Neurology Flashcards
When might you see febrile convulsions in a child?
6 months to 5 years
FHx
How are febrile convulsions treated?
Paracetamol/ibuprofen
What are the differentials for febrile convulsions?
Rigors
Intracranial infections
What do infantile spasms look like on EEG?
Hyposarrythmia
What can infantile spasm cause?
Developmental delay
What is the most common cause of infantile spasm?
Tuberous sclerosis
What others things should you look for in infantile spasm?
Dysmorphism
Skin changes
How is infantile spasm treated?
Vigabatrin
Prednisolon
What do myoclonic jerks mean in children?
Often benign
No treatment
What is a reflex anoxic seizure?
Triggered by pain/strong emotion Results in decreased perfusion to brain ECG - ?arrhythmia EEG - normal Most will grow out of it
What is juvenile myoclonic epilepsy?
Myoclonic jerks
Absence seizures
Worse when tired
Treatment - sodium valproate NOT carbamazepine
How is status epilepticus treated?
ABCDE 5 mins - benzodiazepine 10 mins later - further benzodiazepine eg IV lorazepam Prepare PR paraldehyde Prep phenytoin Call for senior support 10 mins later - IV phenytoin
How do you take a neurological history and what questions should you ask?
From parent/carer (but don’t forget child)
If for funny turns need proper witness description
Thorough history helps plan examination
Family history (neurogenetic/neurometabolic)
Birth history - prenatal, perinatal and postnatal (25% referrals for developmental impairment)
Developmental milestones
- Need to know if there is developmental delay/developmental regression)
- Normal for one age may be abnormal for another age
- Reflection of maturation of child’s nervous system
- Delay and abnormal pattern are indicators of underlying neurological diseases
What are the key principles in neurodevelopment?
Motor development proceeds head to toe fashion
Primitive reflexes are normally present in the term infant and diminish over the next 4 to 6 months of life
Postural reflexes emerge at 3 to 8 months of life
Persistence of primitive reflexes and lack of development of postural reflexes are hallmark of UMN abnormality of infant
Why do we do head circumference in babies?
Accurate reflection of brain size and development Serial important so can plot them on graph Small head (microcephaly) or a large head (macrocephaly or hydrocephalus) can be key findings in explaining the neurological abnormalities of a child
What are the different types of abnormal head shape (sysnostosis)?
Trigonocephaly - fusion of metopic suture - pointed shaped forehead
Brachycephaly - fusion of coronal suture - flat back of head
Solichocephaly/scaphocephaly - fusion of sagittal suture - AP breadth of skull much bigger than lateral breadth - seen in extreme preterm
Plagiocephaly - unilateral premature closure of lambdoid and coronal sutures - flattening of sutures
Oxycephaly - fusion of coronal and lambdoidal sutures - tower skull
What is Gower’s sign?
Hip girdle weakness, when asked to rise from prone position patient uses hands to walk up legs to compensate for proximal lower leg extremity
What should you do in a general physical examination in neurology?
Somatic growth - measure height and weight, compare percentiles with head circumference
Skin search - stigmata of neurocutaneous syndromes eg cafe au lait spot, ash leaf macule, iris lisch nodule, port wine stain, adenoma sebaceum, neurofibroma
Dysmorphic features - look especially at the midface, face reflects the brain, anomalies of the midface often associated with underlying brain malformations
- Facial measurements can be done to determine these
- Williams syndrome, Angelman syndrome, Rett syndrome, Smith-Magenis syndrome
Eyes - can be difficult due to poor concentration, retina is window to brain, fundoscopy
What should you do in a neurological examination to make it easier for you and the child?
Stop look listen - Don't cooperate for standard neuro examination - Tailor to child's age - Learn more by hands off observation - Mental status, cranial nerve examination, co-ordination by watching spontaneous activity Make it a game - Engage curiosity and imagination - Less threatening and child more cooperative when toys used - Not much gained testing power and reflexes Save the worst for last - Undressing child - Looking at fundus - Using auroscope - Testing gag reflex (if essential) - Measuring head circumference Cranial nerves including eye movements
What eye movement problems can you see in children?
Paroxysmal tonic upgaze Opsoclonus myoclonus syndrome Tick disorders Nystagmus Horner's syndrome
What is paroxysmal tonic upgaze?
Initially described as benign phenomenon with negative investigations and eventual complete resolution of symptoms
Similar clinical picture may arise from structural brain lesions, channelopathies, neurotransmitter disorders, and epileptic seizures
CACNA1A related disorders - spectrum of episodic ataxia 2, hemiplegic migraine, benign paroxysmal torticollis of infancy, and paroxysmal vertigo
What is opsoclonus myoclonus syndrome?
Associated with neuroblastoma
Eyes flick in all different directions
Also get myoclonus
What should you do with someone with nystagmus and why?
CT scan to make sure no cerebellar lesion
What is palatal myoclonus?
Rhythmic involuntary jerking movement of soft palate and pharyngopalatine arch
Surprisingly little effect on swallowing
How is palatal myoclonus treated?
Drugs used - clonazepam, carbamazepine, baclofen, anticholinergics, tetrabenazine, valproic acid, phenytoin, lamotrigine
Botulinum toxin but caution as spread of toxin may cause dysphagia and other problems
What are 4 common neurological problems?
Seizures
Syncope
Movement disorders
Cerebral palsy
Name 3 common types of seizure in children
Atonic seizures
Absence seizures
GTCS
Frontal lobe seizures - can progress to tonic-clonic
Name 3 common types of movement disorders in children
Hyperekplexia Chorea Shuddering Sleep myoclonus Tremors Motor tics Motor stereotypy
What is hyperekplexia?
Tonic phase after shocking baby, baby will stop breathing, flex neck will abort attack, genetic condition
What is motor stereotypy associated with?
ASD
What is cerebral palsy?
Umbrella terms covering group of motor impairment syndromes secondary to non progressive lesions or anomalies in the brain arising in the early stages of development
In most cases anomaly occurs pre or perinatally
What nutrition concerns are there in children with disabilities?
10-50% severely impaired children may be undernourished
Growth retardation due to inadequate intake as a result of self-feeding inadequacy and/or oromotor coordination difficulties
On the whole, the more severe disability the more severe the feeding difficulties and growth retardation
Name 2 consequences of undernutrition?
Poor growth with reduced muscle strength
Poor circulation due to reduced activity
Increased susceptibility to infection
What can cause feeding difficulties?
Oral motor dysfunction
Disrupted sensitive period
Dystonia
- Extensor patterns, limiting oral movements becoming stereotyped and abnormal
- Tactile hypersensitivity may be greatest around mouth Postural deformity - control of head/trunk/compression abdomen
Drug treatment - baclofen/diazepam/tone/sedation/anticonvulsants/sedation, effect of appetite
Impaired hand function
Immobility
Vision impairment
Deafness
GORD - pain/irritability, Sandifer syndrome
When do stereotypies present?
< 2 years
What do stereotypies look like?
Fixed, identical, foreseeable Arm-hands, wavelike, fluttering, jiggling Rhythmic Intermittent, repeated, prolonged No pre-movement sensorimotor phenomena Triggered by excitement, stress Suppress by distraction, seldom conscious effort May be positive family history Rarely responsive to medication
When might tics develop?
6-7 years
What do tics look like?
Variable pattern Blinking, grimacing, warping, jerking Quick, sudden, aimless, but not rhythmic Intermittent, short, abrupt Pre-movement sensorimotor phenomena Triggered by excitement/stress Self-directed, short suppressibility Often positive family history Primary neuroleptic treatment
How would you manage a child presenting with DKA in shock?
ABCDE Consider intubation Blood gases 20ml/kg bolus of 0.9% NaCl over 10-15mins Manage in resus
What fluids would you give to a child presenting with DKA who is NOT in shock?
10ml/kg over 60mins
What fluids would you give someone with DKA over the next 48 hours?
0.9% NaCl
Dehydration correction over 48 hours minus bolus
Maintenance over 24 hours
Give insulin after fluids for an hour
How can you treat cerebral oedema?
3% NaCl
Name 3 differentials for a seizure
Febrile convulsion Epilepsy Meningoencephalitis Tumour Metabolic - hypoglycaemia Head injury NEAD
What can cause non-epileptic seizures?
Organic - infection, hypoglycaemia, tumour, trauma
Non-organic - NEAD
What is a febrile convulsion?
Seizure in a febrile child with the absence of CNS infection, metabolic imbalance, or other neurological cause
How are febrile convulsions classified?
Simple < 15 mins
Complex > 15 mins
Status > 30 mins
What are the potential risk factors of febrile convulsions?
FHx
Viral infection
What does a febrile convulsion look like?
Generalised tonic-clonic seizure lasting < 5 mins, rapid and full neurological recovery
What are the rules of 6 for febrile convulsions?
Usually occur in 6 months - 6 year olds
Occur in 6% of pre-school children
1/6 chance of recurrence after 1 episode of febrile convulsion
What education should you give to parents about seizures?
How to manage seizure
Call ambulance if seizure lasts > 5 mins
When to admit
Risk of epilepsy
When should you admit a child with seizures/
First seizure Seizure recurring within the same febrile illness Incomplete recovery after 1 hr Suspected serious infection < 18 months Anxious parent
What is the risk of epilepsy in children with febrile seizures?
2% risk
(1% in child w/o febrile seizures)
Complex seizures have higher risk
What is West syndrome/
Salaam attacks
Hypsarrhythmia on EEG
Developmental delay
What is childhood absence epilepsy?
5-20 second episode of arrest of movement and awareness - blankly starring into space
Can have automatism
No developmental delay but impaired learning due to multiple episodes during the day
Spontaneous remission in adolescent
What differentials can you have for seizures in childhood?
Myoclonic jerks during sleep in babies (normal)
Jittering - can be normal but check BM
Breath holding attacks - when child gets angry or frustrated they can hold their breath and go cyanotic and lose consciousness
Reflex anoxic seizures - pain, minor head trauma can trigger a vaso-vagal where the child gets a short episode of asystole or bradycardia and goes pale and faint and has seizure activity with spontaneous resolution
Name 3 viruses that can cause meningitis in children
Enteroviruses EBV CMV VZV Adenovirus Herpes simplex - from birth canal
What bacterias can cause meningitis in children?
< 3 months - listeria, E coli, GBS
> 3 months - N meningitidis, strep pneumo, HiB
What can cause meningitis in immunocompromised people?
Fungal - cryptococcus
How do you manage meningitis?
3rd gen cephalosporin IV
Resuscitate
Add amox in < 3 months to cover listeria
Report to PHE
What are the complications of meningitis?
Sepsis DIC SIADH Hydrocephalus Hearing loss - all children get hearing tests after 6 weeks LD
What is cerebral palsy?
Disorder of movement and posture
Due to non-progressive lesion of the motor pathways in the developing brain
First 2 years of life - after 2 years it’s termed ABI
What can cause cerebral palsy antenatally?
Congenital infections (TORCH) Radiation IVH Metabolic Ischaemic Periventricular leukomalacia
What can cause cerebral palsy intrapartum?
Birth asphyxia
What can cause cerebral palsy post-natally?
Neonate - IVH, meningitis, head trauma, meconium aspiration, hypogylcaemia, jaundice (kernicterus)
Infant - hydrocephalus, hypoglycaemia, head injury - NAI, CNS infection
What are the TORCH organisms?
Toxoplasmosis Other - VZV, parvovirus B19, syphilis, listeria Rubella CMV HSV
How is cerebral palsy classified?
Spastic/pyramidal - cortex UMN - tremors, hypertonicity, brisk reflexes, scissor gait
Dyskinetic/extrapyramidal - basal ganglia - involuntary movements - chorea, dystonia, athetosis
Ataxic - cerebellum - broad based gait
Mixed
What is the most common type of cerebral palsy?
Spastic/pyramidal
What do you get in spastic hemiplegic CP?
Tiptoe gait
Circumduction gait
Arm bent - hand spastic or floppy, often of little use
Other side almost completely normal
What do you get with diplegic/paraplegic spastic CP?
Both legs with slight involvement elsewhere
Upper body normal or with minor signs
Child may develop contractures of ankles and feet
What do you get with quadriplegia?
Child may develop contractures of ankles and feet
When walks arms, head, and mouth may twist strangely
Children 4 limbs affected often have such severe brain damage that they are never able to walk
Knees press together
Legs and feet turned inward
What happens in a dyskinetic baby?
Initially baby hypotonic with poor head control
Increased tone and dyskinetic movements develop
What is dystonia?
Involuntary, sustained contraction of opposing muscles leading to abnormal posture, twisting repetitive movements
What is athetosis?
Slow involuntary writhing movements
What is chorea?
Irregular, non-repetitive dance like movements
What non-motor features can you get in CP?
Epilepsy Swallowing problems - bulbar palsy GOR Hearing and visual - strabismus Communication problems Bladder/bowel problems Behaviour difficulties MSK - scoliosis, contractures, hip dislocation
How is CP diagnosed?
MRI
Chromosome testing
Metabolic workup
How is CP managed?
MDT!!
- Nutrition - faltering growth so may need NG/PEG
SALT - assess swallow
OT and PT - promote mobility and physical activity to prevent contractures
Adaptive equipment -walkers, splints, wheelchairs
Special needs education
Antimuscarinics to prevent drooling
Anti-reflux medication
Anti-spasmodics
Bisphosphonates - prevent osteoporosis
Surgery - achilles tendon release, fix scoliosis
