Paeds Neuro Flashcards
Define cerebral palsy
Permanent disorder of movement and/or posture, and of motor function due to a non-progressive abnormality in developing brain
What if brain is damaged after 2yrs old?
Acquired Brain Injury
NOT cerebral palsy
What causes 80% of cerebral palsy?
Antenatal factors eg. cerebrovascular haemorrhage/ischaemia, maldevelopment, congenital infection
What makes up the other 20% of cerebral palsy?
10% hypoxihc-ischaemic injury intrapartum
10% postnatal
Why are preterms more at risk of CP?
Vulnerable to periventricular leukomalacia, severe intraventricular haemorrhage, and venous infarcts
What are the post-natal causes of CP?
I+4H
Infection Head injury Hypoglycaemia Hydrocephalus Hyperbilirubinaemia
What are the types of CP?
Spastic = 90% Dyskinetic = 6% Ataxic = 4%
What are the early features of CP?
Delayed milestones Difficulties feeding eg. slow, gagging, vomiting Asymmetric hand function <12m Abnormal gait Primitive reflexes may persist
Gross Motor Classification System - 5 levels
1 = walks without limitation 2 = walks with limitation 3 = walks with hand-held mobility device 4 = self-mobility with limitation eg. powered mobility 5 = manual wheelchair
What are the features of spastic CP?
Tone increased
Brisk tendon reflexes
Clasp-knife rigidity
Presents early
What is unilateral spastic CP?
Presents 4-12m with fisting of hand, pronated forearm and flexed wrist
Tiptoe walking on affected side
Affects arm more than leg
Spares the face
What is bilateral spastic CP?
Affected all limbs
Trunk tendency to opisthotonus (extensor posturing)
Poor head control
Why is bilateral spastic CP bad?
Severe
Associated with seizures, microcephaly and mod-severe intellectual disability
What is bilateral diplegia?
Legs affected more than arm
Hand function may appear normal
Associated with Periventricular Leukomalacia on MRI
What the features of dyskinetic CP?
Involuntary, uncontrolled, stereotyped movements
Primitive reflexes remain
Signs = chorea, athetosis and dystonia
What is chorea?
Chorea = irregular, sudden and brief non-repetitive movements
What is athetosis?
Athetosis = slow, writhing, distal movements eg. fanning fingers
What is dystonia?
Dystonia = contraction of agonist and antagonist muscles at the same time, causes twisting appearance
When do chorea, athetosis and dystonia arise in dyskinetic CP?
10-12m
What causes chorea, athetosis and dystonia?
Basal ganglia dysfunction - shown on MRI
What causes basal ganglia dysfunction leading to CP?
Hypoxic-ischaemic encephalopathy
Previously kernicterus secondary to hyperbilirubinaemia
What causes ataxic CP?
Generally genetically determined
May be due to ABI, features are often ipsilateral to lesion and symmetrical
What are the early features of ataxic CP?
Trunk and limb hypotonia
Poor balance
Delayed motor development
What are the later features of ataxic CP?
Uncoordinated movements
Intention tremor
Ataxic gait
What are the 4 medical/surgical treatment options for spasticity?
Botox injections
Selective dorsal rhizotomy
Intrathecal baclofen
Deep brain stimulation of basal ganglia
What is epilepsy?
2 or more unprovoked epileptic seizures
What is a febrile seizure?
Seizure due to a fever but not intracranial infections
Who gets febrile seizures?
6m - 6yrs
10% have family history
When do febrile seizures occur?
When pyrexial
Generally during viral illness
What type of seizures are febrile seizures?
Usually brief tonic-clonic
40% have more than one
Do febrile seizures increase the risk of epilepsy?
1 = no increased risk
Focal, prolonged, or repeated = 4-12% risk
What is the management of febrile seizures?
Rule out meningitis - give Abx if can’t get LP
Buccal midazolam if >5mins
Paracetamol won’t prevent but will make child more comfortable
Antipyretics and anti epileptics do not prevent
What happens in frontal epilepsy?
Motor symptoms
Clonic movements travelling proximally = Jacksonian march
Tonic seizures eg. raise both arms for a few seconds
What happens in temporal epilepsy?
Aura - taste/smell Lip smacking Clothes plucking Automatism Deja vu
Impaired consciousness
What happens in parietal epilepsy?
Contralateral dysaethesias = altered sensations
Distorted body image
What happens in occipital epilepsy?
Stereotyped vial hallucinations
What investigations are useful in epilepsy?
ECG - exclude long-QT syndrome
EEG - ictal/interictal/ambulatory-24hr
MRI-FLAIR - looking for mesial temporal sclerosis which can be cured surgically
PET/SPECT - if considering surgery
What are the features of a generalised seizure? (4)
Loss of consciousness >3sec
No warning
Symmetrical seizure
Bilateral synchronous discharge on EEG
What are the features of absence seizures? (4)
Loss of consciousness
Abrupt start and end
No motor Sx
May be trigged by hyperventilation
What is a myoclonic seizure?
Jerking of limbs/neck/trunk
What is a tonic seizure?
Generalized increase in tone
What is a tonic-clonic seizure?
Tonic = stiff, fall, cyanosed
then Clonic = jerking, irregular breathing, biting tongue, urinary incontinence
Lasts minutes, followed by deep sleep for few hrs
What is an atonic seizure?
Initial myoclonic jerk then loss of muscle tone -> fall
What is the management of epilepsy?
Antiepileptics until 2yr seizure-free
Need them forever if Juvenile absence/myoclonic epilepsy
Valproate/Lamotrigine for generalised
Carbamazepine for focal
What advice is it important to give patients?
Avoid swimming
No driving until 1yr seizure-free
Need contraception for girls
Epilepsy Action = support group
What is status epilepticus?
Seizure lasting 30mins or longer, or repeated seizures without recovery
What are the side effects of valproate?
Weight gain
Hair loss with curly regrowth
Idiosyncratic liver failure
What are the side effects of lamotrigine?
Rash
Insomnia
Ataxia
What are the side effects of carbamazepine?
Rash
Ataxia
Hyponatraemia
What is West’s Syndrome?
3-12m
Appears similar to colic in 20-30sec bursts
What does an EEG show for West’s Syndrome?
Hypsarrhythmia
What is the management of West’s Syndrome?
Vigabatrin and steroids
70% response but lots of S/Es
Prognosis poor - many develop LD
What is Juvenile Absence Epilepsy?
10-20yrs
Absence and tonic-clonic seizures, generally with photosensitivity
Good response to treatment but will need lifelong
What is Juvenile Myoclonic Epilepsy?
10-20yrs
Myoclonic, tonic-clonic and absence seizures may occur, generally in the morning
Typical Hx = throwing breakfast due to myoclonus
Good response to treatment but will need lifelong
What is Childhood Absence Epilepsy?
4-12yrs, 2% childhood epilepsy, 2/3 female
Momentary unresponsive state <30sec
No recollection, only knows that they’ve missed something
No LD
80% remission
Can may induce Childhood Absence Epilepsy?
Hyperventilation/blowing for 2-3mins
Useful for conducting EEG
What is Benign Rolandic Epilepsy?
4-10yrs, 15% childhood epilepsy
Have tonic-clonic seizures in sleep
EEG shows focal sharp waves from rolandic area
Completely benign, remits in teens, no Tx
What is Parayiotopoulos Syndrome?
1-5yrs, 5% childhood epilepsy
Vomiting and unresponsive staring in sleep with head and eye deviation
May progress to convulsions
EEG shows posterior sharp waves and occipital discharge even with eyes closed
Remits in childhood, no Tx, may have some specific LD
What are breath-holding spells?
Upset toddler cries, holds breath on expiration, turns blue, briefly loses consciousness
Recovers rapidly
What is the treatment for breath-holding spells?
Behaviour modification with distraction
Drug therapy = unhelpful
