Neurology Flashcards
Difference between seizure and epileptic seizure?
Epileptic seizure is an excessive and hypersynchronous self-limiting activity of neurons in the brain
Taking a history of a seizure, what do you ask about before the event?
Time of day What were you doing at the time? Any triggers Any warnings like behavioural changes Was it sudden or gradual Well before the episode? were they feeling funny? Chances of being poisoned?
When taking a history of a seizure, what do you ask about the event itself?
Movement - eyes, head, which limbs, symmetrical, flex or extend, hypertonic or hypotonic, jerking (clonic), sudden jerks Pallor Cyanosis Eyes open or closed Awareness Tongue biting Incontinence
When taking a history of a seizure, what do you ask about after the event?
When did it stop? Gradual/sudden? How long till returned to normal? Post-ictal confusion sleepy?
What is it always important to have before epilepsy diagnosis can be made?
Eye witness
What is the differential diagnosis to epilepsy?
Non-epileptic paroxysmal events
List 10 types of non-epileptic paroxysmal events?
Vasovagal syncope Cardiogenic syncope Breath holding Reflex anoxic spells Blank spells/day dreaming Sandifer Syndrome fabricated illness sleep phenomena self gratification benign neonatal sleep myoclonus benign paroxysmal vertigo and torticollis Tics Pseudoseizures
Cardiogenic syncope often is found to have what? Triggers?
QT prolongation
FH of sudden death
Triggered by exercise and sleep
What is Sandifer syndrome?
Present in complex neurodisability triggered by feeding and leaves patients with GORD and pain
2 examples of idiopathic generalised seizures in children?
CAE (childhood absence seizures)
JME (juvenille myoclonic epilepsy)
A type of idiopathic focal epilepsy in children?
BREC (benign rolandic epilepsy of chidhood)
2 epileptic encephalopathies that can develop in children?
Wests syndrome
Dravet Syndrome
Describe benign neonatal sleep myoclonus?
Brief jerks whilst asleep that don’t bother neonate
Grows out of it in 6-8 months
Infantile spasms - when do they present? symptoms? cry? assocaited to what syndrome? 2 underlying causes?
Presents 2 months-1year
Symptoms = myoclonic jerks of head, limbs or trunk, clusters on waking
Cry between spasms whereas colic cry during
Assocaited to Wests syndrome - infantile spasms, developmental delay and Hips arrythmia on EEG
underlying causes can be Down’s Syndrome or Tuberous Sclerosis
How do you treat infantile spasms?
Steroids
How do you treat infantile spasms if the underlying cause is tuberous sclerosis?
Vigabatrin
2 things that make a febrile illness become complex?
More than 2 seizures in a 24 hours period
Seizure for longer than 15 minutes
Age group usually effected by febrile seizures?
6 months to 5 years
When do you treat febrile illness?
If they enter status epilepticus which is prolonged seizure for 15-20 minutes then treat with buccal Midazolam
Describe the typical picture of breath holding spells? resolves by what age?
child crying, louder then collapses and seizes, return to normal
Resolve by age 2/2.5
What causes reflex anoxic seizures? typical picture?
Hypoxic drive for seizure activity
Suddenly go limp then clonic jerks
pallor
Usual age onset of CAE? features during and after?
4-8 years old eye or mouth flickering Little finger movements Peri-orbital and periocular flickering Last 20 seconds No post-ictal confusion
How do you diagnose CAE alongside clinical appearance?
2 second spike and wave on EEG
Key feature of BREC? age?
facial or periorbital onset of focal epilepsy
3-13 years
What are the 2 classic prodromal symptoms of BREC do children tend to suffer?
Other potential prodromes?
Unilateral numbness and paraesthesia of the lips, gums, cheek
Involuntary movements of the jaw
Expressive speech arrest
4 post-ictal features of BREC?
guttural sounds
speech arrest
hypersalivation
difficulty swallowing
EEG characteristic feature of BREC?
bilateral or unilateral centrotemporal spikes
Prognosis of BREC?
90% remission by 16 years old
Juvenille myoclonic epilepsy? age of onset? EEG findings? treatment options? prognosis in comparison to CAE?
onset at 12 years old
Absence seizures, GTC, myoclonic
EEG sees 3 per second spike and wave
Treatment with sodium valproate and lamotrigine
Juvenile myoclonic epilepsy onset age? type of seizure? often confused with what? EEG shows what? Treatment? Prognosis?
12-18 years onset
GTC seizures
Often confused with daydreaming - clumsy, dropping stuff in morning
EEG shows polyspike waves with irregular slow waves
Treatment is with sodium valproate and lamotrigine
Prognosis is lifelong disorder
Conditions that relate to development of cerebral palsy?
Prematurity
Hypoxic ischaemic encephalopathy
metabolic disease
Acquired brain injury
Define cerebral palsy
Non-progressive non changing disorder due to malformations in brain development
What are common comorbidities suffered from those with cerebral palsy?
Difficulties feeding Epilepsy Sensorineural deafness Visual impairment Gut motility issues Retinopathy of prematurity
GMFCS 1 vs GMFCS 5 in terms of cerebral palsy classification
GMFCS 1 = no walking aids needed
GMFCS 5 = wheelchair and poor head control
Which cause of cerebral palsy is most likely to end up as a quadriplegiac?
CP caused by prematurity
Cardinal features of cerebral palsy?
dystonia
spasticity
pain
What is dystonia and which type of cerebral palsy cause usually presents with it?
involuntary movements of the limbs
Usually in full term hypoxic events that damage the basal ganglia
Dystonia causes that arent caused by damage to the basal ganglia?
Dopa responsive dystonia Biotinidase deficiency Methyl malonic aciduria Glutaric aciduria type 1 GLUT 1 deficinecy Inherited DYT1/DYT11
How does spasticity form in cerebral palsy and what does it present as?
Damage to motor pathways
High tone movement disorder
Increased reflexes
Clonus
3 main forms of pain in cerebral palsy?
dental pain, bone pain and gastric pain
What can the pain in cerebral palsy impact the persons life?
Seizures become worse dystonic positioning difficult sleeping poor feeding irritable and challenging behaviour
What kind of MDT team is involved in CP?
Paediatrician Physiotherapist OT GP Respite SLT Neurologist SW
4 main MSK issues suffered by patients with CP?
scoliosis
Hip dislocation
Muscle contracture
Plantar flexed feet and toe walking
Main prevention methods of worsening MSK issues in children with CP?
Prevention schemes - walking and moving
Medication to decrease tone
Postural management - chairs, beds
Common sites for Botulinum toxin injections to be administered? Who needs to be involved in management of patients recieving them? Benefit? How long do they last?
Iliopsoas, hamstrings, adductors
OT - train antagonist muscle whilst the agonist are paralysed
last for 3-4 months with peak at 1 month
What is the CDC input in post injection therapy for cerebral palsy patients?
8 weeks once a week treatment sessions, monitoring, review Goal setting Liaison with school Ongoing monitoring 2/3 review sessions in months 3/4
What GABA agonist is commonly used to treat cerebral palsy that presents as a high tone disorder such as spasticity?
Intra-thecal baclofen
What is the gold standard trio of medication for managing dystonia in cerebral palsy? alternative?
Haloperidol + tetrabenazine + trihexyphenidyl
Deep brain stimulation
How is a Baclofen pump placed in situ and who is it administered to?
Placed inside abdomen with leads into spinal cord
Spasticity and dystonia indications in older ambulatory children with either severe spasticity in upper and lower extremities or spinal injury
In deep brain stimulation what the rods target?
Basal Ganglia
Define Neural tube defect?
developmental disorder of the brain, spine or spinal cord occurring in the first trimester of pregnancy
3 types of spina bifida?
Spina bifida occulta, meningocele, myelomeningocele
How does spina bifida occulta present?
dimples in back, hair tuft, lipoma, adhesions can potentially cause sensory or motor disturbances
Meningocele?
Vertebral cleft and meninges in CSF
Myelomeningocele?
Vertebral cleft with meninges and nervous tissue
Antenatal USS reveals what signs of spina bifida?
Lemon sign
Banana sign
Ventriculomegaly
Posterior vertebral arch defects
What screening blood test can be done in early pregnancy to assess for spina bifida? how many weeks gestation?
AFP in maternal blood
Preventable causes of spina bifida?
Folic acid deficiency
AEDs - sodium valproate, phenytoin, carbemazepine
Maternal diabetes
Why do AEDs cause spina bifida?
Reduce folate stores
If the spina bifida occurs at the S2-S4 level what are the symptoms?
can usually walk with shoe inserts
If the spina bifida occurs at the level L5-S1 level what are the symptoms?
Walk with short ankle braces
Crutches
If the spina bifida occurs at the L4 level what are the usual symptoms?
Can use braces that go above the knee
Wheelchair often preferred for long distances
Braces for short distances
If the spina bifida occurs at the level L2-L3 what are the usual symptoms?
Can have braces above knee/hip
Usually end up using wheelchair majority of time
is the spina bifida occurs at the level T12-L1 what are the usual symptoms?
Younger may walk with braces above hip
Usually end up just using wheelchair
Why is surgical intervention often needed with higher lesion spina bifida?
Scoliosis
Apart from walking, what other complications does spina bifida often come with?
Sensory issues
Neuropathic bowel
Neuropathic bladder
Management of neuropathic bowel?
laxatives
suppositories
bowel training
rectal washouts
2 main types of neuropathic bladder that can develop in spina bifida? complications associated?
sphincter tight shut - reflux, renal damage, CKF
Sphincter loose and open - incontinence, stagnant bladder, UTI
80% of myelomeningocele patients will suffer with what?
Associated with what?
What is needed as treatment?
Hydrocephalus
Chiari 2 malformation
Ventriculoperitoneal shunt
Infant presenting with shunt malformation?
Lethargy Poor feeding Vomiting Irritable Seizure LOC
Child presenting with shunt malformation?
Headache Visual loss Dizzy Vomiting Personality changes developmental delay LOC Seizure
High AFP on 15-20 week screen of mother suggests what 2 possibilities?
Myelomeningocele and anencephaly
How does USS determine spina bifida?
Lemon and banana sign - ventriculomegaly and microcephaly
In motor defects of spina bifida where are the main referrals made to?
OT
Physio
Orthopaedics
Child development clinic
Urinary issues in spina bifida investigated and managed how?
MCUG
USS
Videourodynamics
Self-catheterisation and UTI prophylaxis
What is given for bowel problems suffered in spina bifida patients?
Glycerol suppositories
In spina bifida, what support group is a good one to suggest?
SHINE charity
Symptoms of VP shunt blockage in baby?
Lethargy Poor feeding Vomiting Seizure LOC
Symptoms of VP shunt blockage in child?
Developmental delay Headache Visual loss Dizziness Vomiting Seizures LOC Unsteady Irritable
How do you diagnose shunt malfunction?
CT brain
Shunt series
AFO? KAFO? HKAFO?
Ankle foot orthoses
Knee ankle foot orthoses
Hip knee ankle foot orthoses
Child with spina bifida is starting to hate having to self-cathetrise, who do you refer to?
Specialist continence nurse
SHINE
If a child with spina bifida presents with incontinence every day what are investigations performed?
MRI spine - tethering
Videourodynamics - pressures, compliance
CT brain - shunt malformations
Symptoms suggestive of cord tethering?
Progressive limb deterioration
Change in urinary habits
Progressive scoliosis
Pain
Associated symptoms to ask about in headache history?
nausea vomiting aura visual disturbances balance fever personality changes numbness or paraesthesia
In terms of the pattern and time course to a headache what questions do you want to ask?
Morning? night?
after exercise? argument? eating? trauma? travel?
What relieving factors do you want to ask about with headache history?
Lying down Dark room Paracetamol Sitting down Eating
What questions can you ask to assess how much headaches are effecting a childs quality of life?
If they have to leave school? how many times?
Sleep effected?
Daily activities effected?
Medication history of a child with a headache what is it important to ask about?
Anticoags, antoplatelet, contraception
Allergies
Vaccines up to date
OTC - eye drops, nasal decongestants, herbal
FH questions to ask about migraines?
headaches, migraines kidney problems, high BP eye problems - any one wear glasses? Familial tumours Blood vessel abnormalities - aneurysms, malformations, coagulopathy
SH questions to ask about migraine?
Who lives at home? Any social worker? Pets? School? progress? enjoy? any developmental concerns? Hobbies and activities?
Things to assess in a neurology exam to assess headaches?
Gait
Coordination
CNS - power, tone, coordination, reflex, sensation
PNS - cranial nerve 1-12
Eye exam - movements, acuity, nystagmus, diplopia, pupils, fundoscopy
What symptoms make you worried about a space occupying lesion with a headache?
Headache worse in morning, vomiting, sleep disturb
If CT is performed for papilloedema and finds no brain abnormality, what 2 tests can you perform?
Lumbar puncture for pressure reading
MRI brain for more accurate imaging
Differential diagnosis of seizures in children?
Epilepsy - idiopathic or secondary to damage
Metabolic - dka, hypocalcaemia, hypomagnesaemia, hypo or hypernatraemia
Trauma - intracranial haemorrhage, NAI
Infection - meningitis, encephalopathy
Cardiac - arrhythmia, long QT syndrome
Breath holding
Toxins
Poisoning
Causes of a seizure - febrile illness, reflex anoxic attacks, sleep apnoea, pseudoseizures, fabricated illness
Difference between simple and complex febrile illness?
Simple - 6 months to 5 years, fever, GTCS, less than 15 minutes, full neuro in an hour, no CNS infection
Complex - Focal onset, does not fully recover in an hour, more than 15 minutes, reoccurrence within 24 hours
When do you admit a child to hospital for a seizure?
Complex seizure First seizure Hasn't been assessed by paediatrician Parental anxiety Fever not present More serious infection suspected Child is on antibiotics
2 deficiencies that can lead to increased risk of febrile seizures?
Zinc and iron
What infection can predispose a child to their first febrile seizure?
Herpes virus 6
Dose of paracetamol given to children under 1 years old? 1-3 years old? 4+ years old?
120mg
240mg
360mg
rectal and buccal rescue treatments for febrile seizures?
Rectal diazepam
Buccal midazolam
What physical examinations need to be performed for a child experiencing seizures? (including one examiantion with light)
Facial dysmorphic features cafe au lait spots, woods light HC fundoscopy Bruits BP Neurodevelopmental exam Mental state
What investigation should never be forgotten in a child presenting with seizure?
Blood glucose
Epilepsy support groups?
British epilepsy association and epilepsy action
If buccal midazolam isnt working what can be given IV in hospital setting?
IV lorazepam 0.1mg/kg
If IV lorazepam isnt working then what is given IV to resolve seizure?
Phenytoin 20mg/kg over 20 minutes
