Paed Neuro

Question 1

Core Neuro Topics

Answer 1

-Seizures
-Headaches
-Neuromuscular disorders
-Investigations (MRI/EEG etc)
-Cerebral palsy
-Tic disorder and co-morbidities
-Delayed development
-Genetic disorders

Question 2

History taking

Answer 2

-Consanguinity , parents age and health, Family history
-Pregnancy (folic acid, nutrition, infection, screening, scan, foetal movement)
-Delivery and complication
-Perinatal adaptations, feeding
-Early development
-Immunisations
-Early CNS infections/Brain injury
-School progress
-Mental health

Question 3

Paed Neuro Exam

Answer 3

-Age appropriate neurological and systemic examination, what is their baseline (neurodisability)
Hopping, walking on toes, developmental milestones
-Head circumference (if abnormal check parents OFC): growth charts
-Ophthalmological examination
-Neuro cutaneous markers (Neurofibromatosis: axillary freckling, Tuberous Sclerosis)
-Dysmorphic features/Syndromes

-Mental state and level of consciousness
-General
-Meningeal signs
-Cranial
-Motor system
-Cerebellar
-Autonomic

Question 4

Paed Neuro investigations

Answer 4

-Neuroimaging (CT/MRI/MRS )
-CSF studies (infection, inflammation, metabolic disorders)
-Genetic studies (microarray, genetic panel, specific genes)
-Metabolic (amino acid, organic acid, MPS)
-TFT, CK
-Neurophysiological (EEG, VEP, ERG, Nerve conduction, EMG)
-Hearing test

Question 5

Causes of fits in children

Answer 5

The commonest cause of fits in children is a ‘febrile fit’ or ‘febrile convulsion’. However other causes of fits must be excluded including metabolic causes (such as low glucose), head injury, and CNS infection. It is important to understand that in some of these instances the child will also have a high fever, but the fever was not the cause of their fit.

-Epilepsy (4/1000): primary and secondary (cerebral palsy, structural brain lesions, syndromes)
-Child abuse
-Encephalitis/ meningitis
-Poisoning
-Head injury
-Reflex anoxic seizure
-Metabolic (sodium, calcium, glucose)

Question 6

Differential diagnosis of epilepsy

Answer 6

-Syncope/faints
-Cyanotic(blue) breath-holding Attacks
-Pallid Syncopal Attacks(Reflex Anoxic)
-Delirium
-Night Terrors
-Migraine
-Cardiac Dysrhythmias: prolonged QT
-Narcolepsy

Question 7

Neurocutaneous syndromes

Answer 7

-Sturge-Webber
=Reddish discolouration of skin on one side of face

Question 8

Epilepsy syndromes

Answer 8

-Seizure type
-Age of onset
=New-born: benign familial neonatal convulsions/ pyridoxine BG dependent seizures
=Infancy: Infantile spasms
=Pre-school: Lennox-Gastaut
=School age: typical absence epilepsy
=Adolescence: juvenile myoclonic epilepsy

-Seizure type
-Neurodevelopmental findings (neurodevelopmental disorder)
-Family history
-Complex febrile seizures
-EEG(ictal AND interictal)

Question 9

Overview of Infantile spasms/ West’s syndrome

Answer 9

-Brief spasms beginning in the first few months of life

-P: flexion of head, trunk, limbs → extension of arms (Salaam attack); last 1-2 secs, repeat up to 50 times, progressive mental handicap

-I: EEG: hypsarrhythmia, usually secondary to serious neurological abnormality (e.g. tuberous sclerosis, encephalitis, birth asphyxia) or may be idiopathic

-M: possible treatments include vigabatrin and steroids, has a poor prognosis

Question 10

Overview of typical absence seizures (petit mal)

Answer 10

-Onset 4-8 yrs
-Duration few-30 secs; no warning, quick recovery; often many per day
-EEG: 3Hz generalized, symmetrical
-Sodium valproate, ethosuximide
-Good prognosis: 90-95% become seizure free in adolescence

Question 11

Overview of Lennox-Gastaut syndrome

Answer 11

-May be an extension of infantile spasms
-Onset 1-5 yrs
-P: atypical absences, falls, jerks, 90% moderate-severe mental handicap
-I: EEG: slow spike
-M: ketogenic diet may help

Question 12

Overview of Juvenile Myoclonic Epilepsy

Answer 12

-Typical onset is in the teenage years, more common in girls
-P: infrequent generalized seizures, often in morning//following sleep deprivation, daytime absences, sudden, shock-like myoclonic seizure (these may develop before seizures)
-M: usually good response to sodium valproate

Question 13

Overview of Benign Rolandic Epilepsy

Answer 13

-Benign Rolandic epilepsy is a form of childhood epilepsy that typically occurs between the age of 4 and 12 years.

-P: seizures characteristically occur at night, seizures are typically partial (e.g. paraesthesia affecting the face) but secondary generalisation may occur (i.e. parents may only report tonic-clonic movements), the child is otherwise normal

-I: EEG characteristically shows centrotemporal spikes

The prognosis is excellent, with seizures stopping by adolescence

Question 14

Overview of febrile convulsions

Answer 14

-Usually in toddlers, not usual for first after 3 years (3 months to 5 years): 3% children
-Usually a family history
=Simple 70%: <15 minutes, generalised, typically no recurrence within 24 hours, should be complete recovery within hour
=Complex 30%: 15-30 minutes, focal seizure, may have repeat seizures within 24 hours

-P: Usually stop before 5 minutes, usually generalised tonic-clonic, usually only occur early in a viral infection as temp rises rapidly

-I: ABCDE, glucose

-M:
=First seizure or complex: admitted to paediatrics
=If a child has a febrile convulsion you will need to find where the fever is coming from, and treat that illness. If it is fever due to a viral illness the treatment will be supportive.
=Treat a fit with anticonvulsant medication if it continues at 5 minutes; parents phone ambulance
=Regular antipyretics not shown to reduce chance o febrile seizure occurring
=If recurrent convulsions occur then benzodiazepine rescue medication may be considered (specialist advice, rectal diazepam/ buccal midazolam)

-Prognosis: 1/3 risk of further convulsion depending on age of onset <18 months, fever <39, shorter duration of fever before seizure, family history

-Complications
=Brain damage after 30 minutes (status epilepticus)
=Injuries/ death
=Educational/ psychosocial/ treatment related

Question 15

History of fits

Answer 15

-What were they doing when the fit started?
-How did the fit start?
-Was there loss of consciousness?
-Were bladder/bowels opened?
-What the movements were like, and in which parts of the body?
-Was there eye rolling or staring, eyes open or closed, eyelid flutter, jerking or deviation?
-Was there tongue biting/ facial twitching/ body stiffness/ chaotic or rhythmic jerking of limbs?
-What was the tone?
-What was the colour (pallor or cyanosis)?
-How long did it last?
-How did it stop? (was it self resolving?) Duration of recovery
-Were they sleepy afterwards (how long was this post-ictal phase?)
-Was there headache afterwards?
-Was there any injury sustained?

-After a description of the fit you need to ask about what happened before the fit:
=Was there a fever?
=Has the child been unwell recently?
=What is the past medical history?
=What is the birth history?
=Has the child been developing normally?
=Did the child complain of anything prior to the fit?
=Were they exercising? (cardiac syncope may come suddenly at rest, or during exercise, Wolff Parkinson White)
=Any warning signs? Awareness of surroundings, aura

-With fever
=Do not assume a ‘febrile convulsion’. Remember that CNS infections (meningitis and encephalitis) also cause fever and fits. Check for signs of meningism / bulging fontanelle. Check for personality or behaviour change.
=Even in the case of a febrile convulsion, remember to make sure that you find the cause of the fever and assess the child for serious bacterial infection. This includes a thorough examination and urine check at the least. Blood tests may be required.
=Children who have epilepsy may also have their fit threshold lowered by fevers. We do not call these febrile convulsions.

Established epilepsy: Fit pattern and fit nature

Question 16

Examples of non-seizure events

Answer 16

-Faints
-Cardiac syncope: rapid recovery once flat, not younger children

Question 17

Fit examination

Answer 17

-Generalised fits
=Tonic-clonic fits are the most common and involve whole body jerks (the tonic phase is the initial stiffening, the clonic phase is the jerking)
=The eyes are usually rolled upwards, the jaw is clenched and the child is unresponsive, salivation, sweating, blue: grand mal
=Petit mal (absence seizure)

-Focal fits
=The child is awake and the fit only affects a part of the body, partial seizure if aware
=Neuroimaging may be required for a first focal fit (CT scan)
=Sometimes a generalised fit starts as a focal fit.

-After fit
=Check Airway (obstructed by unconsciousness?), Breathing depressed from medication?), Circulation (Dehydration, sweating, sepsis) and Disability (watch for airway problems and slow breathing post-ictally)
=As part of D (disability) check the AVPU score (P/U is common initially post-ictally) and the blood glucose. Full neuro exam when awake
=Children may have a headache or be agitated or drowsy: paracetamol
=Examine for complications of the fit and the cause of the fit
=Check for urine infections after febrile fits

Question 18

Fit Investigation

Answer 18

-CT or MRI?
-1-1.5% of children with epilepsy have brain tumour (brain tumours RARELY present with seizures)
-Imaging indicated in :
=Focal seizures
=Neuro deficit or not responding to treatment
=Early onset

Question 19

Management of fits and epilepsy

Answer 19

-During fits
=Remain calm and place the child in the recovery position
=Check the time that the fit started
=Give facial oxygen (nasopharyngeal airway)
=Check the blood sugar and treat if low
=If the fit continues for 5 minutes it will need benzodiazepine drug treatment
=If you have IV access give lorazepam IV according to guidelines
=If IV access is unobtainable give buccal midazolam or rectal diazepam according to guidelines
=Watch out for respiratory depression after using these benzodiazepines
=Don’t forget to check the blood glucose

-Epilepsy
=Introduce anti-epileptic drugs slowly (7-14 days)
=SV/ Carbamazepine/ Lamotrigine/ Levetiracetam/ Phenytoin
=Lifestyle changes
=Epilepsy specialist nurse involvement
=Surgery, ketogenic diet, electrical stimulators

Question 20

Red flags in fits

Answer 20

-Fits in babies
-Aspiration
-Hypoglycaemia
-Status Epilepticus
-Pseudoseziures

Question 21

Fits terminology

Answer 21

-Grand mal, Petit mal
-Convulsion
-Seizure/ Epileptic seizure
-Epilepsy/Epilepsies
-General/Focal

Question 22

Fits in babies

Answer 22

-Are worrying and are sometimes difficult to identify. They may be subtle and involve eye deviation or lip smacking only.
-Often atypical
-Some go floppy, less violent jerking

Question 23

Aspiration in fits

Answer 23

-Vomitus can be aspirated during or after a fit.
-The recovery position helps to prevent this, throughout post-ictal phase
-Aspiration may be suspected if oxygen saturations are low or there is respiratory distress after a fit, admit to hospital

Question 24

Hypoglycaemia in fits

Answer 24

-Low glucose can cause fits and also jitteriness in small babies, can cause brain damage
-Glucose below 3.5 should be corrected with glucogel or IV dextrose
-Causes: systemically unwell, on insulin, congenital metabolic disease, alcohol

Question 25

Status Epilepticus in children

Answer 25

-Fit lasts ore than 30 minutes, no recovery in between (single seizure lasting >5 minutes or more than 1 within 5 minute period without recovery in between) -Airway adjunct, high flow O2, check glucose -Lorazepam 100 micrograms/kg PO//IO IV vs midazolam (buccal) or diazepam rectal if no IV access -Repeated one after 5-10 minutes then phenytoin/ levetiracetam 18mg/kg IV/IO over 20 minutes -RSI with thiopentone: If no response ('refractory status') within 45 minutes from onset, then the best way to achieve rapid control of seizure activity is induction of general anaesthesia or phenobarbital

Question 26

Pseudoseizures in children

Answer 26

-Children and young people occasionally present with pseudo seizures, but they are very difficult to tell apart from real seizures. (Rare), starts mid teens -P: pelvic thrusting, family member with epilepsy, female, crying after seizure, does not occur when lone, gradual onset WHEREAS true: tongue biting, raised serum prolactin -I: video telemetry -Diagnosis can be difficult and advice should be sought.

Question 27

Presentation and causes of headache

Answer 27

-Common, usually benign (50% 7 year olds, 80% 15 year olds. Equally common then girls at puberty) -Migraine without aura most common primary cause =A: >=5 attacks fulfilling B to D =B: Headache lasting 4-72 hours =C: At least 2: bilateral or unilateral (frontal/temporal) location, pulsating, moderate to severe, aggravated by routine physical activity =D: At least 1: N/V, photophobia and phonophobia -Tension 2nd most common =A: at least 10 previous headache episodes B to D =B: lasting 30 mins to 7 days =C: 2: pressing/ tightening (non/ pulsating), mild to mod, bilateral location, no aggravation by routine activity =D: Both: no N+V, photophobia or phonophobia -ENT/refractory errors -Triggers: dehydration, dietary, sleep, screen time, bullying, social -Red flag symptoms: Early morning headache, waking up from sleep with headaches, weight loss, ataxia

Question 28

Investigation of headache

Answer 28

-Detailed history: associated features, behaviour during headache (keeping still/ restless), frequency of analgesia, COCP, psychosocial history, triggers (hunger, snack, caffeine, screens) -Examination: cranial nerve palsy, papilloedema, ataxia, BP and HR, weight, BMI, OFC, skin, scalp, face, neck, oral, fundoscopy, pubertal status -None if no red flag symptoms -MRI brain scan (incidental findings) -Ophthalmology assessment

Question 29

Management of headache

Answer 29

-Treat the cause -Life style changes -Analgesia/Sumatriptan: ibuprofen, triptans may be used in children >=12 (sumatriptan nasal spray), SE: tingling, heat and heaviness/ pressure sensations -Prophylaxis: Propranolol/Pizotifen -Psychology input

Question 30

Examples of neuromuscular disorders

Answer 30

-Muscular dystrophies -Myasthenia Gravis -Congenital myopathies -Neuropathies -Metabolic myopathies

Question 31

Overview of DMD

Answer 31

-Duchenne muscular dystrophy is an X-linked recessive inherited disorder in the dystrophin genes required for normal muscular function. -P: progressive proximal muscle weakness from 5 years (boys), calf pseudohypertrophy, Gower's sign (child uses arms to stand up from a squatted position), 30% of patients have intellectual impairment -I: raised creatinine kinase, genetic testing has now replaced muscle biopsy as the way to obtain a definitive diagnosis -M: largely supportive as unfortunately there is currently no effective treatment. Steroids? -Prognosis: =Most children cannot walk by the age of 12 years patients typically survive to around the age of 25-30 years =Wheel chair bound by 2nd decade =Associated with dilated cardiomyopathy

Question 32

Overview of Becker muscular dystrophy

Answer 32

-In Becker muscular dystrophy there is a non-frameshift insertion in the dystrophin gene resulting in both binding sites being preserved leading to a milder form -Develops after age of 10 -Intellectual impairment much less common

Question 33

Overview of cerebral palsy

Answer 33

-Disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain. -It affects 2 in 1,000 live births and is the most common cause of major motor impairment

Question 34

Causes of cerebral palsy

Answer 34

-Antenatal (80%): e.g. cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV) -Intrapartum (10%): birth asphyxia/trauma -Postnatal (10%): intraventricular haemorrhage, meningitis, head-trauma

Question 35

Presentation of cerebral palsy

Answer 35

-Abnormal tone early infancy -Delayed motor milestones -Abnormal gait -Feeding difficulties. -Children with cerebral palsy often have associated non-motor problems such as: =Learning difficulties (60%) =Epilepsy (30%) =Squints (30%) =Hearing impairment (20%)

Question 36

Classification of cerebral palsy

Answer 36

-Spastic (70%) =Subtypes include hemiplegia, diplegia or quadriplegia =Increased tone resulting from damage to upper motor neurons -Dyskinetic =Caused by damage to the basal ganglia and the substantia nigra =Athetoid movements and oro-motor problems -Ataxic =Caused by damage to the cerebellum with typical cerebellar signs -Mixed

Question 37

Management of cerebral palsy

Answer 37

-As with any child with a chronic condition a multidisciplinary approach is needed -Treatments for spasticity include oral diazepam, oral and intrathecal baclofen, botulinum toxin type A, orthopaedic surgery and selective dorsal rhizotomy -Anticonvulsants, analgesia as required

Question 38

History of head injury

Answer 38

-What was the mechanism and force involved in the head injury? Bullseye in car accidents -If it was a fall, did they fall from standing height or from higher up? -Was there Loss Of Consciousness (LOC)? (Did they cry immediately/ in older children do they remember who was there and who called for help?) -How long was the LOC? -Was there any fitting? Generalised/ focal/ breath-holding attack/ duration -Has the child been their normal self since? -Has the child been drowsy since? Over 2 hours: CT -Has the child been irritable since? Cerebral oedema, haemorrhage -Has the child complained of headache since? -Has the child vomited since? How many times? 3 or more CT -Vomiting, drowsiness or loss of consciousness may be indications for a scan. Follow local protocols or NICE guidelines. -Be aware of the possibility of Non Accidental Injury, particularly think of the possibility of an inflicted injury in a non-mobile, non-verbal child.

Question 39

Head injury exam

Answer 39

-Observe the child and decide if they are just upset, or if they are truly irritable after a head injury -Check for drowsiness or confusion -Check and record AVPU scale or GCS -Examine the head for the size of any swelling or laceration -If there is a bump feel to see if it is a depressed fracture or a soft boggy swelling (fractures require CT scan to look for underlying damage). Also, any sign of basal skull fracture such as battle sign or CSF leak is an indication for CT scan. -Examine fully for neurological deficits and boggy swellings require CT scan to look for underlying damage. Any suspected NAI needs a CT scan. -For a child <1 year of age a laceration or swelling on the head of more than 5cm is an indication for CT scan. Also any sign of basal skull fracture is an indication for CT scan. -Vomiting 3 or more times is an indication for CT scan. -Be aware of the possibility of Non Accidental Injury. -If discharging a child home then give the parents ‘head injury advice’ for home.

Question 40

Criteria for immediate request for CT head in children

Answer 40

-Loss of consciousness lasting more than 5 minutes (witnessed) -Amnesia (antegrade or retrograde) lasting more than 5 minutes -Abnormal drowsiness -Three or more discrete episodes of vomiting -Clinical suspicion of non-accidental injury -Post-traumatic seizure but no history of epilepsy -GCS less than 14, or for a baby under 1 year GCS (paediatric) less than 15, on assessment in the emergency department -Suspicion of open or depressed skull injury or tense fontanelle -Any sign of basal skull fracture (haemotympanum, panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign) -Focal neurological deficit -If under 1 year, presence of bruise, swelling or laceration of more than 5 cm on the head -Dangerous mechanism of injury (high-speed road traffic accident either as pedestrian, cyclist or vehicle occupant, fall from a height of greater than 3 m, high-speed injury from a projectile or an object

Question 41

Overview of Tic Disorder

Answer 41

-Rapid, repetitive, involuntary contractions of a group of muscles. -Not harmful but can severely interfere with daily life -Motor and phonic (vocal tics) =Tourette’s syndrome (motor and vocal tics over 12 months) =Clinical evaluation =Anxiety, ADHD, ASD =Treatment to reduce problems (Social, school, bullying etc) -No investigations unless there are any co-morbidity -Treatment mainly behavioural therapy -Habit reversal therapy (HRT) – which aims to help you learn "responses" (other movements) that "compete" with tics, meaning the tic can't happen at the same time. HRT teaches you to use these competing responses when you get the feeling you need to tic, until the feeling goes away

Question 42

Presentation of brain tumour: Under 5

Answer 42

-Persistent/ recurrent vomiting -Balance/ co-ordination/ walking problems -Abnormal eye movements -Behaviour change, particularly lethargy -Fits or seizures (not with a fever) -Abnormal head position such as wry neck, head tilt or stiff neck

Question 43

Presentation of brain tumour: 5-11

Answer 43

-Persistent/ recurrent headache -Persistent/ recurrent vomiting -Balance/ co-ordination/ walking problems -Abnormal eye movements -Blurred or double vision -Behaviour change -Fits or seizures -Abnormal head position such as wry neck, head tilt or stiff neck

Question 44

Presentation of brain tumour: 12-18

Answer 44

-Persistent/ recurrent headache -Persistent/ recurrent vomiting -Balance/ co-ordination/ walking problems -Abnormal eye movements -Blurred or double vision -Behaviour change -Fits or seizures -Delayed or arrested puberty, slow growth

Question 45

Examples of neonatal neurology

Answer 45

-Hypoxic ischaemic encephalopathy -Congenital infections (TORCH) -Inherited metabolic disorders -Congenital malformations

Question 46

Overview of spina bifida

Answer 46

-Prevention (nutrition) -Antenatal detection and counselling -Surgery -Management of hydrocephalus -Bladder and bowel management -Mobility support -MDT -Support

Question 47

Common genetic disorders

Answer 47

-Downs' syndrome -Fragile X