Neuro Flashcards Preview

Paeds > Neuro > Flashcards

Flashcards in Neuro Deck (94):
1

causes of abnormal motor development

central (CP), myopathy, spinal cord lesion (spina bifida), global developmental delay

2

what is cerebral palsy

abnormality in posture and tone, brain injuries before 2 years.

3

CP are the lesions progressive

no, clinical manifestations emerge over time

4

causes of CP antenatal

placental insufficiency- occlusion, chromosomal, genetic, infection

5

causes of CP

antenatal 80%, hypoxic-ischaemic encephalopathy 10%, postnatal 10%

6

causes of CP postnatal

meningitis, encephalitis, encephalopathy, hypoglycaemia, kernicterus, hydrocephalus

7

how does CP present

abnormal posture and tone, delayed motor milestones, feeding difficulties, abnormal gait, hand asymmetry before 1y

8

what happens to primitive reflexes in CP

may persist and become obligatory

9

what are the 3 types of CP

spastic, dyskinetic and ataxic

10

what signs are seen in spastic CP

UMN lesion so increased tone, brisk reflexes, extensor plantar responses. tone is velocity dependent so clasp knife can be seen. initial hypotonia at birth

11

3 main types spastic CP

hemiplegia, diplegia, quadriplegia

12

how does hemiplegic spastic CP present

unilateral involvement arm and leg; fisting affecting hand, flexed arm, pronated forearm, asymmetric hand function; tip toe walking. birth history may be normal

13

how does quadriplegic spastic CP present

all 4 limbs involved, severe, trunk involved- opisothonus, poor head control, low central tone.

14

what is quadriplegic assoc with and what can happen in birth to lead to it

seizures, microcephaly, intellectual impairment. hypoxic-ischaemic encephalopathy

15

how does diplegic spastic CP present

all 4 limbs involved but legs more than arms so hand function may appear normal. abnormal walking.

16

what can happen to cause diplegic

periventricular brain damage

17

what is asymmetrical hand function before 12m likely to be due to

hemiplegic spastic CP

18

what tracts are affected in spastic CP

pyramidal and corticospinal tracts

19

what is damaged in dyskinetic CP

basal ganglia, extra pyramidal

20

how does dyskinetic CP present

abnormal involuntary movements more obvious with active movement and stress. tone is variable. chorea, athetosis, dystonia. intellect can be unimpaired

21

what is ataxic CP

early trunk and limb hypotonia, poor balance, delayed motor development

22

what are the causes of ataxic CP

most genetically determined. if brain injury- signs on same side as lesion. cerebellum

23

causes of headache

tension- tight band like headache, common in adolescents usually with abdominal pain; migraine; raised ICP; sinusitis; benign intracranial hypertension

24

symptoms raised ICP

headache worse on lying down and when waking up, vomiting especially in the morning, visual field defects, CN 6 palsy, gait abnormalities, papilloedema (late sign)

25

causes of hydrocephalus

congenital, arnold-chiari malformation, subarachnoid haemorrhage, meningitis

26

features of hydrocephalus

large head circumference, sutures separated, bulging anterior fontanelle, if left untreated- setting sun sign and signs of increased ICP

27

what signs can be seen from anterior fontanelle

closes around 1 and a half. bulging- raised ICP, sunken- dehydrated

28

what is microcephaly

head circumference below 2nd centile

29

causes of microcephaly

familial (take mean of parents), congenital infection, brain injury- meningitis, hypoxia, hypoglycaemia, accompanise CP and seizures; autosomal recessive- developmental delay

30

what is macrocephaly

head circumference >98th centile

31

causes of macrocephaly

tall stature, familial, hydrocephalus, raised ICP, tumour, chronic subdural haematoma

32

what is a febrile seizure

seizure accompanied by fever in absence of intracranial infection due to meningitis/encephalitis

33

what happens in febrile seizure

usually brief generalised tonic clonic, usually early in infection when temperature is rising rapidly

34

what age is febrile seizure likely

6m-5y

35

how many patients will have further febrile seizures and what will make further seizures more likely

30-40%. more likely if younger child, started earlier on in the illness, family history, lower temperature when the convulsion occured

36

what is the chance of developing epilepsy with febrile convulsions

1-2% in simple seizures. 4-12% if it is complicated by having focal signs, prolonged seizures, repeated in the same illness

37

in febrile convulsion what must you always consider

bacterial cause eg meningitis as classical features may not be present if patient is

38

if history of prolonged seizures what can be given

rectal diazepam or buccal midazolam.

39

what is the normal length of a febrile convulsion and above which you would get worried

5 mins

40

causes of paroxysmal events in children

breath holding attacks, reflex anoxic seizures (head trauma, cold food, fright, fever), syncope, BPPV, pseudoseizures, epilepsy, induced illness (NAI), cardiac- prolonged QT

41

what are examples of generalised seizures

arise from both hemispheres. generalised tonic-clonic, myoclonic, atonic, absence

42

what is the most common focal seizure and what does it present with

temporal- aura, lip smacking and other automatisms, deja vu

43

what does frontal present with

motor/pre motor. clonic movements, asymmetrical tonic can be seen

44

what does parietal present with

altered sensation- dysaesthesia, distorted body image

45

what investigations should be done in child with seizures

EEG, 24h ambulatory EEG, video telemetry, MRI/CT to exclude brain lesions (tumour, infarct, malformation), PET to see hypometabolism

46

what is West syndrome

infantile spasms. occurs 4-6m. triad- spasms, learning difficulties and hypsarrhythmia on EEG. multiple bursts 20-30 spasms lasting 1-2s each

47

what can you use to treat West syndrome

vigabatrin and corticosteroids. poor prognosis

48

what is Lennox-Gastaut syndrome

slightly later onset seizures- 1-3years. drop attacks- astatic seizures, tonic, atypical absences. neurodevelopmental arrest. poor prognosis

49

what is childhood absence epilepsy and when does it come on

4-12 year olds. absence- for seconds not longer than a minute, then may twitch eyelids or hand. developmentally normal, females 2/3.

50

how could childhood absence be induced in the clinic

hyperventilation

51

what is the prognosis of childhood absence

good, 5% develop tonic clonic in adults

52

when does juvenile-myoclonic epilepsy set in and what happens in it

adolescence- adult. myoclonic but can have clonic and absences. learning unimpaired. remission unlikely

53

what is the most common syndrome and is it generalised or focal

rolandic- benign epilepsy with centro-temporal spikes. focal

54

what is benign epilepsy with centro temporal spikes (rolandic) and does it remit by adolescence

4-10 years. facial movements and strange movements of the lips. remits by adolescence

55

what AED can make absence and myoclonic seizures worse

carbamazepine

56

first line in tonic clonic

valproate, carbamazepine

57

first line in absence and myoclonic

valproate

58

first line in focal

carbamazepine, valproate, lamotrigine

59

second line tonic clonic

lamotrigine, topiramate

60

second line absence and myoclonic

lamotrigine

61

second line focal

topiramate, levetiracetem, gabapentin

62

other options other than AED for seizures

ketogenic diet, VNS, surgery

63

what is status epilepticus

>30 mins of seizure. lorazepam IV if access, rectal diazepam/buccal midazolam if not. if no response- phenytoin

64

side effect valproate

weight gain, hair loss

65

side effect carbamazepine

rash, neutropenia, hyponatraemia, ataxia

66

side effect lamotrigine

rash. steven johnson

67

side effect benzos

sedation

68

side effect topiramate

weight loss, drowsy, withdrawal

69

how does an ataxic child present

abnormal gait, tremor

70

how would you describe the ataxic/cerebellar gait

broad based, staggering/lurching, fall towards side of cerebellar lesion

71

what is ataxia

inabillity to coordinate muscle activity- jerky and incoordination. inability to perform precise movements and loss of balance

72

what is true ataxia and what are the types

sign of cerebellar dysfunction. sensory and cerebellar

73

what is sensory ataxia

peripheral nerve or posterior columns damaged. wide based gait. can look normal whilst sitting, loss of position and vibration sense. positive romberg.

74

what is cerebellar ataxia

problem in the vermis of the cerebellum. lurching, staggering gait. unable to sit balanced

75

what viral infection is most implicated in ataxia

varicella

76

acute causes of ataxia

drugs, trauma, post infectious immune eg GBS, tumour, labyrinthitis

77

insidious ataxia

tumours, BPPV, hereditary- ataxia telangectasia, Friedrichs, congenital malformations- Dandy Walker, chiari malformation

78

how does hemiplegic gait look

flexed arm, straight leg, foot makes circle round0 circumduction, upper limb flexed, lower limb extended, distal more affected than proximal so get weak fingers and foot drop

79

what does diplegic gait look like

both arms flexed, both legs extended. tip toe walking. adduction prominent feature. can get scissoring gait

80

what is the gait in patient with myopathy

waddling gait- lean trunk away from side of lesion, compensate for the weak pelvic muscles which cant lift hip when lift leg to take a step

81

central causes of floppy baby

causes of developmental delay eg Downs, hypothyroid, hypocalcaemia, HIE, evolving CP

82

peripheral causes of floppy baby

spinal muscular atrophy, MG, myopathy

83

what is hydrocephalus

obstruction to the flow of CSF leading to dilatation of the ventricular system proximal to the site of obstruction.

84

where is the obstruction in non communicating hydrocephlus

within ventricular system or aqueduct

85

where is the obstruction in communicating hydrocephalus

arachnoid villi

86

causes of non communicating

aqueduct stenosis, dandy walker, chiari

87

causes of communicating

SAH, meningitis

88

signs hydrocephalus

bulging ant fontanelle, scalp veins distended, downward gaze or sun setting sign

89

what is needed for diagnosis neurofibramotosis type 1

2 or more of : 6 or more café au lait, more than one neurofibroam, axillary freckles, optic glioma, lisch nodule, bony lesions, first degree relative with NFM1

90

dominant feature of NFM2

acoustic neuroma

91

what are the lipid storage disorders causing neurodegeneration

tay sachs, gaucher, niemann pick

92

what enzyme is deficient in tay sachs

hexosaminidase A. auto somal recessive. Ashkenazi Jews. developmental regression. death by 2-5 years. cherry red spot macula

93

what enzyme is deficient in gaucher

beta glucosidase. Ashkenazi jews. splenomegaly, bone marrow suppression.

94

what enzyme is deficient in niemann pick

sphingomyelinase. death by 4 years. feeding difficulties, failure to thrive, cherry red spot macula