Paediatric Neurology Flashcards
When does maximum head growth occur?
approx. 2 y / o
What is the young brain very susceptible to?
insult
What is symbolic play?
Pretend play
What is the second most common cancer in children?
Brain tumours
Types of headaches in childhood
Isolated acute Recurrent acute - migraines Chronic progressive Chronic non progressive (TTH)
What is a red flag sign of a headache?
If the headache is localised to the back of the head
What is looked at in a headache exam?
Growth parameters, OFC, BP Sinuses, teeth, visual acuity - sinusitis, carious teeth, headaches from visual problems Fundoscopy - papilloedema Visual fields (craniopharyngioma) Cranial bruit Focal neurological signs Cognitive and emotional status
What would the identification of a bruit be indicative of in children?
AV fistula
Aneurysm
Examples of primary headaches in children
Migraine
Tension headache
Presentation of childhood migraine
Pain - hemicranial - throbbing - pulsatile Nausea Vomiting Focal symptoms / signs - visual disturbance - paraesthesia - weakness Pallor
When can focal symptoms / signs of migraine occur in the attack?
During
Before
After
What are migraines aggravated by?
Bright light
Nosie
What are migraines related to?
Fatigue
Stress
What are migraines helped by?
Sleep
Rest
Dark
Quiet room
What is often found in the history of migraine?
FH
Treatment of childhood migraine
Acute attack - effective pain relief - Triptans Preventative (at least 1 week) - Pizotifen - Propanolol - Amitriptyline - Topiramate - Valproate
What is a key feature of tension headaches?
Featureless and never severe
How often do tension headaches last?
They are there all of the time
But there may be symptom free periods
Features of pain in tension headaches
Diffuse, symmetrical pain
A band
Constant ache
Treatment of tension headahce
Aim at reassurance; no sinister cause Attention to underlying chronic, physical, psychological or emotional problems Acute attacks - simple analgesia Prevention - amitriptyline
What is discouraged in chronic TTH?
Analgesics
Clues to raised ICP
Aggravated / increased by - coughing - straining at stool - bending Woken from sleep with headache
Features of analgesic overuse headache
headache is back before allowed to use another dose
What analgesics give analgesic overuse headache?
Paracetamol
NSAIDs
particular problem with compound analgesics e.g. cocodamol
Indications for neuroimaging
Features of cerebellar dysfunction Features of raised ICP New focal neurological deficit e.g. new squint Seizures esp focal Personality change Unexplained deterioration of school work
What describes paroxysmal disorders?
Fits, faints and funny turns
Seizure vs convulsion
Seizure / fit = any sudden attack where there is prominent motor activity
Convulsion = Seizure where there is prominent motor activity
Pathology of an epileptic seizure
An abnormal excessive hyper synchronous discharge from a group of (cortical) neurones
Chemically triggered by imbalances in your neurotransmitters
- decreased inhibition (GABA)
- excessive excitation
- excessive influx of Na and Ca ions
Chemical stimulation produces an electrical current
Depolarising current can be measured on EEG
Paroxysmal change in motor, sensory or cognitive function
Definition of epilepsy
A tendency to recurrent, unprovoked / spontaneous epileptic seizures
How many seizures do you need to have to be diagnosed with epilepsy?
2 attacks
What is a partial / focal seizure?
Abnormal activity restricted to one hemisphere or part of the hemisphere
What is a general seizure?
Abnormal activity coming from both halves of the brain
When is an interictal EEG used?
When they are NOT having a seizure
EEG is used in identifying…
Seizure types
Seizure syndromes
Aetiology
Investigations for epilepsy
History Recording of event ECG in convulsive seizures Interictal / ictal EEG MRI brain Genetics Metabolic tests
What does an MRI of the brain tell you about epilepsy?
The cause e.g. damage / aetiology
Treatment of epilepsy in children
Anti-epileptic Drugs (AED) - Sodium valproate (General) - Carbamazepine (focal) Steroids Immunoglobulins Ketogenic diet (mostly for resistant therapies) VNS (palliative procedure for treatment of epilepsy) Surgery
S/Es of AED
CNS related Drowsiness effect of learning cognition behavioural
Examples of non epileptic seizures
Acute symptomatic seizures due to acute insults Reflex anoxic seizures Syncope Sleep Parasomnias e.g. night terrors Behavioural stereotypes Psychogenic seizures (NEAD)
Causes of acute symptomatic seizures
Hypoxia-ischaemia Hypoglycaemia Infection Trauma Pyrexia
Who is reflex anoxic seizure common in?
Toddlers
What triggers reflex anoxic seizures?
Pain
Upset
Fright
What does NEAD stand for?
Non epileptic attack disorder
What is a febrile convulsion?
An event occurring in infancy / childhood, usually between 6 months and 5 years of age, associated with fever but without evidence of intracranial infection or defined cause for the seizure
What is the commonest cause of acute symptomatic seizure in childhood?
Febrile convulsion
Types of seizures
Jerk / shake - clonic = rhythmic - myoclonic = one attack - spasms Stiff - tonic seizure usually Falls - atonic - hypertonia / tonic Vacant attack / absence / partial seizure
Features of a myoclonic seizure
One attack
Fully aware but cannot control the jerks
Can be very subtle
What happens in an atonic seizure?
Sudden drop of tone
What is a risk of atonic seizures?
Injury
What happens in a hypertonic seizure?
Sudden increase in tone
Posture maintained
Presentation of a vacant attack / absence seizure
Acute onset
Eye flickering
Does not last long
What are vacant attacks / absence seizures triggered by?
Hyperventilation
What happens in a tonic clonic seizure?
Go blue
Go stiff
What do neuromuscular disorders affect?
The peripheral nervous sysem
Features of when to suspect a NM disorder
Baby 'floppy' from birth Slips from hands Paucity of limb movements Alert but less motor activity Delayed motor milestones Able to walk but frequent falls
Presentation of NM disorders
Belly sticks out
Thin, weak thighs except from the front
Poor balance
- falls often
- awkward clumsy walking
Weak muscles in front of the leg cause “foot drop” and tip toe contractures
Tight heel cord (contracture) child may walk toes
Thick lower leg muscles (mostly fat and not strong)
Myopathic facies
Sway back
Shoulders and arms held back awkwardly when walking
Weak butt muscles (hip straightners)
Myotonia - difficulty in relaxing the muscles (eyes, fists)
Knees may bend back to take the weight
Very high foot arch - pess cavis
Hammar toes
Features of myopathic facies
Ptosis
Cant close mouth due to shape
Lack of muscle control around mouth
Poor head position control
NM muscle conditions
Muscle dystrophies
Myopathies congenital / inflammatory
Myotonic syndromes
NM NMJ conditions
Myasthenic syndromes
NM nerve conditions
Hereditary or acquired neuropathies
NM anterior horn cell conditions
Spinal muscular atrophy
Genes of Duchenne Muscular Dystrophy
Xp21
Dystrophin gene
Who gets Duchenne Muscular Dystrophy?
Female carriers
Males have the disease
Presentation of Duchenne Muscular Dystrophy
Delayed gross motor skills Symmetrical proximal weakness - waddling gait (pelvic waddle) - calf hypertrophy (replaced by fat) - Gower's sign positive Elevated Creatinine kinase - > 1000 Cardiomyopathy Respiratory involvement in teens
What is creatinine kinase a measure of?
Muscular breakdown
What value of creatinine kinase is normal?
200
Treatment of Duchenne Muscular Dystrophy
Steroids
No cure
Prognosis of Duchenne Muscular Dystrophy is what and depends on what?
With treatment can live to early 30s
Depends on gene type
Characteristics of febrile seizures
Temp > 38.4C Age 6 months - 5 years Peak 18 - 22 months Usually after 1 - 24 hours of recognised fever Seizure type is generalised or focal
Two broad types of seizures
Typical
Atypical
Features of a typical seizure
Generalised convulsion for < 15 mins
Single convulsion in one illness
Features of an atypical seizure
Focal
Lasts for > 15 mins
Abnormal neurological signs
Developmental delay
What condition protects against febrile seizures?
Gastroenteritis (apart from shigella)
Does febrile seizures run in the family?
Yes
Investigations of a seizure
PLASMA GLUCOSE! Electrolytes CSF examination FBC Neuroimaging EEG
Treatment of febrile convulsion
Antipyretics
Do antipyretics reduce the risk of recurrence in febrile convulsions?
No
Complications of febrile seizures
Todds paresis
Recurrent of FS
Epilepsy
What is the chance of a febrile seizure happening again?
1 in 3
Atypical febrile seizures increased he risk for developing epilepsy in the future by how much?
8-9%
How long is a prolonged seizure?
5 mins or more
Definition of an epileptic seizure
An electrical phenomenon in which an abnormally excessive synchronous discharge from a group of neurones (usually from cerebral cortex) occurs
Definition of epilepsy
Tendency to recurrent, unprovoked, spontaneous epileptic seizures
Definition of a symptomatic seizure
A seizure from a secondary cause e.g. brain tumour, hypoglycaemic etc
Types of seizures
Focal/partial - simple (consciousness retained) - complex (consciousness impaired) - secondary generalised Generalised - absence - myoclonic jerks - tonic / clonic / GTC - atonic / drop attacks
Differential diagnosis of a seizure
Reflex anoxic seizure Vasovagal syncope Breath holding attack Sleep related events (parasomnias) GORD Shuddering attack, temulousess/startles (hyperekplexia) Benign positional vertigo Pseudo seizures
1st line treatment for generalised epilepsy
Valproate
1st line treatment for focal epilepsy
Carbamazepine
Other treatments for seizures
Steriods IgS Ketogenic diet Vagus nerve stimulation Surgery
Who gets reflex anoxic seizures?
Toddlers
Pathology of reflex anoxic seizures
Triggers cause vasovagal overactivity
Treatment for reflex anoxic seizures
None
When do frontal lobe seizures particularly happen?
At night
Features of GORD that look like a seizure
Babies arch their back and clench hands to prevent symptoms
Features of infantile spasms
Repetitive
Recovery of reflex anoxic seizures vs epileptic seizures
Reflex anoxic - rapid recovery
Epileptic - prolonged recovery
What are the seizure patterns in west syndrome?
Infantile spasms
When does west syndrome present?
First 4 - 8 months
Which gender is west syndrome more common in?
Males
What is west syndrome often associated with?
A serious underlying condition and carry a poor prognosis
Presentation of west syndrome
Characteristic ‘salaam’ attacks; flexion of head, trunk and arms followed by the extension of the arms
Lasts 1 - 2 seconds but may be repeated up to 50 times
Progressive mental handicap
Investigation of west syndrome
EEG - hypssarrhytmia in 2/3rds of infants
CT demonstrates against diffuse or local brain disease in 70% (e.g. tuberous sclerosis)
Prognosis of west syndrome
Poor
Treatment of west syndrome
Vigabatrin 1st line
ACTH
Most common cause of headache in children
Migraine
