio, respiratory support (e.g. non-invasive ventilation, Type 1 SMA may require tracheostomy and mechanical ventillation) PEG feeding if swallow unsafe

Answer 41

seen between the ages of 4-12 focal seizure, involving their face, drooling, and one side or one limb twitching (that can sometimes progress to secondary generalised seizure) Before or after bedtime. It is common for children to be sleep deprived. Good prognosis - stop by adolescence