Neuro Flashcards
(45 cards)
1
Q
What is cerebral palsy?
A
Group of disorders that cause a non progressive disorder of movement and/ or posture and of motor function which is permanent but unchanging. The motor disorders are often accompanied by other abnormalities such as sensation, perception, cognition and communication problems as well as epilepsy
2
Q
What can cause cerebral palsy?
A
Damage to immature brain, mostly in first 24 weeks gestation.
- haemorrhage (intraventricular)
- hypoxia (HIE)
- teratogens
- genetic/ congenital defects and cortical migration defects
- infections (sepsis, meningitis esp)
- toxins (hyperbilirubinaemia)
- metabolic problems (mitochondrial or inborn errors of, hypoglycaemia)
- trauma
3
Q
What are the 4 types of cerebral palsy
A
- spastic: intermittently increased tone and pathological reflexes
- athetoid: increased activity (hyperkinesia/ stormy movements)
- ataxia: loss of orderly muscle coordination so movements are performed with abnormal force, rhythm or accuracy
- mixed
4
Q
give 5 risk factors for cerebral palsy
A
- preterm birth and LBW
- congential malformations
- multiple births
- taxoplasmosis, rubella, CMV, HIV infections
- maternal smoking, alcohol and drug abuse
- maternal illness
- neonatal sepsis/ infection/ encephalopathy
- post natal meningitis, intracranial haemorrhage, trauma, infection, hypoxia, seizures
5
Q
Describe the early clinical features of cerebral palsy
A
- delay in reaching milestones (no head control by 3 months, not sitting by 8 months, not walking by 18 and hand preferance before 1 yr are commonest)
- scissoring of legs, pronated forearm and fisted hand
- persistence of primative reflexes
- unusual fidgety/ asymmetrical movements
- low apgar score 5 mins after birth
- floppiness/ spasticity or dystonia
- feeding difficulties
- they will NOT regress in development
6
Q
Give 5 features associated with cerebral palsy
A
- learning disability
- communication difficulty
- emotion and behaviour difficulty
- sensory difficulty
- visual impairment
- scoliosis, hip sublaxation/ dislocation
- consitpation
- vomiting
- GORD
- epilepsy
- incontinence/ infections
- drooling/ poor saliva control
- low bone density- osteoporosis
7
Q
give 3 differentials for cerebral palsy
A
- transient hypotonia
- acute poliomyelitis
- charcot marie tooth disease
- stroke motor impairment
- neonatal brachial plexus injuries
- muscular dystrophy: beckers or duchennes
- fredriches ataxia
8
Q
How should cerebral palsy be investigated?
A
- diagnosis made on clinical exam and parent observation. tests are to exclude other diagnosis
- thyroid studies
- chromosomal analysis
- pyruvate and lactate levels to exclude mitochondrial cytopathies
- organic amino acid levels to exclude inborn errors of metabolism
- CSF for protein, lactate and pyruvate levels to determine if asphyxia in neonatal period
- sequential USS to detect major intracranial lesions
- MRI if USS not finding cause but usualy lesions that could explain cause not seen till age 2
- EEG can help detect damage due to hypoxia and vascular insult
9
Q
How is cerebral palsy managed?
A
- oral diazepam (rapid) or baclofen (long acting) can help if spasticity causes discomfort, pain, muscle spasms or functional disability
- levodopa/ baclofen if dystonia is causing problems
- botulinum toxin a injections if spasticity is impeding motor function, cauing pain etc
- intrathecal baclofen if non invasive is ineffective
- surgery to correct skeletal deformity, hip dislocations, contractures
- mobility aids and orthopaedic devices splints
- physio, SALT, OT, recreational therapist input
- preventative measures: maternal iodine, prolonging pregnancy in preger labour, improve neonatal intensive care (cooling)
10
Q
Give 3 complications of cerebral palsy
A
- contractures
- GI: reflux, constipation, swallowing difficulties, failure to thrive
- Resp: aspiration pneumonia, chronic lung disease
- dental problems
- learning difficulties
- hearing loss
11
Q
Briefly describe the 6 types of generalised seizures
A
- tonic clonic: tonic (hypertonia/ contraction then extension phase, then tremour/ shaking followed by clonic contraction and relaxation)
- atonic: sudden loss of muscle tone
- myoclonic: quick movements/ sudden jerking of muscles in clusters throughout day or for several days in a row)
- absence: brief altered consciousness, maintains posture, commonest age 4-12
- febrile: associated with fever
- infantile spasm: before 6 months old, when waking or falling asleep
12
Q
What is epilepsy?
A
- recurrant tendency to experience epileptic seizures- abnormal excessive or synchronous neuronal activity in the main
- at least 2 seizures more than 24 hrs apart
13
Q
What can cause epilepsy?
A
- genetic (multiple gene loci, complex inheritance, AKA idiopathic)
- cerebral dysgenesis/ malformation
- neurocutaneous syndromes
- cerebral damages (by HIE, trauma, infection, intraventricular haemorrhae/ ischaemia)
- cerebral tumour
- neurodenerative disorders
14
Q
give 4 common triggers for epileptic seizures
A
- photosenstivitiy
- hyperventilation
- sleep deprivation
- alcohol
- menstruation
- videogames
- low blood sugar
15
Q
How should epilepsy be investigated?
A
- EEG- 24hr tapes, 5 day tapes, sleep deprived, with photic stimulation and hyperventilation, helps determine type of epilepsy but not needed for diagnosis
- MRI if ?structural abnormality
- bloods: BMs, ABG, LFT, U&E, FBC etc
- video recordings by familly
- genetic testing if FHx
16
Q
how should a seizure be managed in the first 5 mins?
A
- start timer
- protect from injury (remove glasses, pillow around head, remove harmful objects nearby)
- dont restrain
- when stops put in recovery position
- examine and manage for injuries
- if 1st seizure, struggling for breath or difficult to wake up- arrange admission/ senior r/v
17
Q
How is a seizure managed from 5 mins onwards?
A
5 mins: high flow o2, do BM, midazolam buccal 0.5mg/kg or lorazepam 0.1mg/kg IV
15 mins: lorazepam 0.1mg/kg IV
25 mins: phenytoin 20mg/kg IV over 20 mins or phenobarbitone if not on phenytoin previously. Inform ITU.
45 mins: rapid sequence induction of anaesthesia using thiopental sodium
18
Q
What general measures need to be put in place for epilepsy?
A
- educate parents and child
- identify and avoid triggers
- supervise around water
- inform school
- epilepsy medical bracelet
- driving restrictions
- drinking and recreations drugs education
19
Q
What drugs are used for generalised seizures and for focal seizures?
A
generalised: sodium valproate, lamotrigine (not for myoclonic), carbemazepine (for TC), levitericetam, clobazam (2nd line for any)
Focal: carbemazepine, lamotrigine, valproate, levitericetam
- use one them move up to max dose then stop and try another if inefficient
20
Q
What are the 6 criteria for a febrile convulsion?
A
- age 6 months- 5yrs
- no cns infection or severe metabolic disturbance
- less than 15 mins duration
- generalised tonix clonic nature
- once in 24 hr period
- no history of afebrile seizures
21
Q
How are febrile convulsions managed?
A
- monitor duration
- protect from injury
- check airway put in recover position and observe till recovery
- manage other injuries
- is lasts >5 mins, call ambulance and give rectal diazepam if advised by specialist for child with recurent febrile seizures
- rule out meningitis
- immediate peads r/v if first febrile seizure, <18 months, diagnostic uncertainty, forcal neuro deficit, recent abx, LOC before seizure
- Antipyretic and anti epilepitic meds dont prevent them reoccuring
22
Q
Describe the features of a floppy infant
A
- no neck control- head flops
- limp when held
- difficulty sucking and swallowing
- weak cry
- frog legged posture (hips adducted, knees flexed)
- delay in development
- tip to walking/ cant heel walk
- jont hyperlaxity
- hypereflexia and clonus (cns dysfunction)
- diminished or absent reflexes (LMN dysfunction)
- signs of cardiac failure may indicate mitchondrial disease
- hepatomegaly suggests lysosomal or glycogen storage disease
23
Q
Describe the causes of a floppy infant (give 2 at each level there can be a problem - 10 total)
A
CNS: chromosome disorder (prader willi), metabolic disease, spinal cord injury, cerebral dysgenesis, HIE, cerebral palsy, infections, prematurity
Motor neurone: spinal muscular atrophies
Nerve: congenital hypomyelination neuropathy, familial dysautonomia, infantial neuraxonal degeneration, brachial plexus injury
Neuromuscular junction: congenital and transient myasthenia gravis, infantile botulism
Muscle: muscular dystrophy, metabolic myopathy, fibre myopathy
Other: hypothyroid, tay saches, downs, benign congenital hypotonia (no cause found)
24
Q
How should a floppy infant be investigated?
A
- infection screen (fbc, csf, blood cultures)
- bloods for glucose, electrolytes, ck (increased in muscular dystrophy)
- CT, MRI
- EEG
- EMG
- nerve conduction studies
- muscle biopsy
- genetic testing
- head circumference (often small in central hypotonia)
25
What is charcot marie tooth syndrome and how does it present?
- inherited peripheral neuropathy
- distal limb muscle wasting and sensory loss which progresses proximally overtime
- 4 types, each progresses at different rates with different prognosis
26
What is spinal muscle atrophy?
- degeneration in anterior horn cells
- progressive weakness of skeletal muscles due to mutations in survival motor neurone gene
- second commonest cause of NMD after duchenne
- 3 types, type 1 is the worst- die of resp failure within a year
27
What are the 3 commonest types of primary headaches?
migrane (with and without aura)
tension
cluster
28
Give 5 causes of secondary headaches?
- raised ICP/ space occupying lesion
- medication overuse headaches
- head/ neck trauma
- vascular malformation
- infections
29
Describe the clinical features of a raised ICP headache
- worse when lying down and in morning
- night time wakening
- change in mood, personality and educational performance
- visual field defects, diplopia, facial nerve palsy
- early or late puberty, growth failure, squint, abnormal coordination, bradycardia
30
How can migraines be managed?
- analgesia (paracetamol/ nsaids)
- antiemetics
- tripitans- sumatriptan nasally is effective if used early
- cold compress, warm pads, topical forehead balms
31
What can be used for migraine prophylaxis?
- sodium channel blockers
- betablockers
- tricyclics
- acupuncture and relaxation techniques
- tigger avoidance
32
What is reflex asystolic syncope?(aka reflex- anoxic seizures)
Cardiac asystole due to vagal inhibition due to a triggering event. The child becomes pale and falls to floor, the hypoxia may cause a generalised tonic- clonic seizure. Common triggers are minor head trauma, cold food, fright or fever. They occur in infants or toddlers and more common if FHx of faints. Paroxysmal slow wave discharge on EEG
33
How is reflex asystolic syncope managed?
- only a minority of children require drugs- if very frequent and due to minor triggers
- anticholinergics and atropine as well as cardiac pacing are options
- most need no specific management
- the seizures are usually only brief
34
What could trigger a child to have a syncope (simple faint)
hot stuff environments, standing for long periods or fear. colonic movements lasting a few seconds are common
35
Give 5 causes of ataxia in a child
- friedreich ataxis
- ataxia telangiectasia
- cerebellar agenesis/ dysgenesis
- post infectious cerebellitis (varicella)
- posterior fossa tumours
- other hereditary cerebellar ataxia (many, most benign course in childhood)
- miller fisher (similar to guillian barre)
- mitochondrial disease
- drugs (carbamazepine, lamotrigine)
- toxins (ethanol)
36
What is fredreichs ataxia?
- genetic defect leading to lack of frataxin protein
- causes worsening ataxia and dysarthria, distal wasting to lower limbs, absent reflexes and pes cavus
- similar to charcot marie tooth but impairment of proprioception and vibration only
- evolving kyphoscoliosis, diabetes, cardiomyopathy and resp compromise cause death a 40-50
37
what is ataxia telangiectasia? what is their prognosis like?
- protein kinase DNA defect
- mild delay in motor development in infancy and oculomotor problems, difficulty with balance and coordination becomes apparent at school age
- nystagmus, dystonia, spasms, jerks, tremours, cerebellar ataxia and dysarthria develops in time
- telangiectasia develops in conjunctiva, neck and shoulders around age 4
- raised susceptibility to infection, malignancy, pulmonary disease and raised alpha fetoprotein
- will die of malignancy or lung disease in their 20s
38
Other than epilepsy, what can cause seizures?
- Acute symptomatic seizures- caused by intracranial infections, traumatic brain injury, stroke, posions/ toxins, electroyte disturbance (low Ca2+, low or high Na, low Mg, low glucose)
- Febrile seizures
- Non epileptic seizures- may be functional, sudden increase in ICP, hyponic jerks, or convulsive syncope caused by breath holding spells, arrhythmias, hypovolaemia etc
39
What is childhood and juvenile abscence epilepsy?
- absence seizures lasting <30s in child age 3-12 (childhood) and 10-20 (juvenile)
- juvenile often photosensitive and may have TC seizures
- 80% remission for childhood
- juvenile need AEDs lifelong usually but respond well
- Hyperventilation EEG shows fast spike and wave for childhood, juvenile also has characteristic EEG
40
What is lennox gasthaut?
- disorder causing multiple seizure types, neuo development arrest and behaviour disorder
- generalised spike and wave on EEG
- poor prognosis
41
Describe infantile spasms/ west syndrome and state how it is managed?
- violent spasms of head trunk and limbs followed by extension of arms, often mistaken for colic. Lasts 1-2s and get bursts of 20-30. often occur in morning
- presents between 3 and 12 months
- social interaction deteriorates
- Hypsarrhythmia on EEG
- Vigabatran and corticosteroids control well initially but most relapse, loose skills and develop LD
42
What is juvenile myoclonic epilepsy?
- myoclonic seizures, +/- TC and absence
- on walking/ shortly after waking- 'throws milk everywhere'
- leaning unimpaired
- good response to AEDs
43
What can cause communicating and non communicating hydrocephalus?
communicating: sub arach harmorrhage, meningitis
Non communicating: intraventricular haemorrhage, posterior fossa neoplasm/ vascular malformation, congenital (chiari malformation, atresia of outflow foramina of 4th ventricle, aqueduct stenosis)
44
How does hydrocephalus present?
- large cranial circumferance
- bulging of anterior frontanelle
- prominent scalp veins
- sun setting gaze (fixed downwards)
- increased ICP signs in older children
45
How is hydrocephalus managed?
- USS (infants) or CT
- monitor head circumferance
- venticulo- perionteal shunt
- furosemide
