Neurology Flashcards
CEREBRAL PALSY
i) what is it? name two antenatal/perinatal/postnatal causes
ii) what are the four types? what is the most common?
iii) what is mono/hemi/di.quadreplegia?
iv) name five signs and symptoms? which condition puts a child at increased risk?
v) what will be seen on neuro exam in terms of tone/reflexes/muscle bulk (UMN lesion)
i) permanent non progressive neurological condition due to damage to brain around birth
antenatal - maternal infection or trauma during preg
perinatal - birth asphyxia, pre term birth
postnatal - meningitis, severe neonatal jaundice, head injury
ii) spastic (most common( - hypertonia and UMN damage
dyskinetic - hyper and hypotonia, basal ganglia damage
ataxic - lack of co-ordinate due to cerebellum
mixed
iii) mono - one limb aff
hemiplegia - one side of body affected
diplegia - four limbs aff but mostly legs
quadrepelgia - four limbs aff severley
iv) fail to meet milestones, inc or dec tone, hand preference below 18m, probs with co-ord/speech/walkingm feeding or swallow probs, learning difficulty
hypoxic ischaemic encephalopathy increases risk
v) UMN - hypertonia, brisk reflexes, muscle bulk preserved, slightly reduced power
STRABISMUS (SQUINT)
i) what is it? what will the person experience?
ii) what is amblyopia? what is a co cominant squint?
iii) name four causes?
iv) name three things that can be tested in examination? which two tests can be done?
v) before what age does tx need to start? what can be put on the eye? which eye drops may be given?
i) misalignment of the eyes - images on the retina do not match and there will be double vision
ii) amblyopia - affected eye becomes passive and reduced function compared to dominant eye
co cominant - due to diffs in control of extra ocular muscles
iii) idiopathic, hydrocephalus, cerebral palsy, space occ lesion, trauma
iv) eye movements, fundoscopy to rule out Rb, cataracts, retinal pathology
visual acuity test
hirschbergs test and cover test
v) tx needs to start before 8 years as thats when the visual fields are developing
eye pacth over the dominant eye forces the weaker eye to develop
can also use atropine eye drips in the good eye causing vision to be blurred
HYDROCEPHALUS
i) what is it? what can it be due to? (2)
ii) where is CSF created? where is it absorbed?
iii) what is the most congenital cause? name three other causes
iv) how may it present? what may happen to head circumference? name three other signs
v) what can be used to treat it? name three complications of this
i) CSF build up in brain and sp cord due to over produc of CSF, problem with drainage/absorption
ii) created in choroid plexus and abs into venous system via arachnoid granulations
iii) most common is acqueductal stenosis (narrowing on ceb acq - blocks normal flow of CSF)
other causes - arachnoid cysts, arnold chiari (cerebellum herniates into foramen magnum and blocks CSF flow) and chromo abnorms
iv) px with enlarged head and rapidly increasing head circumference
also see bulging anterior fontanelle, poor feeding, poor tone, sleepy
v) ventriculoperitoneal shunt - tube from ventricle to peritoneum to drain excess CSF
complications - infection, blockage, excess drainage, intraventric haemmorhage, outgrowing (need to be replaced every 2 yrs child grows)
CRANIOSYNOTOSIS
i) what is it? what does it result in? what can happen if its left untreated? (4)
ii) what does the presentation depend on? name two presenting features
iii) what is the first line investigation? what can then be done to confirm dx
iv) how are mild cases managed? how can severe cases be managed? what is the prognosis?
i) skull sutures close prematurely > abnormal head shape and restricted brain growth
if untreated - raised ICP > dev delay, cog impair, vom, irritable, visual impair, neuro symptoms
ii) px depends on which cranial suture is affected
also see anterior fontanelle closure before 1 years and small head in proportion to body
iii) first line is skull x-ray then CT head
iv) mild cases - follow up over time
severe - surgical reconstruc of skull
good prognosis if properly managed
PLAGIOCEPHALY AND BRACHYCEPHALY
i) what are they? what is plagioceph? what is brachy ceph? at what age do they normally px
ii) what is positional plagio? why has this become more common?
iii) what dx needs to be excluded? how is it this done? what is congenital muscular torrticollis?
iv) name three measures that can be taken to help minimise it?
i) conditions that cause abnormal head shapes
plagio - flattening of one area of head
brachy - flattening of back of head
usually px 3-6m
ii) positional - baby rests head on one particular point causing flattening - happens more as patient advised to plays babies on back to reduce SIDS
iii) exclude craniosynotosis by properly palpating the sutures
CMT is shortening of SCM which can cause baby to always rest on one side of head
iv) position on rounded side of head for sleep, supervise tummy time, use rolled towels, minimise time in pram or cat seat
MUSCULAR DYSTROPHY
i) what is it?
ii) what is gowers sign? which children may exhibit this?
iii) which gene is defective in duchennes musc dystrophy? which chromo is it on? what is the inheritence pattern?
iv) when do patients usually px with DMD? which muscles are weak first? what is avg life expectancy?
v) which drugs have been shown to slow progression of DMD? which other supplement may help muscle strength?
i) genetic condition that causes gradual weaknening and wasting of muscles
ii) gowers sign is the technique a child may use to stand up from a laying position - hands and knees > downward dog > walk hands to knees to standing - seen in DMD
iii) DMD - dystrophin gene (holds muscles together) is defected and lays on X chromosome
inheritence is X linked recessive (girls can be carriers but boys will have the condition)
iv) patient px around 3-5 years with weakness in muscles around pelvis > progressive
wheelchair by teenager and life expect of 25-35yrs
v) oral steroids may slow progression
creatine supplements can help support muscle
SPINAL MUSCULAR ATROPHY
i) what is it? what is the inheritance? which type of motor neurons does it affect?
ii) which signs are likely to be seen in relation to the MNs affected? (musc bulk, tone, power, reflexes)
iii) which type is most severe? which is the most common type?
iv) which MDT members may be important in care/ what type of resp support may be needed? what may be required to feed?
i) progressive loss of LMNs in spinal cord > progressive muscle weakness
ii) LMN - fasiculations, reduced muscle bulk, reduced tone, reduced power, reduced/absent reflexes
iii) type 1 is most severe and px in first few months of life > death in a few years
type 2 is the most common - px in first 18m
iv) physio for max strength in muscles and retaining resp function
may need NIV
may need PEG feeding if swallow is unsafe
FEBRILE CONVULSIONS
i) what are they? is there underlying pathology? between which ages do they occur?
ii) what is a simple febrile convulsion? how long do they last? how many times do they occ in febrile illness?
iii) what is a complex feb conv? how long do they last? how many times do they occ in febrile illness?
iv) name four differential dx for feb conv? what should be managed first?
v) over what time should an ambulance be called?
i) seizure that occurs when a child has a high fever - no underlying pathology and usually occ between 6m and 5yrs
ii) simple is generalised tonic clonic seizure lasting <15 mins and occuring once in a single feb illness
iii) complex consists of partial or focal seizure, lasts >15mins or occ multiple times within a feb illness
iv) DDs: epilepsy, menhingitis, enceph, space occ lesion, syncopal episode, electrolye abnorm, trauma
first manage underlyign cause if fever with paracet and ibuprofen
v) call ambulance if lasting more than 5 min or bring to hospital if first seizure
EPILEPSY
i) what is it? what is a tonic clonic seizure? which phase comes first? name three symptoms that may be assoc
ii) what usually follows a generalised TC seizure? what is first line mx? what is second line? (2)
iii) where do focal seizures start? what do they affect? (3) name four ways they may px? what is the tx first and second line? (opposite of TC)
iv) what is an absence seizure? how long do they usually last? when do they stop for most pts? what is first line tx?
i) abnormal brain activity
TC = muscle tensing, clonic = muscle jerking (tonic before clonic)
may be assoc with tongue biting, incont, groaning, irregular breathing
ii) post TC > post ictal phase where patient is drowsy, confused, irritable, low mood
mx first line is sodium valproate
second line is lamotrigine/carbamazepine
iii) focal seizures start in temporal lobe
affect hearing, speech, memory, emotion
px with deja vu, hallucinations, memory flashbacks, strange behaviour
first line tx is lamotrigine/carbamaz
second line is sodium valproate or keppra
iv) absence is blank/stares into space then suddenly comes back
lasts 10-20seconds and most stop happening as they get older
first line tx is valproate or ethosuximide
EPILEPSY CONTINUED
i) what is an atonic seizure aka? what are they charac by? how long do they last? what is first and second line tx?
ii) what is a myoclonic seizure? does patient usually lose conc? what is the most common type in childhood? what is first line tx? name two other tx that may be given?
iii) what are infantile spasms? what syndrome is it aka? name two potential tx?
i) atonic = drop attack - charac by brief lapses in muscle tone that dont usually last more than 3 mins
first line valproate, second line lamotrigine
ii) myoclonic = sudden brief muscle contractions - pt usually stays awake
most common form is juvenile myoclonic epilepsy
mx with valproate or lamotrigine, keppra, topiramate
iii) infantile spasm - west syndrome (rare)
clusters of full body spasms
tx with prednisolone or vigabatrin
EPILEPSY MANAGEMENT
i) what ix can show patterns of epilepsy and support a dx? when is this done?
ii) what other imaging can be used to dx structural problems in the brain? name three instances this may be considered?
iii) which type of ep the only type that valproate is not first line for? how does valp work? name three side effects
iv) name three side effects of carbamazepine, pheytoin, ethosuximide, lamotrigine
i) EEG - do after second simple tonic clonic seizure
ii) brain MRI - consider if first seizure in child under 2, focal seizure, no response to first line anti epileptics
iii) valproate not first line for focal seizures
increases GABA activity > SE are teratogenic, liver damage/hepatitis, hair loss, tremor
iv) carbamaz - agranulocytosis, aplastic anaemia, p450 inducer
phenytoin - folate/b12 defic, megaloblastic anaemia, osteomalacia due to vit D defic
ethosux - night terrors and rashes
lamotrigine - SJS, leucopenia
STATUS EPILEPTICUS
i) what is it?
ii) what approach should be taken? what needs to be secured? what should be given to the patient?
iii) which drug is given? how quickly is it repeated if seizure continues?
iv) what drugs can be given if seizure further persists? what may be needed?
v) which two drugs can be given in the community?
i) seizure lasting more than 5 mins or 2+ seizures without regaining conc
ii) A-E approach as medical emergency
secure airway and give high conc o2
iii) give IV lorazepam and repeat after 10 mins if seizure continues
iv) persists - give IV phenobarbital or phenytoin
may need to ventilate/ITU
v) community - buccal midazolam or rectal diazepam
